Practical General Practice: Guidelines for Effective Clinical Management 4th Edition

Practical General Practice

For Buttenvorth-Heinemann: Commissioning Editor: Heidi Allen Project Development Manager: Robert Edwards Project Manager: Andrea Hill Designer: George Ajayi

Practical General Practice Guidelines for Effective Clinical Management

Edited by AleX KhOt MA, MB, BChir, DCH General Practitioner, East Sussex, UK

Andrew PolrnearMA,Msc,FRcp FRCGP Senior Research Fellow, Academic Unit of Primary Care, The Trafford Centre for Graduate Medical Education and Research, University of Sussex, UK

FOURTH EDITION

BUTTERWORTH-HEINEMANN An imprint of Elsevier Science Limited © Reed Educational and Professional Publishing Ltd 1999 © 2003, Elsevier Science Limited. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without either the prior permission of the publishers, or a licence permitting restricted copying in the United Kingdom issued by the Copyright Licensing Agency, 90 Tottenham Court Road, London WIT 4LP. Permissions may be sought directly from Elsevier's Health Sciences Rights Department in Philadelphia, USA: phone: (+1) 215 238 7869, fax: (+1) 215 238 2239, e-mail: [email protected]. You may also complete your request on-line via the Elsevier Science homepage (http://www.elsevier.com), by selecting 'Customer Support' and then 'Obtaining Permissions'. First edition 1988 Second edition 1992 Third edition 1999 Fourth edition 2003 Reprinted 2003 ISBN 0 7506 4911 9 British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging in Publication Data A catalog record for this book is available from the Library of Congress Note Medical knowledge is constantly changing. As new information becomes available, changes in treatment, procedures, equipment and the use of drugs become necessary. The editors and the publishers have taken care to ensure that the information given in this text is accurate and up to date. However, readers are strongly advised to confirm that information, especially with regard to drug usage, complies with the latest legislation and standards of practice. Disclaimer The clinical advice and the organizational details contained in this book refer to the United Kingdom, unless otherwise stated. The book is intended for the use of medical practitioners and not for patients. While strenuous efforts have been made to ensure the accuracy of the information in this book, it is the responsibility of the doctor who is caring for the patient to make his or her own judgement on the best treatment for that patient at that time. Some of the information contained in this book will become out of date. Some of it may even be wrong, written as it is by mere human beings. In particular, we recommend that prescribing details are checked with the British National Formulary or equivalent, or with the manufacturers' date sheets. We do not even attempt to list contraindications or adverse effects, nor do we always point out when a product is unlicensed for the use we are suggesting. The authors, editors and publishers cannot accept liability for information that is subsequently shown to be wrong. your source for books, journals and multimedia in the health sciences

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Contents

Preface

vii

17. Substance abuse

321

The structure of the book ix

18. Ear, nose and throat problems 335

Acknowledgements

19. Eye problems

355

20. Skin problems

365

x

Feedback and updating List of contributors

xi

xii

List of abbreviations

21. Allergic problems 383

xiv

22. Older people 389 23. Disability 403

1. General 1 2. Infectious diseases

24. Haematology 415

19

3. Sexually transmitted infections 41 4. Childhood problems 51

26. Health promotion and screening 441

5. Cardiovascular problems 89

Appendices 457

6. Respiratory problems 115 7. Gastroenterological problems

131

8. Endocrine problems 147 9. Rheumatological problems

25. Pain, palliative care and bereavement 421

161

10. Neurological problems 189 11. Gynaecological problems 209 12. Contraception and sexual problems 227

Appendix 1 Routine schedule of immunizations 459 Appendix 2 Incubation period and infectivity of common diseases 459 Appendix 3 A suggested schedule of immunization for travellers 460

13. Obstetric problems 253

Appendix 4 Notification of infectious diseases 460

14. Urinary problems 275

Appendix 5 Child health promotion 460

15. Surgical problems 287

Appendix 6 Injury prevention strategies 461

16. Psychiatric problems 301

Appendix 7 Stages of child development

462

vi CONTENTS

Appendix 8 Stages of puberty 465

Appendix 20 Testing peripheral nerves 479

Appendix 9 Predicted normal peak flow values in children (under 15 years of age) 466

Appendix 21 Drug levels 480

Appendix 10 Peak expiratory flow in normal subjects 467 Appendix 11 FEV^FVC charts 468 Appendix 12A Management of chronic asthma in schoolchildren and young adults 469 Appendix 12B Management of asthma in children under 5 471 Appendix 13 Pill ladders 472 Appendix 14 Obstetrical table 473 Appendix 15 Edinburgh Postnatal Depression Scale 474 Appendix 16 International prostate symptom score (IPSS) 475 Appendix 17 Body mass index 476 Appendix 18 Blood data 477 Appendix 19 Dermatomes and myotomes 478

Appendix 22 Suggestions for monitoring patients taking disease-modifying anti-rheumatic drugs 481 Appendix 23 Problems associated with specific causes of disability 484 Appendix 24 The Community Dependency Index 486 Appendix 25 Nottingham Extended Activities of Daily Living Questionnaire (EADL) 487 Appendix 26 Admission procedures for patients with mental health problems 487 Appendix 27 The early warning form for use in psychotic illness 488 Appendix 28 Drug stabilities 489 Appendix 29 Guidelines for the urgent referral of patients with suspected cancer 491 Index 497

Preface

In the preface to the first edition of this book we wrote about our belief that it was possible 'to draw up guidelines of management which are logical and justifiable on the evidence'. The subsequent development of evidence-based medicine and of clinical guidelines shows that we were not alone in our belief and the evidence on which guidelines can now be based is far more extensive than it was then. For this reason we have recruited other authors to write some of the chapters, and they have indeed brought a new breadth to the book. We want to stress that this is a manual of clinical practice, not a collection of national guidelines. Each section is structured to try to mirror the thought processes of the doctor (see The structure of the book) in a way that few national guidelines do. Even less is it a textbook designed to give the reader an all-round knowledge of the subject. Anything that does not help the doctor decide what to do with the patient in the consulting room has been purged. Furthermore, we are deeply suspicious of textbooks as instruments of stagnation. We urge our readers to realize that this book will have started to go out of date by the time it goes on sale. Our hope is that the reader will use it as a template on which to write new information and new management strategies. This new edition is different from previous editions in a number of ways: 1. The search for evidence has been conducted in a more systematic way than before. Each author has searched for national guidelines, in the UK and abroad. If a reliable guideline was not found,

a search for evidence was conducted by working through the Cochrane Library, Clinical Evidence, Evidence-Based Medicine, Bandolier, Drug and Therapeutics Bulletin, Effective Health Care, Medline and other sources of evidence until reliable information was found. 2. The use of new authors has allowed us to submit the material for peer review. All chapters, except those submitted too late to allow time for review, were posted on a dedicated website. Authors reviewed each others' work and submitted comments to the editors. These comments were then argued over until they were accepted or rejected. 3. Contributors to three chapters have come from abroad - Australia, New Zealand and Mexico. The chapters that they have contributed therefore contain practical information, about patient organizations for instance, local to those authors, as well as the equivalent information for the UK. In some ways this has been a trial run. We have learnt what we suspected: that the core of the book is applicable to any country where a system of primary care exists that resembles that in the UK. We have also learnt that the local details differ so much that a single book cannot contain them. The way forward, for the fifth edition, must be multiple editions, differing electronic versions or a combination of the two. We therefore ask that you excuse our inconsistency in this edition. Our move to an international version is not due to unbounded ambition, but to our realization that previous editions have sold well abroad, and that we need to respond to what is clearly a need in

viii PREFACE

countries other than the UK. Indeed, the first Spanish edition of the book is in preparation. 4. The book contains many more references than previous editions. This partly reflects the systematic way in which the evidence has been collected,

but also it serves as a fairly complete guide to the literature on clinical management in primary care. Alex Khot Andrew Polmear

The structure of the book

ASTERISKS AND BULLETS

BOXES

Central to the structure are instructions preceded by an asterisk:

These are used to highlight information that might otherwise get lost in the text: guidelines, a list of tests as a 'work-up' for a patient with a particular condition, or patient organizations.

* Ask the patient x, y and z; * Examine for a, b and c; * Take blood for d, e and f, etc. At the same time the reasons for these steps are explained, either in the same sentence or nearby, signalled by a round bullet. LISTS Where we present a list in no particular order we use (a) chest pain; (b) hypotension; or (c) heart failure. Where the order is important we number the list: 1. Sit the patient up. 2. Give oxygen.

3. Give diamorphine ...

REFERENCES Our aim is to reference every statement of fact. Where such a statement is not accompanied by a reference, the reader can assume it is taken from the reference in a box at the start of that section. Most instructions are, however, not referenced because they are original to this book.

Acknowledgements

We would like to thank the staff at ButterworthHeinemann for their help and support, especially Melanie Tait, Zoe Youd, Heidi Allen, Robert Edwards and Andrea Hill in the production of this edition; Caroline Makepeace, Mary Seager and Geoff Smaldon for previous editions; and Sue Deeley, our original editor, whose faith in our book from the start made the whole project possible. We are deeply grateful to Trish Maxwell at the University of Sussex for her work in transforming the work of so many authors into a single

format. As editors we would like to thank the contributors for their work and for their willingness to shrink their masterpieces into the required format. In turn the contributors would like to thank Professor R West (Chapter 17), Professor David Price, Dr Ron Neville, Dr David Bellamy, Dr Mike Thomas, Dr Daryl Freeman, Dr Rachel Joce, Dr Angela Iveson (Chapter 6) and the NHS R&D National Primary Care Award Scheme (for support of Dr A Sheikh).

Feedback and updating

We are keen to receive feedback from readers that could inform a future edition. This could take the form of ideas for new topics, new evidence that should be incorporated, or correction of errors of fact or judgement. A reader who would like to provide feedback should email [email protected]

A reader who does not have access to email may provide feedback by post by writing to Product Manager - General Practice, Elsevier Science, 32 Jamestown Road, London, NW1 7BY, UK.

List of contributors

EDITORS

Chapter 6

Alex Khot, MA, MB, BChir, DCH General Practitioner, Portslade on Sea, East Sussex, UK

Hilary Pinnock, MB, ChB, MRCGP General Practitioner, Whitstable Medical Practice, GPIAG Clinical Research Fellow, Dept of General Practice & Primary Care, University of Aberdeen, UK

Andrew Polmear, MA, MSc, FRCP, FRCGP Senior Research Fellow, Academic Unit of Primary Care, The Trafford Centre for Graduate Medical Education and Research, The University of Sussex, Falmer, UK

Nurse Adviser Primary Care & Public Health

CONTRIBUTORS

Chapter 8

Chapter 3

Ross Lawrenson, MD, MRCGP, MFPHM, FAFPHM Professor of Primary Health Care, University of Surrey, Guildford, UK

Jackie Cassell, MSc, MRCP, Dip GUM, DFFP Clinical Research Fellow, Department of Sexually Transmitted Diseases, Royal Free and University College Medical School, London, UK Barbara Rasburn, MB BS, BSc (Rons), MRCGP, DRCOG, DSFP General Practitioner, South Islington, London, UK

Marilyn Eveleigh, BA(Hons), PGCE, RGN, SCM, HV, FWT, NP, FRSH

Chapter 9 Kevork Hopayian, MB BS, BSc, FRCGP, DCH, DRCOG General Practitioner, Aldeburgh, Suffolk, UK

Chapter 10

Pierre Pellegrino, MD Associate Specialist, Department of Sexually Transmitted Diseases, Royal Free and University College Medical School, London, UK

Stephen Cox, MSc, MB BS, MRCGP, DRCOG General Practitioner, Ardingly, West Sussex, UK

Chapter 4

Multiple sclerosis and essential tremor

Paul Seddon, MB ChB, DCH, FRCP, FRCPCH Consultant Paediatrician, Brighton and Sussex University Hospitals, NHS Trust, East Sussex, UK

William A Rhoton, MD Planning & Development Unit, Hospital Civil de Guadalajara, Guadalajara, Mexico

Stroke

LIST OF CONTRIBUTORS xiii

Fernando Petersen-Aranguren, MD, FACC Chair of Medicine, University of Guadalajara, Guadalajara, Mexico

Chapter 11 Lisa Argent, MB BS, DRCOG General Practitioner, GP Tutor, Hospital Practitioner in Obstetrics and Gynaecology, Brighton, East Sussex, UK

Chapter 12

Chapter 20 CB Del Mar, MA, MD, MB BChir, FRACGP, FAFPHM Professor of General Practice, University of Queensland Medical School, Australia Geoff Mitchell, MB BS, FRACGP, FAChPM

Senior Lecturer in General Practice, University of Queensland Medical School, Australia

Chapter 21 Aziz Sheikh, BSc, MSc, MB BS, MRCGP, MRCP, DCH, DRCOG, DFFP

Sam Rowlands, MD, MRCGP, MFFP, DCH, DRCOG Lead Clinician in Reproductive & Sexual Health, South East Hertfordshire Primary Care Trust, General Practitioner, Luton, UK

NHS R&D National Primary Care Training Fellow, Department of Primary Health Care & General Practice, Imperial College of Science, Technology & Medicine, London, UK

Chapter 13

Chapter 22

Des Holden, BSc, PhD, MB BS, MRCOG Consultant Obstetrician, Brighton and Sussex University Hospitals, NHS Trust, East Sussex, UK

Ngaire Kerse, PhD, MBChB, FRNZCGP General Practitioner and Senior Lecturer in General Practice

Chapter 15 David Lewis, B Med Sci, FRCSEd, MRCGP, DFFP General Practitioner, Watford, UK

Matthew Parsons, BSc (Hons), MSc, PhD, RGN Senior Lecturer in Gerontology, Faculty of Medical and Health Sciences, University of Auckland, New Zealand

Chapter 16

Chapter 23

Ben Essex, MB BS, MSc, FRCGP, FRCP Medical Advisor, Lewisham Primary Care Trust, London, UK

Rehabilitation

Chapter 17

Jane Crawford-White, DipCOT, BSc (Hons), SROT Lead Practitioner Occupational Therapist, East Cambridgeshire and Fenland Primary Care Trust Disability

Steven Laitner, MB BS, MSc, MRCP(UK), DCH, MRCGP DFPHM Specialist Registrar, Public Health Medicine, Brent and Harrow Health Authority, Middlesex and General Practitioner, St Albans, UK

Nick Lennox, MB BS, B Med SC, Dip Obst, FRACGP Director, Developmental Disability Unit, School of Population Health, University of Queensland, Australia

Chapter 18

Chapter 25

Philip Cotton, MRCGP Lecturer, Department of General Practice, University of Glasgow, UK

Catherine Neden, MA, FRCP, FRCGP, Dip Pall Med John Neden, MA, FRCGP, Dip Pall Med General Practitioners, Ramsgate, Kent, UK

List of abbreviations

5-FU 5-HT A/C AA AAA ABI ABPM ACBS ACE AD ADHD AF AFP AI AIDS ALP ALT AOM APTT APV AS ASO ASW a-v AV b.d. BA BBT BCC BCG BMA BMD BMI BMJ BNF

5-fluorouracil 5-hydroxytryptamine albumin/creatinine ratio attendance allowance abdominal aortic aneurysm ankle-brachial index ambulatory pressure monitoring Advisory Committee on Borderline Substances angiotensin converting enzyme Alzheimer's disease attention deficit (hyperactivity) disorder atrial fibrillation alpha-fetoprotein aortoiliac acquired immune deficiency syndrome actinomyces-like organism alanine transferase acute otitis media activated partial thromboplastin time acellular pertussis vaccine ankylosing spondylitis antistreptolysin O (titre) approved social worker arteriovenous atrio-ventricular twice daily (bis die) Benefits Agency basal body temperature basal cell carcinoma bacillus Calmette-Guerin British Medical Association bone mineral density body-mass index British Medical Journal British National Formulary

BP BSER BTB CABG CCF CDH CDSC CEG CF CHD CI CIN CJD CMO CMV CNS COAD COC COMA COPD CPAP CPHM CPK CPN CPR CRP CSA CSF CSM CSOM CT CTD CVA CVB CVD

blood pressure brainstem evoked response breakthrough bleeding coronary artery bypass graft congestive cardiac failure congenital dislocation of the hip Communicable Disease Surveillance Centre clinical effectiveness guidelines cystic fibrosis coronary heart disease confidence interval cervical intraepithelial neoplasia Creutzfeld-Jakob disease Chief Medical Officer cytomegalovirus central nervous system chronic obstructive airways disease combined oral contraceptive Committee on Medical Aspects of Food and Nutrition Policy chronic obstructive pulmonary disease continuous positive airways pressure Consultant in Public Health Medicine creatine phosphokinase community psychiatric nurse cardiopulmonary resuscitation c-reactive protein child sexual abuse cerebrospinal fluid Committee on Safety of Medicines chronic suppurative otitis media computerized tomography connective tissue disease cerebrovascular accident chorionic villus biopsy cardiovascular disease

LIST OF ABBREVIATIONS

CXR D&C DC DEET DEXA DLA DMARD DMPA DNA DoH DRO DSM DSS DTB DTP DU DVLA DVT DXR DXT ECG ED EDD EEG EIA ELISA EMA ENT EPA ERPC ESR FAP FBC FBS FDA FEVj FHSA FM3 FM5 FM6 FOB FP FPA FSH FT3 FT4 FTU FVC GA GCA

chest X-ray dilatation and curettage direct current diethyltoluamide dual energy X-ray absorptiometry disability living allowance disease-modifying antirheumatic drug depot medroxyprogesterone deoxyribonucleic acid Department of Health disablement resettlement officer Diagnostic and Statistical Manual Department of Social Security (now the Department of Work and Pensions) Drug and Therapeutics Bulletin diphtheria, tetanus and pertussis duodenal ulcer Driver and Vehicle Licensing Agency deep vein thrombosis deep radiotherapy deep radiotherapy electrocardiogram extended delivery expected date of delivery electroencephalogram enzyme immunoassay enzyme-linked immunosorbent assay endomysial antibody ear, nose and throat enduring power of attorney evacuation of the retained products of conception erythrocyte sedimentation rate familial adenomatous polyposis full blood count fetal blood sample Food and Drugs Administration (USA) forced expiratory flow volume in 1 second Family Health Services Authority Form Med 3 Form Med 5 Form Med 6 faecal occult blood femoropopliteal Family Planning Association follicle stimulating hormone free tri-iodothyronine free thyroxine fingertip unit forced vital capacity general anaesthetic giant cell arteritis

GCS GGT GI CMC GORD GP GPC GTN GTT GUM H2 HA ART Hb HbAlc HBeAg HBIG HBsAg HCG HDL HGV Hib HIV HMSO HNIG HNPCC HR HRT HSE HSV HTN HV HVS IBS IDD IFG IgG IgM IGT IHD im 1MB INR IOP IQ ISA ISDN ISMN ITP iu IUCD IUD

xv

Glasgow Coma Scale gamma-glutamyl transferase gastrointestinal General Medical Council gastro-oesophageal reflux disease general practitioner General Practitioners' Committee (of the BMA) glyceryl trinitrate glucose tolerance test genitourinary medicine histamine receptor type 2 highly active antiretroviral therapy haemoglobin glycated haemoglobin hepatitis B virus e antigen hepatitis B immunoglobulin hepatitis B virus surface antigen human chorionic gonadotrophin high density lipoprotein heavy goods vehicle Haemophilus influenzae b human immunodeficiency virus Her Majesty's Stationery Office normal human immunoglobulin hereditary non-polyposis colorectal cancer heart rate hormone replacement therapy Health and Safety Executive herpes simplex virus hypertension health visitor high vaginal swab irritable bowel syndrome insulin dependent diabetes impaired fasting glycaemia immunoglobulin G immunoglobulin M impaired glucose tolerance ischaemic heart disease intramuscular intermenstrual bleeding international normalized ratio intraocular pressure intelligence quotient intrinsic sympathomimetic activity isosorbide dinitrate isosorbide mononitrate idiopathic thrombocytopenic purpura International Units intrauterine contraception device intrauterine device

xvi

LIST OF ABBREVIATIONS

IUGR IUS iv IVU JVP KUB LBC LDL LFT LH LHRH LMP LNG-IUS LOC LR LSCS LTOT LVF MA MAOI MASTA MCP MCV MDA MDI MI miu MMR MMSE MR MRI MS MSBP MSU MTP NE NETOEN NHS NI NICE NIDD NNH NNT NRT NSAID NSF NSPCC NSU o.d.

intrauterine growth retardation intrauterine system intravenous intravenous urogram jugular venous pressure kidneys, ureter and bladder liquid-based cytology low-density lipoprotein liver function test luteinizing hormone luteinizing hormone releasing hormone last menstrual period levonorgestrel releasing intrauterine system loss of consciousness likelihood ratio lower segment caesarean section long-term oxygen therapy left ventricular failure Maternity Allowance monoamine oxidase inhibitor Medical Advisory Service for Travellers Abroad metacarpophalangeal mean cell volume Misuse of Drugs Act metered dose inhaler myocardial infarction millions of international units measles, mumps and rubella Mini-mental State Examination modified release magnetic resonance imaging multiple sclerosis Munchausen's syndrome by proxy mid-stream urine metatarsophalangeal norethisterone enantate 1,9-nortestosterone National Health Service National Insurance National Institute for Clinical Excellence non-insulin dependent diabetes number needed to harm number needed to treat nicotine replacement therapy non-steroidal anti-inflammatory drug National Service Framework National Society for the Prevention of Cruelty to Children non-specific urethritis every day

o.m. OA OME OR ORS OT OTC p.r.n. PA PAM PCO PCOS PCP PE PEFR PEG PET PF PFI PHLS PHN PID PIH PIP PKU PMR PMS POP PPH PPI PPm PSA PSV PTA PUVA PV PVD q.d.s RA RAST RCGP RCP RCT RDC REM RIDDOR RMO RMS RNA RR RR

every night (omni node) osteoarthritis otitis media with effusion odds ratio oral rehydration salts occupational therapy/therapist over the counter as necessary posteroanterior postauricular myogenic response primary care organization polycystic ovary syndrome Pneumocystis carinii pneumonia pulmonary embolus peak expiratory flow rate percutaneous endoscopic gastrostomy positron emission tomography peak flow pill-free interval Public Health Laboratory Service post-herpetic neuralgia pelvic inflammatory disease pregnancy-induced hypertension proximal interphalangeal phenylketonuria polymyalgia rheumatica premenstrual syndrome progestogen-only pill post-partum haemorrhage proton pump inhibitor parts per million prostate-specific antigen public service vehicle post-traumatic amnesia psoralens with UVA plasma viscosity peripheral vascular disease to be taken four times a day (quarter die sumendum) rheumatoid arthritis radioallergosorbent testing Royal College of General Practitioners Royal College of Physicians randomized controlled trial research diagnostic criteria rapid eye movement Reporting of Injuries, Disease and Dangerous Occurrences Regulations Regional Medical Officer Regional Medical Service ribonucleic acid relative risk (according to context) respiratory rate (according to context)

LIST OF ABBREVIATIONS

RRR RUQ SBE sc SCC SD SHBG SIDS SK SLE SLR SMP SpA SPECT SPF SSD SSM SSP SSRI stat STD STI t.d.s. T4 TB TCA TENS

relative risk reduction right upper quadrant subacute bacterial endocarditis subcutaneous squamous cell carcinoma standard deviation sex hormone binding globulin sudden infant death syndrome solar keratosis systemic lupus erythematosus straight leg raising statutory maternity pay spondylarthropathies single photon emission computerized tomography sun protection factor Social Services department superficial spreading melanoma statutory sick pay selective serotonin reuptake inhibitor immediately sexually transmitted disease sexually transmitted infection to be taken three times a day (ter die sumendum) thyroxine tuberculosis tricyclic antidepressant transcutaneous electrical nervous stimulation

TFT TIA TIBC TM TOP TPHA TSH TURF TV U&Es UPSI URTI USS UTI UV UVA UVB UVC VA VAT VDRL VF VTE VZIG WB WBC WHO

xvii

thyroid function test transient ischaemic attack total iron-binding capacity temporomandibular termination of pregnancy Treponema pallidum haemagglutination (test) thyroid stimulating hormone transurethral resection of the prostate Trichomonas vaginalis urea and electrolytes unprotected sexual intercourse upper respiratory tract infection ultrasound scan urinary tract infection ultraviolet ultraviolet wavelength A ultraviolet wavelength B ultraviolet wavelength C visual acuity value added tax Venereal Disease Reference Laboratory ventricular fibrillation venous thromboembolism varicella-zoster immunoglobulin withdrawal bleed white blood cell (count) World Health Organization

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1

CHAPTER CONTENTS Medical certificates 1 Statutory Sick Pay 1 The Medical Service of the Benefits Agency Employment rehabilitation 3 Food handlers 4 Maternity benefits 4 Financial benefits for patients 4 If you disagree with the BA decision

3

General

7

Fitness to drive 7 Common medical problems that affect driving 8 Driving and the elderly 10 Driving for the disabled 10 Exemption from the wearing of a car seat belt 11 Fitness to fly

11

Fitness to scuba dive Mental capacity

13

13

Advance directives

15

Victims of crime or accidents 16 Rape and indecent assault 16 Occupational disease reporting 17 References

18

MEDICAL CERTIFICATES (SOCIAL SECURITY, STATUTORY SICK PAY AND MATERNITY BENEFITS) Advice: Benefits Agency (BA) booklet Medical Evidence for SSP, SMP and Social Security Incapacity Benefit Purposes: A Guide for Registered Medical Practitioners leaflet IB 204 available from local BA offices. Details are also available on www.dwp.gov.uk/index.htm

Patients are deemed unfit for work because of: (a) illness; or (b) the danger of their passing on infection; or (c) the fact that they are receiving treatment on a regular basis (e.g. dialysis, chemotherapy or radiotherapy). STATUTORY SICK PAY To qualify for Statutory Sick Pay (SSP), a patient must be employed, be under age 65 and have been sick for 4 consecutive days. This includes days when the patient would not normally have worked. A leaflet for patients (NI224 SSP) is available from local Benefits Agency offices. Periods of sickness that are =£ 8 weeks apart count as one. An employee not eligible for SSP may claim incapacity benefit instead. Self certification: If patients are sick for 4 to 7 days they should, on the last day of illness, certify themselves on form SCI if claiming incapacity benefit or form SC2 if claiming SSP. The GP is not 1

2 GENERAL

required to fill in a certificate for the first 7 days of illness. * Use form Med 3 (FM3) if the patient has been seen and examined not more than 1 day before. Otherwise use form Med 5 (FM5). * Assess whether patients are fit to work at their normal occupation. If unemployed, consider the patients' fitness for their last job, or the job for which they are registered with the Employment Service.

Notes on form Med 3 Open certificates

(a) If the patient is not expected to return to work within 2 weeks, an 'open' certificate should be issued, i.e. in Section (b) write in an appropriate number of weeks. In the first 6 months of illness, this period should not exceed 6 months. (b) If the patient is unfit continuously for more then 6 months then the statement can be filled in for longer periods and, in cases of severe illness, 'until further notice' can be used. (c) If an 'open' statement is issued, a patient cannot return to work without a 'closed' certificate. Closed certificates

If a patient is expected to return to work within 2 weeks, a 'closed' statement can be issued (i.e. in Section (b), after the phrase 'OR until', the date that the patient is expected to return to work should be filled in). Backdating

The GP cannot backdate a certificate. However, when a patient has either failed to consult a doctor at the start of the illness or has allowed a gap to occur between expiry of one 'open' Med 3 and the signing of the next, the GP can, in the space for comments, write: 'continuously unfit since ....

Duplicates

If a certificate is lost a duplicate can be issued, but 'DUPLICATE' should be written in the space for comments.

Notes on form Med 5 Use it if:

(a) no statement has been issued since the patient was examined and the GP wishes to issue a certificate more than 1 day after examination; or (b) it is felt, on the basis of a report from another doctor (not more than 1 month previously), that the patient should refrain from work; or (c) the patient has returned to work without a 'closed' form Med 3 (see below). Note: Form Med 5 should not be issued for a future period of more than 1 month.

Diagnosis The diagnosis should be as precise and as accurate as possible, unless the patient's interests dictate otherwise. If the GP does not wish to write the precise diagnosis, an imprecise diagnosis can be used. At the same time, form Med 6 (FM6) (one copy with each pad of FM3s) should be filled in with the correct diagnosis and sent to the local Benefits Agency office.

Notes on form Med 4 Form Med 4 (FM4) is issued when requested by patients, who will have been instructed to ask for it by the Benefits Agency. This usually happens after 28 weeks illness. The doctor should: (a) certify whether patients should refrain from their usual occupation; and (b) give the full diagnosis and further information, on which the BA will base the decision whether patients are fit for any work (the Personal Capability Assessment).

MEDICAL CERTIFICATES

Once given, the GP is not required to issue further certificates.

THE MEDICAL SERVICE OF THE BENEFITS AGENCY The Medical Service may be involved: (a) at the request of an employee because the employer has refused to pay SSP. This happens when an employer does not accept that the employee is unfit to work; (b) where an employer asks for a second opinion, as in the case of prolonged or frequent illness, e.g. more than four periods of illness of 4 to 7 days each in any one year; (c) where an adjudication officer at the local BA office cannot find sufficient reason to grant incapacity benefit to a patient who is claiming it. Doctors who want a second opinion while continuing to issue certificates may refer cases to the Medical Service via the local BA office using form RM7 (one copy with each pad of FM3s). Patients will not be told that the GP made the referral. The Service is, however, unlikely to review patients if they are claiming SSP and not incapacity benefit.

When the GP disagrees with the decision of the BA When an adjudication officer declares that a patient is fit for work, a GP may only continue to issue form Med 3 if the patient's condition has deteriorated or the diagnosis has changed. It is important to detail the change under 'Doctor's remarks'. If neither of these circumstance applies, the patient should appeal against the decision of the adjudication officer to the Independent Tribunal Service. The GP can assist the appeal by: (a) writing in support of the patient; (b) asking a consultant for an opinion; (c) referring the patient to the Disability Employment Adviser for an opinion; (d) advising the patient to obtain support from a trade union or local rights organization.

3

EMPLOYMENT REHABILITATION As soon as it becomes clear that a patient will never be fit to return to previous work, consider whether rehabilitation or a change of job would be advisable. It is worth raising this topic before the BA applies the Personal Capability Assessment.

Options when the patient may be fit for alternative work (a) Issue a closed form Med 3, and in the comments section write 'fit within limits'. State what work might be suitable; or (b) continue to issue an open form Med 3 and refer, with the patient's consent, to the Disability Employment Adviser at the Jobcentre. Write a letter or complete form DP99 (available from the Jobcentre). The Adviser can arrange for the patient to be registered as disabled, which can lead to a number of facilities including retraining and placement in sheltered workshops or in vacancies reserved for the disabled. The Adviser also has access to schemes which provide special equipment and assistance with fares, and to the Job Introduction Scheme (which finances a disabled person in a job for a 6-week trial); or (c) continue to issue an open form Med 3 and recommend that the patient takes on part-time or light work as therapy. This can be done without loss of benefits, provided the doctor recommends it as therapeutic and the patient earns less than a certain amount and works for fewer than 16 hours a week. After April 2002 it will be called 'permitted work' and prior approval by a doctor will not be required. Prior approval from the local BA office is necessary before starting work to avoid accusations of 'moonlighting'.

Council tax discount Patients are entitled to a council tax discount if they are 'substantially and permanently disabled' or severely mentally impaired or they come into certain categories of carers.

4 GENERAL

FOOD HANDLERS Advice: Department of Health (DoH). Food Handlers' Fitness to Work. London: HMSO, 1995. Available from DoH, PO Box 410, Wetherby, LS23 7LN

Food handlers with diarrhoea or vomiting that is likely to be infectious should refrain from work and not return until 48 hours after the diarrhoea or vomiting has ceased. Indicators of infection are the presence of fever, or more than one episode of diarrhoea or vomiting. They should be notified as suspected food poisoning. Negative stool samples will only be needed from those with Salmonella typhi or paratyphi (six samples) or toxin-producing Escherichia coli (two samples). Food handlers with hepatitis A may return to work 7 days from the onset of symptoms. Food handlers with a staphylococcal or streptococcal infection may return to work once the lesion has healed, or sooner if it can be adequately covered. This applies to infections of the skin, eye, ear or mouth.

starting the time during which she draws her allowance until, at the latest, 1 week after the baby's birth. SMP or maternity allowance may be paid for a shorter period if a woman: (a) continues to work into the last 6 weeks of pregnancy (i.e. after 34 weeks of pregnancy); or (b) claims late.

Form Mat B1 * A form Mat B1(A) should be issued either by the GP or by the midwife not earlier than 20 weeks before the expected week of delivery (i.e. after 20 weeks of pregnancy). Examination may have been before this time. * If maternity allowance is claimed after confinement, use form Mat Bl (B).

FINANCIAL BENEFITS FOR PATIENTS

MATERNITY BENEFITS Guidance: The Benefits Agency. A Guide to Maternity Benefits. Leaflet Nl 17A from local BA offices.

Review: Teale C. Money problems and financial help. BMJ 1996; 313: 288-90. Guidance: www.dwp.gov.uk/index.htm

Most working women will be entitled to Statutory Maternity Pay (SMP) or Maternity Allowance (MA). The regulations are complex, and it is for the woman's employer to tell her the details. A woman not entitled to SMP nor to MA should claim incapacity benefit from 6 weeks before the expected date of delivery (HDD) until 2 weeks after the birth. The woman should: (a) inform her employer at least 21 days before she intends to stop work (she must not stop work earlier than 14 weeks before the expected delivery, i.e. when 26 weeks pregnant); (b) hand in medical evidence of the expected date of confinement (form Mat Bl, see below). Both SMP and maternity allowance are paid for a maximum of 18 weeks from, at the earliest, 11 weeks before the expected week of confinement (i.e. 29 weeks pregnant). A woman may delay

Figures in brackets (below) refer to the BA leaflet number, and these leaflets are available from local BA offices. It is worth the GP using the same terminology as the BA when writing in support of patients' claims.

Patients with a low income and little savings In 2001 the threshold for savings was £8000 for patients aged <60, £12,000 for those 60 years old and over, or £16,000 for permanent residents in care homes. These patients are entitled to: (a) Income support (IS 2). (b) Housing (RR1) and Council Tax benefit (CTB 1).

FINANCIAL BENEFITS FOR PATIENTS 5

(c) Family credit (FC 31) (for those working 16 hours or more per week and bringing up one or more children). (d) Certain NHS benefits (AB 11): free prescriptions, dental treatment and sight tests, vouchers for glasses, help with hospital travel costs (form AG1 from hospital or BA offices). (e) Payments from the social fund (form SB 16): funeral payments, cold weather payments, maternity payments, budgeting loans, crisis loans and community care grants, which are designed to help people who would otherwise not manage to stay in the community to do so. These grants are discretionary, and may help with the purchase of beds, bedding, cooker, refrigerator, vacuum cleaner, clothes, shoes, furniture, heaters, washing machine, house repairs, removal costs, etc. They may be loans, and applicants considered too poor to repay them may be turned down. (f) Disabled persons tax credit for working disabled people on low earnings and low savings. The credit is reduced if savings are over £3000. The person must be aged 16 or over, work 16 or more hours a week and be restricted in the type, hours or amount of work by the disability. He or she must also be receiving one of the other disability benefits.

Patients who are chronically ill or disabled (regardless of income) These patients may be eligible for: (a) Incapacity Benefit (IB 200), which is paid for the first 28 weeks of illness except to those receiving SSP. Receipt of Incapacity Benefit in the first 28 weeks is dependent on a doctor issuing a medical certificate; thereafter, it depends on the Benefits Agency performing the Personal Capability Assessment. (b) Disability Living Allowance (DLA) (DS 704), which is for those aged under 65 who need help with personal care or who are aged 3 or over and have difficulty walking or aged 5 or over and need help getting around. People who did not qualify for the old Attendance and Mobility Allowances may qualify for the DLA, e.g. those over 16 who are unable to prepare a main cooked meal or who

have difficulty taking medication reliably. To qualify, a patient must have needed help for 3 months (except in the case of the terminally ill) and be likely to need help for at least a further 6 months. (c) Attendance Allowance (AA) (DS 702), which is for people aged 65 or over who need help with their bodily functions or to stop them being a danger to themselves or to others. Bodily functions include hearing, seeing, eating, dressing, washing and going to the toilet. The need must have existed for 6 months before applying (except in terminally ill patients, see below). It is not necessary actually to have someone provide that care. (d) DLA and AA in terminal illness. Patients likely to die within 6 months can claim DLA or AA immediately, whether or not they need looking after. They need not know that they are terminally ill. Patients should submit the ordinary DLA or AA claim form, accompanied by the doctor's report on form DS 1500. (e) Invalid Care Allowance (FB 31), which is for those under 65 when first claiming and who care every day, for at least 35 hours a week, for someone who is getting or waiting to hear about: AA, DLA, Industrial Injuries Disablement Benefit Constant Attendance Allowance or War Pensions Constant Attendance Allowance. It is not paid to someone who is receiving more than a minimal earning from another job. Married women and women who are separated from their husbands can now claim. (f) Industrial Injuries Disablement Benefit (N 16), which is for those disabled by an accident at work or by disease related to their work. Benefit can be paid from 15 weeks after the onset of disablement. Patients who suffer even a minor injury should seek a declaration from the BA on form BI 95, in case they need to claim benefits later. Only 1% disablement is required for some respiratory conditions. Conditions include: asthma, chronic bronchitis, deafness, pneumoconiosis (including silicosis and asbestosis), tenosynovitis and vibration white finger. (g) War pensions (MPL 153) For the war disabled and for their dependants. In addition to the pension, the disabled person is entitled to priority treatment for the disability from the NHS and payment of funeral expenses if death is due to the

6 GENERAL

disablement. There is provision for a widow or widower's pension, and allowances for children, parents and other relatives in certain circumstances. (h) Criminal Injuries Compensation. The injury must be severe enough to need sutures or to have caused a fracture, or hospital admission, or medical care for over 6 weeks, and can include care for psychological stress. Patients should apply to the Criminal Injuries Compensation Authority, Tay House, 300 Bath Street, Glasgow G2 4LN, tel. 0141 331 2726. Claims should normally be made within 2 years of the injury, (i) Vaccine damage payments, which are for people severely disabled as a result of immunization, performed in the UK, against diphtheria, tetanus, pertussis, polio, measles, mumps, rubella or Haemophilus influenzas type b. Claims should be made on form HB3 from the Vaccine Damage Payments Unit, Palatine House, Lancaster Road, Preston PR1 1HB. Free prescriptions (a) age <16 or 60 or over; (b) age 16-18 and in full-time education; (c) pregnancy or delivery of a baby in the last 12 months; (d) on a low income with little savings; (e) family in receipt of certain benefits, e.g. Income Support; (f) certain conditions: -permanent fistula requiring continuous surgical dressing or requiring an appliance; -epilepsy requiring continuous anticonvulsant drugs; -diabetes mellitus, except where treated by diet alone; -hypothyroidism; - hypoparathyroidism; -diabetes insipidus or other forms of hypopituitarism; -hypoadrenalism for which specific substitutional therapy is indicated; -myasthenia gravis; -continuing physical disability which makes it impossible to go out without the help of another person.

Free NHS sight tests (a) age <16 or 16-18 and in full-time education; (b) age 60 or over; (c) registered blind or partially sighted;

(d) diabetes; (e) glaucoma or aged over 40 with a first-degree relative with glaucoma; (f) receiving Income Support or certain other benefits; (g) people on low income; they need to apply in advance (using form HC1) for an HC2 or HC3 certificate; or to apply on form HC5 for reimbursement within 2 weeks of the test; (h) under the care of a hospital ophthalmologist; (i) requiring complex lenses.

Free NHS dental treatment (a) age <16 or 16-18 and in full-time education. Being aged <18 entitles patients to free treatment but not free appliances; (b) pregnant or delivered of a baby in the last 12 months; (c) receiving Income Support or certain other benefits; (d) on low income; they need to apply in advance (using form HC1) for an HC2 or HC3 certificate; or to apply on form HC5 for reimbursement within 2 weeks of the test; (e) resident in a residential care or nursing home and receiving help from the local authority with the fees.

Patients who are chronically ill or disabled and on low income with little savings These patients are, in addition, eligible for: (a) Disabled Persons Tax Credit. This is for those aged 16 or over with an illness or disability that puts them at a disadvantage in getting a job, but who are working at least 16 hours a week with low earnings. It is handled by the Inland Revenue. (b) Independent Living Fund. This is for those receiving DLA or AA who are not able to pay for the care they need to live at home. Application forms are available from the Independent Living Fund, PO Box 183, Nottingham NG8 3RD, tel. 0602 290423. (c) Community Care Grants. These are more likely to be awarded to patients on low income, especially if they are supported by a letter from their GP detailing the medical need.

FITNESS TO DRIVE

IF YOU DISAGREE WITH THE BA DECISION Advise your patient to get BA booklet NI 246, How to Appeal. The appeal must be made within 3 months of the original decision. Advise your patient also to seek guidance from an advice centre or solicitor. Support the patient's appeal by supplying a medical report. Further advice is available from: (a) local BA offices or Citizens' Advice bureaux; (b) Disability Rights Handbook, published by the Disability Alliance, 1st Floor, 88 Wentworth Street, London El 7SA, tel. 020 7247 8776; www. disability alliance. org (c) Welfare Benefits Handbook, published by the Child Poverty Action Group (CPAG), 94 White Lion Street, London Nl 9PF, tel. 020 7837 7979; www.cpag.org.uk (d) Disablement Income Group, PO Box 5743, Finchingfield, Essex CM7 4PW, tel. 01371 811 621. (e) The Disability Discrimination Act Information line 0345 622 633.

FITNESS TO DRIVE Advice: At-a-Glance Guide to the Current Medical Standards of Fitness to Drive. DVLA, 2001. Available on www.dvla.gov.uk/at_a_glance/content.htm

• It is the duty of licence holders to inform the DVLA if they suffer from: (a) a prescribed, relevant or prospective disability; or (b) a previously notified disability that has become worse. • The patient should notify the Drivers Medical Unit, DVLA, Swansea, SA99 ITU, tel. 0870 600 0301, or, in Northern Ireland, Driver and Vehicle Licensing, Castlerock Road, Coleraine, BT51 3GS, tel. 01265 41200. • The inclusion of prospective disability means that the patient is obliged to notify the DVLA as soon as an illness is diagnosed if that illness could

7

lead to the patient being unfit to drive in the future. Insulin-dependent diabetes is an example. The DVLA is likely to issue a licence for 1,2 or 3 years, which permits them to review the case. Temporary disabilities (e.g. fractured bones) that are not expected to last more than 3 months are exempt. • It is the duty of the doctor to inform patients with a disability of their obligation to notify the DVLA and the vehicle insurance company. Record that such advice has been given in the patients' notes. • Epilepsy is the commonest cause of collapse at the wheel, followed by blackouts, diabetes and cardiac conditions. In almost all these cases the majority of patients are aware of their condition, and, if they have not notified the DVLA, their insurance is liable to be invalid. Doctors unable to demonstrate that they warned patients of the need to notify the DVLA could be sued by that insurance company. • If there is doubt about a case, contact: (a) England, Wales and Scotland: the Medical Adviser, Drivers Medical Unit, DVLA, Longview Road, Swansea SA99 1TV, tel. 01792 761119. (b) Northern Ireland: Driver and Vehicle Licensing, Castlerock Road, Coleraine BT51 3GS, tel. 01265 41200.

Confidentiality Patients' names should not normally be disclosed to the DVLA without their consent. If, however, there is any doubt about whether the patient will inform the DVLA, the UK General Medical Council recommends the following steps1 (reproduced with permission): 1. The DVLA is legally responsible for deciding if a person is medically unfit to drive. The Agency needs to know when driving licence holders have a condition which may now, or in the future, affect their safety as a driver. 2. Therefore, where patients have such conditions you should: (a) Make sure that patients understand that the condition may impair their ability to drive. If a patient is incapable of understanding this advice, for example because of dementia, you should inform the DVLA immediately.

8 GENERAL

(b) Explain to patients that they have a legal duty to inform the DVLA about the condition. 3. If patients refuse to accept the diagnosis or the effect of the condition on their ability to drive, you can suggest that the patients seek a second opinion, and make appropriate arrangements for the patients to do so. You should advise patients not to drive until the second opinion has been obtained. 4. If patients continue to drive when they are not fit to do so, you should make every reasonable effort to persuade them to stop. This may include telling their next of kin. 5. If you do not manage to persuade patients to stop driving, or you are given or find evidence that a patient is continuing to drive contrary to advice, you should disclose relevant medical information immediately, in confidence, to the medical adviser at the DVLA. 6. Before giving information to the DVLA you should try to inform the patient of your decision to do so. Once the DVLA has been informed, you should also write to the patient, to confirm that a disclosure has been made. Table 1.1

The doctor has a similar responsibility to breach confidentiality in the case of a patient whose licence has been revoked on medical grounds and who continues to drive. In this case, with the provisos above, the doctor should notify the police.

What to do if the doctor or patient disagrees with the DVLA's decision The patient has the right of appeal, within 6 months in England and Wales and within 21 days in Scotland. This may be costly. An informal letter to the Medical Adviser from the doctor may be more appropriate.2

COMMON MEDICAL PROBLEMS THAT AFFECT DRIVING If a patient's medical condition poses a danger to the public, he or she must stop driving and

Neurological problems that affect driving

Condition

Notification of DVLA

Advice on driving a car or motorcycle

Epilepsy

Notify

A 'provoked' seizure

Notify

Withdrawal of anticonvulsants

No need to notify

A solitary fit

Notify

Chronic neurological disorders Disabling giddiness Stroke

Notify Notify Notify only if there is residual neurological deficit after 1 month (other than mild limb weakness) Notify

Stop driving. The licence is likely to be restored when free from seizures for 1 year, or when seizures in the last year have occurred only while asleep and the patient has had a previous seizure while asleep >3 years ago with no attacks while awake since Stop driving. Restoration of the licence is likely once the provoking factor has been removed Recommend stopping driving for 6 months after stopping treatment. The risk of a seizure is 40% greater in the first year than if treatment is continued Stop driving. The licence is likely to be restored after 1 year if there are no more fits Continue driving if performance is not impaired Stop driving. Restart if symptoms are controlled Stop driving for 1 month

Multiple frequent transient ischaemic attacks (TIAs) Loss of consciousness (LOG): simple faint - clear provoking factors LOG: probable syncope but no provoking factors - risk of recurrence low LOG: probable syncope but no provoking factors - risk of recurrence high (e.g. heart disease) LOG with no clinical pointers Unwitnessed LOG or altered awareness with seizure markers (e.g. incontinence or bitten tongue)

Stop driving for 3 months

Do not notify

No restrictions

Notify

Stop driving for 4 weeks

Notify

Stop driving for 6 months (or 4 weeks if the cause has been treated)

Notify Notify

Stop driving for 6 months Stop driving for 1 year

FITNESS TO DRIVE 9

Table 1.2

Cardiovascular problems that affect driving

Condition

Notification of DVLA

Advice on driving

Angina at rest or while driving Myocardial infarction Angioplasty Coronary artery bypass graft (CABG) Arrhythmias

No need to notify No need to notify No need to notify No need to notify No need to notify unless symptoms are distracting or disabling Notify

Stop driving until symptoms are controlled Stop driving for 4 weeks Stop driving for 1 week Stop driving for 4 weeks Stop driving if arrhythmia is causing incapacity until symptoms have been controlled for at least 4 weeks Stop driving for at least 1 week

Pacemaker insertion

Table 1 .3

Diabetes mellitus - effects on driving

Condition

Notification of DVLA

Advice on driving

On insulin

Notify

On oral drugs On diet alone

Notify No need to notify

Will be allowed to drive but on certain conditions. Should stop driving if frequent hypoglycaemic episodes occur or if awareness of hypoglycaemia is lost Need not stop driving unless other disabilities develop May drive unless other disabilities develop

Table 1.4

Psychiatric problems that affect driving

Condition

Notification of DVLA

Advice on driving

Severe anxiety or depression

Notify

Acute psychosis

Notify

Chronic schizophrenia

Notify

Dementia

Notify

Learning disability

Notify

Persistent behaviour disorder

Notify

Stop driving. Licence may be restored once a period of stability has been reached Stop driving. Licence may be restored after up to 12 months of stability Stop driving until the DVLA permits it. Licence may be restored once a period of stability has been reached Stop driving. Licence may be restored if symptoms are mild Do not start driving. Licence may be granted if the disability is mild Stop driving. Licence may be restored if behaviour is controlled satisfactorily

Table 1.5

Drug and alcohol misuse - effects on driving

Condition

Notification of DVLA

Advice on driving

Alcohol misuse

Notify

Alcohol dependency

Notify

Cannabis, ecstasy, LSD, etc.

Notify if dependent or using them persistently Notify if dependent or using them persistently Notify if dependent or using them persistently

Stop driving. Licence may be restored after 6 months abstinence or controlled drinking Stop driving. Licence may be restored after for 1 year Stop driving. Licence may be restored after for 6 months Stop driving. Licence may be restored after for 1 year or stabilisation on a maintenance Stop driving. Licence may be restored after for 1 year

Heroin, cocaine, etc. Benzodiazepine misuse

at least abstinence abstinence abstinence programme abstinence

10

GENERAL

Table 1.6 Visual problems that affect driving Condition

Notification of DVLA

Advice on driving

Severe bilateral cataract

Notify

Monocular vision Visual field defects

Notify Notify

Diplopia

Notify

Stop driving if unable to read a number plate at 20.5 metres. This lies between 6/9 and 6/12 on the Snellen chart May drive once the patient has adapted to the disability Stop driving until DVLA confirms that the national requirements are met: a field of at least 120° horizontally plus binocular vision 20° above and below the horizontal meridian Stop driving. Restart only on confirmation to the DVLA that it is controlled by glasses or a patch

notify the DVLA whether or not the condition is listed above. The details of the more common conditions above are taken from the DVLA publication At-a-Glance Guide to the Current Medical Standards of Fitness to Drive March 2001 updated August to September 2001. A more recent and detailed version is available on www.dvla.gov.uk/at_a_ glance/content.htm * Advise patients to notify their insurance company of any relevant medical condition, even if it is not necessary to notify the DVLA. Failure to do so could lead to a refusal by the company to support the patient if an accident occurs that is related to the condition.

DRIVING AND THE ELDERLY • Drivers over the age of 70 have to renew their licence every 3 years, making a declaration of health. • Elderly patients are more likely to have impaired concentration, memory loss and slower reaction times, as well as being more likely to have other conditions that make them unfit to drive. • The GP may need to assess elderly patients at the latter's request when they are applying for renewal of a driving licence, or at the request of the insurance company. Modifications to the car may enable the patient with locomotor problems to drive. It may be easier to ask a partner to perform the medical examination. • If there is doubt, a single trial driving lesson from a professional instructor will give the patient an impartial opinion. Patients who have

lost confidence or have not driven for some time may benefit from a course of lessons.

DRIVING FOR THE DISABLED A disabled patient who wishes to drive Most patients who are disabled can drive cars with suitable modifications, including paraplegics, hemiplegics and those who are wheelchair-bound. They should be aware of the following: • DVLA. Whether already holding a driving licence or applying for one for the first time, the first step is for the patient to notify the DVLA (see p. 7) of the disability. • Driving Assessment Centres. There are ten driving assessment centres in the UK that will assess the patients disability and advise on modifications to vehicles. Most are private, but one is run by the Department of Transport and offers advice free (see below). An information pack is available from Mobility Advice and Vehicle Information Service (MAVIS), DETR, O Wing, Macadam Avenue, Old Wokingham Road, Crowthorne, Berkshire RG45 6XD, tel. 01344 661000; www.mobility-unit.detr.gov.uk/mavis.htm

• Car modification firms. Once advice has been obtained, the assessment centre will provide a list of firms able to carry out modifications. The Disabled Living Foundation (www.dlf.org.uk) also has a list. • A driving instructor specially trained to teach the disabled may be needed. For information, contact The Association of Driver Educators of People

FITNESS TO FLY 11

with Disabilities, Banstead Mobility Centre, Damson Way, Orchard Hill, Queen Mary's Avenue, Carshalton, Surrey SMS 4NR. • Finance. The Disability Living Allowance (see p. 5) entitles the driver to other advantages: exemption from vehicle excise duty, a Blue Badge, and access to the Motability Scheme, which helps applicants to buy or hire cars or outdoor electric wheelchairs. Contact: Motability, Goodman House, Station Approach, Harlow, Essex, CM20 2ET, tel. 01279 635666; www.motability.co.uk

The Blue (formerly Orange) Badge • Patients over the age of 2 are eligible for a Blue Badge if they have: (a) a permanent and substantial disability that causes very considerable difficulty in walking. As a guideline, patients normally able to walk more than 50 metres unaided are unlikely to be considered suitable; or (b) have a severe disability in both upper limbs, regularly drive a motor vehicle but cannot turn the steering wheel even if that wheel is fitted with a turning knob; or (c) are registered blind; or (d) receive the higher rate mobility component of the DLA; or (e) receive a war pension mobility supplement; or (f) use a motor vehicle supplied for disabled people by a Government Health Department. • The patient may use the badge either as a driver or as a passenger. • The patient should apply to the local Social Services department for the Badge. • More information is to be found on www. mobility-unit.detr.gov.uk/bluebadge/advice/ 01.htm Refusal to support the patient's application for the badge may cause friction between doctor and patient. Minimize this by: (a) walking with the patient to establish his or her limits; (b) pointing out that the final decision lies not with the GP but with the issuing office, to whom complaints should be made.

EXEMPTION FROM THE WEARING OF A CAR SEAT BELT * Advise patients who ask for an exemption certificate: (a) to read the booklet Seat belts and child restraints, published in 1999 and available from the Department of Transport, Local Government and the Regions, www.think.dtlr.gov.uk/ childcarseats/pdf/seatbelt.pdf (b) that (except in specific cases) a fee is payable for the examination, but that a free examination may be arranged by the Department of Transport (see the above leaflet); (c) that it is very unlikely that a certificate will be issued. There are few conditions, if any, which permit patients to drive and yet render them unfit to wear a seat belt. * Accompany the patient to the car and explain how the repositioning of attachments or the 'Clever Clip' to reduce pressure across the chest or abdomen may solve the problem. Certificates. If you do intend to issue a certificate, use the official ones obtainable from the Department of Health, PO Box 410, Wetherby LS23 7LN. If in doubt or in difficulties, the doctor or patient can discuss the problem with: Advice for disabled drivers: RADAR (Royal Association for Disability and Rehabilitation), 12 City Forum, 250 City Road, London EC1V 8AF, tel. 020 7250 3222; www.radar.org.uk MAVIS Transport Research Laboratory, DETR, O Wing, Macadam Avenue, Old Wokingham Road, Crowthorne, Berkshire RG45 6XD, tel. 01344 661000; www.mobility-unit.detr.gov.uk/mavis.htm

FITNESS TO FLY Guidance: British Airways Health Services: Your Patient and Air Travel: A Guide to Physicians. Available from British Airways Health Services, Waterside (HMSG), PO Box 365, Harmondsworth UB7 0GB and on www.britishairways.com/health (click on 'before your flight').

• An ill or disabled passenger who is contemplating flying should notify the airline in advance

12 GENERAL

on a form obtainable from the travel agent or the airline. Urgent cases should be discussed by telephone with the airline medical service. • Such a passenger should inform his or her insurer in writing of all pre-existing medical conditions. • Patients who might need medication during the flight must carry it in their hand luggage. Airlines stress that each case is considered on its merits but that the following rules are likely to apply: • Locomotor disability. Patients with arthritis or other lower limb problems may need the extra legroom of First Class unless the airline can provide economy seating with extra space. Electric buggies and hoists can get patients on to the plane. Airlines will normally accommodate a stretcher, at a price, but any patient on a stretcher must be accompanied by a trained person. Special wheelchairs can be used to transfer patients from seat to toilet, but cabin staff can not assist a patient in the toilet because they are food handlers. • Hypoxia. Patients who cannot tolerate mild hypoxia are either unfit to fly or will need oxygen during the flight. These patients include those suffering uncontrolled heart failure, stroke in the last 3 days, even if recovering, myocardial infarction in the last 7 days, a grand mal fit in the last 24 hours or anaemia below 7.5g/dl. Patients with chronic respiratory disease who cannot walk 50 metres on the flat, or climb one flight of stairs are unfit to fly. Patients with arteriosclerotic dementia may become confused. A patient may not fly within 10 days of a sickle-cell crisis and anyone with the disease, although not the trait, must travel with supplementary oxygen. • Difficulties due to gas expansion. Gas in the body is likely to expand by about 30% during the flight. Patients who cannot tolerate this include those: (a) with blocked Eustachian tubes and sinuses; (b) with a tonsillectomy or middle ear surgery in the last 2 weeks; (c) within 10 days of bowel or chest surgery; (d) within 3 weeks of a gastrointestinal bleed; and those with an active peptic ulcer;

(e) within 7 days of air encephalography or intraocular surgery; and not within 6 weeks if gas has been used in the treatment of retinal detachment; (f) with a pneumothorax; (g) who have been scuba diving in the 12 hours before the flight; (h) with a recently applied plaster cast (patients should wait for 24 hours before a flight of less than 2 hours and 48 hours before a longer flight, unless the cast is capable of expansion). • Time zones. Epilepsy may be precipitated by fatigue when flying, and patients should increase rather than decrease their medication as they change to the new time zone. Insulin-dependent diabetics should carry everything they need in their hand luggage in case of delays, and should take enough food with them for them to be able to keep to their own time zone during the flight. Melatonin is remarkably effective in reducing jetlag,3 although it is not licensed in the UK. Between 0.5 and 5 mg should be taken at bedtime on arrival at the destination. Patients with epilepsy and those on warfarin should avoid it. Otherwise it is recommended, especially for those crossing at least 5 time zones, particularly if travelling east. • Pregnancy. A pregnant woman may fly up to and including the 35th week of pregnancy on international flights, and the 36th week on domestic flights. A woman with a multiple pregnancy may not fly after the 31st week. If a woman is >28 weeks pregnant she should carry with her a certificate confirming her EDD. Newborn babies should not fly in the first 2 days of life while their alveoli are still expanding. • Deep vein thrombosis (DVT). A patient with an acute DVT may not fly. All travellers should keep well hydrated, exercise the legs and walk about the cabin as much as possible. Older patients on long-haul flights and others at high risk should consider the use of compression stockings. In one study, below-knee UK class II stockings (pressure 18-24mmHg) reduced the incidence of asymptomatic DVTs in travellers on long-haul flights with an average age of 62 but no other risk factors from 10% to nil.4'5 High-risk cases should consider taking aspirin and may even merit warfarin, although there is

MENTAL CAPACITY

no direct evidence of benefit in this situation. The Department of Health in November 2001 (http:/ / www.doh.gov.uk/dvt/index.hrm) defines high risk as: (a) those who have ever had a DVT or pulmonary embolus (PE); (b) anyone with a family history of clotting conditions; (c) those with an inherited tendency to clot (thrombophilia); (d) people with cancer or who have had treatment for cancer in the past; (e) those who have undergone major surgery in the last 3 months; (f) those who have had a hip or knee replacement within the last 3 months; (g) anyone who has suffered a stroke. • Other conditions (a) Contagious patients may not fly. (b) Psychiatric patients should be accompanied by someone competent to handle them if they are unsettled by the stress of flying. (c) Airlines will not accept the terminally ill if they are liable to die on the flight. (d) Patients with offensive conditions are not welcomed by airlines. (e) Patients with wired jaws must be able to release the mechanism in the event of vomiting. FITNESS TO SCUBA DIVE Review: Sykes JJW. Medical aspects of scuba diving. BMJ 1994; 308: 1483-8. Study: Glen S, White S, Douglas J. Medical supervision of sport diving in Scotland: reassessing the need for routine medical examinations. Br J Sports Med 2000; 34:375-8.

Until recently, diving was only possible in the UK for those in possession of a medical certificate certifying them as fit to dive. At the time of writing a new system is proposed in which divers complete an annual Medical Declaration Form and only those answering 'yes' to one or more questions would need to contact their local medical referee. General practitioners are, however, still likely to be asked for advice on this subject.

13

The combination of cold water, high pressure and strenuous exercise makes great demands on the diver. The 'buddy' system of diving means that any diver must be capable of rescuing another. The following conditions are likely to exclude an applicant from diving: (a) asthma, although some authorities consider controlled asthma to be a relative contra-indication only; (b) spontaneous pneumothorax, pulmonary cysts or other respiratory disease; (c) cardiac disease (this is the cause of almost half the deaths in divers over 40 years old); (d) orthopaedic problems including acute and chronic back pain; (e) problems with balance; (f) diabetes, unless controlled on diet alone; (g) neurological disorders including epilepsy and other causes of loss of consciousness; (h) a past history of head injury; (i) poor vision and, especially, a history of eye surgery; (j) problems with Eustachian tubes, drums, middle or inner ear disease, other than presbyacusis; (k) upper airways disorders, including laryngectomy; (1) certain upper GI problems including severe reflux; (m) certain haematological disorders: sickle-cell disease, polycythaemia, acute anaemia; (n) neuromuscular disorders; (o) psychiatric difficulties; (p) obesity (BMI >30) although an individual who can demonstrate adequate agility in the pool may be considered fit; (q) pregnancy. More information from the UK Sport Diving Medical Committee is available on www.uksdmc.co.uk

MENTAL CAPACITY Guidance: The Law Society and the BMA. Assessment of Mental Capacity; Guidance for Doctors and Lawyers. London: British Medical Association, 1995.

14 GENERAL

In all the following situations, a doctor asked to give an opinion on a patient's mental capacity, should: (a) have access to the patient's records; (b) seek information from friends, relatives and carers; (c) examine the patient to assess the type and degree of mental disorder as well as the patient's ability to comply with the specific requirements listed below; (d) check whether assessment should be postponed while measures are taken to improve the patient's capacity; (e) record all the above information. A doctor who thinks that a proposed action is in the patient's best interests should not be swayed by this into judging that the patient is capable if this is not clearly the case. If there is doubt, it is wise to seek a second opinion.

Testamentary capacity (the capacity to make a will) Patients who are mentally disordered can make a valid will provided they understand: (a) the nature and effect of making a will; (b) the extent of the property being disposed of; (c) the claims others may have on that property; and provided their decision is not the result of the mental disorder, for instance, the result of a delusion. The decision need not appear sensible to others, especially if it is consistent with the patient's previous personality.

Power of attorney This may be an ordinary power of attorney, which ceases to have effect if the patient (the donor) becomes mentally incapable; or an enduring power of attorney (EPA), which continues if the donor is mentally incapable, provided it is registered with the Court of Protection. (In Scotland, an ordinary power of attorney signed after January 1991 remains valid even if the donor becomes mentally incapable.) Power of attorney only covers

financial matters, not, for instance, consent to treatment. For an ordinary power of attorney, it is sufficient that the donor understands the nature and effect of what he or she is doing. For an EPA the donor must understand: (a) that the attorney will be able to assume complete authority over the person's affairs; (b) that the attorney will be able to do anything with the donor's property that the donor could have done; (c) that the authority will continue if the donor becomes mentally incapable; and (d) that the authority will be irrevocable while the donor remains incapable.

Court of Protection If a person, by reason of mental disorder, becomes incapable of managing his or her affairs, and has not previously signed an EPA, it may be necessary for someone, usually the nearest relative, to apply to the Court of Protection for the appointment of a 'receiver' to do so. The medical practitioner will be asked to complete form CP3. If the patient's affairs are simple, e.g. the drawing of the state pension, arrangements can be made with the Social Security authorities without involving the Court.

The capacity to consent to marry and engage in sexual intercourse In order to be able to consent to marry, a person with a mental disorder must be able to understand broadly what marriage entails. Similarly, those who are mentally disordered can consent to sexual intercourse if they understand what is proposed and its implications, and are able to exercise choice.

The capacity to consent to medical treatment Guidance: The BMA's views on the capacity to consent to and refuse medical treatment can be found on www.bma.org.uk/ (choose 'ethics' then 'guidelines').

ADVANCE DIRECTIVES

A patient must be able to: (a) understand what the treatment is and why it is proposed; (b) understand the benefits, risks and alternatives; (c) understand the consequences of not having the treatment; (d) retain the information long enough to make a decision; and (e) make a free choice. If competent, an adult can refuse a procedure whether the decision appears rational or irrational. However, if the decision appears to contradict the patient's own previously expressed attitudes it calls into question the patient's competence. If an adult is unable to consent, because, for instance, of coma or mental incompetence, no-one else can do so, but treatment can be carried out if it is 'necessary'. Indeed, it is the doctor's duty, under common law, to give treatment to an incapacitated adult if it is in the patient's best interests. The opinions of the relatives are important, as possibly shedding light on the choice the patient might have made, but a consent form signed by a relative carries no legal force. Age

Patients aged 16 and above can consent to treatment. Those under 16 can either consent to treatment themselves if they have 'sufficient discretion to be able to make a wise choice in their best interests', or their parents can consent for them. A child's consent to treatment cannot be overridden by the parents if the doctor judges that the child has the above discretion. The BMA advises that, to be judged capable, a child should have: (a) the ability to understand that there is a choice and that choices have consequences; (b) the willingness and ability to make a choice (including the option of choosing that someone else make treatment decisions); (c) understanding of the nature and purpose of the proposed procedure; (d) understanding of its risks and side-effects;

15

(e) understanding of the alternatives to the proposed procedure and the risks attached to them and the consequences of no treatment; (f) freedom from pressure. If a child refuses treatment and is judged by the doctor to be competent, however, there is a possibility that his or her decision can be overruled by the parents or by a court. In Scotland parental consent is needed for any child under age 16.

ADVANCE DIRECTIVES (ADVANCE STATEMENTS OR LIVING WILLS) Advance statements: the BMA's views can be found on www.bma.org.uk (choose 'ethics' then 'guidelines').

These are statements where a person makes a decision about medical treatment in case he or she becomes incapable of making that decision later. A patient may decline specific treatments. A patient cannot insist on specific treatment but can authorise one, should it be offered. It is legally binding if: (a) it is in writing and is clear what the patient means; and (b) it is applicable to the current situation; and (c) the patient is competent and is broadly informed about the treatment in question; and (d) it was made without undue pressure from others. The BMA recommends that doctors should not withhold 'basic care' (e.g. symptom control) even in the face of a directive which specifies that the patient should receive no treatment. Despite an advance directive, a doctor in the UK cannot do anything whose prime purpose is to hasten death. Information and the relevant forms are available from: The Terence Higgins Trust, 52-54 Gray's Inn Road, London WC1X 8JU; www.tht.org.uk The Voluntary Euthanasia Society, 13 Prince of Wales Terrace, London W8 5PG, tel. 020 7937 7770; www.ves.org.uk

16

GENERAL

VICTIMS OF CRIME OR ACCIDENTS Advice: Victim Support, Treating Victims of Crime: Guidelines for Health Professionals. Available from the national office of Victim Support (see box below).

Immediate management

The doctor's role is to support, treat and ensure the safety of the patient, as well as to gather information for legal purposes. * Ensure privacy. * Offer a woman a female doctor or chaperone if she has been assaulted by a man. * Check whether the patient intends to report the assault to the police. Respect the patient's decision, except in the case of child abuse, which must be reported. Explain that reporting is necessary if the victim wishes to claim Criminal Injury Compensation. * Discuss whether the patient is safe to return home. If not, recommend a woman's refuge (via the duty Social Worker) or urgent homeless accommodation from the Council Housing Department. * For legal purposes: (a) document the date, time and place of the event; (b) document any injuries in detail, both physical and psychological; (c) arrange for a photograph to be taken, unless the police have already done so. * Tell the patient about Victim Support. * At subsequent meetings look for evidence of post-traumatic stress disorder (see p. 315). * Sexual assaults: See below. Victim support: local schemes and the witness service in the crown court may be found in the local directory. Victim Support National Office (for England, Wales and Northern Ireland), Cranmer House, 39 Brixton Road, London SW9 6DZ, tel. 020 7735 9166; e-mail [email protected]; www.victimsupport.com Victim Support Scotland, 15/23 Hardwell Close, Edinburgh EH8 9RX, tel. 0131 668 4486; e-mail [email protected]; www.victimsupport.com

RAPE AND INDECENT ASSAULT The implications of the examination of an alleged victim of a sexual offence are so great that it is best for this to be done by a doctor trained and experienced in such work. The police or the patient may, however, request a GP to perform the examination. The police will provide a kit for taking samples. Strict attention to procedure is essential: (a) Obtain written consent from the woman concerned, or from the parents or guardian if under 16 years of age. Include the wording that this is 'a declaration of consent to medical examination for non-treatment purposes'. (b) Examine the patient where lighting and facilities are adequate. (c) Obtain: - a detailed history: this may determine how you look for forensic evidence; - the date of her last menstrual period; -how long ago she last had intercourse other than the alleged assault; -any drugs or alcohol taken. (d) Record everything. (e) Note the appearance and mental state of the patient. (f) The patient should undress on a large sheet of new brown paper, which can be folded and sent to the laboratory. The state of the underclothing should be noted, and whether any stains are visible. All clothing worn at the time of the alleged incident should be sent in a clean polythene or paper bag for analysis. (g) The whole body should be examined for injuries or stains. Scrape off stains if possible, or remove with a moistened swab if unable to remove dry. Label each one with details of which part of the body it came from. (h) Examine genitalia and: - remove stains as above; -comb the pubic hair and send any loose hairs; -pluck a pubic hair from the patient and send that separately. (i) Aspirate vaginal fluid before digital examination; also fluid from the anus if anal intercourse

VICTIMS OF CRIME OR ACCIDENTS 17

is alleged. Smear on to a microscope slide only if there will be a delay of more than 24 hours before it is examined. Spermatazoa survive in the vagina for up to 6 days and in the rectum for up to 3 days. (j) Note specifically the state of the vulva, hymen, vagina, anus and mouth. (k) Take swabs for sexually transmitted diseases. (1) Take fingernail scrapings. (m) Take 10ml clotted blood. (n) Take saliva. Examination of an alleged assailant follows the same pattern. Informed consent is essential.

Once the examination is complete Discuss with the patient the need for: (a) emergency contraception; (b) prophylactic antibiotics, e.g. ciprofloxacin 500 mg and azithromycin 1 g orally stat; (c) follow-up for counselling - inform the patient of the service offered by the nearest Rape Crisis Centre, or the victim support scheme; (d) a blood test 3 months later for syphilitic serology and, at 3 and 6 months, for HIV and hepatitis B and C antibodies; (e) prophylaxis against hepatitis B and HIV.

Male victims of sexual assault The procedure to be followed is as described for women, with obvious anatomical differences.

Patient organizations: Independent Care after Incestuous Relationships and Sexual Abuse, Gatehouse, Great Chesterford, Essex, CB10 1NX, tel. 01799 530520 Survivors, PO Box 2470, London W2 1NW, helpline 020 7613 0808 for male rape victims. www.survivorsuk.co.uk Rape Crisis Federation, www.rapecrisis.co.uk HAVOCA (Help for Victims of Child Abuse); e-mail: [email protected] (general enquiries) or [email protected] (for support); www.havoca.org

OCCUPATIONAL DISEASE REPORTING Advice: RIDDOR: Information for doctors. London: Health and Safety Executive, 1995. Available from HSE Books, PO Box 1999, Sudbury, Suffolk CO10 2WA; tel. 01787 881165; www.hsebooks.co.uk

Since 1 April 1986, doctors have not been obliged to notify occupational diseases. They are, however, required to inform the employers, in writing and with the patients' consent, if patients develop a reportable disease known to be linked with their occupation. The doctor need not decide whether the disease is, in each particular case, caused by the occupation. Employers are, in turn, obliged to notify the local office of the Health and Safety Executive (HSE). The self-employed are obliged to report the disease themselves. Reports should be made to the Incident Contact Centre 0845 3009923; www.riddor. gov.uk If the patients do not consent to the notification of employers, they might agree to the notification of their employers' occupational health service or to direct notification to the Employment Medical Advisory Service of the HSE. Addresses are contained in the RIDDOR booklet above. Some examples of reportable diseases that are commonly not reported are: (a) occupational asthma; (b) irritant dermatitis; (c) folliculitis, acne or skin cancer in workers exposed to mineral oil, tar, pitch or arsenic; (d) vibration white finger in workers using vibrating or percussive machinery; (e) tenosynovitis in workers doing movements of the hand or wrist; (f) bursitis (e.g. 'housemaids knee') in workers involved in kneeling; (g) writer's cramp and all repetitive strain injuries; (h) occupational deafness; (i) hepatitis B in health workers. Patients should be referred to the BA booklet NI.2, obtainable from their local BA office.

18

GENERAL

REFERENCES 1. General Medical Council. Confidentiality: Protecting and Providing Information. September 2000; Appendix 2. London: GMC. Online. Available: www.gmc-uk.org 2. Rouse E. DVLA Senior medical adviser's reply. BMJ 1995; 311: 1163. 3. Herxheimer A, Petrie KJ. Melatonin for the prevention and treatment of jet-lag (Cochrane Review).

In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 4. Scurr JH, Machin SL, Bailey-King S et al. Frequency and prevention of symptomless deep-vein thrombosis in longhaul flights: a randomised trial. Lancet 2001; 357: 1485-9. 5. Anderson R. Deep-vein thrombosis in long-haul flights (letter). Lancet 2001; 358: 837.

CHAPTER CONTENTS Specific infectious diseases and immunizations 19 Incubation and infectivity periods of common diseases 19 Notification of infectious diseases 19 Procedure when giving an immunization 19 Vaccine damage payments 20 Live vaccines 20 HIV-positive patients 21 Diphtheria immunization 21 Haemophilus influenzae type b 22 Hepatitis A - active immunization 22 Hepatitis A - passive immunization 23 Hepatitis B: recommendations for active immunization 23 Hepatitis B: recommendations for passive immunization 24 Chronic hepatitis 24 Influenza 25 Measles, mumps and rubella 26 Measles 27 Mumps 27 Rubella 27 Meningococcal infection 27 Pertussis 28 Pneumococcal infection 29 Polio 29 Tetanus 30 Tetanus-prone injury 30 Tuberculosis 31 Varicella (chickenpox) and herpes zoster - passive immunization 31 Advice for travellers and immunization for travel abroad 32 Travellers' diarrhoea 33 Advice for doctors on immunizations 33 A suggested schedule of immunizations 33 Cholera 33 Japanese encephalitis 34 Malaria 34 Meningococcal immunization 35 Rabies 35 Tick-borne encephalitis 36 Typhoid 36 Yellow fever 36 Symptoms in travellers returning from abroad 37 Fever 37 Diarrhoea 38 Jaundice 39 Pharyngitis 39 Sexually transmitted diseases 39 Screening of immigrants and those who have spent a long time abroad 39 Screening returning travellers 39 Screening of immigrants 40 References

40

2 Infectious diseases

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATIONS Guidance: Immunization against Infectious Disease 1996 (known as the 'Green Book'), published by the DoH, the Scottish Home and Health Department and the Welsh Office. Available from The Stationary Office, PO Box 29, St. Crispins, Duke St, Norwich NR3 1GN and authorized bookshops and from www.the-stationary-office.co.uk More recent information, for doctors and patients, is available on the website of Health Promotion England, www.immunisation.org.uk Department of Health. Health Information for Overseas Travel, 2nd edition, 2001. London: The Stationary Office, 2001.

INCUBATION AND INFECTIVITY PERIODS OF COMMON DISEASES See Appendix 2.

NOTIFICATION OF INFECTIOUS DISEASES See Appendix 4.

PROCEDURE WHEN GIVING AN IMMUNIZATION * Establish that the immunization is needed. * Check that the patient is fit. 19

20

INFECTIOUS DISEASES

* Check that consent has been obtained. In childhood immunizations this usually consists of: (a) written consent to the programme in general; and (b) verbal consent to the specific immunization at the time (bringing a child to an immunization clinic has been viewed as giving consent, but parents may not realise which vaccines are going to be given); * Check that the immunizations are in-date and that the cold chain has not been interrupted; check that facilities for resuscitation are available. * Record the date, vaccine and batch number in each patient's medical record. * Record the site if more than one injection was given.

Contraindications It is important to be clear about the genuine contraindications to immunization (see under individual vaccines) and not to be deflected from immunizing children because of: (a) eczema; (b) a family history of allergy; (c) a personal history of allergy unrelated to the vaccine in question; (d) a minor infection, without fever or systemic upset; or (e) a minor reaction to a previous immunization.

Table 2.1

Major reactions A major reaction may be: (a) a local reaction, where induration involves most of the anterolateral surface of the thigh or most of the circumference of the upper arm; or (b) a general reaction, where fever is s=39.5°C within 48 hours, or convulsions or encephalopathy occur within 72 hours, or there is prolonged inconsolable or high-pitched screaming for more than 4 hours, or other signs of major systemic reaction.

VACCINE DAMAGE PAYMENTS Vaccine damage payments are for those suffering 80% or more disablement as a result of immunization with one of the recommended childhood vaccines administered in the UK under the age of 18. Claim forms are available from the Vaccine Damage Payments Unit, Department of Social Security, Palatine House, Lancaster Road, Preston PR11HB.

LIVE VACCINES These are: (a) BCG; (b) measles; (c) mumps;

Routine schedule of immunizations

Age

Immunization

2 months 3 months 4 months 12-15 months 3-5 years 1 0-1 4 years 1 5-1 9 years 20-24 years 65 onwards

DTwP DTwP DTwP MMR DTaP BCG (if tuberculin negative) Td

Hib Hib Hib Men C if not already given MMR Men C if not already given MMR if not already given

polio polio polio

Men C Men C Men C

polio polio

Men C if not already given Men C if not already given

flu annually

aP, acellular pertussis; D, diphtheria; d, low dose diphtheria; flu, influenza; Hib, H. influenzae b; Men C, meningococcal C conjugate vaccine; MMR, mumps, measles and rubella; T, tetanus; wP, wholecell pertussis. Note: 3 doses of Meningococcal C conjugate vaccine should be given to anyone age < 25 not already immunised with the conjugate vaccine or immunised with the polysaccharide vaccine over 3 years ago.

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATIONS 21

(d) (e) (f) (g)

oral typhoid; oral polio; rubella; yellow fever.

They should not normally be given:

than normal, and so inactivated vaccine may be more appropriate.

DIPHTHERIA IMMUNIZATION Recommended for:

(a) To pregnant women (this is a theoretical risk only, and may be outweighed by the risk of infection). (b) To the immunocompromised. This category includes the following: -Patients who have received high dose steroids in the last 3 months. This means children who have received 2mg/kg per day for at least 1 week or 1 mg/kg per day for at least 1 month, or adults who have received 40 mg prednisolone per day for at least 1 week. Lower doses may suppress immunity, especially if combined with cytotoxic drugs. Discuss each case with the specialist concerned. -Patients with malignancy of the reticuloendothelial system. -Patients who are immunosuppressed due to radiotherapy or chemotherapy in the last 6 months, or are in some other way immunodeficient. (c) Within 3 weeks of each other. If they must be given within 3 weeks, they should be given on the same day. This is, however, only based on the observation that smallpox immunization may be less effective if given within 3 weeks of another live vaccine. If it is unavoidable, immunizations should be given within three weeks of each other rather than omitted. (d) As MMR with immunoglobulin. Normal human immunoglobulin (HNIG) should be given at least 3 weeks before MMR or at least 3 months after. Contrary to previous advice this does not apply to other live vaccines, except possible the primary course of oral polio vaccine.1

HIV-POSITIVE PATIENTS Patients carrying HIV may receive live vaccines, except BCG, oral typhoid and yellow fever. They may, however, excrete live polio vaccine for longer

(a) Infants, preschool children and school leavers (see Table 2.1). (b) Travellers to areas where diphtheria is endemic or epidemic. (c) Health-care workers exposed to diphtheria. (d) Contacts of a diphtheria case. Previously immunized contacts need a booster dose. Nonimmune contacts need immunization and penicillin or erythromycin. Note: In patients likely to be exposed to the risk of diphtheria over a length of time, check antibody levels at least 3 months after their most recent immunization and give 10-yearly boosters of low-dose vaccine.

Side-effects Minor local reactions are common. Minor general reactions occur.

Contraindications (a) Acute illness. (b) Severe reaction to a previous dose. If the previous dose was in the form of triple vaccine, assume that the reaction was to the pertussis component and give the next dose as diphtheria and tetanus only.

Practicalities • Use low dose vaccine in adults and children over 10 years old. Give as a booster if previously immunized. If not, give as three vaccinations 1 month apart. Full strength vaccine in those over 10 years old is more likely to lead to severe reactions. • Antitoxin is only indicated for suspected cases of diphtheria. The frequency of hypersensitivity

22

INFECTIOUS DISEASES

reactions makes it too dangerous for prophylaxis. It is available from the Communicable Disease Surveillance Centre, tel. 020 8200 6868 (or, in Northern Ireland, the Public Health Laboratory, tel. 028 9032 9241).

HAEMOPHILUS INFLUENZAE TYPE b (Hlb) Indications (a) As per national schedule (at 2, 3 and 4 months), see Table 2.1. (b) Children aged 13-48 months who missed their primary immunization. They need only receive a single dose. Thereafter, it is not needed because the risk of infection is low, except for patients at high risk. (c) Household contacts of invasive Hib disease if aged under 4 years and not already immunized. Household contacts (except immunised children under the age of 4) should also receive rifampicin 20mg/kg/day for 4 days. (d) Non-household contacts aged under 4 at a creche, nursery or playgroup should be offered the vaccine if not already immunised. Older nonhousehold contacts may be offered rifampicin if two or more cases occur within 120 days. (e) A patient with invasive Hib disease, regardless of age, should be immunized. (f) High-risk patients: children and adults who have lost their spleens, with sickle-cell disease, or receiving treatment for malignant disease.

Side-effects Mild local reaction is common.

Contraindications (a) Acute illness. (b) Severe local or general reaction to a previous dose. If the previous general reaction followed the administration of Hib together with diphtheria, tetanus and pertussis (DTP), omit the pertussis component in future immunizations.

Chemoprophylaxis Give rifampicin to: (a) All household contacts of invasive Hib disease, except those under 4 years old who are already immunized. (b) All non-immunized room contacts, e.g. teachers and school children where two or more cases have occurred within 120 days. Note: Rifampicin is contraindicated in children under 3 months of age, pregnant or breast-feeding women and people with severely impaired hepatic function. Dose: 20mg/kg per day (maximum 600 mg daily) once daily for 4 days.

HEPATITIS A - ACTIVE IMMUNIZATION Indications Immunization is recommended for: (a) Laboratory staff working with the virus. (b) Haemophiliacs treated with Factor VIII or IX, or who have liver disease, or who have contracted hepatitis B or C. (c) Travellers to areas of poor sanitation, especially if travelling to areas of high or medium risk repeatedly or for more than 3 months. (d) Individuals who are at risk because of their sexual behaviour. Immunization should be considered for: (a) Patients with chronic liver disease. (b) Close contacts of those with hepatitis A. Take advice from the Consultant in Communicable Disease Control or, in Scotland, from the Consultant in Public Health Medicine (CPHM). (c) Outbreaks in schools and closed communities. Take advice as in (b). (d) Workers at risk, e.g. sewage workers and those working in institutions for those with severe learning disabilities.

Side-effects Mild transient local and general reactions.

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATION

Contraindications (a) Severe febrile illness. (b) Pregnancy, unless the risk of infection overrides the probably negligible risk to the fetus.

Practicalities Give one dose of vaccine intramuscularly into the deltoid. This gives immunity for at least a year. A second dose 6 to 12 months later gives immunity for up to 10 years.

HEPATITIS A - PASSIVE IMMUNIZATION

Indications Normal human immunoglobulin (HNIG) may be given to patients in any of the categories for whom active immunization is recommended. The protection it provides is inferior to that of active immunization and it is only indicated if immediate protection is required. The benefit lasts for about 4 months.

Contraindications If possible, live viruses should be given at least 3 weeks before or at least 3 months after. This does not apply to yellow fever vaccine.

HEPATITIS B: RECOMMENDATIONS FOR ACTIVE IMMUNIZATION (a) Occupational exposure - All health personnel in contact with blood products or with possibly HBsAg-positive patients. Most at risk are those working in mental institutions, renal units, liver units, and those working in high risk areas abroad. -Others at risk by virtue of occupation: prison staff and inmates, dental workers, morticians and embalmers, and prostitutes. The risk for police and ambulance crews is not increased, but individuals within those services may be considered to be at increased risk.

23

(b) Life style exposure -Those with multiple partners, especially male homosexuals (in 1990, 50% of male homosexuals attending a London STD clinic were positive); - iv drug users (in the UK, 50% are positive); -Sexual partners, themselves negative, of patients who are HBsAg positive; -Prisoners. (c) Babies born to mothers who are chronic carriers of hepatitis B virus or who acquire it during pregnancy. The UK National Screening Programme recommends that all pregnant women be offered antenatal screening for hepatitis B.2 If screening is omitted during pregnancy it should be offered at the time of delivery and a result obtained within 24 hours. (d) Travellers to areas of high prevalence likely to require medical or dental treatment there or to expose themselves to the risk of sexual transmission. (e) Immigrants. Members of families who have immigrated from high-risk areas. These are South and Central America, Africa and Asia, with South East Asia (especially Vietnam) having the highest incidence (50%). Families adopting a child from a high-risk area. (f) Patients at risk. Patients undergoing high-risk treatment, e.g. repeated transfusions, haemodialysis; patients entering institutions for the mentally handicapped (50% of Down syndrome inpatients, for instance, may be positive). Note: Those not yet exposed to the risk of hepatitis B should be immunized without prior screening for hepatitis B antibodies. As immunity takes 6 months to develop, immunization should be started well before exposure. Those with a substantial chance of already being positive should be screened before immunization to avoid an unnecessary course of injections.

Side-effects Reactions are uncommon and usually mild.

Contraindications Severe febrile illness.

24

INFECTIOUS DISEASES

Practicalities

Practicalities

* Give three injections at 0, 1 and 6 months. In cases of urgency, give the third injection at 2 months with a booster at 12 months. * Give 1 ml (0.5 ml in children) into the deltoid or anterolateral thigh (injection into the buttock is less effective). * Test for antibodies 2-4 months later; 10% will still be negative, mainly those over 40 years old.

Give hepatitis B immunoglobulin (HBIG) as soon as possible after exposure as well as active immunization. If a patient declines active immunization, give a second dose of immunoglobulin 4 weeks later. In someone previously immunized, give a booster dose of vaccine unless they are known to have antibody levels above lOOmiu/ml.

(a) Non-responders (<10 million International units (miu)/ml) need a repeat course of vaccine. (b) Poor responders (10 to lOOmiu/ml) need a single booster. (c) The 'Green Book' recommends a single booster 5 years later in those who remain at risk. (d) Those with levels of less than lOOmiu/ml should be warned that they would still need hepatitis B immunoglobulin if exposed to risk.

HEPATITIS B: RECOMMENDATIONS FOR PASSIVE IMMUNIZATION

* Newborn babies (see (b) above). Give 200 iu of hepatitis B immunoglobulin within 1 hour of birth, or at the latest within 48 hours. Start active immunization at the same time (in a contralateral site). * Sexual contacts. Test the partner for hepatitis B antibodies at the time of giving HBIG. If negative, give a rapid course of active immunization. * A person who is accidentally inoculated. Give 500 iu of hepatitis B immunoglobulin (HBIG) within 48 hours of the injury, although passive immunization up to 7 days later may still be worthwhile. Consider the need for prophylaxis against HIV (see p. 48).

Indications (a) Those who are accidentally inoculated or who contaminate eye, mouth or fresh wounds with infected blood (the risk of hepatitis B from an inoculation of infected blood is 6%). All these patients will require active immunization as well. (b) Babies born to mothers who are HBeAg positive, or HBsAg positive without e markers (or where e marker status is unknown), or who had acute hepatitis B during pregnancy (as well as active immunization, see above). (c) Sexual contacts of patients with acute hepatitis B and of carriers who are highly infectious (i.e. HBsAg and HBeAg positive but anti-HBe negative).

Contraindications Immunization with live virus should be avoided in the 3 weeks before and the 3 months after administration of immunoglobulin. This does not apply to yellow fever, and possibly not to a booster of oral polio vaccine.

CHRONIC HEPATITIS Guidelines: Gow PJ, Mutimer D. Treatment of chronic hepatitis. BMJ 2001; 323:1164-7. National guideline on the management of the viral hepatitides A, B and C. Clinical Effectiveness Group of the Association for Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases 2001. Online. Available: www.mssvd.org.uk/CEG/ceguidelines.htm

• Hepatitis B. In the UK up to 4% have serological evidence of exposure to hepatitis B and 10% of these (approximately 240,000) have chronic infection. • Hepatitis C. The estimated prevalence in the UK is between 0.3 and 0.6% of the population (approximately 200,000-^00,000). About 85% of those infected develop chronic infection and of these 30-50% go on to develop cirrhosis over 20-30 years. Up to 50% of intravenous drug users are infected.

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATION

* Drug treatment can significantly alter the progression of both types of chronic hepatitis: (a) In hepatitis B conversion from a high level of virus production to a low level can be achieved with interferon or lamivudine with an improvement in liver function tests and liver histology. (b) In hepatitis C interferon and ribavarin combined therapy permanently eradicates the virus in at least 40% of patients and may prevent progression or even reverse hepatic fibrosis in those who are not cured. * Consider screening the following patients: (a) those with a past history of hepatitis; (b) gay men; (c) sex workers (either sex); (d) intravenous drug users; (e) those who are HIV positive; (f) needle-stick victims; (g) sexual partners or babies of positive or high risk patients; (h) patients transfused in the UK before 1991. * Refer all patients with evidence of chronic infection to a specialist in the disease, for consideration of active treatment. Request for the patient to be seen soon if there is measurable viral DNA (hepatitis B) or raised alanine transferase (ALT) and detectable hepatitis C RNA. * Monitor liver function in all cases and advise against alcohol consumption.

25

hepatitis B. Consider vaccination (see p. 23). A positive test indicates natural immunity, (b) Positive for HBsAg: indicates that the patient has either acute hepatitis or is a chronic hepatitis B carrier. Check IgM, anti-HBc, HBeAg, HBeAb, and possibly Hepatitis B DNA.

Screening for hepatitis C Check serum hepatitis C antibody and if positive check hepatitis C RNA. Information for patients and professionals: National Hepatitis C Resource Unit, PO Box 31844, London SE11 4DT, tel. 020 7735 7705; http://www.hep-ccentre.com/Contact.htm Hepatitis C Your questions answered www.doh.gov.uk/hepatitisc

INFLUENZA • UK studies show that immunization can prevent, or reduce the severity of, influenza with a reduction of hospital admissions by 60% and of mortality by 40% compared to controls.3 • The UK target for 2001 /2 is that at least 65% of people age 65 and over should be immunized.

Indications4

Using serum HBsAg

(a) Those with: - chronic respiratory disease, including asthma requiring continuous or repeated use of inhaled or systemic steroids or with previous exacerbations requiring hospital admission; - chronic heart disease; - chronic renal disease; -diabetes requiring insulin or oral hypoglycaemic drugs; - immunosuppression whether due to disease or to treatment, including systemic steroids equivalent to 20 mg prednisolone daily for more than 2 weeks. (b) all aged 65 years and over. (c) Those living in long-stay residential and nursing homes and other long-stay facilities.

(a) Negative for HBsAg: check anti-HBc. Negative anti-HBc indicates no previous exposure to

Those outside these groups may request immunization, and GPs may give it but the Chief

Screening for hepatitis B Using serum anti-HBc (a) Negative for anti-HBc: indicates no previous exposure to hepatitis B. Consider vaccination (see p. 23). (b) Positive for anti-HBc: test for HBsAg. If this is negative this indicates natural immunity. If positive, it indicates that the patient has either acute hepatitis or is a chronic hepatitis B carrier. Check IgM, anti-HBc, HBeAg, HBeAb, and possibly hepatitis B DNA.

26

INFECTIOUS DISEASES

Medical Officer (CMO) warns that excessive immunization of people outside the target groups could lead to vaccine shortages.

Side-effects Reactions other than mild local inflammation are rare.

Contraindications (a) Previous anaphylactic reaction to egg. (b) Pregnancy, unless strongly indicated.

Practicalities Give one injection yearly to adults. Children (under 13 years old) need two injections 4-6 weeks apart as a primary course, followed by yearly boosters.

Prophylaxis and treatment * Amantadine. Consider prophylaxis with amantadine lOOmg daily for non-immunized at-risk patients during an outbreak, either during the 2 weeks that immunization takes to be effective, or throughout the outbreak, if immunization is contraindicated. It only protects against influenza A. It reduces the number of clinical cases by 23% (95% CI11 to 34%).5 * Zanamivir. Consider zanamivir by inhalation for the treatment of influenza A or B within 48 hours of the onset of symptoms and when influenza is circulating in the community. The National Institute for Clinical Excellence recommends that its use be restricted to high risk individuals, namely those age 65 or over, or those with chronic respiratory or cardiac disease or with diabetes or immunosuppression. It appears to reduce the duration of symptoms by 1 day.6

MEASLES, MUMPS AND RUBELLA (MMR) Indications (a) Vaccinate all children between 12 and 15 months of age, and with the preschool booster.

(b) Offer it to students entering college who have not previously been given MMR.

Side-effects (a) Malaise, fever and a rash 1 week after the injection. Febrile convulsions occur at this stage in 1:1000 immunizations. Parents of a child with an increased tendency to convulsions should be warned of this and of the need to reduce the child's temperature. They can be reassured that in the UK the child is ten times more likely to convulse after catching measles than after irnrnunization. (b) Parotid swelling occurs in 1%, usually in the third week. (c) Arthralgia or arthritis occurs rarely 2-3 weeks after immunization. (d) Meningoencephalitis due to the mumps component occurred in 1:400,000 cases until 1992, when the mumps virus strain was changed. No cases have been reported since. (e) Thrombocytopenia, usually transient, occurs in 1:22,300 after the first dose but less frequently after the second. The rate of thrombocytopenia after natural infection is higher, being 1:3000 for rubella and 1:6000 for measles. Thrombocytopenia after natural infection tends to be more severe (see the DoH paper on Measles Mumps and Rubella (MMR), 8 October 2001. Online. Available: www.doh.gov.uk/mmr.htm). (f) Autism. The evidence available is overwhelmingly against a connection between MMR and autism.7 The CMO warns that children should not be given separate doses of measles, mumps and rubella vaccines as there is no evidence of benefit and a clear risk of harm.

Contraindications (a) As for all live vaccines (see p. 20). If given to a woman, pregnancy should be avoided for 1 month. (b) Anaphylactic reaction to egg is not now a contraindication because anaphylaxis after the vaccine is rare even in children who are hypersensitive to eggs (it occurs in 0.16% of those who are hypersensitive to egg). If there is concern, the vaccine can be given in hospital.

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATION

(c) Systemic allergy to gelatin, neomycin or kanamycin. Contact dermatitis is not a contraindication.

MEASLES Measles vaccine is not available in the UK except as MMR.

Indications Certain contacts of measles who have not been immunized: (a) Normal children who have not been immunized can be protected if given MMR within 72 hours. (b) Immunocompromised children and adults who are not immune to measles should be given normal human immunoglobulin, as should children debilitated by severe illness.

MUMPS Indications There is no indication for mumps vaccination as a separate immunization. Active and passive immunization are not effective in protecting contacts.

RUBELLA Indications (a) Seronegative women of child-bearing age who are not pregnant. All women should be screened before pregnancy and offered screening during the first pregnancy, whether immunized or not. Once two positive results have been obtained, further testing is unnecessary. If the woman is pregnant, immunization should be postponed to the postpartum period. (b) Seronegative staff (male and female) working in antenatal clinics who might, if infected, pass rubella on to women in the first trimester.

Side-effects Sore throat, lymphadenopathy and arthralgia may occur between 1 and 3 weeks after vaccination.

27

Contraindications (a) As for all live vaccines (see p. 20). (b) Anaphylaxis to neomycin or polymyxin.

Details • Women immunized with rubella should avoid pregnancy for 1 month. There is no evidence that the vaccine can damage the fetus and pregnancy occurring within 1 month of immunization is not grounds for termination. Surveillance in the UK, the USA and Germany has failed to detect a single case of congenital rubella due to the vaccine. • Screening. All women should be screened for rubella antibodies at least 3 months before they want to embark on their first pregnancy (2% are likely to be susceptible). Up to 5% of women fail to develop antibodies following immunization. Once acquired, immunity does not seem to decline. • Passive immunization. Active and passive immunization are ineffective in protecting contacts. However, passive immunization with normal immunoglobulin is available in the first trimester of pregnancy for women with proven rubella who do not wish to have the pregnancy terminated. Its value is, however, doubtful. Consult the local Public Health Laboratory in England and Wales; the Community Medicine Specialist (Communicable Diseases) in Scotland; the Public Health Laboratory, Belfast City Hospital, in Northern Ireland.

MENINGOCOCCAL INFECTION Indications (a) Children in the first year of life (see Table 2.1). (b) Children over the age of one, or young adults aged <25, if not previously immunized (see Table 2.1). (c) Close contacts of a patient with meningococcal infection (A or C) if not already immunized. They should also receive antibiotic prophylaxis (see below). (d) To control outbreaks, especially of group C organisms, in institutions such as schools.

28

INFECTIOUS DISEASES

(e) Travellers going for at least 1 month to the meningitis belt of Africa during the dry season, North India, Nepal, Bhutan, Pakistan and the Arabian peninsula (especially Mecca). Use meningococcal A and C vaccine or, for Mecca, the ACWY vaccine. (f) Laboratory staff working with N. meningitidis. (g) Patients with an absent or dysfunctional spleen?1 Note: The meningococcal C conjugate vaccine should be used except in travellers or contacts of non-C meningococcus.

Side-effects Only mild local reactions are common.

Contraindications (a) Pregnancy, unless strongly advised. (b) Febrile illness. (c) Previous reaction to the vaccine.

Prophylaxis Give close contacts rifampicin or ciprofloxacin (according to local policy). Rifampicin is given for 2 days at: adults 600 mg b.d.; children 1 to 12 years 10 mg per kg b.d.; 3 months to 1 year 5mg per kg b.d. Ciprofloxacin is given to adults only as a single dose of 500 mg.

Side-effects Minor general and local reactions are very common. The fear of neurological damage may be unfounded, see below. Reactions to wholecell pertussis vaccine are commoner in older children, hence the use of acellular vaccine for this age group.10 It is not licensed for use in children over the age of 6.

Contraindications (a) Acute illness. (b) Progressive neurological disease. (c) Severe reaction to a previous dose, in which case acellular pertussis vaccine should be used. A severe local reaction is one where indurated redness and swelling involves most of the anterolateral surface of the thigh or a major part of the circumference of the upper arm. A severe general reaction is one where there is fever of 39.5°C or higher within 48 hours; or any of the following within 72 hours: prolonged inconsolable screaming, anaphylaxis, bronchospasm, laryngeal oedema, collapse, prolonged unresponsiveness or convulsions.

Practicalities Use acellular pertussis vaccine (APV) in children with a severe local reaction to a previous dose. Use monovalent APV when a child is given pertussis immunization alone.

Immediate treatment of a suspected case * Give parenteral benzyl penicillin: 300 mg (children under 1); 600 mg (children 1-9 years) or 1.2 g (adults). This reduces mortality without making subsequent diagnosis more difficult.9

PERTUSSIS (WHOOPING COUGH) Indications All children at age 2, 3 and 4 months, and a preschool booster in the form of acellular pertussis vaccine.

Comments The interim report of the National Childhood Encephalopathy study in 1981 appeared to show a risk of neurological damage of 1 in 310,000 vaccinations. However, the High Court judgement of 1988 found no evidence of neurological damage from a review of the final results. The 'Green Book' cites the evidence that the vaccine is either not related to acute neurological illness or long-term brain damage or, if it is related, the incidence is too low to show up in the studies carried out.

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATION 29

Children with a personal or family history of febrile convulsions are more likely to develop febrile convulsions after receiving the vaccine. The British Paediatric Association's advice is that the benefit for these children outweighs the risk, and that they should receive pertussis vaccine. In the end, it is for the parents to make the decision. The usual warnings about how to avoid febrile convulsions should be stressed. Doctors are urged to take consultant advice before deciding not to vaccinate. Children with a personal or family history of epilepsy appear no more likely to convulse after pertussis vaccine than other children.

Treatment and prophylaxis of pertussis Give erythromycin for 7 days.11 This: (a) renders a patient non-infectious; (b) should be given to non-immunized children in recent contact with whooping cough so that they might avoid illness (treatment should always be given to a neonate returning to a home where a sibling suffers from pertussis).

PNEUMOCOCCAL INFECTION Indications Patients aged 2 months and above with: (a) asplenia or severe splenic dysfunction; (b) sickle-cell disease; (c) coeliac disease; (d) nephrotic syndrome or chronic renal disease; (e) HIV infection or other cause of immunodeficiency; (f) diabetes mellitus; (g) chronic heart, lung or liver disease. Note: Use the polysaccharide vaccine for patients over 2 years old and the conjugate vaccine for children under 2. Patients with hypo- or asplenism should also receive immunization against H. influenzae b, influenza and meningococcal C infection. They also need penicillin (phenoxymethylpenicillin

250 mg b.d.) until at least age 16, and should take special care to avoid malaria and tick bites.12

Side-effects Local and general reactions to a first immunization are almost always mild.

Contraindications (a) Acute infection. (b) Pregnancy. (c) Breast feeding.

Practicalities Give immunization at least 2 (and preferably 4 to 6) weeks before splenectomy. Revaccination after 5 to 10 years should only be considered in those at greatest risk, e.g. asplenia or nephrotic syndrome. It is associated with severe reactions and is not otherwise recommended for adults. Infants should receive three doses at 2, 3 and 4 months. Children age 5 months to 2 years should receive two doses at least 1 month apart.8

POLIO Indications (a) As per national schedule (at 2, 3 and 4 months, 3-5 years and 15-19 years; see Table 2.1). (b) Non-immune parents of children receiving their first polio vaccination should also receive a basic course (parents born before 1958 are unlikely to have been immunized). (c) Anyone who has never been immunized needs the basic course. (d) Health-care workers at high risk need a single booster dose every 10 years. (e) Travellers to areas outside Europe, North America and Australasia need a single booster every 10 years.

Side-effects Poliomyelitis due to the vaccine occurs once per 2,000,000 doses, and in the same number of

30

INFECTIOUS DISEASES

contacts of vaccinees. Those who will be changing the nappies of a recently vaccinated baby should be warned to wash their hands for 6 weeks after each dose.

(d) If the whole course of five injections is interrupted, continue from where the course was left off to make a total of five doses. Immunity is likely to be lifelong, so further routine boosters (after the basic five) are unnecessary.

Contraindications (a) As for all live vaccines (see p. 20). (b) Febrile illness, vomiting or diarrhoea. (c) Immunocompromised patients should not receive live vaccine, nor should their close family. Use inactivated vaccine instead. (d) Hypersensitivity. Avoid in extreme cases of hypersensitivity to penicillin, streptomycin, polymyxin B and neomycin.

Practicalities (a) The basic course is three doses at 4 week intervals. (b) Parents should be warned to treat the baby's stools as infectious for 6 weeks to any in the family who have not been immunized. (c) There is no need to stop swimming after immunization nor to advise parents against letting their children swim before immunization.

TETANUS Everybody should be immunized against tetanus. One practice in Yorkshire found that in 1988 three-quarters of their patients were inadequately covered.13 In the UK, 75% of cases occur in patients over the age of 45, and mainly in women.

Indications (a) Vaccination should be by means of a primary course of three injections over 3 months followed by two boosters as per schedule. (b) Children who have their primary course after the age of 1 should wait 3 years before having their first routine booster. (c) People having their primary course after the age of 10 should have boosters 10 and 20 years later.

Side-effects Mild local reactions are common. A nodule may remain if the injection is given superficially. General reactions are uncommon, and serious ones are rare.

Contraindications (a) Acute febrile illness. (b) Anaphylactic reaction to a previous dose. Other general reactions do not seem to carry a risk of being repeated with subsequent doses.

TETANUS-PRONE INJURY This is defined as one that: (a) was sustained more than 6 hours before surgical treatment; or (b) is showing one or more of the following: - a significant degree of devitalized tissue; - a puncture-type wound; - direct contact with soil or material likely to harbour tetanus spores; -clinical evidence of sepsis. * Non-immunized patients and any whose last booster was more than 10 years ago. Give: (a) a tetanus toxoid booster or primary course as appropriate; and (b) tetanus immunoglobulin. This is available on prescription as Tetabulin, or from the Regional Blood Transfusion Centre. Give one vial (250 iu) i.m. or two vials if more than 24 hours has elapsed, the wound is heavily contaminated or is a burn, or the patient weighs over 90 kg. * Patients of unknown immune status. If patients are uncertain whether a primary course has been given, assume that they will have received primary immunization only if they were born in the UK after 1960, or were in the Armed Forces in or after 1938.

SPECIFIC INFECTIOUS DISEASES AND IMMUNIZATION 31

* Fully immunized patients who have received a booster in the last 10 years: only give immunoglobulin if the risk is especially high, e.g. contaminated with manure.

TUBERCULOSIS Indications (a) All those at normal risk of contracting tuberculosis: -all children aged 10 to 14 years; - anyone else who requests it. (b) Those at higher risk: -contacts of patients with open respiratory tuberculosis; -workers in contact with potential cases, e.g. health workers and staff in prisons, old people's homes and hostels for the homeless or for refugees; -veterinary staff in contact with potential cases; -immigrants from areas of high prevalence. Infants born in this country to immigrants from areas of high prevalence should be vaccinated within a few days of birth; -travellers to high prevalence areas (Asia, Africa, Central and South America) who intend to stay for more than 1 month.

Side-effects Local ulceration or abscess formation, especially if the dose has been given subcutaneously instead of intradermaHy. Axillary adenitis is common, and usually subsides within weeks.

Contraindications (a) As for live vaccines (see p. 20). (b) Septic skin lesions. Patients with eczema may be vaccinated, but avoid eczematous patches.

* Once BCG has been given, do not use that arm for any other immunization in the next 3 months because of the risk of axillary lymphadenitis.

VARICELLA (CHICKENPOX) AND HERPES ZOSTER - PASSIVE IMMUNIZATION (VZIG) Indications (a) The immunosuppressed. This does not include HIV-positive patients unless they have HIV-related symptoms. It does include those on systemic corticosteroids, or those who have taken them in the last 3 months, at the equivalent of prednisolone 40 mg daily for at least a week (children 2 mg/kg/day for at least a week). (b) Debilitated patients. (c) Non-immune pregnant women, to protect them from severe illness. It will not protect the fetus from congenital varicella syndromes. (d) Neonates, if: -the mother develops chickenpox between 7 days before and 7 days after delivery; or -there has been contact with chickenpox or zoster and the mother is not known to be immune. Check maternal serology before giving immunoglobulin. (There is no risk to the baby if the mother develops zoster, since she clearly has had chickenpox in the past.) Contact is significant if the contact has: (a) chickenpox; or (b) disseminated zoster; or (c) exposed zoster; or (d) localized zoster that is not exposed, but where the patient with the zoster is immunosuppressed (and so may be shedding more virus).

Side-effects Anaphylaxis is very rare.

Practicalities * Refer to a Chest Clinic, where a tuberculin test will be performed before administering BCG except in infants less than 3 months old.

Practicalities * Arrange an urgent antibody screen on potential candidates while ordering VZIG from

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INFECTIOUS DISEASES

the local Public Health Laboratory. The immunoglobulin is in short supply and can be returned if antibodies are present. It must be given within 10 days of contact, and preferably within 3 days. * Ignore a previous history of chickenpox if the patient is likely to have lost previous immunity, as in bone marrow transplants. * Believe a history of a previous episode of chickenpox in pregnant women, but ignore it in the immunosuppressed, who may have been immune but lost it. Active immunization is available on a namedpatient basis from GlaxoSmithKline for the immunosuppressed. If an immunosuppressed patient develops chickenpox, refer urgently for iv acyclovir.

ADVICE FOR TRAVELLERS AND IMMUNIZATION FOR TRAVEL ABROAD Advice: DoH. Health Information for Overseas Travel, 2nd edition, 2001. London: The Stationary Office. Available from The Stationary Office, PO Box 29, St. Crispins, Duke St, Norwich NR3 1GN and authorized bookshops and from www.the-stationary-office.co.uk Information about recent disease outbreaks is available on www.doh.gov.uk/hat/emerg.htm Advice for doctors and travellers can be found on the website of the Foreign and Commonwealth Office, www.fco.gov.uk/travel

* Check that the potential traveller is fit. Most serious illness occurring abroad is due to preexisting cardiovascular or pulmonary disease, not to tropical diseases. Some elderly travellers do not realise the stress that travel imposes. * Check that the traveller has received all the routine immunizations recommended for the UK, including BCG and tetanus. Consider giving the following boosters if the last immunization was over 10 years ago and the planned travel poses more risk than is present in the UK: polio,

tetanus and diphtheria. The combined dip/tet vaccine may be used even if only diphtheria immunization is needed. * DoH leaflets. Recommend that the patient reads the DoH leaflet T6, Health Advice for Travellers available on www.doh.gov.uk/traveladvice/index. htm. Patients can obtain individual copies free of charge from a post office or by ordering on tel. 0800 555777. Doctors can order in bulk from the Department of Health, PO Box 777, London SE1 6XH, fax 01623 724524. It is not published sufficiently frequently for the immunization recommendations to be up to date, and updates are available on PRESTEL. * Prevention. Stress the importance of preventive measures against gastroenteritis, insect bites, sunburn, accidents and sexually transmitted diseases. Excellent advice is contained in the above leaflets. Travellers should know that accidents are the greatest cause of morbidity and mortality in travellers, and that more become HIV positive than contract typhoid or malaria. * Recommend the appropriate immunizations from an up-to-date source, e.g. Travax, the Scottish Centre for Infection and Environmental Health. The website for health professionals is www.travax.scot.nhs.uk but a fee is charged for those working outside Scotland. The public can access similar information free at www.fitfor travel.scot.nhs.uk. * Unusual itineraries. Advise patients with unusual itineraries or with other health problems to contact MASTA (Medical Advisory Service for Travellers Abroad), tel. 0906 822 4100. www.masta.org * Medical equipment. Advise patients travelling to remote areas to purchase a sterile medical equipment pack, also obtainable from MASTA. * Altitude sickness. Warn climbers and walkers who are planning to go above 2500 metres about the danger. Nine per cent suffer from altitude sickness in the high Alps,14 and over 50% can expect to get it on the trek to Everest base camp if they fly in.15 Prescribe acetazolamide 750 mg daily from 2 days before the ascent to 3 days after. Warn of the risk of paraesthesiae in the hands and feet in the first 2 days of treatment. It is not a reason to stop the drug.

ADVICE FOR TRAVELLERS AND IMMUNIZATION FOR TRAVEL ABROAD 33

TRAVELLERS' DIARRHOEA * Explain that: (a) oral rehydration is the most important aspect of treatment. If commercial sachets of replacement sugar and salt are not available, travellers can add one teaspoonful of sugar and a pinch (but no more) of salt to a large glass (250 ml) of boiled or bottled water. (b) the patient should continue to eat if food can be tolerated. It can shorten the duration and amount of diarrhoea. (c) loperamide, bought over the counter, can reduce abdominal cramps and diarrhoea but it should not be used if there is fever nor in young children. (d) Medical help should be sought if there is significant fever, confusion, blood in the stools or if the diarrhoea does not settle in 72 hours (adults) or 24 hours (small children and the elderly). * Offer travellers to areas of poor hygiene: (a) ciprofloxacin or trimethoprim for 2 days to be taken if diarrhoea occurs; and (b) tinidazole or metronidazole to those going to areas where giardiasis is likely. * Prophylaxis. Offer prophylaxis only to those travellers for whom gastroenteritis would have serious implications, e.g. politicians, Olympic athletes or those already ill with another condition. This is because of the problems of antibiotic resistance if this were widely used. Trimethoprim 200 mg b.d. for up to 2 weeks can prevent up to 95% of travellers' diarrhoea.16

Note: Advice on immunizations which are routinely recommended for those not travelling (e.g. hepatitis A and B, BCG) are included in the previous section.

A SUGGESTED SCHEDULE OF IMMUNIZATIONS Table 2.2 travellers

A suggested schedule of immunization for

Week

Normal schedule

Rapid schedule

0

Typhoid, tetanus, polio, (diphtheria, rabies, Japanese encephalitis)

1

(Rabies, Japanese encephalitis)

Typhoid, tetanus, polio, hepatitis A, (diphtheria, yellow fever, rabies, Japanese encephalitis) (Rabies, Japanese encephalitis) Tetanus, (diphtheria), polio

2 3 4

(Yellow fever) (Rabies, Japanese encephalitis)

6

Typhoid, tetanus, (diphtheria), polio, hepatitis A Tetanus, polio, (diphtheria)

10

Polio, tetanus, typhoid, (diphtheria, rabies, Japanese encephalitis)

Italic type indicates that immunization is unnecessary if a primary course has been given previously. Entries in brackets indicates those immunizations that are needed only in special situations. Meningococcal vaccine can be given at any stage. Diphtheria can be added to a tetanus immunization as the combined dose. For hepatitis A and B, polio, diphtheria, meningococcal infection and BCG, see the previous section.

Note: Drugs prescribed in the UK for use abroad should be on a private prescription.

CHOLERA ADVICE FOR DOCTORS ON IMMUNIZATIONS England: Communicable Disease Surveillance Centre, Travel Medicine Unit, 61 Colindale Avenue, London NW9 5EQ, tel. 020 8200 6868 (weekdays 10 a.m. to 12 noon). Scotland: The Scottish Centre for Infection and Environmental Health, Travel Medicine Division, Clifton House, Clifton Place, Glasgow, G3 7LN, tel. 0141 300 1130 (weekdays 2 to 4 p.m.).

• The vaccine is of limited usefulness (about 50% effective), and does not decrease asymptomatic carriage. • The WHO no longer recommends cholera immunization for travellers. • Cholera vaccination is not an official requirement for entry into any country. Reports continue of officials requiring it at remote borders. If a traveller suspects that this may happen, issue a signed statement on headed notepaper that

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cholera vaccination is not needed. Appendix 1 of Health Information for Overseas Travel has a sample document.

JAPANESE ENCEPHALITIS Recommended for those travelling during the monsoon season (May to October) to rural areas of the Indian subcontinent, Southeast Asia and the Far East for 4 weeks or more. The risk is all year in southern regions of the Far East (Malaysia, the Phillipines and Indonesia). Normal tourist and business trips pose little hazard. * Warn travellers to take precautions against mosquito bites (see under malaria). * Warn the patient that use of the vaccine is unlicensed and that allergic reactions occur, both immediate and delayed. The patient should remain under observation for 30 minutes after each dose and should complete the course at least 10 days before travelling. * The injection schedule varies according to the product used and the duration of protection required. The minimum is two doses, 1 week apart, with a booster at 3 months for those requiring continued protection. * Obtain vaccine from Aventis Pasteur or from MASTA, on a named patient basis.

MALARIA Guideline: Bradley DJ, Bannister B. Guidelines for malaria prevention in travellers from the United Kingdom for 2001. Commun Dis Pub Health 2001; 4: 84-101. Online. Available: www.phls.co.uk

* Assess the degree of risk for a traveller by asking about the destination, duration, likely activities and style of travel. * Explain to patients that they cannot rely on prophylactic drugs as their sole protection against malaria. In high-risk parts of Africa >3% of travellers taking chloroquine develop malaria each month.17 Recommend that patients: (a) cover arms and legs from dusk till dawn when out of doors; (b) use an effective mosquito repellent on exposed skin and under thin clothes, e.g. DEET

(diethyltoluamide) and re-apply it every 2 hours; (c) sleep with closed windows, having sprayed the room with a 'knock-down' spray. If this is not possible, sleep under permethrin-impregnated mosquito netting; the permethrin should be renewed every 4 months. If that is not available, burn a mosquito coil overnight or vaporize a synthetic pyrethroid on an electric mat. Do not use electronic buzzers, which do not work.18 * High-risk patients. Warn certain patients that they are especially at risk: those without a functioning spleen and pregnant women in whom the disease is usually more severe and in whom it may lead to abortion or stillbirth.

Drug prophylaxis * Explain that patients must start all tablets 1 week before departure (except mefloquine, where a start 2 to 3 weeks before departure is recommended so that early adverse effects will occur at home). They should be continued for 4 weeks after leaving the area, except in the case of proguanil plus atovacquone, where 7 days is enough. * Warn the patient that fever occurring while taking the tablets, or for up to 1 year after stopping the tablets, may still be due to malaria. * Follow the current recommendations of the Malaria Reference Laboratory and Ross Institute, available on www.malaria-reference.co.uk/ • The recommendation for areas without drug resistance or where resistance is low is likely to be chloroquine or chloroquine plus proguanil. • For travellers to highly chloroquine-resistant areas, the choice is between (a) mefloquine; (b) Malarone (proguanil plus atovaquone) for up to 28 days; (c) Doxycycline for up to 6 months; (d) Maloprim (dapsone plus pyrimethamine). • Rural areas are more dangerous than cities and the risk increases with the length of stay. • Mefloquine is the usual choice. Adverse neuropsychiatric reactions are rare (1 in 10,000 users) but enough to contraindicate its use in those with epilepsy or a history of psychiatric illness. Doxycycline may cause a photosensitive dermatitis

ADVICE FOR TRAVELLERS AND IMMUNIZATION FOR TRAVEL ABROAD 35

and so is less suitable for sunbathers, for whom Malarone may be more appropriate. Telephone advice. Patients may telephone 09065 508908 for a 24-hour recorded message. They may, however, not remember the advice correctly.19 The British National Formulary (www.BNF.org) has information that is no more than 6 months old. Health professionals may telephone their nearest Department of Tropical Medicine for advice in complicated cases or the PHLS Malaria Reference Laboratory on 020 7636 3924 (weekdays 9 a.m. to 4.30p.m.).

Immigrants and their children Immunity to malaria is lost after 2 years. If immigrants from a malarious area are returning to that area on holiday, they should use prophylactic drugs. If they are returning long-term, they should not take prophylaxis but should be aware that they are at risk from malaria. Children of such immigrants born in the UK should be protected for the first 12 months in a malarious area with proguanil.

Standby drugs for travellers to remote areas Travellers who will be more than 8 hours away from immediate medical care for more than 1 week, especially in sub-Saharan Africa, may be given antimalarials as standby treatment to take at the onset of fever. Current recommendations are: (a) quinine (600 mg t.d.s. for 3 days) followed by three tablets of Fansidar as a single dose. The patient should be warned that the quinine is likely to cause tinnitus; or (b) quinine (600 mg t.d.s. for 3 days) and doxycycline (100 mg b.d. for 7 days); or (c) malarone (four tablets daily for 3 days). Anyone using standby treatment should still contact a health professional as soon as possible thereafter.

Treating malaria * Admit any patient who is sufficiently ill; otherwise telephone one of the above numbers for advice.

* Notify malaria to the local authority. The PHLS Malaria Reference Laboratory (MRL) requests that doctors also notify the MRL on a blue form available from them. This requests more information than the statutory notification.

MENINGOCOCCAL IMMUNIZATION * Travellers entering Saudi Arabia for the pilgrimage to Mecca must provide proof of vaccination with meningococcal ACWY vaccine. The UK Department of Health recommends use of the quadrivalent ACWY meningococcal polysaccharide vaccine. Even those with a current certificate of vaccination against meningococcal A and C need to be vaccinated against the W135 strain if going on the pilgrimage.10

RABIES Eleven cases have occurred in the UK since 1976, all acquired abroad.

Pre-exposure * Immunize anyone at risk from rabies by nature of their occupation in the UK or abroad. * Immunize travellers to high-risk areas where vaccine is unlikely to be available following a bite, scratch or lick from an infected animal.

Immunization schedule Two doses of rabies vaccine 1 month apart; or three doses on days 0, 7, and 28 for those at high risk. Boosters after 6 to 12 months and then every 2 to 3 years are necessary for those at continuing risk, especially if remote from medical care.

Post-exposure * If a patient is bitten, licked or scratched by a potentially rabid animal, the wound should be thoroughly scrubbed with soap and water for 5 minutes. * Ascertain the risk of rabies in the country concerned from the booklet Health Information for Overseas Travel; or telephone the Communicable

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Disease Surveillance Centre 020 8200 6868 (weekdays 10 to 12 noon); or 020 8200 4400; or The Scottish Centre for Infection and Environmental Health, 0141 300 1130 (weekdays 2 to 4 p.m.) or 0141 946 7120. * If recommended, give the first dose of vaccine 1 ml deep sc or im. Subsequent doses are on days 3, 7,14 and 30. Stop if the animal is under observation and still well after 10 days. Fully immunised patients need only receive the boosters at day 0 and 3-7. * If the risk is high, give human rabies immunoglobulin 20iu/kg, infiltrating half around the wound and giving the other half intramuscularly. TICK-BORNE ENCEPHALITIS Patients at risk are those walking or camping in certain forested areas of Europe from April to October. Countries most affected are Austria, Croatia, Czech Republic, Germany, Hungary, Poland, Slovakia, Slovenia, Switzerland and the western parts of the former USSR. * Advise walkers to tuck long trousers into socks and spray clothes with insect repellent, e.g. DEET or permethrin. * Instruct those at risk to inspect the skin for ticks. If found, they should be pulled off by applying steady traction. Only 1 to 2% are likely to be infected and 90% of patients who contract the virus remain asymptomatic. If a tick is found, specific immunoglobulin may be available locally but the risk from the immunoglobulin may outweigh that from the disease. It is unlicensed in the UK. * Offer vaccine to those planning to walk in the infected areas. Various regimens exist.

TYPHOID Indications (a) All travellers outside Europe, North America and Australasia, and travellers to some Eastern European countries if eating in local restaurants. (b) Laboratory workers handling specimens from suspected cases.

A single injection of Typhim Vi or Typherix gives 70% protection for 3 years. Thereafter, a 3-yearly booster is necessary. If the oral vaccine is used, three doses are needed, repeated annually. It should not be given to children under 6 years old.

Contraindications (a) Children below the age of 1 year. (b) Patients suffering from a febrile illness. (c) The presence of an outbreak of typhoid fever (as this may increase the susceptibility to infection). (d) Pregnancy, unless a strong indication exists. (e) Severe reaction to a previous dose. (f) Oral typhoid carries the contraindications common to all live vaccines. In addition, allow at least 12 hours between the administration of oral typhoid and mefloquine. Ideally, complete the course of oral typhoid at least 3 days before starting mefloquine. Practicalities

Whole cell vaccine (Typhoid/vac) is still indicated in those who have previously been immunized with it without a significant reaction, and in children aged 12-18 months. An intradermal injection of 0.1 ml may be used for these doses, and repeated 3-yearly.

YELLOW FEVER Indications (a) Laboratory workers at risk. (b) Travellers to endemic areas of Africa and South America. A single injection gives excellent immunity for at least 10 years.

Side-effects Mild local and systemic reactions occur in up to 10%, 5 to 10 days after the injection.

Contraindications (a) As for all live vaccines (see p. 20). (b) Children under 9 months old.

SYMPTOMS IN TRAVELLERS RETURNING FROM ABROAD

37

(c) Pregnancy; however, if a pregnant woman is travelling to a high-risk area, the safer course would be to have the vaccination. (d) Systemic hypersensitivity to neomycin or polymyxin or an anaphylactic reaction to egg.

• Consider a cause unrelated to travel, e.g. respiratory or urinary infection. • Consider the following diseases.

Practicalities

• There are about 2000 cases of malaria imported into the UK each year, and failure to diagnose is regularly referred to in the Medical Defence Associations' reports. Falciparum accounts for over half of UK cases, and can kill within 24 hours. Conversely, the first presentation of falciparum may be up to 12 months after exposure. Vivax may cause illness as late as 18 months after return. • Fever in a traveller from tropical Africa should be assumed to be due to malaria until proven otherwise. Malaria can be contracted by patients in an aircraft on the runway in an endemic area. Malaria does exist even in countries for which prophylaxis is not recommended, e.g. the North African coast. • If the first blood film is negative, the patient may still have malaria. Continue to send blood films until the fever abates or until another diagnosis is reached.

Vaccination can only be given at yellow fever vaccination centres, where the certificate will be issued. A list of centres is available on http://tap.ccta.gov.uk/doh/yellcode.nsf/pages/ Home?open

SYMPTOMS IN TRAVELLERS RETURNING FROM ABROAD Advice: DoH. Health Information for Overseas Travel, 2nd edition, 2001. London: The Stationary Office. Available from PO Box 29, St. Crispins, Duke St, Norwich NR3 1GN and authorized bookshops and from www.the-stationary-office.co.uk/doh/info/index.htm

The history must include: (a) the dates and places visited; (b) visits to rural areas; (c) contact with animals; (d) swimming in inland waters; (e) sexual contact with local people; (f) what immunizations were given, what prophylaxis was taken and for how long. Note: Remember that the patients may be contacts of travellers, and not the travellers themselves. FEVER Review: Humar A, Keystone J. Evaluating fever in travellers returning from tropical countries. BMJ 1996; 312: 953-6.

Work-up: (a) FBC with request for thick and thin films for malaria; (b) LFTs; (c) culture blood and stool; (d) arbovirus serology if suspected.

Malaria

Viral hepatitis • The patient may present with fever, without jaundice. There are about 1000 cases a year in returning travellers to the UK, 20% of them hepatitis B.

Typhoid and paratyphoid fever • These account for 200 cases a year in the UK. They should be suspected in anyone with fever within 3 weeks of returning from an area where typhoid is endemic (e.g. the Indian subcontinent), when malaria has been excluded.

Legionnaires' disease The fever may be associated with headache, myalgia and cough. 25% of cases have watery diarrhoea as a prodrome. About one-third of cases

38

INFECTIOUS DISEASES

in the UK are contracted in foreign hotels. Men over 50 years of age are especially at risk. There will probably be no chest signs, but the CXR will be abnormal.

Amoebic liver abscess * Suspect it in anyone who has visited the tropics or subtropics within the last year. The majority of cases have pain in the right hypochondrium but some have no focal signs.

Lassa fever and other viral haemorrhagic fevers * Suspect it in anyone with persistent fever who has been in an endemic area within the last 3 weeks. Endemic areas: -Lassa fever: West Africa from Senegal to Nigeria; - Ebola fever: Zaire and Sudan; - Marburg fever: Uganda; -Crimean/Congo haemorrhagic fever: East and West Africa, Central Asia and the former USSR. * Arrange for an immediate domiciliary visit by the Consultant in Communicable Disease Control or the Consultant in Infectious Diseases. Do not admit the patient directly into a general hospital. Advice from the Department of Health is available on www.open.gov.uk/doh/vhf.htm. If the patient is admitted to a High Security Infectious Disease Unit, the ambulance service will do so as a category III removal.

Other infections These are less common but possible: (a) Dengue is common in the Caribbean, India and South East Asia. (b) Leptospirosis is worldwide and caught from inland water. (c) Brucellosis is caught from drinking unpasteurized dairy products. (d) Rickettsial infections are tick-borne. They are common in the Mediterranean. (e) Visceral leishmaniasis is also found in the Mediterranean; ten cases a year occur in the UK. (f) Relapsing fever accounts for ten cases a year in the UK. (g) Meningococcal infection may be imported from Africa, the Arabian peninsula, North India or Nepal.

DIARRHOEA

Tuberculosis

* Send stool for microscopy and culture, specifying the places visited. If the stool contains blood and mucus it must reach the laboratory fresh, for microscopy for amoebae. * Warn the patient that if a pathogen is isolated they will be contacted by the local environmental health department. * Mild symptoms: treat with codeine phosphate, loperamide or diphenoxylate as needed. * Moderate to severe symptoms: treat with ciprofloxacin 500 mg b.d. for 5 days without waiting for the stool result. This has been shown to speed recovery in bacterial gastroenteritis.20 Children and pregnant women may be given trimethoprim 200 mg b.d. for 5 days. * Diarrhoea suggestive of giardiasis: treat with metronidazole 2g daily for 3 days or tinidazole 2g as a single dose, even if stool microscopy is negative. Giardiasis is suggested by a long incubation period (2 weeks or longer), by watery stool with a lot of flatus, and no fever. Symptoms respond promptly to the above antibiotics, providing a more reliable test than stool examination.

Tuberculosis may be acquired abroad. Suspect it readily in immigrants from areas of high risk.

Note: Falciparum malaria commonly presents with diarrhoea as well as fever. In a traveller from

Acute schistosomiasis The fever may be associated with abdominal pain, cough, urticaria and eosinophilia. Assume that all rivers, lakes and fresh water in the tropics and subtropics are colonised with snails infected with schistosomiasis.

SCREENING OF IMMIGRANTS AND THOSE WHO HAVE SPENT A LONG TIME ABROAD

an endemic area, exclude this before treating the patient for gastroenteritis. Gastroenteritis may trigger an exacerbation of inflammatory bowel disease or lead to lactose intolerance, which may then continue as prolonged diarrhoea.

JAUNDICE * Send blood for LFTs and hepatitis serology; viral hepatitis is the most common cause. * Send blood for thick and thin films for malaria if the patient has returned from sub-Saharan Africa and is febrile. * Remember that leptospirosis, typhoid, dengue, relapsing fever and yellow fever can present as jaundice.

PHARYNGITIS * Send a throat swab for diphtheria. * Remember that Lassa fever may present with pharyngitis and fever.

SEXUALLY TRANSMITTED DISEASES * Screen all returning travellers who admit to casual, unprotected sexual contact abroad for STD (including HIV). * Consider chancroid, lymphogranuloma venereum and granuloma inguinale in returning travellers with genital ulcers or inguinal lymphadenopathy. Genital herpes and syphilis are, however, much more common.

SCREENING OF IMMIGRANTS AND THOSE WHO HAVE SPENT A LONG TIME ABROAD SCREENING RETURNING TRAVELLERS Advice: DoH. Health Information for Overseas Travel, 2nd edition, 2001. London: The Stationary Office. Available from PO Box 29, St. Crispins, Duke St, Norwich NR3 1GN and authorized bookshops, and from www.the-stationary-office.co.uk/doh/info/index.htm

39

• The Department of Health encourages the screening of returning travellers by GPs, both for their reassurance and for the number of positive results that it yields. One study found positive results in one in four asymptomatic people returning from at least 3 months in the tropics. • The results of screening must be interpreted with care.21 A negative blood film does not exclude malaria between episodes of fever. Conversely, the finding of worms in the stool is of no great significance since, with the exception of Strongyloides, they will die out without treatment in temperate climates. Work-up: (a) Fresh stool microscopy for cysts, ova and parasites. (b) FBC and differential for eosinophilia. (c) MSU. (d) Specific tests for those at special risk (see below).

Schistosomiasis * Check serology, eosinophil count and microscopy of stool and urine of those who have had contact with water in lakes and slow moving rivers in endemic areas (Middle East, China, Japan, South East Asia, sub-Saharan Africa and South America). Allow at least 3 months after exposure for seroconversion to take place.

Sexually transmitted diseases * Test for STD, including hepatitis B, and offer HIV testing for those at risk.

Strongyloides Patients who have ever been in conditions of extremely poor hygiene, e.g. Far East prisoners of war, should have fresh stool sent to test for Strongyloides. Without treatment, subsequent suppression of the patient's immunity can lead to symptomatic disease. In all screening cases, take the opportunity to check BP, weight, and urine protein sugar and blood. These are far more likely to reveal a (non-tropical) condition that needs treatment.22

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SCREENING OF IMMIGRANTS Immigrants unable to provide proof of BCG and normal CXR at the port of entry will be notified to the Consultant in Communicable Disease

Control for the area in which they intend to stay. GPs should offer screening as above, and also any immunizations not given in the country of origin.

REFERENCES 1. UK Department of Health. Health Information for Overseas Travel, 2nd edition, p. 95. London: The Stationary Office, 2001. 2. NHS Executive. Screening of pregnant women for hepatitis B and immunisation of babies at risk. Health Service Circular HSC 1998/127. 3. CMC's letter of 16 July 2001 (PL/CMO/2001/4). Online. Available: www.doh.gov.uk/cmo/cmoh.htm 4. CMO's letter of 16 July 2001 (PL/CMO/2001/4). Online. Available: www.doh.gov.uk/cmo/cmoh.htm 5. Jefferson TO, Demicheli V, Deeks JJ et al. Amantadine and rimantadine for preventing and treating influenza A in adults (Cochrane Review). In The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 6. Ukeyi T, Winquist A. Influenza. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. 7. CMO's letter 9 March 2001 (PL/CMO/2001/1). Online. Available: www.doh.gov.uk/cmo/cmoh.htm 8. CMO's letter 4 January 2002 (PL/CMO/2002/1). Online. Available: www.doh.gov.uk/cmo/cmoh.htm 9. Cartwright K, Kroll S. Optimizing the investigation of meningococcal disease. BMJ 1997; 315: 757-8. 10. CMO's letter 15 October 2001 (PL/CMO/2001/5). Online. Available: www.doh.gov.uk/cmo/cmoh.htm 11. Halperin SA et al. Seven days of erythromycin estolate is as effective as 14 days for the treatment

12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22.

of Bordetella pertussis infections. Pediatrics 1997; 100: 65-71. McMullin M, Johnston G. Long term management after splenectomy. BMJ 1993; 307: 1372-3. Dixon AM, Bibby JA. Tetanus immunization state in a general practice population. BMJ 1988; 297: 598. Maggiorini M et al. Prevalence of acute mountain sickness in the Swiss Alps. BMJ 1990; 301: 853-5. Norboo T, Ball K. High altitude pulmonary oedema in the Himalayas: a preventable condition. Practitioner 1988; 232: 557-60. Luzzi GA, Pasvol G. Travellers' diarrhoea. Prescribers Journal 1988; 28(3): 96-102. Huzly D et al. Malaria chemoprophylaxis in German tourists: a prospective study on compliance and adverse reactions. Journal of Travel Medicine 1996; 3: 148-55. Croft A. Malaria: prevention in travellers. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Phillips-Howard PA et al. Malaria prophylaxis: survey of the response of British travellers to prophylactic advice. BMJ 1986; 293: 932-4. Van Saene R, Damjanovic V, Williets T. Food handlers and food poisoning (letter). BMJ 1990; 300: 747-8. Ellis C. The returning traveller. Practitioner 1990; 234: 831. Carroll B et al. Post-tropical screening: how useful is it? BMJ 1993; 307: 541.

CHAPTER CONTENTS General approach 41 Patients requiring investigation by the GP 41 Patients requiring management by the GP 41 Contact tracing 42 Principles of treatment 42

3 Sexually transmitted infections

Specific management of STIs 42 Gonorrhoea 42 Chlamydia infection 43 Non-specific urethritis 43 Trichotnonas vaginalis 43 Anogenital warts 44 Herpes genitalis 44 Syphilis 45 HIV and AIDS 45 References

49

GENERAL APPROACH • Sexually transmitted infections (STIs) frequently coexist, so patients diagnosed with one STI generally need further investigation to exclude other infections. • Genitourinary (GUM) or sexual health clinics provide full screening, immediate microscopy and assistance for patients in the treating and informing of partners (partner notification or contact tracing). This is important in limiting the spread of disease, and patients diagnosed with an STI should be routinely referred to GUM for further management.

PATIENTS REQUIRING INVESTIGATION BY THE GP (a) Those who are eligible for a local or national screening programme (e.g. for chlamydia in some circumstances). (b) Those who require treatment urgently and cannot attend GUM immediately (e.g. symptomatic pelvic inflammatory disease out of hours). (c) Those who are unwilling or unable to attend a GUM clinic after discussion and recommendation.

PATIENTS REQUIRING MANAGEMENT BY THE GP (a) Those who have tested positive for an STI at the surgery and cannot attend GUM immediately. 41

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SEXUALLY TRANSMITTED INFECTIONS

(b) Those who present as contacts of an STI and refuse to attend GUM for testing, treatment and partner notification advice, after discussion and recommendation of this course of action.

CONTACT TRACING (PARTNER NOTIFICATION) • This is usually best done by GUM, or at least with their support and advice. • Screening of all contacts over the last 1 month (men with urethral discharge) or 6 months (other cases) should be undertaken. • If the patient is diagnosed with non-specific urethritis (NSU), chlamydia, Trichomonas vaginalis or gonorrhoea, the partner should be treated even if tests are negative.

PRINCIPLES OF TREATMENT * Give appropriate antibiotics, bearing in mind any recent travel history. * Advise complete abstinence from all sexual contact until both patient and partner are treated. * Follow-up after completion of antibiotics for compliance and (where appropriate) retesting.

Work-up for cases to be managed in primary care * Take a full sexual history over the preceding 6 months. * Examine the genitalia for discharge, ulcers or warts (including the vagina and cervix in females), the mouth for ulcers, and perform proctoscopy if there are anal symptoms or a history of anal intercourse. Diagnostic tests in women * Gonorrhoea: cervical, urethral swabs; also oropharyngeal and rectal swabs if symptomatic at those sites, if a partner has gonorrhoea, or if suggested by the sexual history. * Chlamydia: endocervical swab (rotate well) (unless first-catch urine is being used for screening). * Trichomonas vaginalis, bacterial vaginosis and Candida: high vaginal swab. * Check that cervical smears are up to date. Diagnostic tests in men * Gonorrhoea and chlamydia: urethral swabs (insert 1-4 cm and rotate), and a slide for the laboratory if there will be a delay in swab transport. Oral and rectal swabs may be indicated by the sexual history.

Tests to be done in all patients suspected of having an STI * Check syphilis serology and repeat it 3 months after exposure. * Check hepatitis B serology in patients who have been exposed in countries with high incidence, and in homosexual men. * Discuss HIV testing with all patients. Where a patient does not wish to attend a GUM, consideration should be given to testing in primary care after due consideration and counselling.

SPECIFIC MANAGEMENT OF STIs See also entries on pelvic inflammatory disease (p. 214), bacterial vaginosis (p. 214) and candidiasis (p. 213).

GONORRHOEA Genital gonorrhoea (uncomplicated) Guideline: Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). 2001 National Guideline on the Management of Gonorrhoea in Adults. Online. Available: www.agum.org.uk (choose 'Clinical Effectiveness Guidelines (CEGs) on management of STIs'). Review: Moran J. Gonorrhoea. Clinical Evidence, 2001; Issue 5. London: BMJ Publishing Group. Available: www.clinicalevidence.org

Referral to, or at the least advice from, GUM is strongly recommended, and screening for other STIs should be routinely undertaken. (a) Ciprofloxacin 500 mg orally stat or ofloxacin 400 mg orally stat if the patient has acquired the infection in the Far East or Africa and is not pregnant; or (b) Ampicillin 2g or 3g orally as a single dose. Ampicillin is not effective in clearing pharyngeal infection. Infection acquired in the Far East or Africa may be ampicillin resistant. (c) Treat all patients for chlamydia infection. The co-infection rate is 40%. (d) Seek advice for all pregnant women. They may require ceftriaxone 250 mg im, or cefotaxime 500 mg im if there is a risk of penicillin resistance.

SPECIFIC MANAGEMENT OF STIs

(e) Trace and treat contacts over the previous 2 weeks (or last partner) in males with symptomatic urethral infection, and over 3 months in all others. (f) Follow up at least once to confirm compliance, resolution of symptoms and partner notification. CHLAMYDIA INFECTION (UNCOMPLICATED) Guideline: Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). 2001 National Guideline on the Management of Chlamydia trachomatis Genital Tract Infections. Online. Available: www.agum.org.uk (choose 'CEGs on management of STIs'). Review: Low N, Cowan F. Genital chlamydia infection. Clinical Evidence, 2001; Issue 5. London: BMJ Publishing Group. Available: www.clinicalevidence.org

* Currently used diagnostic tests vary greatly in their sensitivity, between 50% and 95% on endocervical samples, so treatment and partner notification should be undertaken on strong suspicion even if tests are negative. Urethral sampling in women can increase pick-up rate by 10-20%. Sampling methods greatly affect test performance endocervical swabs should be rotated firmly, and male urethral swabs should be rotated 1-4 cm inside the urethra. * Give: (a) azithromycin 1 g as single oral dose; or (b) doxycycline lOOmg b.d. for 7 days; or (c) erythromycin 500 mg b.d. for 14 days. * In pregnancy, give: (a) erythromycin 500 mg b.d. for 14 days or 500 mg q.d.s. for 7 days; or (b) amoxycillin 500 mg t.d.s. for 7 days; and (c) a test of cure performed 3 weeks after completing therapy. * Patients should be referred to GUM to discuss partner notification with a trained health adviser the UK chlamydia pilots suggest that this is acceptable to patients diagnosed in primary care. A cut-off for 4 weeks is used for symptomatic men - for all others, the lookback should be for 6 months or to the last partner (whichever is the longer).

43

NON-SPECIFIC URETHRITIS (NSU) Guideline: Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). 2001 National Guideline on the Management of Non-gonococcal Urethritis. Online. Available: www.agum.org.uk (choose 'CEGs on management of STIs').

* Test all patients who have urethritis for gonorrhoea and chlamydia. * First-line treatment is doxycycline 100 mg twice a day for 7 days, or azithromycin 1 g orally stat. Follow-up at 2 weeks, and treat partners over the past 4 weeks for symptomatic patients, and 6 months for asymptomatic men. * Refer unresolving cases to GUM. Some men have recurring urethritis due to non-infectious causes - in these cases specialist advice and diagnostics, followed by reassurance, are important. TRICHOMONAS VAGINALIS Guideline: Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). 2001 National Guideline on the Management of Trichomonas vaginalis. Online. Available: www.agum.org.uk (choose 'CEGs on management of STIs').

• Trichomonas vaginalis (TV) infection is almost exclusively sexually transmitted, through urethral or vaginal inoculation. It is sometimes diagnosed on cervical cytology, where there is a false positive rate of about 30%, so tests should be repeated. • Trichomonas infection is strongly associated with other STIs, and full screening should be undertaken. • Metronidazole 2g orally stat, or metronidazole 400-500 mg b.d. for 5-7 days will clear 95% of infections. The stat dose is as effective as the 7-day course but is more likely to be associated with adverse effects.1 Test of cure is required only if symptoms persist, in which case the patient should attend GUM. Alcohol should be avoided during and for 48 hours after treatment. • Symptomatic disease in early pregnancy can be controlled with clotrimazole pessaries lOOmg

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SEXUALLY TRANSMITTED INFECTIONS

daily for 7 days, or Aci-gel, before systemic treatment in the second trimester. • Current partners should be screened for STIs and treated for TV. ANOGENITAL WARTS Guideline: Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). 2001 National Guideline on the Management of Anogenital Warts. Online. Available: www.agum.org.uk (choose 'CEGs on management of STIs'). Review: Wiley DJ, Beutner K. Genital warts. Clinical Evidence, 2001; Issue 5. London: BMJ Publishing Group. Available: www.clinicalevidence.org

• Most anogenital warts cause minor irritation, or are simply cosmetic. However, they cause a good deal of psychological distress. Patients should be given an explanation of their condition, emphasizing that the majority of anogenital warts are caused by types 6 and 11, which are not associated with cervical neoplasia. • Many carriers of human papilloma virus have no visible lesions. • Condom use may prevent transmission of warts to uninfected partners. Their use with regular partners, however, has not been shown to affect the outcome of treatment in patients with visible warts. • Perianal warts are associated with anal sex (although they can occur without) and should suggest the need for anorectal samples for other STIs, and GUM referral. • None of the existing treatments is satisfactory, and all have high recurrence rates. Patients should be made aware of this. The evidence base for distinguishing first and second line treatments is weak. • Pregnancy: pregnant women can be treated using cryotherapy, trichloroacetic acid or other ablative therapies in a specialist setting. However, warts often worsen during pregnancy and improve afterwards. The risk of transmitting warts or laryngeal papillomata to the neonate is small, and caesarean section is indicated only in rare cases of blockage or gross cervical warts.

Soft, non-keratinized warts * Prescribe podophyllotoxin 0.5% (twice daily for 3 days, repeated weekly for up to 4 weeks). Patients using podophyllotoxin at home must take great care to follow the instructions, in order to avoid chemical burns. It should not be used in pregnancy. * Imiquimod 5% cream is an immune response modifier which can be used three times weekly for up to 16 weeks. It should not be used in pregnancy.

Keratin ized warts * Use ablative therapy such as cryotherapy which is available at GUM clinics. HERPES GENITALIS Guideline: Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). 2001 National Guideline on the Management of Genital Herpes. Online. Available: www.agum.org.uk (choose 'CEGs on management of STIs'). Review: Wald A. Genital herpes. Clinical Evidence, 2001; Issue 5. London: BMJ Publishing Group. Available: www.clinicalevidence.org

• New diagnoses of primary herpes may be due to HSV type 1 or type 2. After childhood, HSV1 is equally likely to be acquired in the genital or oral areas.2 Much genital herpes is due to orogenital contact and can therefore occur in patients who may not consider themselves as 'sexually active'. Primary herpes is often asymptomatic, and patients often present many years later with symptoms which are due to recurrence. • Autoinoculation (e.g. from labial herpes) can only occur during primary episodes. Patients who have developed herpes for the first time but had no recent sexual contact should be referred to a consultant in GUM who will be familiar with such cases, and able to advise patients at a time of considerable distress and uncertainty. • Genital herpes can be acquired from asymptomatic partners, sometimes in a long-term relationship. Some patients never develop symptoms, although those with HSV2 will usually develop symptoms eventually.3

SPECIFIC MANAGEMENT OF STIs

45

• Initial episodes are typically far more severe, and of longer duration, than recurrences. Recurrences tend to decrease over time, but are more frequent with HSV type 2.2 • Diagnosis is by isolation and typing of virus using a swab from the base of an ulcer, sent rapidly to the laboratory at 4°C. Typing of the virus, possibly using serology, can be undertaken in some districts, and is best undertaken at GUM where specialist advice is available. • In primary episodes, patients can benefit from practical counselling in relation to the natural history, and issues in relationships (including risk to the partner). This can be provided at the GUM clinic. Psychological stress does not increase recurrences.4 • Partner notification is guided by the individual case and history of symptoms in the partner. Health advisers in the GUM clinic are experienced in this issue and can help with this.

• Severe recurrences may be shortened by oral antiviral agents as above. There is no evidence that topical antiviral creams are effective. Saline bathing, vaseline or lignocaine gel may aid symptoms. • Suppressive therapy. Depending on patient preference, relationship status, and severity of symptoms, it may be appropriate to offer systemic antiviral treatment to reduce the duration and severity of attacks and frequency. This can reduce anxiety, help in adjustment and improve quality of life, usually in patients with six or more recurrences a year. Suppressive therapy should be undertaken for a defined period of time before review, and is best undertaken by or in collaboration with the GUM clinic.

Management of a first symptomatic episode

SYPHILIS

* Oral antiviral therapy, commenced within 5 days of the episode or while new lesions are forming, reduces the severity and duration of that first episode. Options are aciclovir 200 mg five times daily, valaciclovir 500 mg b.d., or famciclovir 125mgb.d. * Offer analgesia, and recommend saline bathing. Topical anaesthetics should be used with caution in case of sensitization. It may be easier to pass urine in a tepid bath. * Admit patients with urinary retention, meningism or severe systemic symptoms. * Offer full counselling, including the risk of asymptomatic shedding and its implications for relationships, and the need to inform health-care workers in the case of later pregnancy. Health advisers at the GUM clinic are experienced in this area.

Management of recurrences • Most recurrences cause minor symptoms, of a few days duration.

Patient support group: The Herpes Virus Association, 41 North Road, London N7 9DR tel. 020 7607 9661; www.herpes.org.uk

This must be referred to the GUM clinic, where further investigation, treatment and partner notification will be undertaken.

HIV AND AIDS Prevention • Prevention of HIV is an essential part of contraceptive care, travel care, and well person clinics. Testing for HIV should now be universally offered as part of antenatal care, so that women avoid vertical transmission, and access services. Advising on safer sex and avoiding high-risk activities • Hugging, kissing and mutual masturbation are safe. • HIV has been transmitted through oral sex, but this is rare. A condom can be used for receptive oral sex, to improve protection. • High-risk sexual activities include penetrative vaginal or anal sex without a condom (which can be lubricated with KY Jelly or similar lubricants but not oils or vaseline, which reduce their strength by 95%). • Other high-risk activities include sharing needles or syringes for iv drug use.

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SEXUALLY TRANSMITTED INFECTIONS

• Sex with local residents in high prevalence areas of the world (e.g. Africa, South America, Thailand, parts of South America), or with people otherwise at high risk (e.g. men who have sex with men) increases the risks in the event of condom breakage or unprotected sex.

• Certain groups are at higher than background risk of HIV in the UK. Sex between men accounted for 57% of all UK HIV infections diagnosed to June 2001,5 and intravenous drug use for a further 8%. • However, among those diagnosed with HIV infection in 2000, 52% acquired it heterosexually6 often through travel abroad. Note: The BMA and RCGP advise doctors not to answer the lifestyle questions on insurance reports, even with consent. These questions allocate a person to an at-risk group, which is discriminatory, while only high-risk behaviour is relevant.

The HIV antibody test • The HIV test is >99.9% sensitive and specific 3 months after exposure to the virus,7 although the 'window period' before becoming positive can occasionally be longer (e.g. after post-exposure antiretroviral prophylaxis). • If HIV primary infection is suspected, P24 antigen and plasma HIV RNA testing may be appropriate, as prognosis may be improved by antiretroviral therapy at this time. GUM specialist advice should be sought. • A positive antibody test should be repeated to exclude identification errors.

HIV testing • Patients who do not wish to have the result recorded in their notes should be referred to the GUM clinic. • The patient should understand that having the test is entirely voluntary.

Pretest counselling • Patients should have an HIV test only with their fully informed consent, and need to come back in person for the result.

• Issues to be covered in pretest counselling for HIV: (a) The difference between HIV infection and AIDS, how the virus is transmitted, whether the patient has any misapprehensions. (b) The latency between transmission and seroconversion. Ensure that the patient is not in the 3-month 'window period' (occasionally longer). If so, discuss the need to postpone or repeat the test. (c) The importance of screening for other STIs and of making plans for safer sex whatever the test result. Pretest counselling Advantages of the test: 1. Effective therapies are available which improve the prognosis. 2. Knowing the result means that partners can be protected if it is positive or make decision to move to safer sex if negative. 3. Decisions about pregnancy can be made. 4. Anxiety due to uncertainty about HIV status is ended. 5. Informed decisions about medical issues, such as live vaccines, can be made. Disadvantages: 1. If positive, the patient will need to cope with a devastating diagnosis. He or she will need to consider whom to tell. 2. A positive (but not a negative) result may affect insurance, and possibly employment prospects.

Post-test counselling Negative results

• Discuss the concerns that gave rise to the test, and how the patient plans to protect him- or herself in future. Discuss the window period and whether further testing will be needed for full reassurance. Positive results

• Ensure that you have time and there will be no interruptions to the interview. Tell the patient early in the interview, to allow time for reflection and questions. • Establish what the patient knows and expects to happen, and ensure that he or she understands the difference between HIV infection and AIDS.

SPECIFIC MANAGEMENT OF STIs

Ensure the patient understands that there are now many effective therapies that improve prognosis. • Explain that the virus is not passed on by normal domestic or work contact, and obtain the leaflet Keep Safe, available free from the Department of Health.8 • Refer to a specialist clinic, usually the GUM service, for specialist follow-up and support. Clinics will fit in a newly diagnosed patient urgently. • Discuss and advise on concerns about transmission to others (see box on safer sex and avoiding high-risk activities), and the need for protected sex. • Find out what the patient's plans are for the rest of the day, and ensure that he or she arranges to meet someone for support. • Arrange to see the patient again in a few days do not overload with information and issues at this initial interview. • Give information on patient helplines. Patient information: Keep Safe - the Department of Health leaflet. Available: www.doh.gov.uk/eaga/ keepsafe.htm Patient support: National AIDS Helpline (24 hours) 0800 567 123, Minicom Service on 0800 521 361, daily 10 a.m. to 10 p.m. Prerecorded information in other languages (varies by day) on 0800 917 2227, and an operator in that day's language in the evenings. Terence Higgins Trust 020 7242 1010 (Helpline) 12 noon-10 p.m. Positively Women 020 7713 0222 10 a.m.-4 p.m. Monday to Friday.

Managing the HIV-positive patient in the community In the last few years, communication between specialist HIV clinicians and primary care has improved, and patients are often managed in collaboration. This allows GPs to gain experience in the condition, while encouraging patients to access care for unrelated conditions appropriately, and to use out of hours care and community nursing appropriately. The Sexual Health Strategy will soon require all HIV practitioners to provide HIV care within a managed service network, which will be expected to support primary care and others in providing non-specialist HIV services.9

47

Routine care • Regular estimates of viral load and CD4 measurements are important in advising patients to start highly active antiretroviral therapy (HAART) at the optimal time (usually when the CD4 count is between 350 and 200 X 106/litre). These are best done through the specialist HIV services (usually GUM). Yearly syphilis serology, baseline hepatitis B and C serology, FBC, LFT, cytomegalovirus (CMV) antibodies, toxoplasma antibodies, and other monitoring tests are needed if on therapy. Women should have yearly cervical smears because of the increased incidence of carcinoma. • Advise patients which symptoms should be treated seriously, including fever, weight loss, diarrhoea, shortness of breath or cough, paraesthesiae, headache, mouth ulceration, or visual disturbances. • Avoid BCG and yellow fever vaccines - seek specialist advice for other live vaccines but otherwise vaccinate as usual. Hepatitis B vaccination should be offered if seronegative. • Advise the patient to avoid exposure to toxoplasmosis (uncooked meat and unwashed salads), cryptosporidium if severely immunosuppressed (unboiled tap water). • Encourage patients with a CD4 count less than 200 to take prophylaxis against Pneumocystis carinii pneumonia (PCP) in the form of co-trimoxazole 960 mg daily, dapsone lOOmg daily, or nebulized pentamidine 300 mg every 4 weeks. • Ensure the patient has adequate psychological support in adjusting to his or her diagnosis. • The issue of pregnancy and contraception, together with safe sex, needs to be discussed with women on an ongoing basis. Effective interventions are now available which may reduce the risk of transmission to the baby to under 5%. Specialist advice should be sought at an early stage.

Symptoms needing special consideration • Cough, especially dry cough with breathlessness in a patient with minimal chest signs, and (initially) a normal CXR can indicate PCP. This requires urgent specialist care. Patients are also at

48

SEXUALLY TRANSMITTED INFECTIONS

increased risk of community acquired pneumonia, tuberculosis (TB), and chest infection with Moxarella catarrhalis. • Headaches can suggest cerebral toxoplasmosis, non-Hodgkin's lymphoma, tuberculoma or, importantly, cryptococcal meningitis, which may not be accompanied by neck stiffness or headache. • Diarrhoea can indicate opportunistic bowel infection, including cryptosporidium, microsporidium or Clostridium difficile, although often no cause is found and symptomatic treatment is required. • Dysphagia can indicate oesophageal candidiasis, treatable with fluconazole 100 mg o.d. for 14-21 days or (in the severely immunocompromised) CMV ulceration. • Visual disturbances including flashes and floaters, can suggest CMV retinitis. This should be referred urgently.

Venepuncture • The same universal precautions should be taken as with patients of unknown serostatus (gloves, avoid resheathing needles, place in appropriately sealed packaging and transport in safe containers). Avoidance of resheathing needles should be standard practice. • Some laboratories require 'high risk' stickers this will depend on local policy. 'High risk' does not require stating the diagnosis of HIV for transport purposes, which is unnecessary for routine tests and can generally be avoided in other cases. This practice is an unnecessary risk to patient confidentiality. • Spills should be mopped up with hypochlorite (10 parts water to 1 part household bleach), or undiluted Milton.

Postexposure prophylaxis for health-care workers (including needlestick injuries) Guideline: DoH. HIV post-exposure prophylaxis: guidance from the UK Chief Medical Officer's Expert Advisory Group on AIDS. London: Department of Health, 2000. Available from Department of Health Publications, PO Box 777, London SE1 6XH.

* The risk of acquiring HIV infection following percutaneous exposure to HIV infected blood is on average around 3 per 1000, and less than 1 in 1000 after mucocutaneous exposure. The risk is increased in the cases of deep injury, visible blood on the device causing the injury, a needle which had entered an artery or vein, or terminal illness in the source patient. An 80% reduction in the transmission rate can be achieved by appropriate prophylaxis, and all districts should have an arrangement for 24-hour access to such supplies for exposed health-care workers. * After any exposure to blood (whether or not the source patient is known to be HIV positive), wash the wound liberally with soap and water, but do not scrub. Antiseptics should not be used. * Telephone for advice on local arrangements for 24-hour access to HIV prophylaxis, and specialist advice on risk assessment. The local consultant in Communicable Disease Control (via the Public Health Department), consultant microbiologist/virologist or consultant in GU medicine will be able to advise if you do not have information to hand. Drug starter packs are often held at the local Accident and Emergency department. * If the risk is assessed as potentially significant, the drugs should be commenced as soon as possible, preferably within 1 hour. At the time of writing, starter packs usually contain zidovudine 250 mg b.d., lamivudine 150 mg b.d. and indinavir 800 mg t.d.s., all of which should be continued for a total of 4 weeks if subsequent risk assessment confirms this is appropriate. * If the source patient is of unknown status, he or she should routinely be approached (by someone other than the exposed patient) to ask consent for testing. * Consider the risk of hepatitis B and C (see p. 24). * Arrange follow-up, by Occupational Health, the GUM department or another appropriate department, for the exposed worker, who will wish to consider testing for HIV in confidence at a later stage.

REFERENCES 49

Notification and surveillance Microbiology laboratories voluntarily notify positive HIV results (confidentially and anonymously) to the Communicable Disease Surveillance Centre (CDSC), 61 Colindale Avenue, London NW9 5EQ, or the Scottish Centre for Infection and Environmental Health, Scottish Centre For Infection & Environmental Health, Clifton House, Clifton Place, Glasgow G3 7LN. These establishments then write to the clinician concerned asking for

further clinical details. Doctors may also notify cases directly. If the patient has not been referred on for further specialist management, the GP who receives a positive test result may be invited to provide information for the voluntary, confidential, anonymised database. Details of HIV and AIDS surveillance, together with recent data, can be seen at http://www.phls.co.uk/facts/HIV/ hivqnotes.htm

REFERENCES 1. Forna F, Gulmezoglu AM. Interventions for treating trichomonas in women (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2000. 2. Langenberg AG, Corey L, Ashley RL et al. A prospective study of new infections with herpes simplex virus type 1 and type 2. New Engl J Med 1999; 341: 1432-8. 3. Benedetti JK, Zeh J, Corey L. Clinical reactivation of genital herpes simplex infection decreases in frequency over time. Ann Int Med 1999; 131: 14-20. 4. Green J, Kocsis A. Psychological factors in recurrent genital herpes. Genitourinary Med 1997; 73: 253-9. 5. CDSC and Scottish Centre for Infection and Environmental Health. Unpublished Quarterly

6.

7. 8. 9.

Surveillance Tables No. 50 01/1, (Table 1), 2001. Online. Available: www.phls.co.uk CDSC, 2001. Online. Available: www.phls.co.ukpublications/CDRelectronic/ CDR%20weekly/CDR%20weekly/archive/ news0401 .html#HIVnews Sloand EM, Pitt E, Chiarello RJ, Nemo GJ. HIV testing. State of the art. / Am Med Assoc 1991; 266 (20): 2861-6. Department of Health, 1996. Online. Available: www.doh.gov.uk/eaga/keepsafe.htm Department of Health, 2001. Online. Available: www.doh.gov.uk/nhsh/bettersexualhealth.pdf

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4

CHAPTER CONTENTS Child health promotion 51 Reducing the risk of cot death 51 Prevention of unintentional injury 52 Nutrition 52 Dental and oral disease 54 Aspects of screening 54

Childhood problems

Child protection 61 Physical abuse 61 Child sexual abuse 61 Neglect and emotional maltreatment 62 Munchausen's syndrome by proxy abuse 62 Management of suspected child abuse 62 Possible outcomes 63 Divorce/marital strife: the effects on the children 64 Infancy 64 Preterm infants 64 Feeding problems 65 Vomiting in infancy 65 Excessive crying (colic1) 66 Watering/discharge from the infant eye 66 Nappy rash 67 Constipation 67 Diarrhoea with or without vomiting 67 Convulsions 69 Bladder and bowel problems 70 Sleep problems 72 Temper tantrums 74 'Breath-holding' attacks 75 Respiratory problems 75 The catarrhal child 76 Cough 76 Asthma in children under 5 years 77 Cystic fibrosis

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Upper airways obstruction Urinary tract infections Vulvovaginitis

Recurrent physical symptoms Recurrent abdominal pain 82 Recurrent headache 83 Night cramps 83

81

82

84

Surgical problems 84 Strawberry naevus 84 Inguinal hernias 84 Hydroceles 84 Undescended testis 84 The foreskin 84 Hypospadias 85 Umbilical hernia 85 Bat ears 85 Orthopaedic problems 85 Preventive health care in adolescents 87 Emotional problems in adolescence 87 References

88

See Appendix 5 for an account of the screening procedures that may be carried out at different ages. Current thinking, however, is moving away from a rigid screening protocol towards a more holistic approach, with an emphasis on child health promotion. REDUCING THE RISK OF COT DEATH

81

82

School refusal

Overview: Hall DM. Health for all children: Report of the Third Joint Working Party on Child Health Surveillance, 3rd edition. Oxford: Oxford University Press, 1996.

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80

The child who appears small or tall Puberty

CHILD HEALTH PROMOTION

* Instruct all parents about the following: (a) Sleeping position. Lie the infant on the back or side, not prone. If laid on the side, the infant should be stopped from rolling onto the front by placing the underneath arm forward. The prone position is associated with an increased risk of cot death. There is no evidence that placing babies on their back results in an increased risk of death by choking or vomiting.1 (b) Overheating. Make sure that the infant does not overheat - clothing and bedding should be no more than for an older child. Avoid duvets. Make the bed up so that the infant cannot slip down under the bedclothes. The bedroom should be maintained at 16-20°C. (c) Smoking. Smokers should smoke outside the house. 51

52

CHILDHOOD PROBLEMS

Parents who have already lost a child from sudden infant death syndrome (SIDS) * Recommend The Foundation for the Study of Infant Deaths. 24 hour helpline 020 7233 2090. www.sids.org.uk * Offer a copy of Baby Check, available from Baby Check Ltd., PO Box 324, Wroxham, Norwich NR12 8EQ, tel. 01603 784400.

PREVENTION OF UNINTENTIONAL INJURY A 'Health of the Nation' target is to reduce accidents in children by one-third by the year 2005. * Be aware of sources of potential injury at every contact with a family (see Appendix 6). Alert the health visitor to any causes for concern.

NUTRITION Breast feeding (see also p. 269) * Although 65% of women are breast feeding on discharge from hospital, only 30% are still feeding after 4 months. Inadequate milk supply is the most common reason put forward for stopping breast feeding.2 * Encourage the mother to have confidence in herself and her abilities. All possible measures should be taken to ensure adequate lactation. * Advise against: (a) Complementary feeding - unless essential; (b) Additional water, which is rarely necessary (even in hot weather), and may increase the risk of gastrointestinal infection. Support groups (head offices): National Childbirth Trust, Oldham Terrace, London N3 6NH, tel. 0870 7703236; http://www.nctpregnancyandbabycare.com La Leche League, BM3424, London WC1N 3XX, tel. 020 7242 1278; http://www.laleche.org.uk

Vitamin K See p. 269.

Vitamins A, D and C These should be given to all breast-fed babies at least until the age of 2 or until formula milks or follow-on milks have been added. They should not be given to a child on formula or follow-on milks.

Maternal diet The mother's diet does not need supplementation with vitamins unless inadequate. Very low calorie diets should be avoided.

Bottle feeding • Formula milk should be given until the infant is at least 12 months old. • Breast-fed babies changing to bottle should take formula feeds until 12 months. • Follow-on milks may be recommended in certain situations, e.g. premature babies or where cows milk is introduced as the main milk at 6 months. Follow-on milks are lower in protein than whole milk and are fortified with iron and vitamins A, C and D. * Advise the parents to use whey dominant milks, where the whey: casein ratio is similar to human milk (e.g. SMA Gold, Cow and Gate Premium, Ostermilk, Aptamil). There is no justification for changing older or 'hungrier' babies onto caseindominant milks (e.g. SMA White, Cow and Gate Plus, Ostermilk Number 2, Milumil), where the whey: casein ratio is similar to cows' milk. * Cows milk is low in iron and vitamins A, C, D and E. 'Doorstep' milk can be used to mix feeds, etc. but should not be used as the main milk until 1 year. After 1 year, whole milk is recommended until the age of 5. * Feed volume. The infant requires about 150ml/ kg per day. Traditionally this is given as 5-6 feeds a day, but 'on demand' feeding does not result in fatter babies. When a baby regularly finishes the feed offered, this can be increased by 30ml per feed. Parents should beware of misinterpreting hand sucking or crying as a request for a feed.

CHILD HEALTH PROMOTION

* Changing milks because the baby is 'not satisfied' or has 'colic' is of no value, and may increase anxiety. If the baby is genuinely not satisfied, the feed volume should be increased, or solids introduced if the baby is over 3 months old. * Additional supplements (e.g. vitamins and fruit juices) are not necessary. * Soya milks do not protect the infant against atopy and should not be given for colic or unconfirmed cows milk intolerance.

53

Other aspects of infant/preschool nutrition Vitamins

* The DoH recommends that all children, once weaned, be given vitamins until the age of 5. 500 ml of formula feed per day contains enough added vitamins. * If prescribing because of poor diet, give five drops of DoH vitamin drops A, D and C from 4-6 weeks after birth until at least 2 years old, and preferably to the age of 5.

Weaning Family history of food allergy, gluten enteropathy or allergic asthma

Committee on Medical Aspects of Food and Nutrition Policy (COMA) The COMA panel recommends that: (a) breast-feeding should be encouraged for 4-6 months, preferably longer; (b) early weaning should be discouraged; (c) foods regarded as potentially allergenic should be avoided until 6 months of age, e.g. cows milk, gluten, eggs, soya proteins, wheat and citrus fruits. General/behavioural principles The general principles are:

(a) Foods should be offered by spoon, in small quantities. If the infant refuses the food it should be removed. (b) Time should be put aside for feeding and infants allowed to feed at their own pace. (c) New foods should be introduced slowly and in small quantities initially. (d) Snacks should not be offered between meals. * Warn the parents to avoid nuts and other foods that could be inhaled until the child is at least 7 years old. * Encourage parents to reduce the fat intake from 1 year onwards, and to introduce semi-skimmed milk from the age of 2.

Iron

* Studies estimate the prevalence of iron deficiency in preschool children to be between 5 and 40%, and in school-age children between 5 and 10%. Iron deficiency is more common in children: (a) born prematurely; or (b) of low birth weight; or (c) who started drinking 'doorstep' milk early; or (d) who drink tea; or (e) who have poor nutrition. * Iron deficiency not only causes anaemia, but can affect behaviour, development and susceptibility to infection. In children with those problems consider: (a) aFBQand (b) a 3-month course of iron 5 mg/kg per day, as ferrous sulphate oral paediatric solution. Sugar

See nutritional advice below. Salt

* Advise against adding salt to food cooked for a baby under 6 months. After 6 months the salt added should be kept to a minimum, with no added salt at the table. Fibre

* Advise against rigorously high-fibre diets, as they do not supply adequate nutrition to a growing child and can cause diarrhoea.

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CHILDHOOD PROBLEMS

Drinks

* Advise the parents (after weaning) to: (a) Give drinks only after meals, and to restrict the milk intake to below 1.5 pints a day to avoid reducing appetite. Encourage water. (b) Offer drinks by feeder cup or beaker from 6 months. (c) Remove the cup from in front of the child after he or she has had enough. (d) Discourage tea, which impairs iron absorption. Fruit juices should be diluted with water.

DENTAL AND ORAL DISEASE • Forty per cent of 5-year-olds nationally have been shown to have active tooth decay. • Young children who attend the dentist 'only when they have trouble with their teeth' have more diseased teeth than children who attend for regular check-ups. • Encourage the parents to: (a) avoid the use of sugared dummies and juices in a 'dinky feeder' or night comforter; (b) wean their children on to food and drink which are free from non-milk extrinsic sugars; (c) discourage the use of a bottle after 12 months; (d) restrict the intake of any sugary food and drinks to mealtimes; (e) beware of 'hidden sugars' in food and drink, even in commercially prepared baby foods; (f) restrict the intake of acidic drinks (fruit juices or carbonated drinks) to mealtimes, and dilute any fruit juices with water; (g) brush children's teeth once they appear using a small toothbrush with a children's fluoride toothpaste (500 parts per million (ppm) concentration). No more than a pea-sized blob is required; (h) commence early and regular dental visits; 6 months of age is the ideal time to seek advice from a dentist on a child's need for fluoride supplements; (i) encourage dental attendance at least once a year.

* Prescribe and promote the use of sugar-free medicines, especially when they are needed long-term. * Refer children with suspected or overt dental disease to a dentist.

Fluoride Topical fluoride is of more value in toothpaste than as oral supplementation. Fluoride supplements should be given only where the water supply contains less than 0.7 ppm.

ASPECTS OF SCREENING Neonatal and 8-week examinations See check-list chart, Appendix 5.

Examination of the hip to exclude congenital dislocation (CDH) • Screening for CDH does not detect all cases at birth. Regular examination and awareness is necessary until the child is walking normally. Note: Overexamination may be harmful, and only one professional should screen on each occasion. • High risk groups: first-born children, girls, those with a family history of CDH, breech delivery, congenital positional deformities of the feet or other joints, oligohydramnios, fetal growth retardation, cerebral palsy or other disorders of the motor system. Examination under 3 months

• Use the modified Ortolani/Barlow manoeuvres. The infant should lie supine on a flat surface in a warm room. The hips should be partially abducted and fully flexed. The pelvis should be stabilized with one hand by holding a thumb over the symphysis pubis and fingers under the sacrum. The leg on the side being checked is held with the other hand. With the upper part of the tibia resting between the examiner's forefinger and thumb, the femur is gripped so that the middle finger is held over the greater trochanter and

CHILD HEALTH PROMOTION 55

the thumb over the lesser trochanter. Assess one hip at a time. (a) Abduct the hip by pressing medially on the greater trochanter with the middle finger. If the hip is posteriorly dislocated it will relocate into the acetabulum with a clunk (Ortolani positive). (b) Place the thumb on the inside of the thigh and press laterally, trying to push the head of the femur backwards out of the acetabulum. The dislocatable or subluxable hip 'clunks' over the posterior edge of the acetabulum (Barlow positive). * Ligamentous clicks without movement are of no importance in themselves, but are associated with an increased incidence of CDH at later testing. Review at 6 weeks; if still present refer to an orthopaedic surgeon.

at 3 months and are unlikely to descend thereafter. * One boy in 120 with an untreated undescended testis will develop malignancy.

Signs and examination over 3 months of age

* Examine the baby shortly after birth and at 6-8 weeks for symptoms and signs of CHD. Any murmur heard in the first 48 hours of life is likely to be significant, and warrants early referral. Any murmur heard at 6 weeks also warrants referral, but not urgently if the child is well. Subsequent routine examination is not necessary, but the opportunity can be taken to examine the heart when examining the child for other reasons. * Always check the femoral or foot pulses. * High risk of congenital heart disease. Ensure that all children at risk have an ECG and echocardiogram in the first few weeks of life. These are children: (a) with conditions that put them at higher risk, e.g. Down syndrome; (b) with a sibling with congenital heart disease.

* Look for the following: (a) shortening of the leg; compare the level of the knees; (b) the thigh lying in partial lateral rotation, flexion and abduction; (c) flattening of the thigh on the affected side when prone; (d) asymmetrical skin creases; (e) a large gap between the thighs, in patients with bilateral dislocation; (f) reduced abduction. * 6-9 months. Abduct the hip when flexed to 90°. If CDH is present, resistance will be felt before 45° is reached. If CDH is unilateral, abduction will be asymmetrical. In some children, a clunk may be felt as the head relocates. In either situation, refer to an orthopaedic surgeon. * 18-24 months. Refer any child with a waddling gait suggestive of CDH.

Undescended testis Review: Davenport M. Inguinal hernia, hydrocele and the undescended testis. BMJ1996; 312: 564-7.

• About 6% of boys have one or both testes undescended at birth; 1.6% are still undescended

* Examine all boys at the neonatal examination, and at 6-8 weeks. Refer to a surgeon at this point if one or both testes are not clearly descended. The surgeon may well choose not to see the child until shortly before his first birthday and not to operate until his second year. * The older child. Examine with the child squatting and lying down. Examination of the standing child is very likely to give rise to error.

Congenital heart disease * The birth prevalence of congenital heart disease (CHD) is 8 per 1000.

'Innocent' murmurs • Innocent murmurs occur in 25% or more of children. Characteristically: (a) they are of low intensity, and are even quieter sitting up; (b) they have a musical quality; (c) they are localized to a small precordial area;

56

CHILDHOOD PROBLEMS

(d) there are no other symptoms or signs of heart disease. • Arrange an echocardiogram or refer if there is any suspicion of underlying disease.

(b) At 6-8 weeks of age. If the plotted results follow the centile lines, no further routine measurements are necessary. Recheck only if there is concern about the child's growth, health or development.

Growth

Where there is concern about growth

• The infant usually regains his or her birth weight by the end of the second week, and then gains 150-200 g per week. A doubling of birth weight is achieved by the age of 4-5 months. Growth then slows, and the birth weight is tripled by 12-13 months. Only 2-3 kg is gained in the second year. • There is no reason to assume that every baby should continue on the same centile from birth onward. • Most babies who cross centile lines are adopting their own genetically determined growth pattern. • It is rare for significant changes in weight to be the only clue to serious abnormality. • All measurements should be plotted on the 1990 nine-centile charts, available from The Child Growth Foundation and now in most parentheld child health records in UK.

* Measure and plot the child's length, weight and head circumference. Include birth weight and head circumference, and any other clinic weights. * Enquire about the child's diet, general behaviour and the character of the stool (irritability and steatorrhoea are indicators of malabsorption). * Examine the infant fully both to reassure the parents and to exclude cardiac, renal or other system disorders. Check the urine for protein, and send a specimen for culture. Refer if the child appears unwell. * Assess the environment in which the child is being raised, and the parents' relationship with the child. * Ask the advice of the health visitor in all cases, but particularly if there is reason to suspect 'nonorganic failure to thrive'.

Charts and measuring equipment are available from the Child Growth Foundation, 2 Mayfield Avenue, London W4 1PW, tel. 020 8994 7625, fax 020 8995 9075.

Recommendations for routine growth monitoring * Check weight: at birth, then regularly at routine well baby checks. Very frequent weighing may pick up small fluctuations and increase anxiety. * Check height/length: at 18-24 months, at about 3.5 years and about age 5. Only check length at the neonatal or 6-week check if there was a low birth weight or if there is some other concern about health; * Check head circumference: (a) Before discharge from hospital (excessive moulding, etc. should be noted, and the head circumference repeated a few days later);

Abnormal patterns of growth * Slow weight gain. Consider referral if the weight gain is slow and crosses two channels (a channel is the space between two centile lines). Refer more urgently if there is a fall-off in head circumference as well. * Single measurement of length/height. Refer any child who has a single length or height measurement above the 99.6 centile or below the 0.4 centile. * Slow gain in height: Refer if the child's height crosses two channel widths. Review a preschool child after a year if the child's height crosses one channel width. Review a school age child after a year if the height crosses half a channel width or more. * Single measurement of head circumference. Refer if a single measurement is above the 99.6 or below the 0.4 centile. The exception may be when development is normal and the baby's head circumference is commensurate with the parents' head circumference centiles.

CHILD HEALTH PROMOTION

• Head circumference crossing centiles upwards. Refer urgently if the child shows symptoms or signs of hydrocephalus or other abnormality. If there are no accompanying symptoms or signs, take two measurements over a 4-week period, and then decide whether to refer. • Head circumference crossing centiles downwards. If the child is thriving there is no cause for concern. Refer if the growth line is below the second centile and falling from it.

Hearing defects • In the absence of appropriate intervention, children with sensorineural hearing loss suffer impairment of language acquisition. Educational difficulties follow and lead to social isolation, an increased risk of mental health problems and reduced prospects for independence in later life. • The estimated birth prevalence of congenital severe (>40dB) hearing impairment is 1.16 per 1000. (A number acquire this severe loss of hearing bringing this figure to 1.3 per 1000. These children require a hearing aid). • At least a further 0.3 per 1000 have a hearing loss which is less than 40 dB but is sufficient for the child to need a hearing aid. • If all high-risk factors are considered, between 40 and 70% of all cases could be identified by testing 5-10% of all babies. • The following are at a high risk of congenital deafness: (a) survivors of intensive care, gestation <33 weeks or birth weight <1500 g, severe asphyxia at birth, meningitis, marked jaundice requiring exchange transfusion, or treatment with aminoglycosides; (b) survivors of severe intrauterine growth retardation; (c) those with a family history of deafness which may be genetic; (d) children of mothers who suffered from rubella, CMV or other relevant infections during pregnancy; (e) children with dysmorphic syndromes, particularly those affecting the head and neck. Paediatric departments should follow up all highrisk infants and assess their hearing by brainstem

57

evoked response (BSER), postauricular myogenic response (PAM) or cochlear echo. * Refer any high-risk infant not followed up, or where there is concern. Parents' recognition of deafness is often accurate. * Refer any child not performing as below. Older children are best seen first by the health visitor. (a) 3 months: infants smile at the sound of parents' voices, even when they cannot see them, and turn the head in response; (b) 6 months: infants turn immediately and accurately across the room to a parent's voice; (c) 9 months: infants listen attentively to sounds and try to localize very quiet sounds. They enjoy making a loud tuneful babble; (d) 12 months: infants respond to their own names.

Vision defects * Examine the eyes of all babies after birth and at 6-8 weeks. Check the pupils, the alignment of the eyes and the red reflex. Use the ophthalmoscope set on +3, held 30cm from the baby's eye. * Neonates: Check that the following infants have been examined by an ophthalmologist: (a) those born prematurely under 1250 g and/or under 32 weeks gestation; (b) those with a family history of inherited eye disease; (c) those with a permanent squint urgently, to exclude retinoblastoma and cataracts. * Infants: Refer if any of the following are present: (a) a lack of fixation or following movements; (b) nystagmus; (c) photophobia; (d) a wandering eye; (e) opacities (best seen with an ophthalmoscope as above); (f) delayed development possibly due to visual problems. Children aged 6-9 months should: look at, recognize and follow their parents with their eyes; examine their hands and feet; look at and reach towards small objects.

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CHILDHOOD PROBLEMS

Squint • Most squints are due to muscle length inequality. However: (a) a few are due to major intra-ocular pathology e.g. retinoblastoma; (b) a few are due to oculomotor palsy. • A persistently squinting eye becomes permanently amblyopic. All children over the age of 6 months with a squint, or where the parents are concerned about a latent squint, should be referred for an orthoptic or ophthamological opinion. Parents' observations and anxieties are usually correct. A persistent squint in the same eye warrants early referral (even before 6 months). A child with an alternating or latent squint should be referred, but not urgently. A squint may be indicated by the following: (a) Appearance. The squint may be apparent on examination. However, broad epicanthic folds can give the appearance of a convergent squint (pseudosquint). A child may hold the head at a tilt to maintain binocular fixation. (b) Corned reflections should be identical in position in both eyes. With the child focusing on a small toy, check the reflection of a small torch held 40 cm away from the eye or the reflection of a window behind the observer. (c) Cover test. This is used to exclude a latent squint. Each eye is encouraged to fixate on a toy while the other eye is occluded. If a squint is present in the occluded eye it will move to take up fixation when uncovered. Ideally, fixation should be tested with both a near object (about 30cm) and one at 6 metres. (d) Eye movements should be assessed in the horizontal and vertical planes. If a squint is found, look for an intact red reflex with the ophthalmoscope.

Disorders of development and behaviour • The more common disorders are: cerebral palsy, 1.5-3 per 1000; Duchenne muscular dystrophy, 3 per 10,000; severe learning disability

(previously called mental handicap), 3.7 per 1000; Down syndrome, 1 per 600; persisting speech and language impairments, 2 per 1000; autistic spectrum disorder, 1-2 per 1000. • The GP who remains vigilant despite normal routine screening results is more likely to detect the milder manifestations of the above. False reassurance is a common cause for a delay in diagnosis. For developmental charts, see Appendix 7. Learning difficulties See p. 409.

Speech and language • Early detection and intervention where speech and language are delayed may avoid irreversible impairment of language acquisition and limit the negative experiences which seriously affect subsequent health and progress. * Discuss with the health visitor whether emotional deprivation may be contributing to the poor language development. * Refer any child where there is parental concern about speech to a speech therapist. * Refer children as follows: (a) 18 months: not using any words; unable to identify common objects. (b) 2 years: words are not joined together; simple instructions are not understood. (c) 3 years: not using sentences; speech is not understood outside the family; simple concepts are not understood. (d) 4 years: difficulty in following a story; sentences are immature; poor understanding of simple concepts, e.g. colour; difficulty in holding a conversation; if stammering is a problem, particularly if the child has physical tension while speaking or gets stuck or repeats words. Autism and Asperger's syndrome • Autism affects about 1:2500, with autistic features occurring in about 1:500 of the population.

CHILD HEALTH PROMOTION

Fifty per cent of autistic people have severe learning disability (IQ <50), 25% have moderate learning difficulties (IQ 50-75). • Children should be referred early to a Community Paediatrician or Child Development Team. In confirmed cases, early diagnosis encourages appropriate behavioural management to limit inappropriate behaviour. • Autistic children have a triad of impairments, although the degree in each category may vary: (a) Impaired social interaction: aloofness/indifference to others, not playing with others; only joining in if an adult assists or insists; onesided interaction with no response to another child; 'odd interactions', e.g. inappropriate questions. (b) Impaired social communication: language not used as a tool for exchange; talking incessantly about one topic; inappropriate laughing and giggling; echolalia - copying words like a parrot; no eye contact. (c) Impaired imagination: lack of creative or pretend play; play becomes stereotyped or obsessional and ritualized. • Asperger's syndrome is usually diagnosed later in life, and is characterized by abnormalities of gaze, poverty of expression, a lack of feeling for others, lack of humour, extreme egocentrism and an idiosyncratic attachment to objects. Intelligence and language are normal. Patient contact: The National Autistic Society, 393 City Road, London, EC1V 1NG, United Kingdom, tel. 020 7903 3599; fax 020 7833 9666; e-mail: [email protected]; http://www.nas.org.uk

Dyslexia Review: Snowling MJ. Dyslexia: a hundred years on. BMJ1996;313: 1096.

• Dyslexia is a specific learning difficulty which results in a significant and persistent difficulty with reading, spelling, written prose and sometimes arithmetic. The individual may also have difficulties with orientation in time, short-term

59

memory, sequencing, auditory or visual perception and motor skills. * It is estimated that around 350,000 UK schoolchildren suffer from dyslexia. About 75% are boys. * Early recognition and management may allow greater attainment of educational potential and limit the stress and frustration that occurs when the problem is not recognized and the child falls behind at school. The preschool child:

(a) Speech or language delay (see p. 58): * Refer the child to a speech therapist if there is any delay in speech, especially where there is difficulty in naming objects correctly, remembering two instructions or recognizing similarities in rhyming words or parts of words. * Encourage the parents to inform the school when the child begins schooling. Many children with early language delay who speak normally by the time they go to school subsequently have difficulty learning to read or write. (b) Poor hand/eye coordination and visual perceptual problems: The child may appear to be developing normally, but has marked difficulty copying shapes and patterns, catching a ball, dressing in the right order, tying shoelaces, clapping in rhythm, etc. * Refer the child to a paediatric occupational therapist, preschool educational psychologist or community paediatrician. The school-age child:

Refer children with: (a) a lack of educational achievement, particularly in learning to read, write and spell. A difficulty in answering questions on paper; (b) a lack of understanding of time and tense; (c) poor concentration in reading and writing; (d) secondary emotional symptoms (e.g. disruptive behaviour, psychosomatic symptoms). The older child failing at school:

* Refer to the appropriate specialist if specific disabilities are apparent, e.g. problems with: (a) visual function;

60

CHILDHOOD PROBLEMS

(b) auditory ability; (c) speech or language difficulties; (d) motor coordination. * // no specific disabilities are apparent, refer for assessment either by a chartered psychologist or local paediatrician. Include a report from the school with the referral. * Advise the parent to get further advice from one of the support organizations. * Encourage the parents to support the child, for example by: (a) helping him or her to maintain self image; (b) encouraging involvement in hobbies where the child can succeed; (c) supporting the child in schoolwork, with an awareness that the child is more likely to be tired than others. * Where the child is suffering significantly at school, encourage the parent to apply to the school to have the child assessed with a view to making a 'Statement of Special Educational Needs'. Patient contact: The British Dyslexia Association, 98 London Road, Reading RG1 5AU, tel. 0118 966 2677; http:www.bda-dyslexia.org.uk

Attention deficit (hyperactivity) disorder (ADHD) Guideline: Scottish Intercollegiate Guidelines Network (SIGN). Attention deficit and hyperkinetic disorders in children and young people. June 2001. Online. Available: www.sign.ac.uk

• ADHD is characterized by the presence of inattention, hyperactivity and impulsivity for at least 6 months. For the detailed Diagnostic and Statistical Manual (DSM) diagnostic criteria, see the ADDnet website in the box below. • In the UK, the estimated prevalence of ADHD is 1.7% of boys of primary school age.3 Only about one-third grow out of their condition completely.4 Only about 10% of children in the UK with ADHD are diagnosed. • Not all are characterized by hyperactivity. A subgroup occurs who are inattentive and do particularly badly at school.

* Hyperactivity in preschool years is associated with conduct disorders in school-age children. * Dietary alteration may have value in a small number of children, especially where ingestion and effect are obvious. Exclusion diets are demanding and tedious, and should only be attempted with a dietitian's support. GP management The child with ADHD may already have seen a number of different agencies (psychologists, social workers, etc.) and may have been recognized as having special educational needs. Where there is concern that a more specific diagnosis and treatment may help: * Refer to a local specialist who has an interest in the problem. This may be a child or adolescent psychiatrist or paediatrician. Include a report by the parents of their child's symptoms, copies of all psychologist's and psychiatrist's reports as well as an up-to-date report from the school. * Do not start drug treatment. A GP may, however, continue treatment once started: (a) Methylphenidate (Ritalin) and dexamphetamine have been shown to be markedly effective in improving behaviour, but do have side-effects. (b) Tricylics may be used in the treatment of behavioural symptoms. (c) A number of other drugs are used, e.g. clonidine, venlafaxine, buproprion but there is little research evidence to support their use. * Conduct disorders/delinquency should be referred. There may be benefit from drug therapy and family and parent interventions.5 * Assess the mental health of the parents. They may benefit from drug therapy or training programmes.6 * Recommend that the parents contact national or local organizations (see box below). * Explain to the parents that treatment may or may not involve drugs, but includes: (a) an understanding of the condition, particularly that it is innate and not their child being naughty; (b) behavioural modification, e.g. where the child is positively rewarded for good behaviour;

CHILD PROTECTION

(c) alteration of the environment, e.g. to reduce distractions. Parent support groups: Hyperactive Children's Support Group, 71 Whyke Lane, Chichester, West Sussex PO19 2LD, tel. 01243 725182. The AD/HD Family Support Groups UK, 1a High Street, Ditton Marsh, Westbury, Wiltshire BA13 4DL, tel. 01373 826045. ADDnet UK: http://www.web-tv.co.uk

Dyspraxia (developmental coordination disorder)

Between 6 and 10% of children in the UK are affected. Children have extreme difficulty in learning motor skills and there may be other difficulties with language, perception and thought. In preschool children there may be: (a) a history of lateness in reaching milestones, e.g. sitting, walking, etc.; (b) a difficulty in running, hopping or jumping; (c) a need to be taught how to do things which other children learn instinctively; (d) difficulty in dressing; (e) slowness and hesitancy in actions; (f) difficulty in gripping a pencil; (g) difficulty in sorting shapes, jigsaws, etc.; (h) difficulty in catching or kicking a ball. The school-age child may: (a) have all the problems above; (b) avoid activities that involve coordination; (c) be unable to remember or follow instructions; (d) have other difficulties, e.g. in reading and writing or a reduced attention span. * Refer for assessment by a local specialist. * Advise the parents to contact the Dyspraxia Foundation for further advice on management and assessment. Patient support: Dyspraxia Foundation, 8 West Alley, Hitchin, Herts SG5 1EG, tel. 01462 454986; http://www.dyspraxiafoundation.org.uk

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CHILD PROTECTION The Children Act Report 2000. Available: http://www.doh.gov.uk/scg/childrenactreport2000.pdf Working together to safeguard children. London: HMSO, 1999. Available: http://www.doh.gov.uk/pub/docs/doh/safeguard.pdf Child abuse and neglect: a clinician's handbook. Edinburgh: Hobbs CJ, Hanks HGI, Wynne JM. Churchill Livingstone, 2000. Munchausen syndrome by proxy abuse: a practical approach. London: Eminson M, Postlethwaite RJ. (Eds), Arnold, 2000.

PHYSICAL ABUSE (NON-ACCIDENTAL INJURY) Physical abuse should be suspected when the: (a) (b) (c) (d)

explanation is inconsistent with the injuries; parents delay seeking help for an injury; parents handle the child awkwardly; child appears wary ('frozen watchfulness').

The following are common injuries that should arouse suspicion: (a) long bone or rib fracture in young children; head or eye injuries; (b) burns or scalds that may have arisen from being held against a hot object or in scalding water; (c) cigarette burns or strap injuries; (d) a torn frenulum (from ramming a bottle into the baby's mouth); (e) bruises - especially finger and thumb grip marks. CHILD SEXUAL ABUSE (CSA) This includes exposure to indecent acts, pornography, masturbation of an adult, being used for intracrural intercourse, or being penetrated orally, vaginally or anally. Children may present with: (a) symptoms of local trauma or infection, perineal soreness, vaginal damage, anal pain or bleeding;

62

CHILDHOOD PROBLEMS

(b) emotional disturbance - loss of concentration, enuresis, encopresis, anorexia or sometimes parasuicide; (c) sexualized behaviour or inappropriate sexual knowledge for their age.

into a range where symptoms may be exaggerated or even invented or procured as in classical MSBP abuse. In this situation children may be abused by lengthy or invasive investigations as well as by the actions of the carer. It may be evident because of:

NEGLECT AND EMOTIONAL MALTREATMENT

(a) claims of illness and 'doctor shopping' when the child is well; (b) enforced invalidism where an ill or disabled child is brought up with a greater degree of mental or physical incapacity than is actually present.

Neglect is a common form of maltreatment in the UK. It is insidious and adversely affects children in many ways, including retardation of growth and development. Poor general health is often a feature. All forms of abuse involve emotional maltreatment. Emotional abuse is complex and its longterm effects on children result in impaired intellectual functioning, disturbed attachments and consequent lack of social and emotional competence. The symptoms of neglect and emotional abuse vary with the age of the child: (a) Infants: failure to thrive, recurrent and persistent minor infections, frequent attendance at casualty or hospital, severe nappy rash, generally delayed development, anxiety and a lack of social responsiveness. (b) Preschool children: short stature, small head, unkempt or dirty, delayed language development, a poor attention span, overactivity, aggressive and impulsive behaviour, indiscriminate friendliness and desire for physical contact from strangers. (c) School children: short stature, poor hygiene and unkempt appearance, learning difficulties, lack of self-esteem, poor coping skills, social and emotional immaturity, few relationships, selfstimulating or injurious behaviour, encopresis or inappropriate voiding of urine.

MUNCHAUSEN'S SYNDROME BY PROXY (MSBP) ABUSE This may also be called factitious illness by proxy but is better known as MSBP abuse. There is a spectrum of parental behaviour in seeking advice about their children's health ranging from neglect of symptoms through a normal response and

MANAGEMENT OF SUSPECTED CHILD ABUSE (a) A full history should be taken from the informant and recorded. (b) The parent's explanation for any injuries should be noted. (c) The child should be fully examined, except in suspected CSA, and the examination fully documented. In suspected CSA, detailed examination should be carried out only after an 'assessment interview' by a multidisciplinary team, and the physical examination should be by a doctor with experience in child protection.

If abuse is likely The urgency of action will be determined by: (a) the severity of injuries and need for immediate medical attention; the type of abuse - physical abuse needs more urgent action than sexual or emotional abuse, which need a planned response; the age of the child - a younger child will be at greater risk; (b) the child's safety, e.g. if the abuser is still living in the house the risk to the child is greater. * Contact the Social Services office when there is suspicion of abuse, after treatment of the acute injuries. * Discuss immediate plans with the parents. Detailed discussion is usually unwise, and it may be best to say no more than that there is a need to seek further advice. * Arrange for a second examination by another doctor: for CSA, by an approved doctor; for

CHILD PROTECTION 63

physical abuse, by the consultant or registrar on call; for failure to thrive, by outpatient assessment by a consultant paediatrician; for emotional abuse by outpatient assessment by a paediatric psychiatrist. In the latter two categories especially, obtain reports from the health visitor and/or school nurse and consider discussing the child with the Consultant Community Paediatrician. * Consider the need to remove the child to a safe place, if the child is not admitted to hospital. In practice, this is usually arranged by a social worker or by the NSPCC. There is now provision for the child to be examined in the home, without removal from it (see below). * Attend the case conference or submit a written report and/or speak directly to the chairperson. * A key worker will be appointed if the case conference decides to place the child's name on the Child Protection Register. Keep the key worker informed about any further worries. * Refer to local guidelines on Child Protection procedures and the DoH publication, Working together to safeguard children (HMSO 1999; see above).

Where access to the child is refused * Contact the Social Services Department (SSD) or the NSPCC, who can apply to the court for a Child Assessment Order. The order will only be granted if the circumstances meet the same criteria as are needed for an Emergency Protection Order. The order lasts for a maximum of 7 days, and the court will direct the nature and type of assessment to be carried out and whether the child should be kept away from home for the purposes of the assessment. * Police protection. If the situation is more urgent, the police have the right of entry, and can remove a child for a maximum of 72 hours.

'Place of safety' The court may issue an Emergency Protection Order where there is reasonable cause to believe that the child is likely to suffer significant harm if: (a) the child is not removed to another place; or

(b) the child's removal from where he or she is accommodated is not prevented. Application is usually from the SSD or NSPCC, but may be made by anyone. The Order is limited to 8 days, and a single extension of 7 days may be granted. The parents can appeal if they were not present initially, but not within the first 72 hours. The court will direct the nature and type of assessment.

POSSIBLE OUTCOMES Child protection register This confers no rights and has no legal standing, but signifies the level of concern and the need for monitoring.

Supervision order This will only be granted if the requirements for Care Orders are met. The court can make a Supervision rather than a Care Order. A Supervision Order can apply for a maximum of 3 years, but is usually reviewed regularly. It does not give the local authority parental responsibility, which is given to a person appointed by the court and supervised by the person taking out the order. Certain conditions may be applied, e.g. regular weighing, regular attendance by the social worker, etc.

Care Order The court may issue a Care Order if: (a) the child is suffering or is likely to suffer significant harm; and (b) the harm or likelihood of harm is attributable either to the care given or likely to be given to the child if the order were not made or to the child being beyond parental control. A Care Order commits a child to the care of the local SSD, usually until the age of 17. Parental responsibility is shared with the SSD, who have the power to decide how much the parents may exercise their parental responsibility. The SSD must allow reasonable contact and try to promote

64 CHILDHOOD PROBLEMS

such contact. The parents or the local authority can return to the court and apply for a revocation' of the Care Order. In some cases, the child may be allowed home on trial by the local authority. While a court is considering its decision, it may make an Interim Supervision or Care Order.

Medical evidence

(c) to express their feelings and talk about what troubles them; (d) to be kept clear of conflict between the parents; (e) continuity. Information for parents and children: a number of useful booklets are available from Relate on www.relate.org.uk

Under the Children Act 1989, a GP may be asked to submit a written report. The DoH gives the following guidelines for reports: (a) confine the report to the facts; (b) present objective and measurable evidence of the child's health and development; (c) where subjective views are given, they should reflect balanced professional judgement; (d) comment and advice should be in the context of the court's considerations about whether to make an order; and (e) the more comprehensive and comprehensible the report, the less likely it is that the GP will be called to give verbal evidence in person.

DIVORCE/MARITAL STRIFE: THE EFFECTS ON THE CHILDREN Review: Hartnup T. Divorce and marital strife and their effects on children. Arch Dis Child 1996; 75:1-3.

• One in three marriages in the UK ends in divorce, with the result that one in 5 to 6 children is affected. One-third of children from divorced parents suffer long-term psychological effects similar to those resulting from traumatic experiences. Children are likely to be worse 1 year after the divorce than at the time. * Advise parents, if the opportunity arises, that children need: (a) clear information in language that is appropriate to their age; (b) to maintain the best possible relationships with both their parents;

INFANCY PRETERM INFANTS There is an increasing tendency for these infants to be discharged early, often on reaching 1.8kg. They may still be tube fed and/or on oxygen.

Feeding * Check that the baby is being given vitamins and iron (see p. 53). * Continue vitamins until 1-2 years old depending on adequacy of diet (i.e. 2 in the fussy toddler). * Continue iron until mixed feeding is achieved, usually around 6 months - again longer if diet is inadequate. * Preterm infants with chronic lung disease may need calorie-supplemented milk (e.g. by adding Duocal or using ready-made high-energy infant formula).

Weight and development Preterm infants will, by the age of 2 years, reach a stage of development and a weight that is appropriate for their date of birth, regardless of their gestational age. Infants who have undergone prolonged ventilation are usually significantly behind in gross motor development, but should eventually catch up.

Immunization Give routine immunizations at the usual times (8, 12 and 16 weeks from birth). Neonatal

INFANCY

convulsions without progressive neurological disorder are not a contraindication to pertussis immunization (but see p. 28). The support of the neonatologist may reassure the parents.

65

will starve. A further reduction of milk may be necessary if the initial reduction is unsuccessful.

The child who won't take lumpy foods

Respiratory infection Preterm infants with chronic lung disease are at high risk of infection, both viral and bacterial, especially with Moxarella. Antibiotics should be given more freely, e.g. co-amoxiclav.

* Advise the parents, using the rules above, and encourage a combination of homogenized and finger foods with the steady introduction of more solid foods.

VOMITING IN INFANCY FEEDING PROBLEMS Advice when the child is being weaned can avoid subsequent feeding problems. The following 'rules' may help: (a) Mealtimes should be organized and unhurried. If necessary, the child should be fed before the family eats; (b) A variety of foods should be offered, of an interest to the child. Finger foods of fun sizes and shapes may help encourage children to take an interest in feeding themselves. However, there is little value in striving too hard to change a child who is resistant and growing well on a bland but nutritious diet; (c) The child should not be forced to eat, or hovered over while eating. However, a parent should remain by or within the sight of the child; (d) Food rejected should be put aside and brought back if requested. A new meal should not be prepared, nor milk offered as a calorie substitute; (e) Anger at rejection or bad behaviour should be avoided. It is likely to lead to a battle, which the child will always win. The child who is unwilling to take solid food The child is usually well nourished, but taking large quantities of milk. * Advise parents to reduce the milk intake by about a half and to introduce a variety of foods. The above rules apply, and it is important that the parents do not weaken because they feel the child

Vomiting is common in infancy, and does not usually indicate severe illness. * Refer any child with: (a) bile-stained vomitus; (b) forceful vomiting; (c) persistent vomiting, especially when it continues through to the next feed; (d) weightless; (e) abdominal distension; (f) with localized tenderness; and any child who is unwell. Note: Congenital pyloric stenosis is most common at the age of 4-6 weeks, and is unlikely to develop after that. Possible causes of vomiting are: (a) overfeeding; (b) possetting; the infant brings up small amounts on winding; (c) gastro-oesophageal reflux; the infant brings up large amounts on winding and after lying down, sometimes with a small amount of altered blood. Consider thickening the feeds and giving a raft antacid. If there is no improvement, especially if there is poor weight gain, refer for consideration of an H2 antagonist or proton pump inhibitor. Troublesome severe reflux may require a prokinetic agent (e.g. domperidone) or fundoplication; (d) upper respiratory infection; the vomitus contains small amounts of mucus; (e) viral enteritis; (f) occasionally, cows milk protein intolerance (see Changing milks below).

66 CHILDHOOD PROBLEMS

EXCESSIVE CRYING ('COLIC')

Behavioural modification

Colic is correlated more closely with socioeconomic factors than with physical ones.7 The classic picture starts when the infant is about 2 weeks of age, and lasts until about 4 months. The infant cries for long periods, is apparently in distress, and may draw the legs up, go pale and pass flatus. In some, there is a marked diurnal variation, 'evening colic', in which the infant is usually well in the morning but as the day progresses the crying becomes worse until, by the evening, the infant appears inconsolable.

Explain that sometimes the cycle of crying sets up a vicious circle of unintentional overstimulation, leading to a tired, distraught infant. Rhythmic rocking and a more structured sleeping and feeding routine may help, as may admission to hospital. Conversely, some infants respond to an increase in care or increased carrying.10

* Establish the parents' perceptions of the problem, and assess whether there are other factors affecting them (e.g. overcrowding, marital strife, etc.). * Exclude important causes, e.g. infantile spasms, recurrent volvulus, intussusception or acute infection (especially otitis media and urinary tract infection). * Reassure the parents that the problem will only last a short time, and that it is not caused by poor feeding, allergy, or poor handling. While there is no easy cure, behaviour modification may help (see below). * Encourage the parents to get as much time off and rest as is possible. This will mean taking turns in looking after the infant and enlisting the help of friends and relatives. * Contact the health visitor, who is very important in supporting and monitoring the situation and for advising on day-care facilities for other siblings. * Follow-up is essential both by the GP and health visitor. * Consider a trial of oral sucrose (2ml of 12%) as a stat dose when needed.8'9 * Sedation should be used only as a last resort. It is essential that the dose is adequate, and that the sedative is only used in conjunction with a change in management and for short periods. * Admit to hospital if the situation has progressed so far that the parents are distraught and desperately need reassurance and/or the child is felt to be at risk.

Changing milks

Changing milks should be avoided on the whole, as only a very small number of children are truly intolerant of cows milk. Problems with tolerating cows milk comprise: (a) Lactose intolerance: usually temporary, causing persistent diarrhoea following gastroenteritis. Watery stools positive for lactose on chromatography. Change to a soya formula and reintroduce cows milk formula after 6 weeks. (b) Cows milk protein intolerance: excessive crying, usually with vomiting and diarrhoea ± blood; occasionally constipation. Short trial of soya formula or hydrolysed formula (e.g. Nutramigen) results in dramatic improvement: no other convenient diagnostic test. May be able to reintroduce cows milk formula after 3-6 months. (c) True milk allergy: usually manifests as an urticarial rash ± anaphylaxis in an atopic child and family. Refer for assessment (skin testing or radioallergosorbent testing (RAST) may be needed if there is doubt) and dietician advice. Hydrolysed formula is recommended as soya allergy often coexists. Wean onto a milk-free diet then review at 1 year. The condition gradually improves over preschool years but other food allergies may coexist. WATERING/DISCHARGE FROM THE INFANT EYE Overview: Young DH, MacEwan CJ. Managing congenital lacrimal obstruction in general practice. BMJ1997; 315: 293-6.

INFANCY

* Reassure the parents that 20% of infants develop the symptoms of lacrimal obstruction, and that 99% of these resolve by 1 year. * Examine to exclude other causes, especially infection. Consider taking a swab for pathogens including chlamydia if under 1 month old. * Advise parents that; (a) topical antibiotics should be used only if there is clear evidence of infection; (b) lid cleaning of debris may decrease the risk of secondary infection; and (c) massage of the lacrimal sac is often recommended, but its value has not been assessed. * Refer at the age of 1 year if symptoms are still present, but explain that surgical intervention may not be necessary and the surgeon may delay operating until 2 years of age.

NAPPY RASH The following are important in preventing napkin dermatitis: (a) frequent nappy changes (5-6 times per day, i.e. when urine is voided); (b) periods without nappies; (c) general skin care.

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(b) boil towelling nappies vigorously to kill ammonia-producing bacteria; and (c) use a barrier cream, e.g. white soft paraffin or zinc ointment, at each nappy change. * Candida dermatitis. Distinctive sign: redness extends deep into the creases, with spotty satellite lesions. Prescribe a topical antifungal agent at each nappy change. Resistant cases: check for oral Candida and treat; consider an antifungal combined with steroid.

CONSTIPATION Infants Infants are very different to older people in the number and volume of stools that they pass. Only consider that the child has constipation if the stool is especially hard or is associated with pain or bleeding. * Exclude an anal fissure. * Consider adding juice from strained fruit to feeds. If necessary, give 2.5 ml of lactulose daily to a newborn and up to 10 ml daily to a 1 year old. Delay in passing the first meconium stool and subsequent constipation raises the possibility of Hirschsprung's disease.

General skin care

(a) Hygiene: at each change, the nappy area should be washed with water and gently dried. (b) Local emollients, e.g. aqueous cream, should be applied at the earliest sign of irritation. (c) Bath oil should be used if the skin is dry or easily irritated.

At the time of weaning Short-term but distressing constipation can occur on the introduction of a varied diet. Strained fruit should be added to the diet. High-fibre diets should not be encouraged, as they can lead to iron and calorie deficiencies.

Management

* Decide whether this is ammoniacal dermatitis or is due to Candida. In both conditions, encourage the parents to allow the infant to be exposed for as long as possible each day. * Ammoniacal dermatitis. Distinctive sign: redness does not extend into the creases. Advise the parents to: (a) change the nappy even more frequently, e.g. every 2 hours if wet;

DIARRHOEA WITH OR WITHOUT VOMITING Review: Childhood gastroenteritis. MeReC Bulletin 1992; 3(7): 25-27.

* Exclude: (a) surgical emergencies; (b) pneumonia;

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CHILDHOOD PROBLEMS

(c) meningitis; (d) otitis media in children under the age of 5; (e) urinary tract infection (UTI). * Refer: (a) if the child is dehydrated; (b) the child is ill out of proportion to the diarrhoea and vomiting. * If satisfied that the illness is consistent with a diagnosis of viral gastroenteritis: (a) treat with hydration; (b) do not use antiemetics, antidiarrhoeal agents or antibiotics (except as below). Antibiotics * Giardia: give metronidazole for 3 days. * Campylobacter: erythromycin may help in campylobacter infection if there is systemic upset or blood is still present in the stools. * Salmonella or shigella infection: children with systemic illness need admission for what is a septicaemia.

strength milk, or 'regrade' (see below). Opinion is divided on the need for regrading in order to avoid lactose intolerance in this age group; for an infant over 6 months, reintroduce full strength milk. Regrading: Give % then 14 then % then full strength milk. Complete the sequence over 24-36 hours.

* Breast-fed children: Breast feeding should continue, with ORS used as supplementary fluid replacement. * Weaned children may continue to eat if they have an appetite. Small, frequent meals of bland foods (e.g. boiled rice) are appropriate. Recurrence of diarrhoea If diarrhoea recurs after reintroducing milk, regrade as above.

Notification Maintenance of hydration

Send a stool sample for culture if:

* Oral rehydration salts (ORS) are used because sodium and water are actively absorbed from the bowel. This absorption is increased considerably if an energy source is presented to the bowel wall. * Preparations marketed in the UK are for mild to moderate diarrhoea, and have a lower sodium concentration (35-60 mmol/L) and higher glucose concentration (up to 200 mmol/L) than WHOrecommended ORS. They can be flavoured with dilute fruit squashes.

(a) the stool contains blood; (b) the diarrhoea is prolonged; or (c) more than one member of the family is affected;

* Bottle-fed children: Tell the parents to: 1. Use the solution as the only oral fluid, with no solids. Plan to give at least as much as the child would normally drink in a day, and give small amounts often (even 5ml every 10 minutes if the parents say that the child vomits everything consumed). 2. Continue the ORS until the diarrhoea is settling. 3. Then feed the child as follows: for an infant under 6 months, either reintroduce full

* Notify the environmental health officer as 'food poisoning' if one of the following organisms is isolated: Shigella, Salmonella, Giardia, Campylobacter, Cryptosporidium and E. coli 157. * Notify the environmental health officer as 'suspected food poisoning', without waiting for the culture result, if two or more members of a family are affected at the same time. Prolonged diarrhoea (more than 2 weeks) * Refer any child with prolonged diarrhoea who is failing to thrive. * Otherwise: (a) Repeat a stool culture (send a fresh specimen and request culture and microscopy). (b) Check fresh stool for reducing sugars. Use Clinitest tablets (add ten drops of water and five drops of liquid stool to the Clinitest tablet.)

INFANCY

If the stool contains more than 0.5% reducing sugars, prescribe a lactose-free formula feed (e.g. Pregestimil) for 3-6 weeks. Explain to the parents that this intolerance of lactose is likely to be temporary. No other food containing milk should be given. The advice of a dietician is recommended. Refer to hospital outpatients if symptoms have not resolved after 6 weeks. (c) Check urine for organisms (especially in young children). (d) If stool cultures are negative and the above tests are normal, try to reintroduce food and only return to a liquid diet if the diarrhoea worsens.

Cryptosporidium This is contracted from animals by milk or water, but can be transferred within families by the faeco-oral route. It can last up to 90 days and does not respond to antibacterial therapy.

Toddler's diarrhoea Many young children (commonly boys) suffer from prolonged watery diarrhoea with undigested food in the stool that starts, or is first noticed, after an attack of apparently infectious diarrhoea. Peas, carrots, sweetcorn and baked beans are especially likely to appear. The child remains well. Once cultures and tests for acid and sugars are negative, the likely diagnosis is 'toddler's diarrhoea'. This is due to a rapid transit time. * Reassure the parents that: (a) the condition is benign and that the child will continue to flourish, despite diarrhoea; (b) the condition usually improves by the age of 5 years. * Review the child regularly and monitor the growth. Refer if there is any parental anxiety or if the child is failing to thrive. * Loperamide 2.5-5 ml t.d.s. should be considered if the diarrhoea is difficult to manage, e.g. on starting nursery school.

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CONVULSIONS

Convulsions associated with a fever Review: Offringa M, Moyer VA. Evidence based management of seizures associated with fever. BMJ 2001; 323:1111-14.

* Look for signs of meningitis and encephalitis. The probability of bacterial meningitis is low (range 0—4%) and a normal physical examination and history make bacterial meningitis highly improbable. * Admit: (a) any child thought to have suffered a febrile convulsion under the age of 18 months, even if he or she appears fully recovered; (b) any child who is unwell or who has suffered a 'complicated' febrile convulsion, e.g. one lasting 15 minutes, focal or with postictal paralysis; (c) any child who suffers from more than one fit in the same febrile illness. * If the child is over 18 months of age and appears to have recovered fully, instruct the parents to telephone you should the child become unwell again later.11 * Information for parents:12 (a) Febrile convulsions are common between the ages of 6 months and 5 years, affecting 4% of the childhood population.13 (b) About 30% of sufferers will have another convulsion, but only 1% go on to develop epilepsy. (c) The majority of convulsions, although frightening, last less than 5 minutes. (d) Further febrile illness: Start paracetamol immediately. The child should be kept cool or sponged down with tepid water if the fever is high.14 If a fit occurs, advise the parents to: - turn the child into the prone position do nothing more; - call for a doctor or an ambulance if it lasts more than 5 minutes; - call for a doctor if recovery is not complete, or if there is any other cause of anxiety; - inform their doctor even when recovery appears complete so that he or she can make a decision about the need for a visit.

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CHILDHOOD PROBLEMS

Recurrent febrile convulsions

* Prescribe rectal diazepam if a child has had recurrent or a single prolonged, febrile convulsions. Show the parents how to use it at the start of a seizure. * Discuss with the parents the question of referral for consideration of prophylaxis with continuous oral anticonvulsants. The risk of recurrence is 20-30%; but four children would have to be treated with valproate, or eight with phenobarbitone, to prevent one seizure. Adverse effects from both drugs make the benefit doubtful.15

Convulsions not associated with a fever * Admit any child under the age of 6 months with obvious fits, absences or abnormal behaviour possibly caused by a seizure. * Admit a child of any age with a history of a seizure and evidence of raised intracranial pressure or other worrying signs. * Refer to outpatients: (a) any child with suspected absence seizures (which can be confirmed in the surgery by encouraging the child to hyperventilate, thereby inducing absences); (b) a child over the age of 6 months with a history of a non-febrile convulsion who, when seen, is well (check the blood pressure and examine the fundi). Note: If a child has had three or more convulsions within a few weeks, consider treating with carbamazepine while awaiting the urgent appointment.

Status epilepticus

syringe - without the needle. In a child over the age of 1 in whom you can get intravenous access, lorazepam is now the preferred intravenous drug. 3. Arrange for immediate admission. BLADDER AND BOWEL PROBLEMS Toilet training * 10% of 5-year-olds still wet the bed (boys more than girls); by the age of 10, about 4% of children still regularly wet the bed (boys much more than girls). If one parent was not dry at night until 6 years old, then children have a 40% chance of not being so either. * Advise parents that: (a) 2 years is the most sensible age to start training for both bladder and bowels; (b) before starting, the child should learn to sit on the toilet or potty and know the difference between being wet and dry. * Bladder training should follow automatically if the child can recognize the difference between wet and dry. In those who appear not to do so, trainer pants will help. Imitating parents is an effective way of encouraging children. It is important not to force the child, who may then use toiletting as a powerful weapon. Children who void appropriately should be rewarded with praise or cuddles. * Bowel training. Advise the parents to sit the child on the potty during the day after meals. This should be fun for the child, who is the focus of attention. If stool is passed in the potty, the child should be rewarded, but if passed in a nappy after the child has given up, this should be ignored or turned into an encouragement to do a 'poo for mummy or daddy next time'.

If the child is still fitting when the doctor arrives: * Rapid and decisive management is important: prolonged seizures (more than 30 minutes) in a child predispose to long-term epilepsy. 1. Lie the patient prone and ensure that the airway is clear. 2. Give diazepam rectally, 0.2-0.3mg/kg, i.e. 1 mg per year of age. Use rectal tubes 5mg and 10 mg, or use an iv preparation in a

Patient information: Green C. New Toddler Taming: A Parents' Guide to the First Four Years. London: Random House, 2001.

Bed wetting * Enquire: (a) at what age the parents were dry;

INFANCY

(b) whether the child is now wetting having been previously dry; (c) whether the child wets during the day; (d) about upsets in the family and stresses at school; (e) whether there are any 'payoffs' when wetting occurs, e.g. cuddles, etc. * Exclude infection, especially in cases of secondary enuresis. * Discuss the problem with the health visitor. * Refer to outpatients any child over the age of 4 years with daytime symptoms. Patient organization: The Enuresis Resource and Information Centre (ERIC), 34 Old School House, Britannia Road, Bristol BS15 2DB, tel. 0117 960 3060; www. enuresis.org.uk

Younger children (aged 5-7) At this age, the problem is likely to be delayed maturation. It seems reasonable for the parents not to struggle to get the child out of nappies at night if a behavioural approach fails. Fluid restriction and lifting may help to reduce the problem without curing it. * Advise the parent to praise the child for any dry beds. Star charts are helpful at this age, although not under the age of 5 years. * Prescribe desmopressin acetate spray 20 (xg or tablets 0.2 mg for nights away from home, holidays, etc. but not as continuous treatment.

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(b) to ignore wet beds; (c) that 'lifting' the child when the parents go to bed may save a wet bed. * If not successful, try a bedwetting alarm (available from the local authority enuresis clinic). Note: Some older children may be able to wake themselves and go to the toilet using an alarm clock set to a time just before they usually wet. * If the use of a buzzer alarm fails, then prescribe desmopressin acetate spray 20-40 |xg or tablets 0.2-0.4 mg a night for 3 months. Assess after a week without treatment. If the response is not maintained, consider a further 3-month course. It probably does not affect the ultimate outcome. If not responding, refer to an enuresis clinic. Note: The CSM warns that, in order to avoid hyponatraemia, patients on desmopressin should: (a) avoid excessive drinking especially at night; (b) omit desmopressin if there is vomiting or diarrhoea; (c) keep to the recommended starting doses; (d) avoid other drugs which increase vasopressin secretion, e.g. tricyclic antidepressants. There may be a place for a tricyclic antidepressant, e.g. imipramine 25-50 mg at night. It may work by lightening sleep. Improvement usually takes 3-4 weeks, and treatment should be continued for at least 4 months. This is only likely to reduce the bedwetting by one day a week.18 The controversy relates to the danger of the drug in overdose.

Older children (aged 7-15) Desmopressin and tricyclic antidepressants seem to be equally effective. Alarms are more trouble to set up but may be associated with longer lasting improvement.16'17 * Advise the parents: (a) to establish a means of positive encouragement or reinforcement whenever a child has a dry bed, with an increased reward if the child has three consecutive dry beds (usually this is done using a star chart or an equivalent sticker chart);

Adolescents 16 years and over * Refer for urological assessment.

Constipation and soiling Soiling is abnormal after the age of 4 years. The majority of soilers are constipated, with a loaded rectum. * Distinguish, from the history, between encopresis (normal stool passed in the wrong place) and soiling (leaking liquid stool into pants).

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CHILDHOOD PROBLEMS

* Enquire as to: (a) whether the child has ever been continent; (b) the frequency and nature of the episodes; (c) the attitude of the parents and child. * Examine: (a) the abdomen for faecal masses; (b) the anus for fissure; and (c) the rectum for faecal masses. Empty rectum

* Intentional defecation to offend the parent (encopresis) - refer to a child psychiatrist. * Poor training - should respond to a star or 'poo' chart, provided the emotional rewards are sufficient. * Stress - this may be factor in regression, and should respond to a star chart. Full rectum

The most common cause is a full, distended rectum with loss of the urge to defecate. Liquid faeces seep around the obstruction, which also comes away piecemeal. * Explain the problem to the parents and child. Normal rectal sensation will only return once the rectum has been empty for a time. * Empty the rectum using a stimulant laxative, e.g. senna as a syrup (2-6 years, 2.5-5 ml; 7-12 years, 5-10 ml, at night), increasing the dose by 5ml each week until a significant result is achieved. At the same time prescribe a stool softener (e.g. lactulose 2-6 years 10ml at night, 7-12 years 20 ml at night) and increase as needed until a comfortably soft stool is passed. Docusate is a useful alternative as combined stimulant and softener. If severely constipated and still loaded after a month on the above routine, refer to hospital. * Once the rectum has been cleared, a maintenance dose of both stimulant and softener laxative should be given and the diet of child and parents discussed. * Retraining is necessary: the child should be encouraged to use the toilet after every meal, and rewarded with heavy praise when he or she

defecates in the appropriate place. Rewards for clean pants may encourage retention to continue; a star or 'poo' chart should be started, which also acts as a reward. * Review the child and charts regularly. Reduce the dose of laxative when continence has been maintained for 3 months. Reassure both child and parents that accidents are inevitable because it takes a long time for the bowel to regain activity. * If the above routine fails, refer to a paediatrician.

SLEEP PROBLEMS Parent information: Green C. New toddler taming: a parents'guide to the first four years. London: Random House, 2001. Review: Ramchandani R Wiggs L, Webb V, Stores G. A systematic review of treatments for settling problems and night waking in young children. BMJ 2000; 320: 209-13.

* Check that the development of a sleeping difficulty in a child with a previously good sleep pattern is not due to an underlying emotional problem. If it is, assessment and referral should focus on the emotional problem rather than on the behavioural approach outlined below.

Assessment of the problem Difficulty in getting a child to sleep, or recurrent waking, are common problems. They require a lot of time and understanding from the GP, and an alteration in the way the parents manage the situation. * Try to see both parents together. Ask whether there are other factors that are making the situation worse, e.g. living in a bedsit or with grandparents. * Explain to the parents that for a plan to succeed: (a) both parents must agree on the approach; (b) that approach must be followed consistently; (c) for the parents' sake, the workload should be shared as far as possible;

INFANCY

(d) neighbours should be told. * Sleep diary. Ask the parents to fill in a diary for a few days, noting the following: (a) the time the child wakes in the morning; (b) the time and lengths of naps during the day; (c) the time the child went to bed in the evening; (d) the time the child settled in bed; (e) the times and duration of waking during the evening, the mood of the child, and the action taken by the parents; (f) the times and lengths of waking at night, the mood of the child, and the action taken by the parents on each occasion.

Difficulty in getting a child to settle Children who won't go to sleep at an appropriate time may be: (a) alert because of naps during the day; (b) tired, but overstimulated just before bedtime; (c) frightened or anxious about being left alone; (d) confused because of shifting ground rules, e.g. one parent puts the child to bed but the other parent gets him or her up again on coming home later; (e) attention seeking. Dry-eyed crying suggests that the child is attention seeking rather than distressed. * Advise the parents as follows: 1. Establish a bedtime routine (e.g. warning the child that bedtime is coming, then a ritual undressing, bath, story, then tucked up in bed). 2. Be consistent and firm in the routine and refuse to play 'after hours' or to read more stories. 3. Leave the room once the child is tucked up, saying that they will be back in 5 minutes. Keep coming back until the child is asleep. Once this works, make the intervals longer. 4. Parents should not run in if crying starts, as the child will usually settle within a short time; they should also: avoid picking the child up and hence providing further stimulation; behave normally - a normal background noise

73

encourages the child to feel secure that the parents are still there. 5. Avoid chastisement, but introduce appropriate rewards for good behaviour. * Very distressed parents. Ask them if they feel at the end of their tether or if they have felt tempted to hit the child. They may benefit from short-term admission of the child or use of hypnotics in addition to an alteration in management. Hypnotics need to be in adequate doses to avoid a paradoxical response, e.g. trimeprazine tartrate 2mg/kg. This is the dose recommended for preoperative premedication, and some children may be heavily sedated. Explanation of this to the parents may avoid subsequent difficulties when they present the prescription to the chemist. The hypnotic should not be continued for more than 7 days. If the above fails * Advise the parents on the controlled crying technique (see below).

Waking at night Children and adults normally cycle into a lighter phase of sleep every 60-90 minutes through the night. Children may make a noise and rapidly condition their parents to respond. The child may receive feeds or other attention, and this perpetuates the cycle. * Encourage the parents to keep a sleep diary (as above). * Advise the parents on the controlled crying technique, and tell them to warn neighbours before starting it. Controlled crying No child should be allowed to cry him- or herself to sleep without comfort. Instead, recommend the following: 1. Leave the child to cry for 5-10 minutes, depending on the degree of distress. 2. Go into the room, cuddle and comfort, and when the cries give way to sobs put the child to bed and say goodnight.

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CHILDHOOD PROBLEMS

3. The child will probably immediately start crying again. Leave him or her for 2-5 minutes longer than last time before going in again. 4. Once the child is comforted, withdraw again. 5. Subsequent episodes of crying should be left for additional 2-5 minute periods before responding. 6. The parent must try to rest. 7. If the crying time is about 30 minutes, sedation should be considered (see above). 8. Heap praise on the child when he or she starts to sleep for longer periods.

The child who comes into the parents' bed at night The principles are the same as previously. Advise the parents to explain to the child that he or she is going to stay in his or her own bed. 1. Put the child back the moment he or she appears. 2. On reappearance, the child is given a warning and returned. 3. At the third reappearance, the child should be returned, the door wedged partially open (but not enough for the child to get through) and the controlled crying technique started.

Other sleep problems Nightmares

Nightmares are common in children over the age of 5, and occur during rapid eye movement (REM) sleep. * Reassure the parents that comforting the child is usually all that is required, and that the phase will pass. * Refer to a child psychiatrist if nightmares: (a) have a recurring theme; (b) are frequent; (c) follow a frightening experience. Meanwhile, consider encouraging the child to express his or her feelings, e.g. through art.

Night terrors

Night terrors occur during deep non-REM sleep, often about 2 hours after falling asleep. They are common in children under the age of 5, but can occur at any age. Classically the child screams in terror, sits up or tries to walk around, appearing to hallucinate. After a short while he or she falls asleep and wakes the next day with no memory of the event. * Reassure the parents that the child will grow out of this and that it does not indicate any severe psychological disorder. * Anticipation. Many children have terrors at 'set times'. If this is so, the sleep pattern can be altered if the child is fully woken 15-30 minutes before the likely event for 1 week. * Sedation may be valuable if terrors are frequent and do not respond to the anticipatory technique. Prescribe diazepam 2mg nightly for 4 weeks, then tail it off. Benzodiazepines reduce non-REM sleep. Sleep walking

* Advise the parents to: (a) ensure that the bedroom is safe and secure; and (b) attempt to wake the child fully 15—30 minutes before the most likely time of attack, for 1 week. * Sedation should only be considered if severe problems occur during the episodes. Prescribe diazepam 5 mg at night, for 1-3 months. TEMPER TANTRUMS Patient information: Green C. New toddler taming: a parents' guide to the first four years. London: Random House, 2001.

Children soon learn what behaviour will irritate their parents. Usually such contrary behaviour is wilfulness (the children testing out their developing strength against the will of the parents), but it can be repayment for not getting their own way in other matters.

RESPIRATORY PROBLEMS

* Ask the parents: (a) what the child does during a tantrum; (b) what they do during and after the tantrum; (c) what causes the tantrums. * Assess whether inconsistent handling of the child by the parents is contributing to the problem.

Principles of management (a) Avoiding provocation. Parents need to realize that telling the child what is wanted is more likely to be successful than subsequent chastisement. Parents need to be consistent in their behaviour and emotions towards the child. (b) Withdrawal of attention: Ignoring a tantrum may not be easy but walking out of the room achieves the same result. It is important that the child is not left in a potentially dangerous situation. The child should be left for 3-4 minutes only. Time out. The child should be removed from the room to a safe but boring part of the house, or told to sit on a chair in the middle of the room and not get off it until allowed. Again the process should only last for 3^4 minutes or until the child has calmed down. If a room is used, it must never be locked, the lights must stay on and a parent should remain outside. (c) 'Punishment' by withholding an expected reward, for example: deny the child a favourite television programme; refuse the child permission to play with friends. (d) Not giving in is essential for any plan to work. (e) Rewarding good behaviour. This is best done with praise and cuddles or permission to watch previously banned programmes, etc. Warn parents about relatives who may confuse a child with undeserved rewards and threaten the management plan. * If defiance is continued, assess whether: (a) the parents are consistent; (b) the rewards and 'punishments' are appropriate; (c) withdrawal of privilege is threatened but not carried out.

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* If still failing to deal with the problem, refer to a child psychiatrist, a clinical psychologist or a family consultation centre.

'BREATH-HOLDING' ATTACKS Blue attacks These occur in children who become angry, cry and then hold the glottis closed. The child quickly becomes cyanosed and then loses consciousness. Recovery is very quick. * Reassure the parents that it is not epilepsy, nor is it serious. * Explain that flicking cold water in the child's face can sometimes abort attacks. * Advise the parents to avoid being 'held to ransom' or becoming overprotective.

White attacks (reflex anoxic seizures) A child who is surprised or hurt may suddenly fall unconscious, and become pale, limp and apnoeic. If this continues long enough the child may have an anoxic seizure. * Advise the parents to leave the child prone when this happens. * Reassure them that their child will not suffer damage, but may be more likely to faints when older. * Stress that this is not epilepsy. * Refer if convulsions are frequent or the parents need reassurance.

RESPIRATORY PROBLEMS • Children may have as many as 20 colds in the first 18 months, and are still getting six a year at age 7. There is no place for antibiotics. • The best guide to whether a small child has pneumonia is the child's general condition, not chest signs. If the child is ill enough to suggest pneumonia, then admission is better than blind antibiotics. • Discourage the parents from smoking anywhere near the child.

76 CHILDHOOD PROBLEMS

THE CATARRHAL CHILD It takes about 6 weeks for the cilia to recover after each cold. It is not surprising that for much of the year the child has nasal catarrh. * Reassure the parent that if the child is well, catarrh is not a sign of illness and does not necessarily need treatment. * Treat any allergic component (see p. 345). * Encourage nose blowing and discourage sniffing. * Decongestants. Prescribe topical nose drops (e.g. xylometazoline 0.05%), but only for 10 days at a time. Oral decongestants (e.g. pseudoephedrine) are less effective and cause hyperactivity. Advise parents not to give a dose before bedtime. * Check the ear drums and the hearing. * Refer any child with: (a) nasal discharge from soon after birth. Primary ciliary dyskinesia occurs in 1 in 20,000 in the UK; (b) unilateral discharge - to exclude a foreign body; (c) purulent discharge - again there may be a foreign body or sinus infection; (d) adenoidal obstruction. This is likely in a child with persistent mouth breathing and an apparently clear nose. Even then, natural improvement will occur by the age of 6-7. Refer only if there is sleep apnoea, otitis media with effusion (OME), or obstruction severe enough to interfere with speech.

* Admit all ill children, especially if they are too breathless to feed. * beta-agonist. In a well child (with no significant difficulty in feeding), it is worth assessing the response to a beta-agonist e.g. salbutamol 2.5 mg t.d.s orally or 200-400 |jig via face mask and spacer. Note that beta-agonists are not licensed in this age group and are only effective in a small number of children. If a beta-agonist is not effective, ipratropium 40-80 |jig by mask and spacer may be. Bronchiolitis A diagnosis of 'probable bronchiolitis' can be made if an URTI occurs with: (a) progressive dry cough in an ill infant; (b) raised respiratory rate (40+); (c) subcostal recession and hyperexpansion with prolonged expiration; (d) bilateral crackles; (e) difficulty in feeding. * Manage at home19 if the infant: (a) is able to feed; (b) is aged over 3 months; (c) has a respiratory rate <50/min; (d) has no other complicating factors. * Management consists of support and monitoring. Antibiotics are of no, and steroids of little, value, and bronchodilators usually ineffective. * Otherwise, admit. The child is likely to need oxygen and nasogastric or iv fluids. The infant who presents with apnoea should always be admitted.

COUGH If a child has had a cough for 1 month or more, arrange a CXR to exclude foreign body inhalation and low-grade pneumonia.

Children under 18 months Upper respiratory tract infection (URTI) followed by wheezing, coughing and breathlessness with signs of hyperexpansion and prolonged expiration - about 50% of infants who suffer repeated episodes of this go on to develop asthma.

Whooping cough * Admit if apnoea occurs, or if there is major distress from coughing. Do so even if the infant seems well when you arrive at the house. (Believe the parents' history of an apnoeic attack the next one could be fatal.) Admit if under 1 month old. * If there is no evidence of respiratory distress, the child can be managed at home. Consider giving the child, and any other susceptible children (especially infants) who have been in contact, a course of erythromycin (see p. 29).

RESPIRATORY PROBLEMS

* Cough does not respond to conventional cough suppressants. Recurrent 'chest infections'

* Arrange a CXR. * Check whether the child is thriving. * Consider referring to exclude cystic fibrosis.

(b) recurrent day-to-day symptoms (presumed atopic asthma). The treatment of these two patterns is different. Children with pattern (a) do not require inhaled steroids unless the episodes are very frequent (e.g. one per month). Even then they are of limited effectiveness.20

Children over 18 months of age

Presumed atopic asthma

Dry cough, worse at night with or without wheezing

Aims of treatment

Always consider that the child might have asthma. * Enquire about wheezing, recurrent chest infections and exercise-induced wheezing and coughing. * Consider a trial of: (a) a beta-agonist (b) steroids (see below). Sudden onset of coughing with no previous URTI

* Exclude an inhaled foreign body. Arrange a CXR (ensure that the film is taken on expiration). * Note that a normal CXR does not exclude a foreign body: if the history is suggestive (e.g. choking then cough in young child) refer for bronchoscopy. Chronic cough, mouth breathing, sniffing and snoring

The symptoms may be due to a postnasal drip in a child with catarrh (see p. 345).

ASTHMA IN CHILDREN UNDER 5YEARS For the management of asthma in children over 5, see p. 115 and Appendix 12A. Asthma in young children does not always follow the picture of older children and adults. There are two clinical patterns: (a) acute wheezy episodes (usually with viral infections);

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(a) Child to be as symptom-free as possible day and night. (b) For beta-agonists to be needed no more than once a day. (c) To avoid adverse effects of treatment, particularly with regard to growth and development. Diagnosis

The diagnosis is a clinical one. If in doubt, consider a trial of oral prednisolone for 3 days (at the same dose as the rescue course, see Appendix 12B) or inhaled steroid (for 6 weeks minimum), in children over the age of 12 months. The diagnosis is hard to make in children under 12 months. A personal or family history of atopy makes the diagnosis of asthma more likely.21 In time a clearer pattern will emerge. Meanwhile refer if symptoms are sufficiently troublesome. * Consider a CXR or referral if: (a) the response to simple treatment is poor; or (b) the child has a productive cough or recurrent chest infections or focal lung signs; or (c) symptoms date back to the neonatal period; or (d) there is failure to thrive. Patient/parent management

Involve both the parents and child closely in the management of the condition. Management includes: (a) understanding the condition;

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CHILDHOOD PROBLEMS

(b) monitoring symptoms, peak flow and drug usage; (c) having a prearranged plan of action should symptoms deteriorate; (d) avoidance of precipitants (see p. 116).

(b) a silent chest; (c) exhaustion; (d) agitation or drowsiness.

Stress that parents who smoke risk worsening their child's symptoms.

hospital

Drug treatment

• In the child with definite asthma, introducing inhaled steroids early, probably protects against decline in lung function.22 • However, the younger the child, the more difficult it is to diagnose asthma and the greater the potential concerns about inhaled steroids. • The efficacy of treatment depends on the age of the child and whether the child can inhale adequate amounts of drug. The delivery system must be tailored to the child. More than 50% of children receiving treatment with conventional metered dose inhalers (MDI) derive little or no benefit because of poor inhalation technique. The following broad guidelines apply: (a) 0-2 years need an MDI via a spacer, preferably with a face mask; (b) 2-5 years need an MDI via spacer; (c) 5-8 years can use a dry powder inhaler or an MDI with a spacer; (d) Over 8 years can use a dry powder inhaler or an MDI. See Appendix 12 for a step wise guide to the management of asthma in children under 5, and for the management of asthma in older children.

Acute asthma in young children • Admit anyone who: (a) is too breathless to talk; (b) is too breathless to feed; (c) has a respiratory rate of >50 breaths per minute; (d) has a pulse >140 beats per minute; (e) is using accessory muscles to breathe. Life-threatening features are: (a) cyanosis;

Those not requiring immediate admission to

1. Give an inhaled beta-agonist 10-20 puffs by MDI using a spacer, with each puff being breathed in before the next puff is given; or by nebulizer 3-4 hourly (salbutamol 2.5 mg under 1 year, 5 mg 1-5 years). 2. give 20 mg of prednisolone orally (in the soluble form). 3. If there is a poor response or a relapse within 3-4 hours, arrange admission. 4. If the response is good, continue the beta-agonist every 3-4 hours. If still requiring 3-4 hourly inhalations after 12 hours, continue oral prednisolone (dose <1 year, l-2mg/kg per day; 1-5 years, 20 mg a day). On recovery Consider the following: (a) Was there an avoidable cause? (b) Was the attack sudden, or did the patient deteriorate slowly? (c) Did the patient/relatives respond appropriately? (d) Was the patient complying with treatment? (e) Was the medical management appropriate? (f) Should the child have a course of steroids at home for acute use? (g) Does the child need regular inhaled steroids if not already on them?

Follow-up * Assess symptoms regularly. Particular questions to ask are: (a) Does the child cough or wheeze at night? (b) Does the child cough or wheeze with exercise? (c) Is exercise limited? * Assess inhalation technique occasionally, and if symptoms deteriorate.

UPPER AIRWAYS OBSTRUCTION

79

* Monitor the frequency of repeat prescriptions. * Continue the education of child and parent.

This strategy will clear new pseudomonas infection in more than 50% of children. Chronic pseudomonas infection requires long-term nebulized antibiotics.

CYSTIC FIBROSIS

Nutrition

All patients suffering from cystic fibrosis (CF) should be managed by a regional cystic fibrosis clinic in conjunction with a district paediatrician and their GP. The principles of management are that:

* Consider oral supplements to provide the necessary calorie intake. * Prescribe pancreatic enzyme supplements to improve fat and protein absorption. If the stools are frequent, foul-smelling or floating, the supplements should be increased.

(a) patients have regular physiotherapy to reduce retention of secretions; (b) aggressive antibiotic therapy is prescribed at the first sign of infection; (c) improved nutrition improves the prognosis. Patients with CF have higher than normal energy requirements and require 120-150% of the normal daily calorie intake to maintain body weight and to grow.

Infection Unlike normal children, respiratory viral infections are frequently associated with bacterial infection, from infancy onwards. * Treat any respiratory infection associated with a cough with a broad spectrum oral antibiotic covering staphylococcus and haemophilus (e.g. co-amoxiclav or cefadroxil) for 14 days. Doses of penicillins/cephalosporins need to be double the normal because of increased excretion, e.g. cefadroxil 125 mg b.d. under 1 year, 250 mg b.d. 1-5 years, 500 mg b.d. 6-12 years, 1 g b.d. 13+ years. * If cough persists, refer or arrange sputum/ cough swab culture and discuss with specialist. Note: Look for non-specific evidence of respiratory infection e.g. weight loss or fever; * Pseudomonas aeruginosa. Start an antibiotic, and discuss/refer immediately if pseudomonas is isolated for the first time. Treat aggressively: (a) If the child is well, treat with ciprofloxacin orally for 2 weeks and start nebulized colomycin/tobramycin. (b) if the child has symptoms as above, admit for intravenous antibiotics.

Diabetes * Ensure that the patient maintains a high calorie intake. Control the blood sugar with adjustment of the insulin dosage, not with dietary restriction.

Fertility Almost all men with CF are sterile, but many women remain fertile. The issue should be discussed with patients in early adolescence.23

Dornase alfa Dornase alfa helps reduce sputum viscosity by breaking down the DNA from dead neutrophils. It is given once a day (2.5mg) by jet nebulizer. It should be initiated and initially monitored by a specialist, and prescribing should only be devolved to the GP with an agreed protocol. Patient organization: Cystic Fibrosis Trust, 11 London Road, Bromley, Kent, BR1 1BY, tel. 020 8464 7211; www.cftrust.org.uk

UPPER AIRWAYS OBSTRUCTION Do not examine the throat of any child with stridor. * Admit if: (a) the child is toxic or drooling. Assume that this is haemophilus epiglottitis or bacterial tracheitis; death can occur within hours; or

80 CHILDHOOD PROBLEMS

(b) the obstruction is severe. The child will be distressed, with a very rapid pulse and intercostal recession; or (c) inhalation of a foreign body is suspected. This is characterized by the sudden onset of stridor in an afebrile child, with no preceding illness; or (d) the child has just returned from an area where diphtheria is common.

URINARY TRACT INFECTIONS (UTI) Systematic review: The management of urinary tract infection in children. Drug Ther Bull 1997; 35: 65-9.

Recurrent or continuing croup

• 3-5% of girls and 0.5-2% of boys have a UTI in childhood. Without adequate investigation, 75% of them will suffer from recurrent UTIs. • 25-55% of children with urinary tract infection have associated urinary tract abnormalities (7-10% have obstruction, 12-20% have renal scarring, 30% have vesico-ureteric reflux). • Renal scarring. Of those with renal scarring, up to 23% will develop hypertension by early adulthood and 10% will develop end-stage renal disease. • Children under 2 are at the greatest risk of renal damage if they develop infection, yet this group is least likely to be investigated with a urine culture. These children are likely to present with nonspecific symptoms such as fever, vomiting and diarrhoea, or failure to thrive without urinary symptoms. • If renal damage is present in a child aged <5, then both subsequent UTIs and vesicoureteric reflux may further damage the kidneys. An onset of scarring after the age of 5 is rare. • Antibiotic prophylaxis in children with a first urinary infection can protect them from subsequent infections and from renal damage. • Urine tests. When screening the urine of a child without urinary symptoms, a dipstick for leucocytes and nitrites is sufficient. If both are positive, their sensitivity is 88% and their false positive rate is 4%.27 If there is a stronger reason to suspect infection, laboratory microscopy and culture are needed.

* Recurrent croup without preceding infection has been linked to allergy and upper airway hyperreactivity. Consider a trial of beta-agonist ± inhaled corticosteroid. * Continuing croup: refer. Children with stridor or an odd cry without upper respiratory infection may have laryngomalacia, subglottic stenosis or a laryngeal web.

• Obtain a urine sample for culture and microscopy as follows: (a) in the very young child, use a urine collection pad,28 a urine bag, or obtain a clean catch; (b) in potty-trained children, loosely stretch cling film over the potty and take urine from the pool;

Note: If you suspect croup and the child's stridor is worsening rapidly, consider giving nebulized adrenaline 4ml of 1:1000 while awaiting the ambulance, but make this clear in admission note: relief is short-lived.

Mild/moderate croup Children with mild stridor and a barking cough who are basically well can be managed at home. * Reassure the parents and encourage them to comfort and settle the child (anxiety makes the stridor considerably worse). * Suggest sitting with the child in a warm steamy room for 15 minutes at a time. If using a boiling kettle, the hot steam must not be pointed straight at the child. * Give a single dose of nebulized budesonide 2mg,24 or oral dexamethasone 0.15mg/kg,25 or prednisolone 1 mg/kg.26 * Do not give antibiotics. The aetiology is viral. * Advise the parents to contact you if there is any deterioration. Explain that the barking cough is likely to be worse at night for about 3 nights.

THE CHILD WHO APPEARS SMALL OR TALL

(c) in the older child, by a mid-stream specimen. * Treat with antibiotics for 5 days. Trimethoprim or a cephalosporin are likely to be appropriate; amoxycillin is not.

Confirmed UTI The growth of a single pathogen at 105/ml is significant, but a pure growth at 104 or fewer may also be. Pyuria strengthens the diagnosis, but is not necessary for it. * Treat as above. * Encourage complete emptying of the bladder and avoidance of constipation. * Refer for imaging unless previously investigated. Refer infants urgently. Include the details of the urine culture. * Start prophylaxis (see below). * Organize a follow-up urine specimen 1 month after the infection. There is no agreement on the value of this or of further routine urine cultures. * Exclude urinary infection in future episodes of unexplained fever, abdominal pain, etc., without stopping prophylactic antibiotics if the child is taking them.

Antibiotic prophylaxis * Start prophylaxis as soon as the treatment course is completed, and continue it at least until the results of imaging are known. Most specialists stop antibiotics if the results are normal, but continue prophylaxis until some time between the ages of 2 and 7 if an abnormality is found. * Give one dose at night: trimethoprim 2 mg/kg or nitrofurantoin 1 mg/kg.

VULVOVAGINITIS Vulvovaginitis in prepubescent girls is usually due to non-specific bacterial infection associated with poor hygiene or with threadworms. If specific bacteria are isolated they are likely to be beta-haemolytic streptococci, not Candida and not

81

sexually transmitted organisms.29 Investigation for possible sexual abuse or foreign body is not justified unless there are other pointers in those directions. * Treat according to severity with: (a) daily baths; (b) local antibiotic cream; or (c) oral penicillin.

THE CHILD WHO APPEARS SMALL OR TALL When there is anxiety about a child's growth, establish: (a) the height and weight of the child, and plot them on the new nine-centile chart; and (b) the expected final adult height of the child (see below). Expected adult height based on the height of the parents The expected adult height is calculated as follows: • for a boy - the mean of the parents' heights plus 7 cm; • for a girl - the mean of the parents' heights minus 7 cm. • Plot the value on the relevant growth chart as age 19 (i.e. adult). Measurement of height Correct measurement of height is essential. (a) The child should not wear shoes and the heels should be against the wall with an assistant or parent holding the feet down gently; (b) With the child standing straight, the thighs and pelvis should be held gently to the wall; (c) The jaw and external auditory meatus should be in a horizontal line; (d) The child should be gently stretched upwards with traction under the angle of the jaw, and the height measured.

82

CHILDHOOD PROBLEMS

Management • A quarter of children with a height at least 2.5 standard deviations (SD) below the mean (which is close to the 0.4 centile) had organic disease in the Wessex growth study.30 * Refer if the child: (a) is above the 99.6 or below the 0.4 centile; (b) is crossing centile channels; (c) differs significantly from the centile corresponding to the expected adult height.

PUBERTY For the stages of puberty, see Appendix 8.

* Pubic hair development under age 8 in girls or age 9 in boys. This is almost always due to an adrenal androgen surge, but again referral is probably wise.

Delayed puberty Most will be boys with constitutional delay of growth and puberty. * Refer any child without secondary sexual characteristics by the age of 14. Even if the delay is shown to be constitutional, these children suffer from psychological problems, increased liability to osteoporosis and a short spine in comparison to leg length. These problems can be avoided by the use of low-dose sex hormones or anabolic steroids.31

Early puberty Most will be girls who will be found to have idiopathic precocious puberty. Once other pathology is excluded, the only treatment is explanation and support and occasionally consideration of gonadotrophin analogues (± anti-androgenic drugs) in an attempt to achieve a higher final height. * Refer any child who develops secondary sexual characteristics before the age of 8. * Refer any child where one secondary sexual characteristic develops out of proportion to the others and out of proportion to the growth rate. This suggests an abnormal source of hormone secretion. Examples would be isolated breast development without the development of pubic hair and apocrine sweat in girls, and penile enlargement without increase in testicular volume in boys. * Refer any child who is passing through the stages of puberty abnormally fast if this is causing concern either for social reasons or because of worries about final height. * Precocious unilateral or bilateral breast development. This usually occurs before the age of 2 and rarely reaches stage 3. It is very unlikely to be due to organic disease but referral is probably wise.

RECURRENT PHYSICAL SYMPTOMS Children commonly develop somatic symptoms where no organic cause can be found. This may reflect psychological distress. It is important to: (a) exclude likely physical causes; (b) help the family change the focus from physical to underlying psychological issues; (c) encourage the child to express his or her feelings; (d) encourage continuing school attendance. Avoiding school makes eventual attendance harder.

RECURRENT ABDOMINAL PAIN In the majority of children with recurrent abdominal pain, the pain is not due to organic pathology. There seems to be a spectrum, with children at one end who seem to have nothing more than an awareness of peristaltic movements, and children at the other end with 'periodic syndrome'. Children at this extreme may vomit, go unusually pale and may be febrile. There is a close connection with migraine.

RECURRENT PHYSICAL SYMPTOMS 83

Features of benign recurrent abdominal pain

severe adult migraine sufferers can remember having severe headaches by the age of 10 years.

(a) The pain is central, episodic and variable in character. (b) The pain is not usually associated with any disturbance in bowel habit. (c) The child continues to thrive. (d) The timing of the pain varies, but tends to be less frequent at weekends and during school holidays.

* Check the blood pressure and examine the fundi. * Refer if there is any anxiety about organic pathology. This is more likely in children under 5, where the headache is not relieved by simple analgesics, the frequency and severity is increasing, the child is not thriving or has signs of intellectual slowing, or where there are neurological signs. * If there is no evidence of organic pathology, reassure the parents that the child does not have a tumour or blood clot causing the headaches. * Migminous headaches. If associated with recurrent abdominal pain, pallor, paraesthesiae, visual disturbances or vomiting, the diagnosis is likely to be migraine. Advise the parents to treat the attack at an early stage with soluble paracetamol and to encourage the child to lie down. Mild hypoglycaemia is a common trigger: try snacks especially before vigorous exercise and avoid long gaps between food. Children with frequent migraines will benefit from prophylaxis, e.g. pizotifen or propranolol, for 6 months in the first instance. See also Migraine (p. 189). * Non-migrainous headaches are commonly tensionrelated. Reassure the child of their benign nature and encourage them to take paracetamol early in the headache.

* Examine the child fully, plot the height and weight and check the urine for protein and infection. * Refer any child who has symptoms of general illness or bowel disturbance, or where the picture differs significantly from that described above. * Assess whether: there are problems at home or school; the child is using the pain as a way of avoiding stress or to gain attention; there is evidence of emotional disorder. * Reassure the parents that the symptoms are not due to a grumbling appendix. Explain that the pain is real but not due to any abdominal pathology and does not require any further investigations. Use the analogy that to have a headache does not mean that there is something wrong with the brain and that, similarly, to have abdominal pain does not necessarily indicate that there is something wrong in the abdomen. * Follow-up regularly, and repeatedly reassure the child and parents. * Consider using prophylactic pizotifen for children with 'periodic syndrome' (as in Migraine, p. 190).32,33 * Refer if the parents are dissatisfied or if the pain does not settle.

Examination of lifestyle and an alteration in behaviour may help. A number of children suffer headaches at the end of a busy day when many factors may have contributed, including hunger. Note: Eye strain is only rarely a cause of headaches, and many children are prescribed glasses unnecessarily for minor refractive errors.

NIGHT CRAMPS RECURRENT HEADACHE Headache is common in children, and the incidence increases with age; 80-90% of secondary school-age children suffer from recurrent headaches! The incidence of migraine in this age group is about 5%, but many children suffer severe non-migrainous headaches. About 25% of

Night cramps are felt more in the legs than in the arms, and are felt between (not in) the joints. They are not growing pains, but reflect stresses in the child's life and are akin to headaches. They are generally more common when the child is at school than during school holidays. The pains commonly wake the child up from sleep.

84 CHILDHOOD PROBLEMS

* Investigations are neither necessary nor helpful. * Emotional support, e.g. cuddles, is all that is needed. Analgesics are not required but may provide a necessary placebo.

SCHOOL REFUSAL * About 1 in 10 of all school pupils are absent from school at any one time, and one-fifth of these have no legitimate reason for being away. The latter fall into three main groups: truants, children voluntarily withheld by parents and school refusers. * School refusal may be based on a fear of separation from one or both parents or fear of school attendance. School-refusing children generally work well at school, and are expressing an underlying neurotic disorder. School refusal tends to occur in three main age groups: 5-7, 11 (after changing to secondary school) and in adolescence. * Exclude an underlying cause if the presentation is of physical illness. * Refer to a specialist unit according to what is locally available. * Encourage the child to go back to school. This involves convincing the parents of the importance of doing so. Even severe emotional problems can improve rapidly on re-establishing attendance.

* Exceptions: (a) large haemangiomas on the nose, lip, eyelid or cheek may grow very large and need steroid treatment or surgery. (b) haemangiomas on the eyelid: refer early to prevent amblyopia. (c) haemangioma on the face + stridor: refer urgently (suggests laryngeal haemangioma).

INGUINAL HERNIAS These usually present in the first 3—4 months of life and are less common with increasing age. * Refer for urgent outpatient appointment; early operation is recommended to avoid incarceration or strangulation.

HYDROCELES These are the most common cause of scrotal swelling. There are two types: (a) Slack, often bilateral, which disappear within the first year; (b) Tense, often unilateral, which exist after the first year and need surgery lest spermatogenesis is impaired.

UNDESCENDEDTESTIS See p. 55.

THE FORESKIN SURGICAL PROBLEMS STRAWBERRY NAEVUS Strawberry naevi usually appear shortly after birth and grow very rapidly for the first 4-5 months of life. They then involute and become pale. Full resolution may take many years. * Reassure parents that surgery is not warranted and that the lesion naturally regresses. * Advise them how to stop any bleeding, should it occur, by applying pressure.

• The foreskin has a protective role and is not usually retractile until 1 year. 'Adhesions' are present in 60% of boys aged 6. They are physiological and regress spontaneously. • Retraction of the foreskin should be encouraged from the age of 6 in order to clean out smegma, but not to break down the 'adhesions'. These will continue to separate naturally throughout childhood and adolescence. Tearing them forcibly risks causing scarring.34 Surgical separation or circumcision should only be considered if they are causing symptoms.

SURGICAL PROBLEMS 85

Circumcision • The General Medical Council (CMC) has given guidelines relating to the circumcision of boys.35 It stresses that, among other issues: (a) the doctor should have the necessary skills and experience both to operate and to treat the patient's postoperative pain; (b) the doctor should explain the risks and benefits of the operation and obtain consent, preferably from both parents. • Circumcision while still in nappies can give rise to meatal ulceration and subsequent stricture.

* At 1 year, if the hernial orifice: (a) is less than 1 cm in diameter then spontaneous resolution is likely; (b) is greater than 1 cm in diameter then surgical intervention may be necessary. * Consider hypothyroidism: check that the child is growing and gaining weight normally.

BAT EARS If operation is desired, do not refer until the child is aged 3 years as the cartilage is not firm enough for surgery until then.

Common indications for circumcision are: (a) Balanitis: prescribe antibiotic ointment (e.g. fusidic acid) to be massaged into the preputial orifice. After three attacks, consider circumcision. (b) Phimosis: true phimosis is uncommon. Circumcision should be considered only if there is difficulty passing urine. Almost all cases of physiological tightness of the preputial oriface will resolve naturally. Even ballooning of the foreskin does not necessarily mean that circumcision is necessary. Sometime aligning the preputial oriface with the meatus before starting to pass urine will avoid the problem. (c) Paraphimosis: this should be reduced as soon as possible. Shrink the glans with an ice pack, and use a lubricant to reduce the paraphimosis. Admit if irreducible. (d) Redundant prepuce: a long prepuce may lead to recurrent irritation and discomfort.

HYPOSPADIAS Refer immediately if the child is not producing a good stream of urine. Non-urgent cases should be seen by 6 months so that subsequent surgery can be planned.

UMBILICAL HERNIA * Tell the parents that: (a) umbilical hernias increase in size in the first 6 months of life but they do not strangulate; (b) bandaging with an old penny is of no value.

ORTHOPAEDIC PROBLEMS

Examination of the hip for congenital dislocation See p. 54.

Club feet Marked congenital talipes equinovarus is easily recognized at birth. Much more common is positional talipes due to the position of the foot in utero. * Refer if the foot cannot be moved into the normal position. * Positional talipes. The foot is easily moved into the normal anatomical position. Reassure the parents that it will correct itself. Exercises may help - teach the parents to stretch the medial border of the foot and to push the foot into eversion and dorsiflexion on a regular basis, e.g. when bathing the baby each day.

In-toeing This may be due to: (a) Metatarsus adductus (varus) or hooking of the forefoot which usually straightens by the age of 3 years. Referral is only needed if the deformity cannot be corrected by passive straightening. (b) Femoral neck anteversion. These children walk with their kneecaps facing each other because of anteversion of the femoral neck. Examine the child

86 CHILDHOOD PROBLEMS

prone with the knees flexed and the feet in the air. Internal rotation (turning the leg outwards) is increased from 70° to as much as 90°. Conversely, external rotation (turning the leg inwards) is considerably reduced. Refer extreme cases for orthopaedic opinion. Reassure the parents of less extreme cases that the femoral anteversion resolves by the age of 8-9 years. (c) Tibial torsion occurs when the normal growth and rotation of the tibia is out of step with the femur. Inward rotation leads to in-toeing. * Reassure the parents that it resolves by the age of 3-4 years, but consider rickets in at-risk children.

Bow legs and knock knees Bow legs and knock knees rarely need treatment. Many infants have outward curving of the tibia, and spontaneous resolution usually occurs by the age of 2 years. Children may then become knockkneed, which reaches a maximum by the age of 4 years. They then improve spontaneously, achieving normal alignment by about 6 years of age. * Refer: (a) if the child is unwell, especially if the bones are tender or joints swollen as in rickets; (b) the legs are asymmetrical; (c) there is anterior curvature of the tibiae; (d) if in bow legs the knee to knee distance is greater than 3 cm; or (e) if in knock knees the medial malleoli are more than 7cm apart when the child lies down with the knees together and the patellae facing forwards. Referral might be made at lesser measurements if the distance is increasing.

Flat feet * Refer if there is neurological abnormality, muscle weakness or a lack of movement at the tarsal joints. * Reassure if the child is asymptomatic, there is a full range of active and passive movements at all joints, and the arch is seen normally when the child stands on tiptoe. The arch is likely to be normal by the age of 10.

Curly toes * Reassure the parents that the toes can be straightened if necessary. * Refer the child to have the toes straightened only if the deformity is fixed and sufficiently severe for one toe to impinge on another in a way that is likely to cause symptoms.

The limping child The child who presents with a painful limp and complains of pain in the knee should be assumed to have a problem in the hip until proved otherwise. Depending on the age of the child, the problem may be due to: (a) Congenital dislocation of the hip. This is painless and usually obvious once the child begins to walk. (b) Perthes' avascular necrosis of the femoral head, which usually occurs between age 5 and 10. (c) Slipping of the upper femoral epiphysis, which usually occurs between age 10 and 15. (d) Acute septic arthritis and acute osteomyelitis, which can occur at any age and are surgical emergencies. (e) Transient synovitis of the hip (irritable hip) this is the most common cause of limp due to hip pain and is commonest between age 4 and 10. Ninety per cent resolve in 7 days, but it cannot reliably be distinguished in general practice from more serious causes of hip pain, and all patients should be referred. * Refer all these to hospital urgently.

Osgood Schletter's disease * Explain the benign nature of the condition. It will resolve spontaneously, leaving only a prominent tibial tubercle. * Explain that exercise will not make it worse. Patients may do any sport that they can manage without excessive pain. * Do not X-ray or refer.

Scoliosis This occurs more commonly in girls than in boys.

PREVENTIVE HEALTH CARE IN ADOLESCENTS 87

* Examine all children over the age of 10 years, when the opportunity arises. Examine from behind, as follows: (a) Stand the child up with the feet together. (b) Assess whether the hips are level (i.e. there is no compensation). (c) Ask the child to touch his or her toes with the knees straight. (d) Look for a 'rib hump'. This will be most marked when the spine is flexed. * Refer to an orthopaedic surgeon if there is any suggestion of 'rib hump' or curvature.

PREVENTIVE HEALTH CARE IN ADOLESCENTS Overview: National Adolescent & Student Health Unit and the Royal College of General Practitioners. The Health of Adolescents in Primary Care. London: Royal College of General Practitioners, 1996. Available from RCGP, 14 Princes Gate, Hyde Park, London SW7 1PU, tel. 020 7581 3232.

• Deliberate self-harm occurs in about four adolescents in a thousand. About half of these have attended their GP in the preceding month, often for a problem connected with the attempt. • About 20% of women and 27% of men have had intercourse by age 16. Half of these had intercourse without using contraception. • Four thousand girls under 16 have terminations in the UK each year. • Only one-third of adolescents believe GP consultations are confidential. • One-third of 16-year-olds smoke regularly. A further third have tried smoking. • The age of initiation of alcohol use is currently around 10-11 years. Teenagers are more likely to have casual sex and are less likely to use contraception when under the influence of alcohol. Many adolescents who overdose do so while under the influence of alcohol. • One study shows that about 60% of adolescents have been offered drugs by the age of 16 (cannabis 52%, LSD 36%, amphetamines 26%,

solvents 22%, magic mushrooms 21%, ecstasy (MDMA) 18%). * Be aware that although they appear to be a healthy group who present with relatively trivial illnesses, the opportunity to explore the above points should not be missed. * Consider special arrangements to see adolescents in order to discuss behavioural issues, e.g. at a dedicated clinic combined with school-leaving immunizations. * Be aware that rational health promotion messages are often ineffective in adolescents, who live more for the moment. Helping a teenager to develop self-esteem is more likely to lead to healthy behaviour than negative messages about the dangers of sex or smoking. * Ensure that the practice is giving the message to adolescents that they are seen as an important group for whom specific services exist.

EMOTIONAL PROBLEMS IN ADOLESCENCE Adolescents with emotional difficulties are unlikely to present themselves. It is more usual for their parents to report behavioural changes, which often they do not understand. These often include: social withdrawal; irritability; running away from home; separation anxiety; recurrent somatic pains; decline in school work; excessive sleep.

Management * See adolescents on their own if possible, and encourage them to express their main concerns. Ask about suicidal thoughts. * School stress. If there are difficulties with peers or schoolwork, talk about the value of discussing this with parents, teaching staff, school nurses. Many schools now have counsellors. * Family relations. Encourage better communication within the family, and recommend both adolescent and parents to read the Family Survival Guide (see below). * Discover whether an adolescent has an excessively low self-esteem. Focus on the patient's positive aspects.

88 CHILDHOOD PROBLEMS

* Refer adolescents with major depression to the appropriate local unit. Where a considerable delay is likely, treatment with an SSRI is reasonable. Tricylic antidepressants seem not to be effective.36 Patients with active suicidal thoughts should be seen urgently.

Patient information: Royal College of Psychiatrists. Help is at hand: surviving adolescence. Online. Available: www.rcpsych.ac.uk Graham L. Teenagers: a family survival guide. London: Chatto and Windus, 1992.

REFERENCES 1. Chief Medical Officer's Expert Group. Report of the CMO's Expert Group on the Sleeping Position of Infants and Cot Death. London: HMSO, 1993. 2. Department of Health and Social Security. Present Day Practice in Infant Feeding. Report on Health and Social Subjects 32. London: HMSO, 1988. 3. Taylor E et al. The epidemiology of childhood hyperactivity. Maudsley Monographs, No. 33. Oxford: Oxford University Press, 1991. 4. Bellak L, Black RB. Attention deficit disorder in adults. din Ther 1992; 14(2): 138-47. 5. Woolfenden SR, Williams K, Peat J. Family and parenting interventions in children and adolescents with conduct disorder and delinquency aged 10-17 (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 6. Barlow J, Coren E. Parent-training programmes for improving maternal psychosocial health (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 7. Crowcroft NS and Strachan DP. The social origins of infantile colic: questionnaire study covering 76,747 infants. BMJ 1997; 314: 1325-8. 8. Markestad T. Use of sucrose as a treatment for infant colic. Arch Dis Child 1997; 76: 356-8. 9. Garrison MM, Christiakis DA. A systematic review of treatments for infant colic. Pediatrics 2000; 106: 184-190. 10. St James-Roberts I. Managing infants who cry persistently. BMJ 1992; 304: 997-8. 11. The management of febrile convulsions. Drug Ther Bull 1987; 25(3): 9-11. 12. Valman HB. Febrile convulsions. BMJ 1993; 306: 1743-5. 13. Verity CM, Golding J. Risk of epilepsy after febrile convulsions: a national cohort study. BMJ 1991; 303: 1373-6. 14. Kinmouth A-L et al. Management of feverish children at home. BMJ 1992; 305: 1134-6. 15. Rantala H et al. A meta-analytic review of the preventative treatment of recurrences of febrile seizures. JPediatr1997; 131: 922-5. 16. Glazener CMA, Evans JHC. Desmopressin for nocturnal enuresis in children (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 17. Glazener CMA, Evans JHC. Alarm interventions for nocturnal enuresis in children (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 18. Glazener CMA, Evans JHC. Tricyclic and related drugs for nocturnal enuresis in children (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2000.

19. Isaacs D. Bronchiolitis. BMJ 1995; 310: 4-5. 20. McKean M, Ducharme F. Inhaled steroids for episodic viral wheeze of childhood (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 21. Chavasse RJ, Bastian-Lee Y, Richter H, Hilliard T, Seddon P. Persistent wheezing in infants with an atopic tendency responds to inhaled fluticasone. Arch Dis Child 2001; 85(2): 143-8. 22. Agertoft L, Pedersen S. Effects of long-term treatment with inhaled corticosteroids on growth and pulmonary function in asthmatic children. Respir Med 1994; 88: 373-81. 23. Sawyer SM. Reproductive and sexual health in adolescents with cystic fibrosis. BMJ 1996; 313: 1095-6. 24. Godden CW et al. Double blind placebo-controlled trial of nebulized budesonide for croup. Arch Dis Child 1997; 76(2): 155-8. 25. Geelhoed GC et al. Efficacy of a small single dose of oral dexamethasone for outpatient croup: a double blind placebo-controlled clinical trial. BMJ 1996; 313: 140-2. 26. Ausejo M, Saenz A et al. The effectiveness of glucocorticoids in treating croup: meta-analysis. BMJ 1999; 319: 595-600. 27. Gorelick MH, Shaw KN. Screening tests for urinary tract infection in children. A meta-analysis. Pediatrics 1999; 104(5): e54. 28. Vernon SJ. Comments on managing urinary tract infection in children were inadequate. BMJ 1996; 313: 491-2. 29. Jones R. Childhood vulvovaginitis and vaginal discharge in general practice. Family Practice 1996; 13(4): 369-72. 30. Voss LD et al. Poor growth in school entrance as an index of organic disease: the Wessex growth study. BMJ 1992; 305: 1400-2. 31. Stanhope R et al. Delayed puberty. BMJ 1992; 305: 790. 32. Abu-Arafeh I, Russell G. Prevalence and clinical features of abdominal migraine compared with those of migraine headache. Arch Dis Child 1995; 72(5): 413-17. 33. Dignan F, Symon DN, AbuArafeh I, Russell G. The prognosis of cyclical vomiting syndrome. Arch Dis Child 2001; 84(1): 55-57. 34. Medical indications for childhood circumcision. Drug Ther Bull 1993; 31(25): 99-100. 35. Beecham L. GMC issues guidelines on circumcision. BMJ 1997; 314: 1573. 36. Kutcher S. Practitioner review: the pharmacotherapy of adolescent depression. J Child Psychol Psych Allied Dis 1997; 38(7): 755-67.

5

CHAPTER CONTENTS Hypertension 89 Whom to treat: The UK BHS guidelines thresholds 90 Non-drug treatment 91 Drug treatment 91 Angina

Cardiovascular problems

93

Unstable angina

95

Myocardial infarction 96 Immediate management 96 Subsequent home management if not admitted The 6-week assessment 98 Other factual advice 99

97

Heart failure 99 GP management 99 Congestive cardiac failure 100 Intractable heart failure 102 End-stage heart failure 102 Acute pulmonary oedema 103 Right heart failure 103 Arrhythmias 103 Ectopic beats in a normal heart 103 Atrial fibrillation 104 Atrial flutter 105 Paroxysmal supraventricular tachycardia Ventricular tachycardia 106 Sick sinus syndrome 106 Bradycardia 106

HYPERTENSION Guideline: Ramsay LE et al. Guidelines for management of hypertension: report of the third working party of the British Hypertension Society. J Human Hyperten 1999; 13: 569-92. Available: http://www.hyp.ac.uk/bhs and choose 'Resources'. 105

Antibiotic prophylaxis of infective endocarditis 106 The prevention of coronary heart disease Primary prevention 108 Secondary prevention 109 References

110

107

• Ten per cent of all adults in the UK have raised blood pressure (BP), using UK definitions. US definitions give a prevalence of 1 in 4 of the population with hypertension.1 • The rule of halves, described in the US in 1972,2 was still operating in Scotland in the late 1980s,3 although there may have been some improvement since then.4 The rule states that half of hypertensives are not known to have a raised BP; of those with known hypertension, half are not on treatment and half of those on treatment are poorly controlled. • All adults should have their BP measured at least every 5 years until age 80. • If hypertension is found it should trigger a search for other risk factors for cardiovascular disease. These factors tend to cluster and their risks are multiplicative. • The elderly: not only is hypertension more common in the elderly, but the benefits of treatment are greater than in the younger patient, at least up to the age of 80.5 Beyond 80 there is a dearth of trial information but what there is suggests a reduction in major cardiovascular events of 22%, of heart failure of 39%, and of stroke 34%.6 89

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• Systolic and diastolic pressures both correlate with cardiovascular risk. The recommendation for treatment at 160/100 means, therefore, that treatment should be started if either the systolic is 160 or the diastolic is 100 or above. • The benefits of treatment: treating mild hypertension reduces the risk of a cardiovascular event by 25%. For patients with mild hypertension and a 10-year CHD risk of at least 15% the number needed to treat for 5 years is 40 (i.e. treating 40 such patients for 5 years will prevent one cardiovascular event).

WHOM TO TREAT? THE UK BHS GUIDELINES THRESHOLDS7 * Low-risk patients. Treat those whose BP is sustained at 160/100 or above. * High-risk patients. Consider treatment if BP is sustained at 140/90 or above in those with: (a) target organ damage; or (b) cardiovascular disease; or (c) a 10-year risk of coronary heart disease of 15% or more; or (d) diabetes (see p. 150).

Targets Aim for a BP <140/85, with a minimum acceptable standard of < 150/90. In diabetics the optimum BP is < 140/80, with a minimum acceptable standard <140/85. Note: These levels for diabetics are conservative. The Joint British Recommendations8 are as follows: • Thresholds: type 1: 130/80; type 2: 160/90 or 140/90 if there is target organ damage or a 10-year CHD risk >15%; • Targets: type 1: <130/80 (or <125/75 if there is proteinuria); type 2: <130/80. Age limits Continue treatment beyond the age of 80, and even consider starting it beyond 80 in a patient who is sufficiently fit, especially if there is target organ damage.

'White coat' hypertension Hypertensives regularly record lower pressures at home or with ambulatory monitoring (ABPM) than in the clinic. The mean difference is 12/7mmHg. Those with white coat hypertension have a substantially greater difference. ABPM is only necessary in those in whom the result would effect the decision to treat. If ABPM or home readings are used to decide on or monitor treatment, thresholds 12/7 lower than the levels quoted above should be used.

Practicalities (a) The patient should be seated, but in older patients and in diabetics check the blood pressure both standing and sitting. (b) Measure the systolic and diastolic pressures (phase V) to the nearest 2 mm. (c) Timing. In mild uncomplicated hypertension, do not start treatment until four readings have been taken over a 3-month period; 25% of blood pressures will settle in that time. Those that settle to below treatment levels need lifelong annual follow-up. If the initial diastolic is 110 mmHg or over, or the systolic is 200 mmHg or over, or there is evidence of end-organ damage, three readings over 2 weeks would be more appropriate.

Work-up (a) A history of family or personal risk factors for stroke or coronary heart disease. Check whether relevant drugs (e.g. NSAIDs) or excess alcohol are taken. (b) Examination, including: -fundi; -femoral pulses; -palpation of kidneys and auscultation for presence of bruit; -signs of left ventricular hypertrophy. (c) Urinalysis, for protein, glucose, and blood. (d) Blood: -creatinine and electrolytes; -fasting blood sugar; -serum cholesterol; -haemoglobin (Hb) to exclude polycythaemia. (e) Look for left ventricular hypertrophy using ECG and CXR, with echocardiography in borderline cases or where the finding of hypertrophy would affect management.9

HYPERTENSION 91

Refer (a) Where treatment is urgent: -accelerated (malignant) hypertension (admit); -severe hypertension (>220/120); treat immediately and refer; -impending complications (e.g. transient ischaemic attack (TIA), left ventricular failure (LVF)). (b) Where there is reason to suspect a primary cause: -age <30 with BP of 160/100 or above; age <20 with BP 140/90 or above; - documented sudden onset of hypertension or sudden worsening in middle age (suggests possible renal artery stenosis); - symptoms suggesting phaeochromocytoma; - signs of coarctation; -evidence of renal disease: proteinuria, haematuria or raised creatinine; -hypokalaemia and/or hypernatraemia; - resistance to at least three drugs. (c) Where the need for ABPM is suggested by the finding of a variable BP or a discrepancy between home and clinic readings.

NON-DRUG TREATMENT Non-drug treatment can lower the systolic pressure by lOmmHg and the diastolic by 8 mmHg.10 Encourage all patients to: (a) Lose weight if overweight. A loss of 8.8kg is associated with a fall in blood pressure of 5/7 mmHg.11 (b) Alcohol: reduce excessive consumption. (c) Diet: reduce intake of salt to <50 mmol (3g) per day and eat a diet rich in fruit and vegetables and low in total and saturated fat. A reduction of 7 mmHg systolic and 3.8mmHg diastolic can be expected.12 Common sources of salt are nuts, crisps, canned foods and bread. Low-salt versions are available. (d) The contraceptive pill: consider stopping, but not until other adequate contraceptive measures are in place.

(e) Exercise: brisk walking for 30 minutes every day is as beneficial as more vigorous exercise three times a week. Brisk walking for an hour, three or four times a week may be even better.13 (f) Stress: possibly adopt a less stressful lifestyle. Studies are equally divided between those that show benefit and those that do not. (g) Smoking: stop. This will not reduce the BP but it will lower the cardiovascular risk. (h) Aspirin: take 75 mg daily if aged 50 or over with a CHD risk of at least 15% or with target organ damage, provided the BP is controlled to the minimum acceptable standard.14

DRUG TREATMENT Choice of drug * The British Hypertension Society Third Working Party recommends the use of thiazides as first-line drugs, followed by beta-blockers or, in the elderly, long-acting dihydropyridine calciumchannel blockers. Angiotensin converting enzyme (ACE) inhibitors, calcium-channel blockers, and alpha-blockers are considered alternative first-line drugs when thiazides and beta-blockers are contraindicated, ineffective, or when side-effects occur. * Evidence that one class is better than another is weak.15 * Omit any steps if contraindications exist. * Do not necessarily abandon a drug that has failed to lower the BP on its own. It may have an additive effect if another drug is added. * If using more than one drug: (a) Use a combination of drugs that has an effect that is more than additive, e.g.: -a beta-blocker with a thiazide and /or a calcium-channel blocker; and/or an alphablocker; or -an ACE inhibitor with a diuretic and/or a calcium-channel blocker. (b) Avoid combinations of drugs that are less than additive, e.g. beta-blockers and ACE inhibitors; thiazides and amlodipine. Beta-blockers should not normally be combined with verapamil nor diltiazem, although the latter is probably safe in low dosage.

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Drug treatment of patients with problems (a) Hyperlipidaemia: the effect of beta-blockers and low-dose thiazides is so small that the presence of hyperlipidaemia does not alter the general drug recommendations.16 (b) Angina: use a beta-blocker or a calciumchannel blocker. (c) The elderly: use a thiazide; then a calciumchannel blocker or ACE inhibitor. (d) Heart failure: use a diuretic, an ACE inhibitor, a beta-blocker then an alpha-blocker. (e) Diabetics: use an ACE inhibitor, a low-dose thiazide, an alpha-blocker or calcium-channel blocker.17 (f) Smokers: do not use a beta-blocker. (g) Peripheral vascular disease: use a thiazide, calcium-channel blocker, or alpha-blocker. (h) Migraine: use a beta-blocker and/or calciumchannel blocker; then all other alternatives. (i) Afro-Caribbeans: use a thiazide, calciumchannel blocker and/or alpha-blocker. These patients respond poorly to ACE inhibitors and to beta-blockers alone, although they respond well to both of these in combination with thiazides, calcium-channel blockers or alpha-blockers. (j) Those active in sports: avoid beta-blockers. (k) Raynaud's syndome: use a calcium-channel blocker. (1) Supraventicular arrhythmias: use a betablocker and/or calcium-channel blocker. (m) Renal failure: get specialist advice. ACE inhibitors may improve renal function, but will need to be given in lower dosage. (n) Gout: avoid thiazides. (o) Asthma: avoid beta-blockers.

Beta-blockers * Use: (a) a once-daily selective agent, e.g. atenolol 50 mg or metoprolol SR 200 mg; (b) a relatively low dose, as above. Adverse effects from higher doses usually outweigh the small benefit; (c) a drug with intrinsic sympathomimetic activity (ISA) in patients with sinus bradycardia, e.g. acebutolol 400 mg o.d.

* Avoid: (a) in the elderly, who are prone to more frequent side-effects, especially tiredness, and in whom they seem less effective; (b) in smokers; (c) in Afro-Caribbean patients. * Observe the usual contraindications, especially a history of asthma or airways obstruction. Perform a peak flow before and after starting treatment if the history suggests the slightest possibility of asthma. * Warn the patient not to stop a beta-blocker suddenly, especially one without ISA. Even those without known coronary heart disease (CHD) have a fourfold increased risk of myocardial infarction or angina in the subsequent 4 weeks.

Thiazides * Use a thiazide equivalent to bendrofluazide 2.5 mg, or 1.25mg if the patient is able to cut the tablet in half. This is as effective as l0 mg, but avoids the metabolic side-effects associated with the higher dose except for a slight rise in uric acid.18 * Observe the usual contraindications. * Recheck serum electrolytes 1 month after starting a thiazide. If no fall in potassium has occurred, further routine checks are not necessary unless there is renal impairment or the patient is taking digoxin. * Ask specifically about erectile difficulties in men. The incidence in patients on thiazides is double that in those on placebo (17% versus 8%), but patients rarely volunteer this information. The difficulties appear early, and may not be sufficiently troublesome to warrant stopping treatment.19

Calcium-channel blockers * The possibility has been raised but not proven that calcium-channel blockers may increase the risk of death from cardiovascular and noncardiovascular causes.20 A meta-analysis has found no evidence for this, although short-acting nifedipine is not recommended in hypertension because of the possibility of an increased risk

ANGINA

of myocardial infarction in patients who have CHD.21 * Use a long-acting preparation, e.g. diltiazem 120-180 mg slow release b.d. or verapamil 120-240 mg b.d. or 240-480 mg slow release daily. Their hypotensive effect is the same as nifedipine, with fewer side-effects. Use brand names. * Use a dihydropyridine calcium-channel blocker, e.g. nifedipine slow release or amlodipine, if: (a) beta-blockers are also being given; (b) there is peripheral vascular disease with skin ischaemia. It will not, however, help intermittent claudication; (c) there is a risk of heart failure, which might be worsened by a non-dihydropyridine. Note: Beta-blockers are not necessary to counteract the tachycardia caused by nifedipine; this settles in 48 hours anyway.22

Alpha-blockers Total cholesterol falls by an average of 4%, with a beneficial rise in HDL cholesterol. * Start with a low dose, e.g. terazosin 1 mg, or doxazosin 1mg, taken at night in case of firstdose hypotension. * Use with caution in the elderly, who may experience continued postural hypotension.

ACE inhibitors and related drugs * Starting them: (a) In volume-depleted patients, discontinue diuretics currently being prescribed for 2 days before starting an ACE inhibitor, unless doing so is too risky because of heart failure. (b) The first dose should be taken at night. Hypotension in once-daily agents may not occur until 6-8 hours after the first dose, and may last for 24 hours. (c) Recheck serum creatinine 2 weeks after starting the drug. A rise suggests renal artery stenosis. * Use them in insulin-dependent diabetics; they improve insulin resistance (whereas thiazides and beta-blockers may worsen it).

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* Avoid them in: (a) Afro-Caribbeans; they are less effective; (b) patients with peripheral vascular disease. As a group they have a 33% incidence of renal artery stenosis.23 An angiotensin II receptor antagonist is indicated in patients who need an ACE inhibitor but cannot tolerate it because of cough.24

ANGINA Guidelines: North of England Stable Angina Development Group. North of England Evidence-Based Guidelines Development Project: summary version of evidence-based guideline for the primary care management of stable angina. BMJ1996; 312: 827-32. De Bono D, for the Joint Working Party of the British Cardiac Society and the Royal College of Physicians of London. Investigation and management of stable angina: revised guidelines 1998. Heart 1999; 81: 546-55.

Work-up (a) Hb. (b) Thyroid function tests (TFTs). (c) Fasting blood sugar. (d) Fasting lipid profile. (e) Resting ECG. (f) Exercise ECG. Symptoms are a poor guide to the severity of the coronary artery disease. Reasons for not performing an exercise ECG are: -the patient is physically incapable of performing the test; -the patient has some other illness or condition which would make him or her unsuitable for coronary artery surgery; -the diagnosis is in doubt (an exercise ECG has low specificity for making the diagnosis of angina); -where symptoms are uncontrolled by medical treatment (such patients should be referred for angiography).

* Check for: (a) diabetes; (b) hypo/hyperthyroidism; (c) anaemia/polycythaemia; (d) hypertension; treatment is warranted if the blood pressure is 140/90 or above. It should be reduced below 140/85.8 (e) hyperlipidaemia (see p. 109); (f) arrhythmias;

94 CARDIOVASCULAR PROBLEMS

(g) valvular disease. Any aortic systolic murmur needs assessment for aortic stenosis unless the patient's general condition is too poor to consider even a valvotomy. * Advise the patient: (a) not to smoke. After 9 years, the risks for an ex-smoker fall to those of a non-smoker25 (see p. 331); (b) to lose weight, if obese; (c) to take adequate exercise. This means 30 minutes moderately intense exercise, e.g. brisk walking, five times a week. The 30 minutes may be divided into separate parts, but each bout of exercise should last at least 10 minutes. * Give all patients aspirin 75 mg per day, or clopidogrel if aspirin is contraindicated. The risk of serious vascular events is reduced by 34%.26 * Give all patients a statin, after assessment of their serum lipids (see p. 109). * Advise the patient about the symptoms of a myocardial infarction or unstable angina and the importance of calling an ambulance if chest pain persists for >15 minutes despite use of glyceryl trinitrate (GTN). * Refer to a cardiologist those patients: (a) with a positive exercise test; (b) with angina not controlled by medication; (c) whose diagnosis is in doubt; (c) with aortic stenosis who develop angina, especially if elderly;27 (d) who are candidates for angiography with a view to CABG or angioplasty.28 Any patient with a positive exercise ECG should be considered, especially those with angina following myocardial infarction (MI). The MI makes patients stronger candidates for coronary artery surgery. * Driving. Advise patients to notify their insurance company and not to drive until symptoms are controlled.

(c) buccal tablets, which may be more effective than sublingual preparations.

Preventive treatment Nicorandil can reduce the risk of future major coronary events (see p. 95). The benefit from beta-blockers post-Mi suggests that they may exert similar protection in angina. Certainly, any patient with angina that is more than occasional should be offered treatment, starting with a beta-blocker, unless contraindicated. In patients for whom beta-blockers are not contraindicated 1. Use a long-acting beta-blocker, e.g. atenolol. Once taken, assess whether the patient is fully beta-blocked. This is indicated by a heart rate of 65 beats/min or less at rest, and a rate of 90beats/min or less after walking up two flights of stairs. Further increases in dose above the minimum required to achieve beta-blockade will probably be unhelpful. Warn the patient not to stop or omit even one dose. If it is necessary to stop for medical reasons, tail off over 4 weeks. 2. If control is unsatisfactory, add a longacting dihydropyridine calcium-channel blocker, e.g. amlodipine. Non-dihydropyridine calciumchannel blockers should not be started in general practice in a patient taking beta-blockers because of their effects on the conducting system. However, note the following problems with all calciumchannel blockers: (a) vasodilation may reduce flow through the coronary artery stenosis and make the angina worse; (b) reflex tachycardia can occur for the first few days of use; (c) aortic stenosis is a contraindication. The coronary blood flow may be further decreased by nifedipine.

Acute attacks * Use glyceryl trinitrate: (a) sublingually: 300-500 ug (300 ug is less likely to cause headaches). The shelf life is only 2 to 3 months; or (b) spray: 400 ug per puff; or

In patients for whom beta-blockers are contraindicated or whose angina is vasospastic, i.e. more prominent at rest than on exertion 1. Begin with a calcium-channel blocker. Consider using verapamil or diltiazem; they are more

UNSTABLE ANGINA 95

powerful than dihydropyridines in angina. Start with verapamil 240 mg or diltiazem 180 mg daily. Increase the dose after 2 weeks only if angina is not controlled, and side-effects are not a problem. Prescribe by tradename because of the variations in bioavailability. 2. If control is still unsatisfactory add: (a) a long-acting nitrate, e.g. isosorbide mononitrate (ISMN) 20-60 mg b.d.; or (b) a sustained-release preparation of GTN, e.g. a buccal preparation (Suscard 1-2 mg t.d.s.) or a nitrate patch containing at least 10 mg of nitrate, if isosorbide mononitrate is not tolerated or fails to control symptoms; or (c) a potassium channel activator, e.g. nicorandil 10 mg b.d. increasing to 20 mg b.d. in combination with any other agents that have proved useful.29 The higher dose has been shown to reduce major coronary events compared to placebo. Problems with nitrates (a) Postural hypotension, syncope and headache. Start with a low dose, and increase once well tolerated. (b) Reflex tachycardia, which may decrease coronary blood flow and worsen the angina. (c) Tolerance, which may develop after several weeks. This can be avoided by allowing a nitratefree period of 8 hours a day. Patches need to be removed at night. Patients on ISMN and isosorbide dinitrate (ISDN) should take the second dose at 4 p.m., and not at bedtime. The alternative is to increase the dose.

UNSTABLE ANGINA Guideline: British Cardiac Society Guidelines and Medical Practice Committee; Royal College of Physicians Clinical Effectiveness and Evaluation Unit. Guideline for the management of patients with acute coronary syndromes without persistent EGG ST segment elevation. Heart 2001; 85:133-42.

• Unstable angina means that there has been an abrupt change in symptoms such that angina is

occurring at rest, or at significantly lower levels of activity, or that the frequency, duration or severity of the attacks has substantially worsened. The recent onset (in the last 2 months) of severe angina should also be treated as unstable. Even with treatment, 5-7% will have a serious outcome (death, MI or emergency revascularisation) within 1 week and 15% within 1 month.30 • Hospital treatment includes rest, beta-blockade, aspirin, nitrates and possibly treatment with a glycoprotein IIb/IIIa inhibitor,31 anticoagulation, coronary angioplasty or CABG. How invasive the treatment should be will depend on the risk stratification performed once the patient reaches hospital. The benefit from aggressive treatment applies to the older as much as the younger patient.32 * Admit by emergency ambulance any patient with cardiac pain lasting >15 minutes despite GTN. This applies whatever the ECG shows. * Arrange for specialist assessment that day for any other patient with unstable angina. * While awaiting admission, give: (a) GTN, e.g. three sprays over 15 minutes if pain continues; and (b) a tablet of soluble or chewable aspirin, 75-300 mg. Continue this after discharge. It can reduce by one-third the rate of MI, stroke and death over the next 6 months with a 'number needed to treat' (NNT) of 20. If the patient cannot tolerate aspirin give clopidogrel; and (c) a beta-blocker without ISA, if there is likely to be a delay in arranging admission. This will slightly reduce the rate of myocardial infarction. * Intensify treatment at home in patients whose angina has gradually worsened but without the abruptness that defines unstable angina. However, warn them to call urgently if symptoms worsen suddenly. In borderline cases, be more ready to admit those who are at higher risk because of: (a) age >65; (b) diabetes or other concurrent illness; (c) impaired left ventricular function; (d) new ECG changes, especially T wave inversion or ST depression.

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* If not admitting, check for a cause for the deterioration: (a) physical or emotional stress; (b) non-compliance with medication; (c) intercurrent illness; (d) a deterioration in cardiac function; (e) anaemia.

• The SHARP guidelines recommend that thrombolysis, if indicated, should be given by the general practitioner if the traveling time to hospital is 30 minutes or more. • Primary angioplasty almost halves the mortality from acute myocardial infarction compared to thrombolysis34 but is not usually available even in the developed world.

MYOCARDIAL INFARCTION (Ml)

IMMEDIATE MANAGEMENT

Guidelines: de Bono DP, Hopkins A. The management of acute myocardial infarction: guidelines and audit standards. Summary of a workshop organized by the Joint Audit Committee of the British Cardiac Society and the Royal College of Physicians of London 1993. Available: www.bcs.com or from the British Cardiac Society, 9 Fitzroy Square, London W1P 5AH. Weston CFM et al. Guidelines for the early management of patients with myocardial infarction. BMJ 1994; 308: 767-71. Braunwald E, Antman EM, Beasley JW et al. ACC/AHA guidelines for the management of patients with acute myocardial infarction, 1999 update. Circulation 1999; 100: 1016-30. Scottish Heart and Arterial Disease Risk Prevention (SHARP). Brief Guidelines for general practitioners giving thrombolytic therapy 2000, available from SHARP, Dept of Medicine, Ninewells Hospital & Medical School, Dundee DD1 9SY.

• Every District needs a policy, worked out between the Health Authority, hospital consultants, GPs and ambulance service, to ensure the provision of the most effective care. The British Heart Foundation Working Group proposes a maximum time of 90 minutes between the patient's first call and the administration of thrombolysis, if indicated. The ideal is for the GP and the ambulance to arrive together, within 1 hour of the onset of symptoms. A GP without an early ambulance entails delay. An ambulance alone cannot provide adequate analgesia and, unless it is a cardiac resuscitation ambulance, cannot provide other aspects of immediate care. Attendance by an ambulance within 1 hour of the onset of pain has been calculated to save 140 deaths over the next 30 days per 1000 patients.33

* Ensure that the ambulance has been called as an emergency by dialling 999. This may enable the service to respond faster than if the same call is telephoned direct to the ambulance control centre. Delay in reaching hospital is associated with a poorer outcome. Those reaching hospital in less than 1 hour from the onset of symptoms appear to derive the most benefit.35 * Insert an iv cannula so that more than one iv drug can be given easily. * Pain relief: For pain relief give either: (a) iv diamorphine 1 mg/min (maximum 5mg);or (b) iv morphine 2 mg/min (maximum 10 mg). Have nalorphine available in case of respiratory depression. These maximums can be repeated after 15 minutes if pain is not relieved. * Antiemesis: Give an antiemetic in the same syringe, e.g. metoclopramide 10 mg or cyclizine 50 mg iv. Avoid cyclizine in heart failure or hypotension because it causes arterial constriction. 'Cyclimorph' has the further disadvantage of being unrepeatable if more morphine is needed. Doses of cyclizine over 50 mg cause confusion and drowsiness. * GTN: Give as the spray or sublingually to oppose coronary vasoconstriction and to reduce myocardial work. Do not give if the pulse is over 100, or the systolic BP is under 90. * Oxygen: Give 100% oxygen routinely. * Bradycardia: If the pulse is below 40, give atropine 300 (ug iv and further doses of 300 ug if needed, up to 1.2mg, whether the bradycardia is due to heart block or to sinus bradycardia. If the pulse is between 40 and 60, only give atropine if there is evidence of low cardiac output.

MYOCARDIAL INFARCTION

* Aspirin: Give 150-300 mg, either in a chewable or dispersible form. This gives a 20% reduction in acute mortality (NNT = 40). * Defibtillation: Defibrillate a patient who develops ventricular fibrillation while awaiting transfer to hospital. If no defibrillator is available, perform cardio-pulmonary resuscitation while waiting for one to arrive. Approximately 5% of patients experiencing an acute myocardial infarction develop cardiac arrest in the presence of the GP. Out of hospital defibrillation has been shown to save lives.36 * Thrombolysis: Make an initial judgement about the patient's suitability. All patients should be considered for thrombolysis, regardless of age, if their quality of life warrants it. It is of benefit in the 24 hours after the onset of symptoms, with more benefit the sooner it is given. The overall decrease in mortality is 18% (NNT 56 but with a 'number needed to harm' (NNH) of 250 for stroke and 143 for an major extracerebral bleed). It is 50% if given in the first hour. The NNT is 25 if given in the first hour, rising to 33 if given in the first 6 hours, 50 if given in the first 9 hours and 100 if given in the first 18 hours. The benefit from aspirin is in addition to this. Thrombolysis involves hospital admission, whether or not it is given at home first. GPs need special training.37 It should be given outside hospital only if: (a) there is strong clinical suspicion of acute myocardial infarction; and (b) chest pain, unrelieved by GTN spray, has been present for at least 20 minutes and for no more than 12 hours; and (c) the ECG shows ST elevation or left bundle branch block; and (d) a defibrillator is available, because of the small but significant increase in risk of ventricular fibrillation (VF) after thrombolysis; and (e) no contraindications exist.

Strong contraindications to thrombolysis Review: French JK et al. Prospective evaluation of eligibility for thrombolytic therapy in acute myocardial infarction. BMJ 1996; 312:1637-41.

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(a) Trauma, major surgery or an invasive procedure in the last 2 weeks, including ribs fractured in cardiopulmonary resuscitation (CPR); or (b) Gastrointestinal or urinary bleeding in the last 3 months; or (c) Hypertension (systolic >180, diastolic >110); or (d) Traumatic or prolonged (>15 minutes) cardiopuLmonary resuscitation in the last 2 weeks; or (e) Stroke or TIA in the last 6 months that could have been haemorrhagic; or any other current intracranial pathology; or (f) Haemorrhagic diabetic retinopathy; or (g) Any other increased tendency to haemorrhage. * Even if thrombolysis is contraindicated, admit if: (a) the patient might be a candidate for immediate coronary artery surgery;38 (b) the patient is seen within 4 hours of the onset of pain (to ensure help is available if ventricular fibrillation occurs); (c) there is evidence of heart failure, shock or arrhythmias, including heart block; (d) chest pain is not relieved by opioids; (e) the patient lives alone or the relatives cannot cope; (f) the doctor is not able to provide proper follow-up (see below). * If thrombolysis is contraindicated, then those who are best treated at home (if well supported) are: (a) those ill with another terminal illness; (b) those first seen more than 4 hours after the onset of pain who are pain-free.

SUBSEQUENT HOME MANAGEMENT IF NOT ADMITTED * Revisit about 6 hours after the first visit, and again daily for a few days. * Explain what is happening and what the future programme is. * Check for pain, arrhythmias, hypotension and heart failure. Mild heart failure occurring from the second day onwards can be treated safely at home with diuretics. * Continue daily aspirin (75 mg) indefinitely.

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* ACE inhibitors. Give an ACE inhibitor: (a) to all patients in heart failure, even if the heart failure was only transient. This will reduce the mortality by 25-30% at 2 years39 and by 11% at 5 years40 with an NNT over the first 2 years of 17. (b) to all aged 55 and over if they are at high risk of death from cardiovascular disease. The benefit of this (relative risk reduction (RRR) for mortality = 16%, NNT = 56) was established by the HOPE study41 in which high risk was defined as having a history of vascular disease plus one other risk factor (hypertension, raised total cholesterol, low high-density lipoprotein (HDL) cholesterol, smoking or microalbuminuria). * Start a beta-blocker on the second day provided it is not contraindicated and, especially, provided there is no heart failure. This will reduce the mortality by 23% (95% CI 15% to 31%) with an NNT of 42 to prevent one death over 2 years.42 The drugs with most evidence of benefit are propranolol, timolol and metoprolol. Most of the benefit is in high-risk patients who have transient arrhythmias or heart failure and then recover well enough to take the drugs. * Pericarditis should be treated with aspirin at full doses, or, if aspirin is contraindicated, an NSAID, e.g. naproxen 500 mg b.d. Occasionally, NSAIDs are needed for up to a year. * Physical rehabilitation should be started after 12 to 24 hours if the patient is stable. Give the patient written instructions: 1. Start to walk about from day 2. 2. By day 7, climb stairs up to four times a day. 3. By day 14, stroll in the garden or street. 4. By week 4, walk1/2a mile a day, increasing to 2 miles a day by week 6. Resume sexual activities gently. 5. From week 6, increase the speed of walking so that 2 miles is covered in less than 30 minutes. Younger patients can then alternate walking and jogging. If a local rehabilitation group is available, the patient should be encouraged to attend from the fourth week.43 * Psychological rehabilitation. Much postinfarction disability is psychological or family-induced. Group sessions and sessions for spouses may be

as important as the exercises.44 Nearly half of all patients are depressed 1 week after an infarct, 18% with major depression.45

THE 6-WEEK ASSESSMENT * Ask about breathlessness and palpitations. * Look for signs of heart failure or arrhythmias and check the blood pressure. * Continue aspirin, at 75 mg daily. * Smoking. Encourage a smoker to stop with the news that stopping even at this late stage probably reduces mortality by 50%. * Exercise. Encourage the continuation of exercise, which will continue to improve cardiac performance and survival and reduce the risk of another infarction by 20%. Exercise can be tennis, jogging, cycling, swimming or circuit training. * Cardiac rehabilitation. Refer the patient if not already enrolled. Studies show reductions in cardiovascular deaths of 20-25% as well as improvements in the quality of life.46 * Weight. Advise the obese to lose weight. * Lipids. Arrange a test for serum lipids at 3 months postinfarction, unless the lipids were assessed within 24 hours of the onset of the infarct. Once blood has been taken, start a statin and continue long term (see p. 109). * Depression. Look again for depression. Even at 1 year, 25% are depressed. * Exercise ECG. Check that an exercise ECG has been arranged (unless contraindicated) with a view to a coronary angiogram and possible surgery if appropriate. CABG can reduce mortality over 5 years by 39% and over 10 years by 17% in selected patients with coronary heart disease and the figures are likely to be greater for patients post-Mi. The initial mortality, however, is higher than with medical treatment with a 45% increase in risk of death or MI in the first year. By the end of the second year the benefit outweighs the harm. Patients with a negative stress test are unlikely to require coronary artery surgery, and so an unnecessary angiogram can be avoided. * Check that the patient continues to take an ACE-inhibitor and a beta-blocker unless contraindicated. The benefit of beta-blockade does not appear to dimmish with time, with an NNT

HEART FAILURE 99

of 42 to save one life over 2 years (a reduction in risk of 23%).42

OTHER FACTUAL ADVICE * Sedentary workers may return to work at 4 to 6 weeks, light manual workers at 6 to 8 weeks and heavy manual workers at 3 months. * Do not fly for 2 weeks, and then only if able to climb one flight of stairs without difficulty. A more cautious policy is to advise against flying for 6 weeks. * Drivers should not drive for 1 month, but need not now notify the DVLA. They should inform their insurance company. HGV and PSV licence holders must notify the DVLA, and may only continue vocational driving after individual assessment.

(b) Myocardial infarction. (c) Severe complicating medical illness, e.g. pneumonia. (d) Inadequate social support. (e) Failure to respond to treatment. (f) ACE inhibitors indicated, and the patient is in one of the categories where they cannot be started at home (see below).

Referral to outpatients Refer patients: (a) aged less than 60; or (b) with a murmur; or (c) with angina; or (d) with arrhythmias; or (e) with severe heart failure; or (f) with renal impairment or other complicating factors; or (g) where the heart failure is unexpected.

HEART FAILURE GP MANAGEMENT Guideline: The Task Force of the Working Group on Heart Failure of the European Society of Cardiology. The treatment of heart failure. Eur Heart J 1997; 18: 736-53.

Investigations

• Consider referral to a cardiac failure clinic if one is available. Such clinics have been shown to achieve improvements in patient functioning and satisfaction with reductions in hospitalizations.48 Exercise training is an important part of any such programme, with the potential to reduce mortality by 63% (95% CI 16% to 83%).49

* Check Hb, creatinine, electrolytes, CXR, ECG and echocardiogram. The echocardiogram is needed to confirm the diagnosis of heart failure, to detect an unsuspected cause for the failure, e.g. valvular disease,50 as well as to quantify the degree of failure. Two 'rules of halves' appear to apply. Only about half of patients with left ventricular systolic dysfunction receive treatment for chronic heart failure and only half of these receive appropriate therapy. Conversely, only about half of patients receiving treatment for heart failure have left ventricular dysfunction when investigated and only half of those are on appropriate therapy.47 The echocardiogram could reasonably be omitted if both CXR and ECG were normal and there were no predisposing factors for heart failure. The chance of heart failure being present would be very small.51

Grounds for admission

General measures

(a) Severe symptoms, e.g. severe dyspnoea or hypotension.

* Explain what is going on and what to do if things get worse.

• Every patient deserves a full cardiac diagnosis. Clinically, it is easy to miss significant valvular and congenital disease with insignificant murmurs, constrictive pericarditis and non-cardiac causes for heart failure, especially drugs (e.g. steroids, non-dihydropyridine calcium-channel blockers and NSAIDs). • The prognosis is poor, with 5-year survivals of 25% for men and 38% for women.47

100 CARDIOVASCULAR PROBLEMS

* Recommend daily weighing. Patients should report if they gain more than 1.5kg over a short period. * Reduce weight, if obese. * Diet: advise a diet with no salty foods or added salt and, unless on ACE inhibitors, one high in potassium (e.g. milk, bananas, oranges, grapefruit juice, tomato juice and chocolate). * Restrict fluid intake to 2 litres a day, unless losing fluids from sweat or diarrhoea or vomiting. * Keep alcohol intake low. * Stop smoking; it causes vasoconstriction. * Rest in the acute phase, but exercise once stable. Moderate exertion (e.g. bicycling or brisk walking) can reduce dyspnoea in as little as 8 weeks.52 It should be carried out for 30 minutes a day on at least 5 days a week. Vigorous exertion should be avoided. * Vaccination: recommend influenza and pneumococcal vaccination. * Check for depression. It is present in one-third. CONGESTIVE CARDIAC FAILURE ACE inhibitors Guideline: Eccles M et al. North of England evidencebased development project: guideline for angiotensinconverting enzyme inhibitors in primary care management of adults with symptomatic heart failure. BMJ 1998; 316: 1369-75.

• All patients with heart failure should be taking an ACE inhibitor, with or without a diuretic, unless there is a contraindication. They increase the ability to exercise, improve well-being and prolong life in all degrees of heart failure.50 Their use can reduce mortality by 20% with greater benefits in the higher risk patients.53 • They may be used alone in patients with fatigue or mild dyspnoea on exertion without overt heart failure. • Exclude both clinically and from the investigations detailed above the absolute contraindications of: (a) aortic and mitral stenosis and outflow tract obstruction; (b) primary pulmonary hypertension;

(c) cyanotic congenital heart disease; (d) constrictive pericarditis; (e) renovascular disease; (f) severe or progressive renal failure; (g) pregnancy. * Refer to hospital, without initiating ACE inhibitors, patients with the following: (a) Severe heart failure, requiring >80mg of frusemide (or equivalent); (b) Low systolic blood pressure (systolic <100mmHg); (c) Low serum sodium (<130mmol/L) or high potassium (>5.5 mmol/L); (d) Possible hypovolaemia (low jugular venous pressure (JVP), recent diuresis or fluid loss, high diuretic dose or combined diuretic treatment); (e) Additional vasodilator treatment other than nitrates; (f) Renal dysfunction (serum creatinine >130 (umol/L) or known renal artery stenosis; (g) Severe diabetes mellitus with associated renal disease; (h) Severe generalized atherosclerosis (especially if intermittent claudication and arterial bruits are present); (i) Severe chronic obstructive airways disease and pulmonary heart disease (cor pulmonale). * Start ACE inhibitors at home, provided the above contraindications are not present.54 1. Check creatinine and electrolytes; 2. Stop potassium supplements or potassiumsparing diuretics; 3. Stop other diuretics for 24 hours and restart 24 hours after starting the ACE inhibitor; 4. Start with a low dose initially, e.g. captopril 6.25 mg, enalapril 2.5 mg, lisinopril 2.5 mg or ramipril 1.25mg. Advise the patient to take the first dose before going to bed to reduce the effect of first-dose hypotension. 5. Repeat the creatinine level after 1 week. A rise suggests renal artery stenosis. Stop the ACE inhibitor and refer. 6. Increase the dose weekly, with a check each time of BP and creatinine and electrolytes, until a dose is reached of captopril 50 mg t.d.s., or enalapril 10 mg b.d., or lisinopril 20 mg o.d., or ramipril 5mg b.d., or side-effects have

HEART FAILURE 101

prevented further increases. Lower doses have not been shown to prolong life. 7. Repeat creatinine and electrolytes at 3 months then 6-monthly or sooner if further dose titration is necessary or there is intercurrent infection. 8. Warn the patient to avoid dehydration, for instance in gastroenteritis or in hot weather, as this can cause a sudden deterioration in renal function. If the weight drops by more than 1 kg because of dehydration, the patient should stop the diuretic and drink more fluid. Note: Potassium-sparing diuretics may be restarted in the rare case where the serum potassium falls to 3.0 mmol/L or below on an ACE inhibitor. Note: Angiotensin-II receptor blockers may be used in patients intolerant of ACE inhibitors.55

Diuretics * Use a thiazide in mild failure, e.g. bendrofluazide 5 mg daily, but not in the very elderly or those with impaired renal function: they respond poorly. * Beyond that dose, or in more severe failure, use a loop diuretic (bendrofluazide l0mg is equivalent to frusemide 20 mg), which will cause less hypokalaemia and impotence than a high-dose thiazide. * Increase the dose gradually until diuresis occurs. Once stable, reduce the dose to as low as possible without oedema or breathlessness occurring. If diuresis does not occur with frusemide 80 mg/ bumetanide 2 mg, consider adding bendrofluazide 5-10 mg or metolazone 5-10 mg daily either for 3 days or long term. Their effect can be synergistic in the short term. Longer use may be associated with electrolyte disturbance. * Add spironolactone 25 mg daily to a loop diuretic and an ACE inhibitor in severe failure. It can reduce the mortality and morbidity by 30%.56 Hyperkalaemia is rare provided the creatinine is <220 umol/L. * In all patients, recheck the serum creatinine and electrolytes after 4 weeks and then 6-monthly. * Other potassium-sparing diuretics are indicated only in patients not suitable for ACE inhibitors.

Add a potassium-sparing diuretic in such patients: (a) who are on digoxin or otherwise prone to arrhythmias; (b) whose initial serum potassium is 3.0 mmol/L or below; (c) who have a potassium-losing condition, e.g. cirrhosis, nephrotic syndrome or chronic diarrhoea; (d) whose potassium falls to 3.0 mmol/L or below; (e) who are elderly or chronically sick, with a poor potassium intake; (f) who are diabetics; (g) who are on oral steroids; (h) who are oedematous, until they are no longer having a diuresis;57 or (i) who are on high doses of loop diuretics, e.g. frusemide 120 mg daily (or bumetanide 2 mg daily).58

Beta-blockers * Consider all patients for beta-blockade once heart failure is controlled, although the BNF currently recommends that treatment should usually be initiated by a consultant. Mortality is reduced by 32%, a benefit which exists for all degrees of severity.59 * Use a non-selective beta-blocker; they seem to be more effective than selective beta-blockers (RRR 49% vs 18%). * Start at a low dose, e.g. bisoprolol 1.25mg daily or metoprolol 6.25-12.5 mg b.d. or carvedilol 3.125 mg b.d. and double the dose every 2-4 weeks until the target dose is obtained (bisoprolol l0 mg daily, metoprolol 150mg daily in divided doses, carvedilol 25 mg b.d.). Symptoms frequently worsen initially then subsequently improve. * Continue ACE inhibitors and diuretics.

Digoxin • Digoxin is indicated in: (a) atrial fibrillation (see p. 105); (b) in heart failure persisting despite optimum therapy with diuretics, ACE inhibitors and

102 CARDIOVASCULAR PROBLEMS

beta-blockers. Digoxin should be given in combination with an ACE inhibitor, and probably needs to be at the upper end of the therapeutic level. Its use can reduce the need for admission without improving mortality.60 * Less than half of all patients on digoxin have levels in the therapeutic range.61 Procedure 1. Give 0.25 mg daily or 0.0625-0.125 mg in the elderly or those with renal impairment. 2. Check digoxin level (see Appendix 21) if in sinus rhythm and serum potassium: (a) 1 month after starting treatment; and (b) if renal function changes. Regular checks of serum creatinine and electrolytes are then needed; or (c) if a drug is added which decreases digoxin elimination, e.g. verapamil, quinine or amiodarone. 3. Aim for a blood level of 1-2 nmol/L, although some further benefit may be obtained from doses up to 3.8 nmol/L without toxicity.62

INTRACTABLE HEART FAILURE * Reconsider the diagnosis. Does the patient have: (a) thyroid disease; (b) infective endocarditis; (c) pulmonary infection; (d) ventricular dysrhythmia; (e) pulmonary thromboembolism; (f) inappropriate bradycardia and a need for pacing. * Review the treatment. Is there: (a) overtreatment with diuretics causing uraemia; (b) non-compliance with drugs; (c) alcohol overuse. * Is there a role for surgery, e.g. valvular surgery, CABG or transplant?

END-STAGE HEART FAILURE There comes a point when the decision has to be made whether treatment of heart failure can be

optimized further or whether care should now be palliative. Individual symptoms may be treated as follows; in addition, the patient may be referred to a multiprofessional palliative care team.

Breathlessness * Still mobile. Consider oral dihydrocodeine 0.5-1 mg/kg. This suppresses respiratory drive and improves dyspnoea but does not improve fatigue.63 * Breathless at rest. Consider oxygen therapy at home (40-80% if there is no COPD).64 Morphine is the most effective palliation. Give a low oral dose (2.5-110mg 4-hourly) as peak plasma levels are higher in heart failure. Nebulized morphine is effective in controlling dyspnoea.

Weakness and fatigue This is usually secondary to a low cardiac output. * Diuretics. Consider reducing diuretics, especially if the serum sodium is low. * Depression. Avoid tricylics; SSRIs may be of value. * Social factors. Increase social care if possible.

Anorexia/nausea * Digoxin. Check serum level and renal function. Consider increasing diuretics if there is hepatic congestion. * Diet. Advise the patient to have small, appetizing meals more frequently. Allow small amounts of alcohol before meals.

Oedema * Mobilization is advisable in theory but rarely practicable at this stage. * Fluid intake. Restrict the fluid intake to 1.52 litres a day with a low salt intake. * Diuretics. Avoid increasing diuretics at this stage. They have little effect on reducing the oedema. * Be cautious about advising the patient to raise the feet. This may increase the venous return to the heart and worsen the dyspnoea.

ARRHYTHMIAS

* Compression stockings/bandages. These have risks of increasing tissue damage and are uncomfortable.

ACUTE PULMONARY OEDEMA 1. Sit the patient up with the legs down. 2. Give oxygen if available. 3. Give iv diamorphine 1 mg per minute up to 5 mg, or iv morphine 2 mg per minute up to 10 mg, mixed with metoclopramide l0 mg iv. Do not use cyclizine; it raises systemic arterial pressure. 4. Give a GTN tablet or spray sublingually (for its immediate vasodilator effect). 5. Give a loop diuretic (iv frusemide 40 mg or iv bumetanide 1 mg). * Look for causes of the heart failure that may need treatment in their own right, especially myocardial infarction. * Admit once the patient is sufficiently stable, for fuller assessment and for continuing treatment. Note: Digoxin and aminophylline should not be used in acute failure outside hospital.

RIGHT HEART FAILURE Acute right heart failure * The clinical picture of low output state, no pulmonary oedema and a raised JVP may be due to massive pulmonary embolism or to acute right heart failure as in a right ventricle myocardial infarction, or acute cor pulmonale. * Admit urgently. Position the patient however is most comfortable. Do not give morphine; respiratory depression and hypotension are very likely to follow. Do not give diuretics: they may precipitate shock.65

Chronic cor pulmonale * Treat the lung disease. Consider: (a) antibiotics for exacerbations of infection; (b) inhaled bronchodilators and steroids via a spacing device (see p. 124); (c) physiotherapy; (d) long-term oxygen therapy (see p. 126);

103

(e) nasal intermittent positive pressure ventilation for those with thoracic deformities and obstructive sleep apnoea. * Admit readily anyone with an acute exacerbation. Oxygen will dilate the pulmonary vessels and so reduce the pulmonary artery pressure. The danger of hypercapnoea makes this hazardous in the acute situation at home. * Use diuretics carefully as they may reduce renal blood flow further. They are only required if oedema becomes troublesome. * Use digoxin only if the patient is in atrial fibrillation. * Do not start ACE inhibitors outside hospital.

ARRHYTHMIAS Arrhythmias can be treated only after accurate diagnosis. * Perform an ECG in a patient with an established arrhythmia or with a paroxysmal arrhythmia who is currently having an attack. In patients with paroxysmal arrhythmias who are between attacks, the history often suggests the diagnosis. * Ask the patient to tap out the rhythm. Patients with attacks of regular tachycardia can usually be accurate to within 10 beats per minute. * If the history suggests an arrhythmia and it is occurring at least once every 24 hours, refer for continuous ambulatory monitoring. A cardiomemo is more helpful when arrhythmias are infrequent, allowing the patient to transmit an ECG by telephone during an attack.

ECTOPIC BEATS IN A NORMAL HEART * Explain their benign nature to the patient. Advise against caffeine, fatigue, smoking and alcohol. * Beta-blockers should be used only if the patient is unable to tolerate the ectopics. * frequent ventricular ectopics (more than 100 per hour) may be grounds for referral. Patients may have a prolapsing mitral valve, or the older patient may have unsuspected ischaemic heart disease.66

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ATRIAL FIBRILLATION (AF) Review: Drug and Therapeutics Bulletin. The anti-arrhythmic treatment of atrial fibrillation. Drug and Therapeutics Bulletin 1996; 34: 41-45.

continued in a patient in whom AF does not recur is not clear; 6 months is a minimum but 5 years may be safer.69

Anticoagulation Work-up (a) History of alcohol intake (either chronic or bingeing); (b) Blood pressure (half of all cases of AF are hypertensive); (c) TFTs and urea and electrolytes (U & Es); (d) ECG (looking for ischaemic heart disease (IHD), LVF and delta waves); (e) CXR (for cardiomegaly); (f) Echocardiogram (for dilated atria or ventricles).

* Admit if rapid AF is associated with: (a) chest pain; (b) hypotension; or (c) more than mild heart failure. * Refer urgently if seen within 48 hours of the onset of AF. A single oral dose of amiodarone (30mg/kg) may achieve cardioversion within 24 hours in 87% against 35% with placebo.67 * Refer to outpatients if: (a) the patient is a candidate for direct current (DC) or chemical cardioversion. This is most likely to succeed in those with no, or mild, organic heart disease, the young, and those with a short history. Anticoagulants will be needed for 3 weeks before the procedure and at least 4 weeks after it, unless the onset is within the previous 48 hours; or (b) the patient is a candidate for anticoagulation (see below); or (c) there is uncertainty about the diagnosis or treatment; or (d) symptoms are uncontrolled by the treatment plan below. * If successfully cardioverted, check that the patient is taking treatment to prevent recurrence. AF will recur in half within 3-6 months. Amiodarone appears the most effective preventive drug, with recurrence occurring in 35% against 63% for sotalol over a mean of 16 months follow-up but the choice depends on whether heart disease is present and whether adverse effects occur.68 How long treatment should be

• All patients with atrial fibrillation are at increased risk of thromboembolism. Overall, atrial fibrillation increases the risk of stroke five times. • In separate placebo-controlled trials, warfarin reduces that risk by two-thirds, but at the expense of seven episodes of severe bleeding for every 25 strokes prevented.70 That equals a rate of major bleeding of 1.3% per year compared to 1% in controls and those on aspirin. • In placebo-controlled trials aspirin may reduce the risk of embolic stroke by 20%.71 In a systematic review of head-to-head trials of warfarin versus aspirin no evidence of superior benefit from warfarin was found,72 but it has been criticized for the trials that were included and excluded. A pragmatic trial in primary care in Holland was also unable to show a difference between aspirin and warfarin.73 • The decision about whether to take warfarin or aspirin must be made with each patient and based on his or her risk. When patients are given information about their risk, roughly half the patients wish to take, or not take, warfarin in a way that is contrary to current guidelines.74 • Using the evidence of placebo-controlled trials, patients may be divided into four categories according to their risk:75-77 (a) Very high risk: rheumatic heart disease or dilated cardiomyopathy. The risk of embolic stroke is >15% per year. Warfarin reduces that risk by 66% with an NNT for 1 year of 12. (b) High risk (history of TIA or stroke, coronary artery disease, or with congestive cardiac failure (CCF) or impaired left ventricular function, or age 75 or over with diabetes or hypertension). The annual risk of stroke is 12%. The recommended treatment is warfarin, which reduces the risk to 4% (NNT = 13). (c) moderate risk (age 65 or over; or age <65 with diabetes or hypertension): the annual risk of stroke is 4% (rather less in those 65-75, rather

ARRHYTHMIAS

more in those over 75). The choice is between warfarin, which reduces that risk to 1.3% (NNT 38) or aspirin, which reduces the risk to 3.2% (NNT 125). (d) Low risk (age <65 without any of the above risk factors): the annual risk of stroke is 1%. For warfarin the NNT is 200 and for aspirin it is 500. Patients are likely to choose aspirin or no treatment. * Consider also the following factors when making a decision about anticoagulation: (a) haemorrhage on warfarin is more common: -in patients aged >75; -when the international normalized ratio (INR) is >3; -when the INR fluctuates; - in uncontrolled hypertension. (b) Upper age limit. Opinion is divided over whether, after the age of 80, the risks from anticoagulation outweigh the benefits. British Heart Foundation Factfile78 comes down against any cut-off point, while DTB79 favours it at age 80. (c) History of previous embolism, however minor. Anticoagulation is essential; the benefit is increased threefold.70 (d) Paroxysmal AF. This carries the same risk of embolism as when AF is sustained. Do not use digoxin; paroxysms may become more frequent and prolonged. Verapamil is ineffective. Use sotalol if necessary while awaiting assessment.80 Note: Echocardiography. Lone AF should not be diagnosed without echocardiography for exclusion of unsuspected valvular disease or chamber dilatation.

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250 ug daily. Halve this if the serum creatinine is raised. A dose of 62.5 ug daily will be too low unless there is severe renal impairment. 5. Check the serum level before exceeding 500 ug daily (or 250 (ug daily in the elderly). 6. If therapeutic levels do not control the ventricular rate, add a low dose of a beta-blocker or verapamil. Note: Digoxin will not control the rate on exercise or in other situations when sympathetic outflow is high. Check that the patient is calm and resting before taking further action on the basis of a high rate. Special cases (a) Thyrotoxicosis. All patients with otherwise unexplained AF should have their thyroid function checked. Control the rate with beta-blockers temporarily. They are likely to need cardioversion once the thyrotoxicosis is controlled. (b) Recent onset. If the onset was within the last year, cardioversion may be considered. (c) Wolff-Parkinson-White syndrome. Digoxin is contraindicated. (d) Sick sinus syndrome. Digoxin, beta-blockers and calcium antagonists may worsen the situation. Pacing is required.

ATRIAL FLUTTER Treat as atrial fibrillation.

PAROXYSMAL SUPRAVENTRICULAR TACHYCARDIA

Treatment of established AF in general practice

If the patient is seen during the attack:

1. Give aspirin 75 mg daily, but only after discussion with the specialist if the patient is on warfarin. 2. Give a loading dose of digoxin 0.5-1.5 mg (in divided doses over 24 hours), depending on the patient's weight and renal function. 3. Check the creatinine and electrolytes; then 4. Give enough digoxin to control the ventricular rate. In an average-sized patient, start with

* Apply carotid sinus massage except where the patient: (a) is elderly; or (b) has ischaemic heart disease; or (c) is likely to be digoxin toxic; or (d) has a carotid bruit; or (e) has a history of transient ischaemic attacks. * Admit if the attack continues and there is no clear history of previous attacks which have

106

CARDIOVASCULAR PROBLEMS

terminated themselves. Even if there is such a history, keep the patient at the surgery until the attack does indeed terminate. * If diagnosed from the patient's history or if the attack has terminated before admission was needed: (a) refer for specialist confirmation and initiation of treatment. Referral should be urgent if attacks are associated with chest pain, dizziness or breathlessness; (b) if the patient is distressed by frequent attacks, start sotalol 80-320 mg daily while awaiting the cardiologist's opinion. Sotalol has an amiodarone-like action as well as being a beta-blocker. A final decision on the most appropriate drug will depend on the electropathology in the individual patient; (c) if sotalol is contraindicated, use verapamil 40-80 mgt.d.s.; (d) instruct the patient in the use of the Valsalva manoeuvre, and check that he or she is not smoking or abusing alcohol or caffeine.

VENTRICULAR TACHYCARDIA * Admit immediately for cardioversion. If the patient is conscious but in extremis, give iv lignocaine l00mg while waiting for the ambulance. If the patient is unconscious from circulatory collapse, give a DC shock as for defibrillation. Accompany the patient to hospital. After discharge, prophylaxis will be needed. If amiodarone is chosen, 6-monthly TFTs and LFTs are necessary.

SICK SINUS SYNDROME This requires pacing. Drugs are likely to make symptoms worse because of the variability of the rhythms.

BRADYCARDIA * Refer all patients with a bradycardia, other than sinus bradycardia, even if asymptomatic. A pacemaker is likely to be needed and may be lifesaving. Untreated second degree and complete atrio-ventricular (AV) block have a mortality of 25-50% in the first year after diagnosis. For this

reason, even asymptomatic patients with a rate of 40 or below should be paced. * Admit a patient in acute AV block with hypotension due to the bradycardia. Give iv atropine while waiting for the ambulance.

ANTIBIOTIC PROPHYLAXIS OF INFECTIVE ENDOCARDITIS Prophylaxis is needed for procedures that cause bacteraemia, in patients with: (a) congenital heart disease (except atrial septal defect); (b) rheumatic and degenerative valve lesions, including the prolapsing mitral valve; (c) prosthetic heart valves and arterial grafts; (d) a recent myocardial infarction. The procedures which require prophylaxis are usually dental. They include extraction, scaling, gum surgery and any other dental treatment which causes bleeding. The current recommendation is that patients with prosthetic joints do not need antibiotic prophylaxis unless they are at especial risk.81 Recommendations are based on those of the British Society for Antimicrobial Chemotherapy:82 (a) amoxycillin 3 g orally 1 hour before the procedure; (b) if allergic to amoxycillin, use clarithromycin or azithromycin 500 mg 1 hour before or clindamycin 600 mg orally 1 hour before.

Special situations * If amoxycillin has already been given twice in 1 month and a third procedure is necessary, use clarithromycin or azithromycin. * General anaesthetic (GA): If a general anaesthetic is used, give: (a) amoxycillin 3g 4 hours before and 3g immediately after; or (b) amoxycillin 3g and probenicid 1g orally 4 hours before; or (c) amoxycillin 1 g iv immediately before.83

THE PREVENTION OF CORONARY HEART DISEASE

* Certain patients requiring a GA are better managed in hospital. These are patients: (a) with prosthetic valves; (b) with a history of endocarditis; (c) who have already received two doses of amoxycillin in the last month; (d) who are allergic to amoxycillin.

Other procedures in vulnerable patients Certain other procedures in general practice require prophylaxis only in especially vulnerable patients, namely those with prosthetic valves or a previous episode of endocarditis. These procedures are: (a) the insertion or removal of an intrauterine contraceptive device (IUCD); (b) proctoscopy, sigmoidoscopy or barium enema; (c) catheterization of a patient with infected urine. * Give: (a) amoxycillin 3g orally and gentamicin 120mg im 1 hour before the procedure; or (b) if the patient is allergic to amoxycillin, admit for iv vancomycin and gentamicin. Patient information: Cards listing these recommendations, which can be given to patients, are obtainable from The British Heart Foundation, 14 Fitzhardinge Street, London W1H 6DH, tel. 020 7935 0185 or may be ordered free on www.bhf.org.uk (choose 'publications' then search on 'endocarditis').

THE PREVENTION OF CORONARY HEART DISEASE (CHD) Guidelines: Wood D et al. Joint British recommendations on prevention of coronary heart disease in clinical practice. Heart 1998; 80 (Suppl. 2): S1-S29. Online. Available: http://www.hyp.ac.uk/bhs Department of Health. The National Service Framework for Coronary Heart Disease. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/nsf/coronary.htm

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National Institute for Clinical Excellence. Prophylaxis for patients who have experienced a myocardial infarction: drug treatment, cardiac rehabilitation and dietary manipulation, 2001. Online. Available: www.nice.org.uk

• Risk factors for CHD do not just add to each other, they multiply. • Risk factors tend to cluster. The presence of one or more of the following increases the chance that other risk factors will also be present: hypertension, raised cholesterol, inactivity, obesity, smoking and glucose intolerance.84 • The most cost-effective intervention is in those who already have CHD. However, lifestyle changes across the whole population would also achieve a major reduction in CHD, especially in groups at high risk (e.g. British South Asians). • According to the yet-to-be published MRC/ BHF Heart Protection Study (2002), cholesterol lowering with simvastatin lowers the risk of a major cardiovascular event by at least one-third, regardless of age, sex and initial cholesterol level. In a patient whose risk of CHD is high, reducing that risk by one-third is worthwhile. In a patient at low risk of CHD, the same reduction would lead to a much smaller health gain. The NNT over 5 years for a patient with a previous myocardial infarction (MI) is 10, with angina it is 13, with a previous stroke, or peripheral vascular disease (PVD) it is 15; for diabetics it is 15. • The UK National Service Framework (NSF) does not recommend the screening of the whole population but urges the concentration of effort on those most at risk. • A systematic approach has been shown to provide more effective care in the management of chronic conditions than an opportunistic approach in some studies but not in others. A nurse-run secondary prevention clinic can improve management in relation to aspirin use, blood pressure control, lipid management, exercise and diet compared to 'usual care' from a GP.85 Systematic care entails a register from which patients can be called and recalled, an agreed management protocol, a computer template or data entry form for the uniform recording of results and, usually, a dedicated clinic. However, a randomized

108 CARDIOVASCULAR PROBLEMS

controlled trial of systematic care in Warwickshire showed that, while it improved follow-up and assessment, it made little impact on clinical care.86

PRIMARY PREVENTION General measures * Stop smoking. Someone who smokes 20 cigarettes a day or less and stops has a risk of CHD 10 years later almost the same as in one who has never smoked.87 Nicotine replacement therapy increases by 1.6 the chance that the patient is not smoking at least 6 months later. This is true for all forms of nicotine replacement and seems to be independent of whether support, other than brief advice, is offered.88 * Alcohol. Keep alcohol intake within safe limits. A higher intake raises low-density lipoprotein (LDL) cholesterol, triglyceride, blood pressure and calorie intake. Modest alcohol intake (1-3 units a day) appears to protect against CHD (relative risk (RR) 0.76, 95% CI 0.64 to 0.91),89 but all-cause mortality is unchanged and promoting alcohol for this purpose could be hazardous. * Take exercise. This must be vigorous (for 20 minutes, three times a week) or moderate, e.g. brisk walking (for 30 minutes five times a week).90 The exercise may be broken into separate bouts as long as they are for at least 10 minutes each. In the short term, the risk of MI is halved91,92 with less risk of death if it does occur, but this benefit is lost if exercise is stopped. Exercise continued for over 1 year improves the coronary collateral circulation enough to be demonstrable on a thallium scan. Simple advice from the GP is unlikely to change behaviour. A more supportive programme is needed. One study, using four visits to the physiotherapist, significantly increased activity even though the patients were women over 80 years old.93 * Manage stress. Stress at work increases the risk of cardiovascular disease, especially where demands on the worker are high but the person's ability to control the situation is low and where social support is also low.94 * Control weight. Central obesity, rather than a raised body mass index (BMI), carries the greater

cardiovascular risk and can be assessed by measuring the waist circumference, with a single cut-off point for each sex. Men with a waist circumference over 94cm and women over 80cm are likely to have other risk factors for cardiovascular disease, and men with a waist circumference over 102 cm (women over 88cm) are two-and-a-half to fourand-a-half times as likely to have other major cardiovascular risk factors.95 Small losses of weight are possible in primary care if advice on diet and exercise is accompanied by a behavioural programme. * Diet. Encourage patients to reduce their fat intake and to eat oily fish, bread, pasta, potatoes, at least five portions a day of fruit or vegetables and to use olive oil and rape seed margarine. For more detailed advice, see page 443. The benefit for an individual of a low fat diet is small, at best, but the potential benefit to a population is large.96 Even in a dietician-run programme, the fall in serum cholesterol is unlikely to be more than 3%97 or 0.3mmol/L. Some studies show that a dietician is more effective than a doctor or nurses.98 Simple advice in primary care is unlikely to achieve even 3% reduction. The effect of fruit and vegetables may be greater than the benefit from the low fat diet. One cohort study found that each extra serving of fruit or vegetable a day was associated with a 4% reduction in the risk of coronary heart disease with no apparent upper limit.99 * Cholesterol. Measure the serum cholesterol in those with other risk factors e.g. diabetes or hypertension. Calculate the 10-year risk of coronary heart disease using the Cardiac Risk Assessor computer program (available on www.hyp.ac. uk/bhs) or use the charts published with the Joint Recommendations. Primary prevention trials show that statins reduce the risk of a major coronary event by 34%100 but that reduction is only worthwhile if the baseline risk is high. If the risk is >30%, the NSF recommends a statin and dietary advice to lower the cholesterol to the same target as for secondary prevention (see the box on p. 110). Once those at >30% risk have been treated, the NSF recommends treatment of those at >15% risk, if resources allow. Those with risk factors but who did not reach the level of risk at which a statin is recommended should have their risk reassessed 5 yearly.

THE PREVENTION OF CORONARY HEART DISEASE

* Familial hyperlipidaemia. Check the family history for CHD. Familial hyperlipidaemia is present in 1 in 500 of the population in the UK and for men carries a 50% risk of a major coronary event by the age of 60. In women the risk is 30%.101 Suspect it if: (a) the total cholesterol is >7.5mmol/L (>6.7 in children <16) or the LDL-cholesterol (LDL-C) is >4.9mmol/L (>4.0 in children <16); and (b) the patient or a first or second degree relative has tendon xanthomas; or (c) a first degree relative had a myocardial infarction before the age of 60 or a second degree relative before the age of 50; or (d) a first or second degree relative has a total cholesterol >7.5mmol/L. Refer all suspected cases to a lipid clinic. * Blood pressure. All adults should have their blood pressure checked at least every 5 years. If they are at high risk of CHD (>15% risk over 10 years) their blood pressure should be treated if it is 140/90 or over and should be controlled to < 140/85; otherwise treatment should start if the blood pressure is 3=160/100 and should be lowered to <140/90.7 * Aspirin reduces cardiovascular events by 15% but at a cost of increasing episodes of bleeding by 69%.102 Benefit outweighs risk when the patient's risk of a major coronary event is at least 15% over 10 years. Work-up of a patient with a raised cholesterol * Re-check fasting lipids at least once. If results vary, use the average. * If blood is taken within 3 months of a major illness, re-check outside the 3-month period. Illness can depress the cholesterol. * If the cholesterol is still raised, exclude a primary cause: (a) Check the history for alcohol excess and drugs, e.g. high-dose thiazides, beta-blockers without ISA, oral contraceptives containing levonorgestrel, retinoids and corticosteroids; (b) Test the urine for sugar and protein; (c) Take blood for TFTs, sugar, urea and LFTs.

Doubt has, however, been cast on the costeffectiveness of biochemical screening for underlying causes, which in British general practice

109

yielded 1.8% of positives only in patients with a cholesterol over 6.5.103 A compromise would be to check the fasting blood sugar in all patients and TFTs in patients with very high cholesterol levels, e.g. over 8mmol/L.

SECONDARY PREVENTION * Recommend the lifestyle changes detailed above. They are even more important than in primary prevention; exercise in patients with coronary heart disease is associated with a 27% reduction in mortality (95% CI 2% to 46%).104 A Mediterranean diet has been shown to reduce the risk of cardiac death and non-fatal myocardial infarction by 72% (95% CI 47% to 85%) over 4 years after an initial myocardial infarction compared to a prudent western diet.105 * Give aspirin 75 mg daily for life; if contraindicated use clopidogrel 75 mg daily. * Perform the full work-up (see box above) initially, then continue regular checks for blood cholesterol and sugar, e.g. yearly. * Check BP yearly and treat if it is 140/90 or above. Maintain it below 140/85. * Prescribe a statin according to the NSF recommendations (see box below). Overall, trials of secondary prevention show that statins reduce the risk of a major coronary event by 30% and the risk of death by 23%.100 The lower thresholds for treatment (i.e. total cholesterol <5mmol/L) would be justified in British Asians,106 those with familial hyperlipidaemia, those with a very strong family history of premature CHD and those with low HDL cholesterol levels. * Refer patients with a very high triglyceride (e.g. >5.0mmol/L) to a lipid clinic. They may benefit from a fibrate rather than, or in addition to, a statin.107,108 * Prescribe a beta-blocker to all those who have had an MI (see p. 98). Start them in patients whose MI was in the last 5 years and who therefore missed the opportunity to have them started in the acute stage. Patients at the highest risk benefit most from beta-blockade, e.g. those aged >50 with angina, hypertension or heart failure. The reduction in mortality risk (RRR 20%, NNT 48) appears to be long term.

110 CARDIOVASCULAR PROBLEMS

* Prescribe an angiotensin converting enzyme (ACE) inhibitor to any patient who has had an MI and who has heart failure or left ventricular systolic dysfunction. Also consider them for patients who meet the criteria of the HOPE study (see p. 98). * Diabetes. Patients with diabetes should have their blood sugar and blood pressure meticulously controlled, with a target for the latter of < 140/80 or below (see p. 90). Lipids and their treatment: The NSF recommendations for secondary prevention The targets: (a) lower the total cholesterol to <5 mmol/L or by 20-25% if that results in a greater fall; or (b) lower the LDL cholesterol to <3 mmol/L or by 30% if that results in a greater fall.109 The threshold for treatment: no clear guidance is given as to whether patients whose untreated total cholesterol is <5 mmol/L should be given statins. The National Institute for Clinical Excellence (NICE) guideline states that the position is unclear but that research evidence exists for treating those with a baseline total cholesterol of 4 mmol/L or above.36 When published, the MRC/BHF Heart Protection Study results are likely to lower this figure. The potential benefit: • Overall, in secondary prevention, the reduction in coronary risk is at least one-third. • Age. Initial thoughts that the importance of raised cholesterol as a risk factor seems to diminish in the very old110 appear to have been countered by the MRC/BHF Heart Protection Study (2002), which found that benefit was undiminished in patients at least up to the age of 80. • The reduction of risk is of most benefit to those at greatest risk. This includes elderly patients aged 65-74

whose benefit in the CARE trial was double that of patients <65 in terms of hospitalizations. Scandinavian patients with CHD aged 35-70 with a total cholesterol 5.5-8.0 mmol/L have a 28% risk of a major coronary event over 5.4 years, which can be reduced to 19% by simvastatin. This is a relative risk reduction of 34%, an absolute risk reduction of 9% and an NNT over 5 years of 12.111

Using a statin (a) Start at the lowest dose that has been shown to be useful in clinical trials, e.g. simvastatin 20 mg daily. (b) Review 3-monthly, with fasting lipids and LFTs, increasing the statin dose until the target level, or the maximum dose, is reached. (c) Thereafter, check cholesterol and LFTs annually. Note: If there is conflict between the total cholesterol and the LDL cholesterol levels, base treatment on the LDL result.

Raised triglyceride levels Less agreement exists on what levels should be treated:112,113 * Refer for treatment if the level is very high, e.g. over 10 mmol/L, to prevent acute pancreatitis. * Refer for assessment patients with levels over 5.0 mmol/L. * Use drug treatment for hypercholesterolaemia more readily if the triglyceride is raised as well.

REFERENCES 1. American Heart Association. 1999 heart and stroke statistical update. Dallas: American Heart Association, 1999. 2. Wilber JA, Barrow JG. Hypertension: community problem. Am J Med 1972; 52: 653-63. 3. Smith WCS, Lee AJ, Cromby IK et al. Control of blood pressure in Scotland: the rule of halves. BMJ 1990; 300: 981-3. 4. Cranney M, Barton S, Walley T. The management of hypertension in the elderly by GPs in Merseyside: the rule of halves revisited. Br J Gen Practice 1998; 48:1146-50. 5. Simon JA. Treating hypertension: the evidence from clinical trials. BMJ 1996; 313: 437-8.

6. Gueyffier F, Bulpitt C, Boissel J-P et al. Antihypertensive drugs in very old people: a subgroup meta-analysis of randomised controlled trials. Lancet 1999;353: 793-6. 7. Ramsay LE et al. Guidelines for management of hypertension: report of the third working party of the British Hypertension Society. J Hum Hyper ten 1999; 13: 569-92. 8. Wood D et al. Joint British recommendations on prevention of coronary heart disease in clinical practice. Heart 1998; 80 (Suppl. 2): S1-S29. 9. Collins R et al. Blood pressure, stroke and coronary artery disease. Part 2: Short-term reductions in blood

REFERENCES 111

pressure: overview of randomized drug trials in their epidemiological context. Lancet 1990; 335: 827-38. 10. MeReC. Essential hypertension - management guidelines. MeReC Bulletin 1993; 4: 25-8. 11. Stevens VJ, Obarzanek E, Cook NR. Long-term weight loss and changes in blood pressure: results of the Trials of Hypertension Prevention, phase 2. Ann Intern Med 2001; 134: 1-11. 12. Vollmer WM, Sacks FM, Ard J et al. Effects of diet and sodium intake on blood pressure: subgroup analysis of the DASH-sodium trial. Ann Intern Med 2001; 135:1019-28. 13. Cleroux J, Feldman RD, Petrella RJ. Recommendations on physical exercise training. Can Med Assoc J1999; 160 (Suppl. 9): S21-8. 14. Hansson L, Zanchetti A, Carruthers SG et al. Effects of intensive blood-pressure lowering and low-dose aspirin in patients with hypertension: principal results of the Hypertension Optimal Treatment (HOT) randomised trial. Lancet 1998; 351: 1755-62. 15. Blood Pressure Lowering Treatment Trialists' Collaboration. Evidence of ACE inhibitors, calcium antagonists, and other blood pressure lowering drugs: results of prospectively designed overviews of randomised trials. Lancet 2000; 356:1955-64. 16. Lakshman MR, Reda DJ, Materson BJ et al. Diuretics and beta-blockers do not have adverse effects at 1 year on plasma lipid and lipoprotein profiles in men with hypertension. Arch Intern Med 1999; 159: 551-8. 17. Curb JD et al. Effect of diuretic-based antihypertensive treatment on cardiovascular disease risk in older diabetic patients with isolated systolic hypertension. JAMA 1996; 276: 1886-92. 18. Carlsen JE, Kober L. Relation between dose of bendrofluazide, antihypertensive effect, and adverse biochemical effects. BMJ 1990; 300: 975-8. 19. Grimm RH et al. Long-term effects on sexual function of five antihypertensive drugs and nutritional hygienic treatment in hypertensive men and women. Hypertension 1997; 29: 8-14. 20. Psaty BM et al. Health outcomes associated with antihypertensive therapies used as first-line agents. JAMA; 277: 739-45. 21. Beevers DG, Sleight P. Short-acting dihydropyridine (vasodilating) calcium channel blockers for hypertension: is there a risk? BMJ 1996; 312: 1143-5. 22. Rufus AB et al. The effect of nifedipine on arterial pressure and reflex cardiac control. Circulation 1983; 67:1084-90. 23. Choudri AH, Cleland JGF et al. Unsuspected renal artery stenosis in peripheral vascular disease. BMJ 1990; 300: 1197-8. 24. DTB. Losartan - a new antihypertensive. Drug Ther Bull; 1995: 73-4. 25. Doll R, Peto R. Mortality in relation to smoking: twenty years observations on male British doctors. BMJ 1976; 2:1525-36. 26. Juul-Moller S et al. (for the SAPAT group). Doubleblind trial of aspirin in primary prevention of myocardial infarction in patients with chronic stable angina pectoris. Lancet 1992; 340: 1421-5. 27. British Heart Foundation Factfile. When to refer patients with angina. London: British Heart Foundation, 1986.

28. Elder AT, Shaw TRD et al. Elderly and younger patients selected to undergo coronary angiography. BMJ 1991; 303: 950-3. 29. IONA. Effect of nicorandil on coronary events in patients with stable angina. Lancet 2002; 359: 1269-75. 30. Natarajan M. Unstable angina. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. 31. Maynard SJ et al. Management of acute coronary syndromes. BMf 2000; 321: 220-3. 32. Johnston FA, Spyt T, Reece I et al. CABG in the elderly: the Glasgow experience. Gerontology 1989; 35:165-170. 33. DTB. Tackling myocardial infarction. Drug Ther Bull 2000; 38. 34. Zijlstra F, Hoorntje JCA, de Boer M-J et al. Long-term benefit of primary angioplasty as compared with thrombolytic therapy for acute myocardial infarction. New EnglJMed 1999; 341: 1413-19. 35. The United Kingdom Heart Attack Study (UKHAS) Collaborative Group. Effect of time from onset to coming under care on fatality of patients with acute myocardial infarction: effect of resuscitation and thrombolytic treatment. Heart 1998; 80:114-20. 36. Pai GR, Haites NE, Rawles JM. One thousand heart attacks in Grampian: the place of CPR in general practice. BMJ 1987; 294: 352^. 37. DTB. Should general practitioners give thrombolytic therapy? Drug Ther Bull 1994; 32: 65-6. 38. DTB. Angioplasty in the management of coronary artery disease. Drug Ther Bull 1993; 31: 45-7. 39. AIRE investigators. Effect of ramipril on mortality and morbidity of survivors of acute myocardial infarction with clinical evidence of heart failure. Lancet 1993; 342: 821-8. 40. Hall AS et al. Follow-up study of patients randomly allocated to ramipril or placebo after acute myocardial infarction: AIRE Extension (AIREX) study. Lancet 1997; 349: 1493-7. 41. The Heart Outcomes Prevention Evaluation Study Investigators. Effects of an angiotensin-converting enzyme inhibitor, ramipril, on death from cardiovascular causes, myocardial infarction, and stroke in high risk patients. New EnglJMed 2000; 342: 145-53. 42. Freemantle N, Cleland J, Young P et al. Beta blockade after myocardial infarction: systematic review and meta regression analysis. BMJ 1999; 318:1730-7. 43. Chua TP, Lipkin DP. Cardiac rehabilitation. BMJ 1993; 306: 731-2. 44. Linden W et al. Meta-analysis: psychosocial interventions augment cardiac rehabilitation programs. Arch Intern Med 1996; 156: 745-52. 45. Schleifer SJ, Macari-Hinson MM, Coyle DA et al. The nature and course of depression following myocardial infarction. Arch Intern Med 1989; 149: 1785-9. 46. Oldridge N et al. Behavioural rehabilitation soon after myocardial infarction. Am J Cardiol 1993; 72: 154-61. 47. Sharpe N, Doughty R. Epidemiology of heart failure and ventricular dysfunction. Lancet 1998; 352 (Suppl. 1): 3-7. 48. Philbin EF. Comprehensive multidisciplinary programs for the management of patients with congestive heart failure. / Gen Intern Med 1999; 14: 130-5. 49. Belardinelli R, Georgiou D, Cianci G et al. Randomized, controlled trial of long-term moderate exercise training in chronic heart failure. Circulation 1999; 99: 1173-82.

112

CARDIOVASCULAR PROBLEMS

50. Dargie HJ, McMurray JJV. Diagnosis and management of heart failure. BMJ 1994; 308: 321-8. 51. Hobbs FDR et al. Heart failure in general practice. BMJ 2000; 320: 626-9. 52. Lloyd-Williams F et al. Exercise training and heart failure: a systematic review of the evidence. B J Gen Pract 2002; 52: 47-55. 53. Flather MD, Yusuf S, Kober L et al. Long-term ACE-inhibitor therapy in patients with heart failure or left-ventricular dysfunction: a systematic overview of data from individual patients. Lancet 2000; 355: 1575-81. 54. Mason J, Young P, Freemantle N et al. Safety and costs of initiating angiotensin converting enzyme inhibitors for heart failure in primary care: analysis of individual patient data from studies of left ventricular dysfunction. BMJ 2000; 321: 1113-6. 55. Pitt B, Poole-Wilson PA, Segal R et al. Effect of losartan compared with captopril on mortality in patients with symptomatic heart failure: randomised trial - the Losartan Heart Failure Survival Study ELITE 2. Lancet 2000; 355: 1582-7. 56. Pitt B et al. The effect of spironolactone on morbidity and mortality in patients with severe heart failure. New Engl J Med 1999; 341: 709-17. 57. MeReC. Combination diuretics. MeReC Bulletin 1994; 5: 37-9. 58. DTB. Drugs for heart failure. Drug Ther Bull 1994; 32: 83-5. 59. Lechat P et al. Clinical effects of beta-blockade in chronic heart failure: a meta-analysis of double-blind, placebo-controlled, randomised trials. Circulation 1998; 98:1184-91. 60. Hood WBJ, Dans A, Guyatt GH et al. Digitalis for treatment of congestive heart failure in patients in sinus rhythm (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2001. 61. Cupples ME, Irwin WG; McDevitt DG. An epidemiological study of digoxin prescribing in general practice. / R Col Gen Pract 1986; 36: 454. 62. Aronson JK, Hardman N. Digoxin. BMJ 1992; 305: 1149-52. 63. Chua TP et al. Effects of dihydrocodeine on chemosensitivity and exercise tolerance in patients with chronic heart failure. / Am Col Cardiol 1997; 29: 147-52. 64. Stewart AG, Howard P. Indications for long-term oxygen therapy. Resp Physiol 1992; 59: 8-13. 65. DTB. What to do about pulmonary heart disease. Drug Ther Bull 1990; 29: 90-2. 66. British Heart Foundation. Factfile. Ventricular extrasy stoles. London: British Heart Foundation, 1995. 67. Peuhkinen K et al. Effectiveness of amioderone as a single oral dose for recent-onset atrial fibrillation. Am J Cardiol 2000; 85: 462-5. 68. Roy D et al. Amioderone to prevent recurrence of atrial fibrillation. New Engl J Med 2000; 342: 913-20. 69. Camm J. Atrial fibrillation: antiarrythmic drugs. Geriatric Med 2001; June: 61-4. 70. McMurray J, Rankin A. Treatment of heart failure and atrial fibrillation and arrhythmias. BMJ 1994; 309: 1631-5. 71. Atrial Fibrillation Investigators. The efficacy of aspirin in patients with atrial fibrillation: analysis of pooled

72.

73.

74.

75. 76.

77.

78. 79. 80. 81.

82.

83.

84.

85. 86.

87. 88.

data from 3 randomised trials. Arch Intern Med 1997; 157: 1237-40. Taylor FC, Cohen H, Ebrahim S. Systematic review of long-term anticoagulation or antiplatelet treatment in patients with non-rheumatic atrial fibrillation. BMJ 2001; 322: 321-6. Hellemons BSP, Langenberg M, Lodder J et al. Primary prevention of arterial thromboembolism in non-rheumatic atrial fibrillation in primary care: randomised controlled trial comparing two intensities of coumarin with aspirin. BMJ 1999; 319: 958-64. Protheroe J, Fahey T, Montgomery AA, Peters TJ. The impact of patients' preferences on the treatment of atrial fibrillation: observational study of patient based decision analysis. BMJ 2000; 320: 1380-4. Lip GYH, Lowe GDO. ABC of atrial fibrillation: antithrombotic treatment for atrial fibrillation. BMJ 1996; 312: 45-9. Segal JB, McNamara RL, Miller MR et al. Anticoagulants or antiplatelet therapy for non-rheumatic atrial fibrillation and flutter (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2001. Hart RG, Benavente O, McBride R et al. Antithrombolitic therapy to prevent stroke in patients with atrial fibrillation: a meta-analysis. Ann Intern Med 1999;131: 492-501. British Heart Foundation. Factfile. Anticoagulation in non-rheumatic atrial fibrillation. London: British Heart Foundation, 1994. DTB. Warfarin or aspirin for non-rheumatic atrial fibrillation? Drug Ther Bull 1994; 32: 57-60. Lip GY et al. Drugs for atrial fibrillation. BMJ 1995; 311: 163-4. American Dental Association, American Academy of Orthopaedic Surgeons. Advisory statement. Antibiotic prophylaxis for dental patients with total joint replacements. / Am Dent Assoc 1997; 128: 1004-8. Endocarditis Working Party of the British Society for Antimicrobial Chemotherapy. Antibiotic prophylaxis of infective endocarditis. Recommendations from the Endocarditis Working Party of the British Society for Antimicrobial Chemotherapy. Lancet 1990; 335: 88-9. Littler WA et al. Changes in recommendations about amoxycillin prophylaxis for prevention of endocarditis. British Society for Antimicrobial Chemotherapy Endocarditis Working Party. Lancet 1997; 350: 1100. Perry IJ et al. Prospective study of risk factors for development of non-insulin dependent diabetes in middle-aged British men. BMJ 1995; 310: 560-4. Campbell NC et al. Secondary prevention in coronary heart disease: a randomised trial of nurse-led clinics in primary care. Heart 1998; 80: 447-52. Moher M, Yudkin P, Wright L et al. Cluster randomised controlled trial to compare three methods of promoting secondary prevention of coronary heart disease in primary care. BMJ 2001; 322: 1-7. Flapan AD. Management of patients after their first myocardial infarction. BMJ 1994; 309: 1129-34. Silagy C et al. Nicotine replacement therapy for smoking cessation (Cochrane Review). In: The

REFERENCES

89. 90. 91. 92. 93. 94. 95. 96.

97.

98.

99. 100. 101.

Cochrane Library, Issue 3. Oxford: Update Software, 2000. Wannamethee SG, Shaper AG. Type of alcoholic drink and risk of major coronary heart disease events and all-cause mortality. Am } Public Health 1999; 89: 685-90. Powell KE, Pratt M. Physical activity and health. BMJ 1996; 313: 126-7. Wannamethee SG, Shaper AG, Walker M. Changes in physical activity, mortality, and incidence of coronary heart disease in older men. Lancet 1998; 351: 1603-8. British Heart Foundation. Factfile. Physical activity and the heart: an update. London: British Heart Foundation, 2001. Online. Available: www.bhf.org.uk Campbell AJ et al. Randomised controlled trial of a general practice programme of home based exercise to prevent falls in elderly women. BMJ 1998; 315: 1065-9. Michie S, Cockroft A. Overwork can kill. BMJ 1996; 312: 921-2. Han TS et al. Waist circumference action levels in the identification of cardiovascular risk factors: prevalence study in a random sample. BMJ 1995; 311: 1401-5. Hooper L, Summerbell CD, Higgins JPT et al. Reduced or modified dietary fat for prevention of cardiovascular disease (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2000. Imperial Cancer Research Fund OXCHECK Study Group. Effectiveness of health checks conducted by nurses in primary care: final results of the OXCHECK study. BMJ 1995; 310: 1099-104. Thompson RL, Summerbell CD, Hooper L et al. Dietary advice given by a dietitian versus other health professional or self-help resources to reduce blood cholesterol (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2001. Joshipura KJ, Hu FB, Manson J et al. The effect of fruit and vegetable intake on risk for coronary heart disease. Ann Intern Med 2001; 134: 1106-14. DTB. Statin therapy - what now? Drug Ther Bull 2001; 39. Neil HAW et al. Extent of underdiagnosis of familial hypercholesterolaemia in routine practice: prospective registry study. BMJ 2000; 321: 148.

113

102. Sanmuganathan PS, Ghahramani P, Jackson PR et al. Aspirin for primary prevention of coronary heart disease: safety and absolute benefit related to coronary risk derived from meta-analysis of randomised trials. Heart 2001; 85: 265-71. 103. Evans P, Gray DP. Value of screening for secondary causes of hyperlipidaemia in general practice. BMJ 1994; 309: 509-10. 104. Jolliffe JA, Rees K, Taylor RS et al. Exercise-based rehabilitation for coronary heart disease (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2001. 105. de Longeril M, Salen P, Martin J-L et al. Mediterranean diet, traditional risk factors, and the rate of cardiovascular complications after myocardial infarction: final report of the Lyon Diet Heart Study. Circulation 1999; 99: 779-85. 106. Balarajan R. Ethnic differences in mortality from ischaemic heart disease and cerebrovascular disease in England and Wales. BMJ 1985; 302: 560-4. 107. The BIP Study Group. Secondary prevention by raising HDL cholesterol and reducing triglycerides in patients with coronary artery disease: the bezafibrate infarction prevention (BIP) study. Circulation 2000; 102: 21-7. 108. Haffner SM. Secondary prevention of coronary heart disease: the role of fibric acids. Circulation 2000; 102: 2-4. 109. Boyle R. DoH explains thinking behind national service framework for coronary heart disease (letter). BMJ 2000; 321: 1083. 110. Weverling-Rijnsburger AWE, Blauw GJ, Lagaay AM et al. Total cholesterol and risk of mortality in the oldest old. Lancet 1997; 350: 1119-23. 111. Scandinavian Simvastatin Survival Group. Randomised trial of cholesterol lowering in 4444 patients with coronary heart disease: the Scandinavian Simvastatin Survival Study (4S). Lancet 1994; 344: 1383-9. 112. Avins AL, Neuhaus JM. Do triglycerides provide meaningful information about heart disease risk? Arch Intern Med 2000; 160: 1937-44. 113. Sattar N, Packard CJ, Petrie JR. The end of triglycerides in cardiovascular risk assessment? BMJ 1998; 317: 553-4.

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6

CHAPTER CONTENTS Asthma 115 Acute asthma 119 Lower respiratory tract infection Tuberculosis

Respiratory problems 121

122

Chronic obstructive pulmonary disease References

123

127

ASTHMA Guidelines: British Thoracic Society. The British Guidelines on Asthma management: 1995 Review and Position Statement. Thorax 1997; 52 (Suppl. 1): S1-20. Scottish Intercollegiate Guidelines Network (SIGN). Primary care management of asthma. SIGN Publication, No. 33, November 1998. Scottish Intercollegiate Guidelines Network (SIGN). Emergency management of acute asthma. SIGN Publication, No. 38, June 1999.

Diagnosis The diagnosis1 of asthma should be confirmed objectively and the process clearly documented. 1. Typical history: (a) Variable symptoms of coughing, wheezing, chest tightness and shortness of breath. (b) Symptoms are often worse at night and are commonly triggered by viral infections, exercise and allergy. (c) A personal or family history of atopy supports the diagnosis. 2. Objective confirmation of variability: Peak flow (PF) variability can be demonstrated by: (a) Peak flow charting.2'3 Morning and evening peak flows recorded over 1 to 2 weeks may show the typical 'saw tooth' pattern due to diurnal variation. (b) Reversibility test. A >15% response to beta2-agonist may be demonstrated in the 115

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surgery if the presenting peak flow is low or the patient is symptomatic. Procedure: Record baseline peak flow. Give a dose of a beta2agonist. Record follow-up peak flow after 15 minutes. The rise in peak flow is expressed as a percentage of the baseline, (c) Response to trigger. A fall in peak flow >15% may be demonstrated by an exercise test4 or undertaken as part of home charting when the patient next meets his or her trigger. 3. Response to treatment: (a) A reduction in peak flow variability and symptoms should be demonstrated after a 6-week course of inhaled steroids. (b) A 2-week course of oral steroids may be appropriate, particularly to distinguish asthma from chronic obstructive pulmonary disease (COPD) (see diagnosis of COPD, p. 123).

Notes • Peak flows should be interpreted in conjunction with symptoms.5 • A normal peak flow at a consultation does not exclude the diagnosis of asthma. Asthma is a dynamic condition and readings need to be taken over time in order to check for variability. • Children under 7, and some elderly or disabled patients, cannot reliably use a peak flow meter. Assessment has to be based on symptoms. Infants Wheezy infants present particular diagnostic difficulties and there is little evidence-based data to guide decisions.6'7 • Exclude serious pathology, e.g. cystic fibrosis, congenital heart defects, inhaled foreign body, oesophageal problems. Referral to a paediatrician is appropriate if the diagnosis is in doubt. • Distinguish between asthma and viral-associated wheeze (Table 6.1). This can be a difficult distinction to make and the diagnosis may only be clear in retrospect, as children with viral-associated wheeze will normally grow out of their symptoms in 2 or 3 years: (a) Consider risk factors and clinical history.

Table 6.1 Distinguishing between asthma and viralassociated wheeze Suggestive of asthma

Suggestive of viral-associated wheeze

Family or personal history of atopy

Prematurity, small for dates Maternal smoking Cough and wheeze only with viral infections

Cough and wheeze not associated with viral illness Good response to treatment and relapse on cessation of treatment

Poor response to treatment

(b) A carefully supervised trial of treatment may be helpful,6'7 e.g. inhaled steroids for 8 weeks (up to beclomethasone 1000 u.g daily, budesonide 800 |xg daily or fluticasone 500 ug daily via a spacer ± mask). (c) Poor response to therapy makes asthma unlikely and treatment can be withdrawn. (d) Good responses to therapy should be confirmed by a withdrawal of treatment in order to exclude coincidental improvement.

Management General measures * Advise on the avoidance of triggers: (a) The National Asthma Campaign produces useful information for patients about allergen avoidance. (b) Common allergens include pets, especially cats, house-dust mite and pollen. (c) Current chemical and physical measures for reducing house dust mite allergen have not been shown to be effective.8,9 (d) Treatment of allergic rhinitis with nasal steroids may improve control of asthma.10 (e) The possibility of occupational asthma should prompt referral. * Encourage smoking cessation. Both active and passive smoking status should be documented. * Consider drug effects: (a) Beta-blockers (systemic or topical eye drops) are contraindicated. (b) Asthmatics sensitive to aspirin should not use non-steroidal anti-inflammatory drugs (NSAIDs).

ASTHMA

Therapeutic management Patients should start treatment at the step most appropriate to the severity at presentation.11,12 (See Appendix 12A & B.) Inhaled steroids are indicated for most symptomatic asthmatics.13,14 Regular treatment improves lung function, reduces symptoms, reduces use of rescue bronchodilation and reduces the risk of exacerbations.13 * Gain control by starting with a moderate dose (beclomethasone 1000 ug daily, budesonide 800 (jig daily or fluticasone 500 |xg) and reduce to the lowest dose compatible with asthma control.12,15 * Discuss compliance. Compliance with inhaled steroid treatment is poor (20-73%) and may be improved by a simple once- or twice-daily regime.16 * Explain ways of avoiding local side-effects. Candidiasis may be reduced by rinsing the mouth after administration or using a spacer device; dysphonia may be improved by reducing the dose. * Reassure patients that important systemic side-effects are unlikely at moderate doses.13 In children, despite an initial reduction in growth velocity, final height is not affected.17'18 Highdose inhaled steroids (beclomethasone or budesonide >1500 (jug daily, fluticasone >0.75 jjig daily) have an increased risk of adrenal suppression, cataracts, reduced bone density and bruising.19 Risks should be balanced against benefits and alternatives considered (see box below).

117

1. Consider an objective trial of long-acting bronchodilators. 2. Monitor response with symptoms and peak flow charting. 3. If no response, stop the additional therapy and consider an alternative (e.g. leukotriene antagonists, increased dose of inhaled steroid). 4. If poor response to any of the options, consider the need for referral.

Beta2-agonists provide symptomatic relief and should be prescribed on an 'as required' basis.12,14,20 They are less effective than inhaled steroids in the management of mild persistent asthma.14,21 * Patients requiring relief medication more than once a day should be considered for regular prevention with inhaled steroids.12 * Exercise-induced asthma which persists despite good asthma control with inhaled steroids may be prevented by using a beta2-agonist prior to exercise. Long-acting bronchodilators are given regularly twice a day as 'add-on therapy' to asthmatics not controlled on inhaled steroids. The addition of long-acting bronchodilators improves lung function, symptom control, reduces the need for relief medication and reduces the exacerbation rate compared with increasing the dose of inhaled steroids.22,23 Leukotriene antagonists are mediator antagonists and are an option as 'add-on therapy'. Their role in comparison with other established therapies is still being investigated.1,12 Other therapeutic options

Management of patients not controlled on a moderate dose of inhaled steroids That is, adult maximum daily maintenance dose: beclomethasone 1000 ug, budesonide 800ug or fluticasone 500 ug. That is, childhood maximum daily maintenance dose: beclomethasone 400 |xg daily, budesonide 400 ug daily or fluticasone 250 u,g. Assessment: (a) Is the diagnosis correct? (b) Is the patient complying with the regular use of inhaled steroids? (c) Can the patient use his or her inhaler device? (d) Does the patient also have uncontrolled rhinitis? (e) Are there any environmental factors?

* Sodium cromoglicate has a small overall treatment effect and should not be first line treatment for the prevention of asthma.24 * Theophyllines may be an option as 'add on therapy' for a minority of patients.1,12 * A short 'rescue' course of oral steroids may be needed at any time and at any step12 but consideration of maintenance therapy with oral steroids should prompt referral to a respiratory physician.1

Devices The effectiveness of inhaled therapy depends on inhaler technique and the particle size produced

118

RESPIRATORY PROBLEMS

by the device.16 Inhaler devices should be selected for individual patients taking into consideration: (a) The patient's ability to use a device. This should always be checked before an inhaler is prescribed, and re-checked regularly. Effective delivery is achieved in about half the patients studied.16 Technique should be observed for: - the patient's inspirational flow rate compared to flow rate recommended by the manufacturer for efficient actuation and inhalation; - hand/breath coordination; - ability to follow the instructions for correct use. (b) The drug and dosage required. High doses of inhaled steroid should be given by a spacer device. (c) MCE guidelines recommend the use of spacers (with masks for infants) for children under the age of five.25 Choice of spacer device should be guided by the information in the Summary of Product Characteristics (see: www.nice.org.uk). (d) Patient preference and lifestyle issues may be relevant. (e) Cost of the device.26

Choice of inhaler device for different age groups Age

Devices to consider

Comments

Preschool

MDI + spacer (with a mask for infants) MDI + spacer

NICE recommendation25

School-age

Dry powder devices

Adults

Breath actuated devices MDI

Breath-actuated devices Dry powder devices MDI, measured-dose inhaler.

Recommended for inhaled steroids because it reduces oral deposition Once a child has adequate inspiration and can be relied on not to blow by mistake Once inspiration is adequate If technique is adequate. Should be used with a spacer to deliver high doses of inhaled steroid

Follow-up Review may be undertaken by GPs or trained asthma nurses and should encompass: * Specific morbidity questions. The Royal College of Physicians recommend that the following three questions should be asked at every asthma review consultation:27 1. Have you had difficulty sleeping because of your asthma symptoms (including cough)? 2. Have you had your usual asthma symptoms during the day (cough, wheeze, tight chest or breathlessness)? 3. Has your asthma interfered with your usual activities (housework, work or school, etc.)? * Review of medication, considering the need to step up or the possibility of stepping down treatment.12 * Review of inhaler technique. * Education and self-management. Patients should be encouraged to discuss issues of importance to them. Education should aim to empower patients to be in control of their asthma and should include the provision of written self-management plans.28

Indications for outpatient referral Referral to a respiratory physician or paediatrician should be considered:12,24 (a) If the diagnosis is not clear. (b) If the patient is not controlled with moderate doses of inhaled steroids ± add-on therapy and their inhaler technique is satisfactory. (c) If there is a possibility of occupational asthma. (d) If there is a history of life-threatening attacks or brittle asthma.

Self-management plans Education involving self-monitoring and written action plans reduce hospitalizations, emergency consultations, reduction in days off work or school, and nocturnal asthma.28 * Offer all asthmatics written self-management plans which should: (a) be tailored to the individual patient, taking into account his or her clinical condition and preference for autonomy;

ASTHMA

(b) involve self-monitoring, based on peak flows and/or symptoms; (c) provide information about features which indicate that the patient's asthma is worsening; (d) advise what action the patient should take.

National Asthma Campaign 'Be in Control' action plan Symptoms are:

Peak flow is:

Action is:

No symptoms

>80% of patient's best

Normal - continue treatment or talk to doctor/nurse about taking less treatment

Getting a cold, symptoms during daytime and/or night time

<80% of patient's best

Take increased dose of inhaled steroids Use blue inhaler for relief

Out of breath Blue inhaler does not help

<60% of patient's best

Continue as above and start steroid tablets. Prednisolone 30 mg and contact GP

Too breathless to speak

<40% of patient's best

This needs emergency action straight away

Entries in italics should be added by the nurse or doctor. The relevant peak flow readings should be added in the middle column.

ACUTE ASTHMA Acute asthma in adults and schoolchildren1229 Delay is the commonest factor identified as contributing to asthma deaths, often related to a failure on the part of the patient or the health-care professional to appreciate the severity of the attack.30 * Assess and record the severity: (a) General condition. Inability to speak, exhaustion, cyanosis and a silent chest indicate lifethreatening asthma. (b) Peak flow. Peak flow (PF) at presentation should be compared with the patient's best (or predicted if best is not known).12'29 PF PF PF PF

>75% of <75% of <50% of <33% of

best best best best

= = = =

mild asthma uncontrolled asthma severe asthma life-threatening asthma

119

(c) Respiratory rate (RR): >25/minute in adults, >40/minute in children indicates severe asthma. (d) Heart rate (HR): >110/minute in adults, > 120/minute in children indicates severe asthma. * Act promptly to provide relief: 1. Bronchodilation: 5mg salbutamol/lOmg terbutaline by nebulizer or multiple (10-20) doses through a large-volume spacer.14,31 Consider adding ipratropium in life-threatening asthma.14,32,33 2. Oxygen: give at high flow rates for severe or life threatening asthma. Note: nebulizers require flow rates of 6-8 litres a minute. 3. Systemic steroids: soluble prednisolone 3060 mg for adults, 20 mg for children should be given at the consultation.14,34 The NNT to prevent one admission in patients with severe asthma is five (95% CI: 4 to 7). 4. Assess response. * Arrange further care 1. Admit: (a) patients with life-threatening asthma; (b) patients with severe asthma who do not respond to emergency bronchodilation. Other factors, e.g. the time of day, social situation, previous history of severe attacks, should influence the decision to admit. 2. Instructions: (a) Bronchodilation may be repeated 3 to 4 hourly. Ideally, progress should be monitored by peak flow. (b) Prednisolone 30-40 mg daily for 10-14 days (adults); 20 mg daily for 3-5 days (children). (c) Increase inhaled steroids (beclomethasone 1000 |JLg daily, budesonide 800 ug daily or fluticasone 500 ug) until full control is regained. (d) Ensure the patient knows when and how to call further medical help. 3. Early follow-up: Arrange to assess progress within 24 hours (severe asthma) or 48 hours (uncontrolled asthma). 4. Review: Arrange for a review of overall asthma control and revision of self-management plans.

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RESPIRATORY PROBLEMS

Acute asthma in preschool children12 * Assess and record the severity: exhaustion, agitation, drowsiness, too breathless to feed, use of accessory muscles, RR >50/minute or HR > 140/minute are signs of severe or life threatening asthma. * Act promptly to provide relief 1. Bronchodilation: 2.5 mg salbutamol/5mg terbutaline by nebulizer or multiple (10-20) doses through a large volume spacer ± mask. 2. Oxygen should be given at high flow rates. 3. Systemic steroids: soluble prednisolone 20 mg. 4. Assess response: RR, HR and general condition. * Arrange further care. Admit: (a) children with any features of severe or lifethreatening asthma; (b) children who do not respond to bronchodilation or who relapse within 3 hours. * Poor social circumstances or a previous history of a severe attack should lower the threshold for admission. * Children not admitted should be given prednisolone 20 mg for 1-3 days and clear instructions for review. Professional groups: General Practice Airways Group (GPIAG): http://www.gpiag-asthma.org Secretariat: 2nd Floor, 9 Windsor Court, Clarence Drive, Harrogate HG1 2PE, tel. 01423 560203; fax, 01423 709993; e-mail, [email protected] The GPIAG is a group of GPs with an interest in respiratory disease. They are active in research education and publish the Primary Care Respiratory Journal. Their website provides online access to the journal, opinion sheets and has a question and answer service. British Thoracic Society: http://www.brit-thoracic.org.uk 6th Floor, North Wing, New Garden House, 78 Hatton Gardens, London NW1 8LD, tel. 020 731 8778; fax, 020 7831 8766; e-mail, adminl @brit-thoracic.org.uk The society for all professionals with an interest in respiratory disease. Their guidelines may be downloaded from their website. Also available are some information sheets for patients on a wide range of respiratory problems.

Guidelines: Scottish Intercollegiate Guidelines Network (SIGN). Primary care management of asthma. Online. Available: www.sign.ac.uk The Global Initiative on Asthma. Asthma prevention and management: a practical guide for public health

officials and health care professionals. Online. Available: www.ginasthma.com

Other useful websites: Scottish Respiratory Site: http://www.srs.org.uk/ This site has a useful Q&A forum and excellent links to other respiratory sites. International Asthma Quality of Care Initiative http://www.iaqoc.com/ This site provides links to international sites and references to key asthma documents. It also has an audit toolbox.

Respiratory training: National Asthma & Respiratory Training Centre: http://www.nartc.org.uk The Athenaeum, 10 Church Street, Warwick CV34 4AB, tel. 01926493313 Respiratory Education Resource Centre: http://www.respiratoryerc.com University Hospital Aintree, Lower Lane, Liverpool L97AL, tel. 0151 5292598 Primary Focus: [email protected] Brackenrigg, Rannoch Road, Crowborough, East Sussex TN6 1RA, tel. 01892 661339 Asthma Management Centre: http://www.amctraining.co.uk 232 Tower Street, Brunswick Business Park, Liverpool L34BJ, tel. 0151 707 1141

Patient groups: National Asthma Campaign: http://www.asthma.org.uk Providence House, Providence Place, London N1 ONT, tel. 020 7226 2260; Helpline 0845 7010203 The NAC provides information leaflets about asthma for patients and resources such as self management plans which can be ordered or downloaded from their website. It also has a Professional Subscription Scheme and publishes the Asthma Journal. British Lung Foundation: www.lunguk.org 78 Hatton Gardens, London EC1N 8LD, tel. 020 7831 5831 The BLF produces information for patients about a wide range of respiratory diseases which may be ordered or downloaded from their website. It runs local 'Breathe Easy' clubs. Action on Smoking and Health: www.ash.org.uk ASH publishes information about smoking and provides information about smoking cessation with links to other useful sites. Quitline: 0800 00 22 00 Provides telephone support line for patients wanting to quit.

LOWER RESPIRATORY TRACT INFECTION 121

LOWER RESPIRATORY TRACT INFECTION Guidelines: The British Thoracic Society. Guidelines for the management of community acquired pneumonia in adults. Thorax 2001; 56(IV): 1-64. European Respiratory Society. Guidelines for management of adult community acquired lower respiratory tract infections. Eur Respir J1998; 11: 986-91.

* The majority of previously well patients who present to their GP with acute respiratory symptoms have a self-limiting illness.35 * Less than half have evidence of a bacterial infection.35,36 Without a chest X-ray (CXR), the 6% of patients with lower respiratory tract infection who have community-acquired pneumonia is imprecise, with no individual sign or symptom being reliably predictive.35,37

Clinical assessment The aim of the initial history and examination is to identify patients with, or at risk of developing, severe or complicated illness.38,39 * Identify high risk groups. The risk of pneumonia increases with age and in institutionalized patients.38,39 * Ask about comorbidity. Premorbid conditions which increase the risk of pneumonia include COPD, congestive heart failure,38,39,40 neurological disease, diabetes, alcoholism, and chronic renal or hepatic failure.38,39 * Examine the chest. The presence of localized chest signs is positively correlated with radiographic evidence of pneumonia, but their absence does not exclude the diagnosis.35,37 * Assess severity. Fever >40°C, RR >30/minute, BP <90/60, pleuritic chest pain, or confusion indicate severe infection39,41,42 and should prompt referral.

Investigations Investigations are rarely needed in primary care 35,38,39

(a) CXR may be indicated if there are focal chest signs and in high-risk patients or those with signs of severe disease even in the absence of focal chest signs.38,39 Persistence of systemic illness, the development of unexpected symptoms (e.g. haemoptysis or pleuritic pain) or failure of respiratory symptoms to resolve should prompt investigation. (b) A raised c-reactive protein (CRP) (>50mg/ litre) is positively correlated with radiographic evidence of pneumonia.35,39 (c) A raised blood urea (> 11 mmol/litre) and a leukocytosis (>20,000 white blood cells (WBC)/ ml) or leukopenia (<4000WBC/ml) indicate severe infection and should prompt urgent referral.39,41,42

Management identification of of well patients with lower respiratory tract symptoms without focal chest signs

* Discuss the natural history of lower respiratory tract illness. The provision of leaflets explaining the nature of a cough and the expected duration (up to 3 weeks) can reduce reconsultation rates.43 Advise that failure to improve as expected, deterioration or development of new symptoms should prompt reconsultation. * Provide advice on symptomatic treatment: (a) Paracetamol, extra fluids, hot lemon and bed rest are commonly recommended in the acute phase, although there are no studies to validate these common remedies. (b) Zinc used within 24 hours of the onset of symptoms has been shown to significantly improve the resolution rate of upper respiratory tract symptoms.44 (c) The use of vitamin C supplements to prevent or treat respiratory infection is unproven. (d) Cough medicines are often bought over the counter but there is no evidence to support effectiveness. * Avoid prescribing antibiotics in well patients with no additional risk factors. Benefits from antibiotics are modest and, for most patients, may not outweigh the risk of adverse effects, the costs or the negative consequences of antibiotic resistance.45 (a) Antibiotics increase the resolution of symptoms by about half a day.45,46,47

122

RESPIRATORY PROBLEMS

(b) The magnitude of benefit is similar to that of the detriment from adverse reactions (NNT = 18, NNH = 14).46 (c) Patients who also have the typical symptoms of URTI and have been ill for less than a week may be least likely to benefit from antibiotics.45 (d) Prescribing antibiotics did not reduce the reconsultation rate in a UK primary care study.48 (e) Declining the request for antibiotics and educating patients on the limitations and disadvantages of treatment is effective in reducing antibiotic use.49 * Use the opportunity to provide smoking cessation advice.

Management of patients with lower respiratory tract symptoms with focal chest signs or where the patient is at high risk of pneumonia or is systemically ill Community-acquired pneumonia is a potentially serious condition with a mortality of 5%.39,40 * Consider the need for a CXR to confirm the diagnosis of pneumonia. An FBC, CRP and U&Es may help to assess severity.39 * Prescribe antibiotics.38,39 Antibiotic treatment should not be delayed if a diagnosis of pneumonia is suspected.39,50 (a) Amoxycillin 500 mg-1 g t.d.s. for 5-7 days39 (erythromycin 500 mg q.d.s. or clarithromycin 500mgb.d. are alternatives). (b) Erythromycin or clarithromycin are indicated for mycoplasma pneumonia. (c) Local microbiologists can provide information about local resistance patterns. * Arrange follow-up.38'39 Fever should settle within 2 days. Consider investigating or admitting patients who fail to respond. Some symptoms such as cough may persist after the antibiotics course has finished. If the initial CXR was abnormal, repeat it 6 weeks later to check that it has cleared. * Consider the need for admission.38,39 Admission is recommended in the presence of: (a) signs of severe disease.39,40,41 (Fever >40°C, RR >30/minute, BP <90/60, pleuritic chest pain, confusion);

(b) high-risk conditions, e.g. pneumonia associated with influenza, chicken pox; (c) suspected complications (e.g. pleural effusion, malignancy); (d) diagnostic uncertainty. Factors which may contribute to the decision to admit are: (a) comorbidity39,40 (COPD, CCF, diabetes, alcoholism, chronic renal or hepatic failure); (b) unsatisfactory social circumstances; (c) failure to respond to first line antibiotics.

Vaccination • Influenza vaccination is recommended for patients in high risk categories:39'51 (a) age over 65 years and institutionalized patients; (b) patients with comorbidity: chronic respiratory disease, including asthma, chronic heart disease, neurological disease, diabetes, alcoholism, chronic renal or hepatic failure; (c) asplenic and immunosuppressed patients. • Pneumococcal vaccination is recommended for the last two of these three categories.

TUBERCULOSIS (TB) Guidelines: Joint Tuberculosis Committee of the British Thoracic Society. Chemotherapy and management of tuberculosis in the United Kingdom: recommendations 1998. Thorax 1998; 53: 536-48. Joint Tuberculosis Committee of the British Thoracic Society. Control and prevention of tuberculosis in the United Kingdom: Code of Practice 2000. Thorax 2000; 55:887-901.

Patients with TB should be managed by physicians or paediatricians with specialist expertise in the management of tuberculosis, supported by tuberculosis nurse specialists or health visitors.52 The role of primary care is to: * Be alert to the increasing incidence of TB.53 This increase is greatest in urban areas, with 56% of notifications occurring in patients from ethnic minorities. New patient medicals may be an opportunity to check the BCG status of immigrants.

CHRONIC OBSTRUCTIVE PULMONARY DISEASE

Other high-risk groups include patients with impaired immunity (including HIV), patients on steroids, the homeless, and those dependent on alcohol or drugs. * Consider the diagnosis. Symptoms of fever and night sweats, cough, weight loss and haemoptysis should prompt investigation. A CXR should be arranged and three sputum samples may be sent for microscopy, culture and sensitivity and should be marked as 'suspected TB'. * Ensure prompt referral. Most patients can be treated as outpatients.52 Inpatient care may be considered because of clinical severity or to ensure compliance with treatment regimes. * Encourage compliance. Treatment regimes will involve a combination of drugs and will continue for at least 6 months.52 Supervision will be arranged by the hospital clinic in accordance with local policy. 'Directly observed therapy' may be needed to ensure compliance. * Support the patient and their family. Information for clinicians and answers to 'Frequently asked questions' are available on the Public Health Laboratory Services website: http://www.phls.co.uk/ facts/TB/Index.htm * Be prepared for concerned enquiries from contacts. Contact tracing is undertaken according to defined procedures usually by a respiratory nurse specialist.54 Close contacts are defined as people from the same household, close friends or frequent visitors to the household. Most other contacts are usually 'casual contacts' and are at considerably lower risk of infection. In the event of a local outbreak the local Consultant in Communicable Disease can provide advice.

CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD) Guidelines: British Thoracic Society. The BTS Guidelines for the management of Chronic Obstructive Pulmonary Disease. Thorax 1997; 52 (Suppl. 5): S1-28. Global Initiative for Obstructive Lung Disease. Global strategy for the diagnosis, management and prevention of chronic obstructive pulmonary disease; NHLBI/WHO workshop report 2001. Online. Available: www.goldcopd.com

123

The aims of the management of patients with COPD is to make an objective diagnosis as early as possible in the course of the disease in order to encourage smoking cessation and prevent progression. Treatment is aimed at providing the best possible relief of symptoms and improving quality of life.55 As the disease progresses treatment levels and professional support should be stepped up to provide adequate palliation.55

Diagnosis History * Ask about smoking. Smoking history may be recorded as 'pack years' (i.e. one pack year = 20 cigarettes a day for 1 year). Note any occupational exposure (e.g. coal mining). * Ask about symptoms of cough, wheeze and breathlessness and their impact on lifestyle. These symptoms have an insidious onset, usually after the age of 50 years, and progress slowly with little variability. Suspect bronchial carcinoma if there is haemoptysis. * Examine the chest. The chest will usually be clear in mild COPD; rhonchi develop during exacerbations and as the disease progresses. Localized signs may indicate an underlying carcinoma. * Check for signs of chronic hypoxia. Cyanosis and confusion can occur with the onset of respiratory failure; ankle oedema suggests cor pulmonale. Investigations * Consider asking the patient to record peak flows. A peak flow chart may be useful to exclude asthma by confirming the absence of variability. * Arrange spirometry: The diagnosis should be confirmed by spirometry.55 Peak flows do not accurately reflect the degree of airway obstruction in COPD. * Consider the need for other investigations: (a) A CXR should be considered at initial presentation to exclude other pathology. (b) The presence of polycythaemia or an oxygen saturation of <92% suggests chronic hypoxia and should prompt referral for blood gas estimations.56

124

RESPIRATORY PROBLEMS

Spirometry should be undertaken when the patient is stable, i.e. at least 6 weeks should have elapsed since an exacerbation. (a) Baseline spirometry in COPD demonstrates an obstructive pattern (forced expiratory volume in 1 second (FEY^ <80% predicted and FEY}/ forced vital capacity (FVC) <70%). (b) FEVj expressed as a percentage of predicted may be used to classify the severity of COPD: -mild COPD is defined as FEVj <80% of predicted; -moderate COPD is defined as ¥EVl <60% of predicted; -severe COPD is defined as FEV1 <40% of predicted.55 (c) Significant reversibility may occur in COPD (defined as an increase in FEVj which is both >200ml and l5%). (d) A substantial response57 (500ml increase in FEY}) to bronchodilators or steroids suggests a diagnosis of asthma. (e) A steroid trial (30 mg prednisolone for 2 weeks) will identify the 10% (95% CI 2-18%) of COPD patients with significant reversibility to steroids.58

Management * Encourage the patient to stop smoking (see p. 331). This is the only intervention that can prevent the accelerated decline in lung function that occurs in patients with COPD.59,60 It is essential that patients understand the implications of continuing to smoke and the benefit of quitting - even with severe disease. * Encourage exercise. Exercise is a key component of all pulmonary rehabilitation programmes which have been shown to improve exercise capacity and the quality of life.61 Explain that breathlessness is uncomfortable but not dangerous. * Encourage weight loss in the obese. Stress the importance of good nutrition in all patients, although there is no evidence to support the use of nutritional supplements.62 * Advise vaccination. Influenza vaccination63 reduces the number of patients who experience an exacerbation (odds ratio (OR) = 0.13; 95% CI

0.04 to 0.45). Reactions to the vaccine are mild and transient. COPD patients are at increased risk of pneumonia and pneumococcal vaccination is recommended.64 * Provide information. The British Lung Foundation publishes patient information about COPD. They also run 'Breathe Easy' clubs in many areas.

Drug management Bronchodilators. These are the cornerstone of treatment for symptomatic COPD. They reduce breathlessness but have a only small effect on lung function.65'66 * Start with an anticholinergic or beta2-agonist. Both are effective in COPD and there may a small additive effect.65 They should be prescribed on an 'as required' basis.55 * Introduce a new drug as a therapeutic trial, accepting improved lung function or subjective improvement as end points.55 Improvement in lung function does not predict subjective benefit.65 * Add a long-acting bronchodilator if necessary. It can produce a small increase in lung function, a significant reduction in breathlessness and improved quality of life.67 * Consider using a theophylline if control is still poor. They have a modest effect on lung function and variable effect on symptoms and exercise capacity.65 Side-effects are frequent, limiting their value in COPD.55 Inhaled steroids

* Inhaled steroids do not reduce the underlying rate of decline in lung function.65,68,69 * In patients with more severe COPD (FEv1 <50% predicted) inhaled steroids have been shown to reduce the exacerbation rate by 25%.68 * Bruising occurs more frequently in patients on inhaled steroids but there is no significant increase in other systemic side-effects.69 * Consider a trial of inhaled steroids in patients with severe COPD who have frequent exacerbations.70 * Prescribe inhaled steroids (up to beclomethasone 1000 (ug daily, budesonide 800 ug daily or

CHRONIC OBSTRUCTIVE PULMONARY DISEASE 125

fluticasone 500(ug) to patients who show a significant, objective response to an oral or inhaled steroid trial.55,70 * Withdraw existing inhaled steroid treatment cautiously if there is no evidence of benefit.

Severe COPD Patients with severe COPD have disabling symptoms and a poor prognosis. * Consider referral to a pulmonary rehabilitation unit. These offer a multidisciplinary, holistic approach to the care of patients with COPD. They are normally hospital based, although some programmes are carried out in the community. They have been shown to increase exercise tolerance, relieve breathlessness and improve quality of life even though they do not improve lung function.71 * Consider the need for a high-dose bronchodilator for symptomatic relief. This may conveniently be delivered by multiple doses through a spacer device. Assessment for a home nebulizer should follow the regime provided in the BTS Guidelines for Nebulizer Therapy72 and should normally be undertaken by a health-care professional with a specialized interest in respiratory disease. * Consider referral for assessment for long-term oxygen therapy. In patients who are chronically hypoxic, oxygen therapy for at least 15 hours a day has been shown to improve 5-year survival; OR 0.42 (95% CI 0.18 to 0.98).73,74 A respiratory physician should assess all patients considered for long-term oxygen therapy. The criteria depend on lung function, blood gas estimations and the presence of

complications.55 The evidence to support the use of short bursts of oxygen to relieve exercise-induced breathlessness is less clear,55 although some severely dyspnoeic patients may benefit.75'76 * Look for depression. It is common in COPD and may require treatment with antidepressants.77 * Advise disabled patients that they may qualify for benefits.^ Advice may be obtained from the Benefits Agency. * Consider the need for practical help.78 Provision of appliances such as walking aids, stair lifts, bath aids may be appropriate. Support with domestic care may be needed. A wheelchair and a disabled parking permit may prevent the COPD patient becoming housebound and day-care may provide a break for both the patient and the carer. * Remember that COPD patients are terminally ill. They may have disabling symptoms for many years but rarely receive support from specialist palliative care services.78 They may welcome the opportunity to be able to discuss their illness, the prognosis and the support available. * Offer palliative care once it is clear that the patient is dying. Such patients need the same attention to symptom control and support as patients dying of cancer.

Palliative prescribing for patients with end-stage COPD79 Note: Sedatives and cough suppressants are normally contraindicated in COPD. Their role is limited to the terminal phase of the illness where control of distressing symptoms is the priority.

Symptom

Advice

Suggested prescription

Dyspnoea

Opiates, titrating the dose to achieve relief of dyspnoea Oxygen may have a small effect on dyspnoea Cool air, e.g. from a fan, sometimes increases comfort

Initial dose: morphine 5mg 4 hourly

Cough

Opiates

Morphine 5mg 4 hourly

Excess secretions

Anticholinergics (but take care to avoid the discomfort of a dry mouth)

sc hyoscine: 400-600 ug 4-6 hourly

Anxiety

Benzodiazepines Note: high doses of beta-agonists can aggravate anxiety

Diazepam 5-1 Omg daily

Confusion

Oxygen may reduce confusion due to hypoxia Chlorpromazine or haloperidol may ease confusion and restlessness

Chlorpromazine 25-50 mg 8 hourly Haloperidol 1-3mg 8 hourly

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Indications for referral to a respiratory physician55 (a) For an objective diagnosis if spirometry is not available in primary care. (b) If the diagnosis is not clear (e.g. apparent COPD in a non-smoker). (c) Aged under 40 or rapid deterioration in symptoms of lung function. (d) Frequent infective exacerbations. (e) Onset of cor pulmonale. (f) Assessment for long-term oxygen therapy or regular nebulizer use. (g) The possibility of industrial disease, e.g. pneumoconiosis, or if there is possibility of an occupational cause for the COPD.

Acute exacerbations Although commonly due to infection, worsening of previously stable COPD may be due to the development of other pathology including lung cancer, left ventricular failure, pulmonary embolism, pneumothorax.55 Assessment The history. Ask about:

(a) onset and duration of current exacerbation; (b) increased volume and purulence of sputum; (c) increased wheeze and shortness of breath; (d) increasing confusion or decreasing conscious level; (e) condition when stable, especially the need for long-term oxygen; (f) severity of previous exacerbations and previous admissions.

(b) the possibility of other respiratory pathology (e.g. carcinoma or TB); (c) the possibility of non-respiratory comorbidity. In one study, 40% of patients with an exacerbation of COPD were found to have other conditions (e.g. hypertension, CHD and diabetes); 14% had more than one condition.80 Initial treatment * Bronchodilators to relieve airway obstruction.55 (a) Increase or add a beta2-agonist and/or anticholinergic drugs. (b) Check inhaler technique (MDI + spacer may be easiest for the breathless patient). (c) Consider nebulized therapy for the severely breathless. * Antibiotics if there is increased volume of purulent sputum and increased breathlessness.81'82 Give amoxycillin or tetracycline for a maximum of 7 days.55 If the response is poor, consider a secondline antibiotic, e.g. a cephalosporin. * Oral steroids reduce hospitalizations83 and improve lung function in hospitalized patients.84 They should not be used routinely in general practice but are indicated in a dose of 30-40 mg daily for 10 days for severe exacerbations55 if: (a) there is a previously documented response to oral steroids; or (b) there is a poor response to bronchodilators; or (c) the patient is already on oral steroids; or (d) this is the first presentation of airflow obstruction. * Diuretics may be given for peripheral oedema due to cor pulmonale (see p. 103).

The examination. Look for:

Arrange further care

(a) cyanosis, confusion, exhaustion, level of activity; (b) severity of breathlessness; (c) peripheral oedema, right heart failure.

* Consider admission if there is:

Consider:

(a) the patient's social circumstances;

(a) confusion, or deteriorating general condition; or (b) cyanosis or the patient is already on longterm oxygen therapy; or (c) severe breathlessness and poor response to treatment; or

REFERENCES

(d) worsening peripheral oedema; or (e) social circumstances are poor or there is an inability to cope at home. * Arrange follow-up bearing in mind the following points: (a) failure to make a recovery should prompt a CXR;

127

(b) smoking cessation should be encouraged and lifestyle advice given; (c) regular treatment should be optimized. Hospital at home. Some chest units are developing support systems to allow for early discharge after an initial assessment in hospital.85,86

REFERENCES 1. Scottish Intercollegiate Guidelines Network (SIGN). Primary care management of asthma. SIGN Publication No. 33. November 1998. 2. Hetzel MR, Clark TJ. Comparison of normal and asthmatic circadian rhythms in peak expiratory flow rate. Thorax 1980; 35: 732-8. 3. Jamison JP, McKinley RK. Validity of peak expiratory flow rate variability for the diagnosis of asthma. Clin Sci 1993; 85: 367-71. 4. Frischer T, Kuhr J, Meinhert R et al. Relation between response to exercise and diurnal variability of peak expiratory flow in primary school children. Thorax 1993; 48: 249-53. 5. Sly PD, Cahill P, Willet K, Burton P. Accuracy of mini peak flow meters in indicating changes in lung function in children with asthma. BMJ 1994; 308: 572-4. 6. Cochran D. Diagnosing and treating chesty infants. BMJ 1998; 316: 1546-7. 7. Bush A. Diagnosis of asthma in children under five. Asthma Gen Pract 2000; 8 (1): 4-6. 8. Hammarquist C, Burr ML, Gotzsche PC. House dust mite control measures for asthma (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 9. Gotzsche PC, Hammarquist C, Burr M. House dust mite control measures in the management of asthma: meta-analysis. BMJ 1998; 317: 1105-10. 10. van Cauwenberge D, Bachert C, Passalacqua G. Consensus statement of the treatment of allergic rhinitis. European Academy of Allergology and Clinical Immunology. Allergy 2000; 55: 116-34. 11. British Thoracic Society. Guidelines on the management of Asthma. Thorax 1993; 48: Sl-24. 12. British Thoracic Society. The British guidelines on asthma management: 1995 review and position statement. Thorax 1997; 52 (Suppl. 1): Sl-20. 13. Adams NP, Bestall JB, Jones PW. Inhaled beclomethasone versus placebo for chronic asthma (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 14. Gates C, FitzGerald M. Asthma. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. 15. Barnes PJ. Inhaled glucocorticoids: new developments relevant to updating of the Asthma Management Guidelines. Respir Med 1996; 90: 379-84. 16. Cochrane MG, Bala MV, Downs KE et al. Inhaled corticosteroids for asthma therapy: patient compliance, devices and inhalation technique. Chest 2000; 117 (2): 542-50.

17. Agertoft L, Pedersen S. Effect of long-term treatment with inhaled budesonide on adult height in children with asthma. New Engl J Med 2000; 343: 1064-9. 18. The CAMP study. Long term effects of budesonide or nedocromil in children with asthma. The Childhood Asthma Management Program Research Group. New Engl J Med 2000; 343 (15): 1054-63. 19. Lipworth BJ. Systemic adverse effects of inhaled corticosteroid therapy. A systematic review and meta-analysis. Arch Intern Med 1999; 159: 941-55. 20. Walters EH, Walters J. Inhaled short acting beta2 agonist use in asthma: regular versus as needed treatment (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 21. Haahtela T, Jarvinen M, Kava T et al. Comparison of a beta agonist, terbutaline, with an inhaled corticosteroid, budesonide, in newly detected asthma. New Engl J Med 1991; 325: 388-92. 22. Pauwels RA, Lofdahl CG, Postma DS et al. (FACET) Effect of inhaled formoterol and budesonide on exacerbations of asthma. New Engl J Med 1997; 337: 1405-11. 23. Shrewsbury S, Pyke S, Britton M. Meta-analysis of increased dose of inhaled steroid or addition of salmeterol in symptomatic asthma (MIASMA) BMJ 2000; 320: 1368-73. 24. Tasche MJA, Uijen JHJM, Bernsen RMD et al. Inhaled disodium cromoglycate (DSCG) as maintenance therapy in children with asthma: a systematic review. Thorax 2000; 55: 913-20. 25. National Institute for Clinical Excellence (NICE). Guidance on the use of inhaler systems (devices) in children under the age of 5 years with chronic asthma. Technology Appraisal Guidance No. 10. 26. DTB. Inhaler devices for asthma. Drugs Ther Bull 2000; 38 (2): 9-14. 27. Pearson M, Bucknall C. Measuring clinical outcome in asthma: a patient focussed approach. R Col Physicians 1999 (July), London. 28. Gibson PG, Coughlan J, Wilson AJ et al. Selfmanagement education and regular practitioner review for adults with asthma (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2000. 29. Scottish Intercollegiate Guidelines Network (SIGN). Emergency management of acute asthma. SIGN Publication No. 38. June 1999. 30. British Thoracic Association. Death from asthma in two regions of England. BMJ 1982; 285: 1251-5.

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31. Gates CJ, Rowe BH. Holding chambers versus nebulisers for beta agonist treatment of acute asthma (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 32. Stoodley RG, Aaron SD, Dales RE. The role of ipratropium bromide in the emergency management of acute asthma exacerbation: a meta-analysis of randomised clinical trials. Ann Emerg Med 1999; 34: 8-18. 33. Plotnick LH, Ducharme FM. Should inhaled anticholinergics be added to beta agonists for treating acute childhood and adolescent asthma? A systematic review. BMJ 1998; 317: 971-7. 34. Rowe BH, Spooner C, Ducharme FM et al. Early emergency department treatment of acute asthma with systemic corticosteroids (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 35. Macfarlane J, Holmes W, Card P et al. Prospective study of the incidence, aetiology and outcome of adult lower respiratory tract illness in the community. Thorax 2001; 56: 109-14. 36. Macfarlane JT, Colville A, Guion A et al. Prospective study of aetiology and outcome of adult lower-respiratory-tract infections in the community. Lancet 1993; 341: 511-14. 37. Metlay JP, Kapoor WN, Fine MJ. Does this patient have community acquired pneumonia? Diagnosing pneumonia by history and physical examination. JAMA 1997; 278: 1440-5. 38. European Respiratory Society. Guidelines for management of adult community acquired lower respiratory tract infections. Eur Respir J 1998; 11: 986-91. 39. The British Thoracic Society. Guidelines for the management of community acquired pneumonia in adults. Thorax 2001; 56 (IV): 1-64. 40. Farr BM, Woodhead MA, MacFarlane JT et al. Risk factors for community-aquired pneumonia diagnosed by general practitioners in the community. Respir Med 2000; 94: 422-7. 41. Fine MJ, Smith MA, Carson CA et al. Prognosis and outcome measures of patients with community acquired pneumonia: a meta analysis. JAMA 1996; 275: 134-41. 42. Ewig S, Schafer H, Torres A. Severity assessment in community acquired pneumonia. Eur Respir J 2000; 16: 1193-201. 43. Macfarlane JT, Holmes WF, Macfarlane RM. Reducing reconsultations for acute lower respiratory illness with an information leaflet: a randomised controlled study of patients in primary care. Br J Gen Pract 1997; 47: 719-22. 44. Mossad SB, Mackin ML, Medendorp SV, Mason P. Zinc gluconate lozenges for treating the common cold. Ann Intern Med 1996; 125: 81-8. 45. Smucny J, Fahey T, Becker L et al. Antibiotics for acute bronchitis (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2001. 46. Smucny JJ, Becker LA, Glazier RH, Mclsaac W. Are antibiotics effective treatment for acute bronchitis? A meta analysis. J Fam Pract 1998; 47: 453-60. 47. Bent S, Saint S, Vittinghof E, Grady D. Antibiotics in acute bronchitis: a meta analysis. Am J Med 1999; 107: 62-7.

48. Holmes WF, Macfarlane JT, Macfarlane RM et al. The influence of antibiotics and other factors on reconsultation for acute lower respiratory tract illness in primary care. Br J Gen Pract 1997; 47: 815-8. 49. Gonzales R et al. Decreasing antibiotic use in ambulatory practice. Impact of a multidimensional intervention on the treatment of uncomplicated acute bronchitis in adults. JAMA 1999; 281: 1512-19. 50. Marrie T. Community acquired pneumonia. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. 51. BNF. Immunological products and vaccines. British National Formulary March 2001; 41: Section 14.4, 559-77. 52. Joint Tuberculosis Committee of the British Thoracic Society. Chemotherapy and management of tuberculosis in the United Kingdom: recommendations 1998. Thorax 1998; 53: 536-48. 53. Ormerod LP, Charlett A, Gilham C et al. Geographical distribution of tuberculosis notifications in national surveys of England and Wales in 1988 and 1993: report of the PHLS/BTS/DOH Collaborative Group. Thorax 1998; 53: 176-81. 54. Joint Tuberculosis Committee of the British Thoracic Society. Control and prevention of tuberculosis in the United Kingdom: Code of Practice 2000. Thorax 2000; 55: 887-901. 55. British Thoracic Society. The BTS Guidelines for the management of chronic obstructive pulmonary disease. Thorax 1997; 52 (S5): Sl-28. 56. Roberts CM, Bulger JR, Melchor R et al. Value of pulse oximetry in screening for long term oxygen therapy requirement. Eur Respir J 1993; 6: 559-62. 57. British Thoracic Society. COPD guidelines summary, December 1997. Thorax 1997; 52 (Suppl. 5): Sl-28. 58. Callahan CM, Citrus RS, Katz BR Oral corticosteroid therapy for patients with stable chronic obstructive pulmonary disease. Ann Intern Med 1991; 114: 216-23. 59. Fletcher C, Peto R. The natural history of chronic airflow obstruction. BMJ 1977; 1: 1645-8. 60. Anthonisen NR. The Lung Health Study. Effects of smoking intervention and the use of an inhaled anticholinergic bronchodilator on the rate of decline of FEVj. JAMA 1994; 272: 1497-1505. 61. Lacasse Y, Guyatt GH, Goldstein RS. The components of a respiratory rehabilitation program. A systematic overview. Chest 1997; 111: 1077-88. 62. Ferreira IM, Broks D, Lacasse Y, Goldstein RS. Nutritional supplementation for stable COPD (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 63. Poole PJ, Chacko E, Wood-Baker RWB et al. Influenza vaccine for patients with chronic obstructive pulmonary disease (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 64. BNF. Immunological products and vaccines. British National Formulary March 2001; 41: Section 14.4, 559-77. 65. Kerstjens H, Postma D. COPD. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. 66. Sestini P, Renzoni E, Robinson S et al. Short acting beta2 agonists for stable COPD (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 67. Appleton S, Smith B, Veale A, Bara A. Long acting beta2 agonists for COPD (Cochrane Review).

REFERENCES

68.

69.

70. 71. 72. 73.

74. 75. 76. 77.

In: The Cochmne Library, Issue 4. Oxford: Update Software, 2000. Burge PS, Calverley PMA, Jones PW et al on behalf of ISOLDE. Randomised, double blind, placebo controlled study of fluticasone propionate in patients with moderate to severe chronic obstructive pulmonary disease: the ISOLDE study. BMJ 2000; 320: 1297-303. Pauwels RA, Lofdahl CG, Laitenen LA et al for EUROSCOP. Long term treatment with inhaled budesonide in persons with mild chronic obstructive pulmonary disease who continue smoking. New Engl J Med 1999; 340: 1948-53. Anonymous. Inhaled corticosteroids: their role in chronic obstructive pulmonary disease. MeRec Bulletin 2000; 11 (6): 21-4. Lacasse Y, Wong E, Guyatt GH et al. Meta analysis of respiratory rehabilitation in chronic obstructive pulmonary disease. Lancet 1996; 348: 1115-19. British Thoracic Society. Current best practice for nebuliser treatment. Thorax 1997; 52 (S2): Sl-106. Medical Research Council Working Group. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Lancet 1981; i: 681-6. Crockett AJ, Cranston JM, Moss JR et al. Domiciliary oxygen for COPD (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. Gar rod R, Paul EA, Wedzicha W. Supplemental oxygen during pulmonary rehabilitation in patients with COPD with exercise hypoxaemia. Thorax 2000; 55: 539-43. Killen JWW, Corris PA. A pragmatic assessment of the placement of oxygen when given for exercise induced dyspnoea. Thorax 2000; 55: 544-6. Light RW, Merrill EJ, Despars JA et al. Prevalence of depression and anxiety in patients with COPD:

78.

79. 80.

81.

82. 83. 84.

85.

86.

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relationship to functional capacity. Chest 1985; 87: 35-8. Gore JM, Brophy CJ, Greenstone MA. How well do we care for patients with end stage chronic obstructive pulmonary disease (COPD)? A comparison of palliative care and quality of life in COPD and lung cancer. Thorax 2000; 55: 1000-6. BNF. Prescribing in palliative care. British National Formulary March 2001: 13-17. O'Brien C, Guest PJ, Hill SL et al. Physiological and radiological characterisation of patients diagnosed with chronic obstructive pulmonary disease in primary care. Thorax 2000; 55: 635-42. Anthonisen NR, Manfreda J, Warren CPW et al. Antibiotic therapy in exacerbations of chronic obstructive pulmonary disease. Ann Intern Med 1987; 106: 196-204. Saint S, Bent S, Vittinghoff E, Grady D. Antibiotics in chronic obstructive pulmonary disease exacerbations. A meta-analysis. JAMA 1995; 274: 1131-2. Wood-Baker R, Walters EH, Gibson P. Corticosteroids for acute exacerbations of COPD. Cochrane Library, Issue 4. Oxford: Update Software, 2000. Niewoehner DE, Erbland ML, Deupree RH et al. Effect of systemic glucocorticoids on exacerbations of chronic obstructive pulmonary disease. New Engl J Med 1999; 340: 1941-7. Gravil JH, Al-Rawas OA, Cotton MM. Home treatment of exacerbations of COPD by an acute respiratory assessment unit. Lancet 1998; 351: 1853-5. Davies L, Wilkinson M, Bonner S et al. 'Hospital at home' versus hospital care in patients with exacerbations of chronic obstructive pulmonary disease: prospective randomised controlled trial. BMJ 2000; 321: 1254-8.

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7

CHAPTER CONTENTS Dyspepsia

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Gastro-oesophageal reflux disease Colorectal cancer

135

Irritable bowel syndrome Constipation 137 Chronic constipation

136

139

Inflammatory bowel disease Ulcerative colitis 140 Crohn's disease 141 Adult coeliac disease

Gastroenteritis

144

References

145

140

142

DYSPEPSIA

143 143

Gastrointestinal bleeding Jaundice

Gastroenterological problems

138

Faecal incontinence

Postgastrectomy

134

144

Guideline: British Society of Gastroenterology. Dyspepsia Management Guidelines. London: BSG, 1996. Available on www.bsg.org.uk Systematic review: Delaney BC, Innes MA, Deeks J et al. Initial management strategies for dyspepsia (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001.

A logical approach is determined by the following points: • Patients who consult with dyspepsia have no more symptoms than those with dyspepsia who do not consult; but they do have more 'life events' and are more likely to fear that they have a life-threatening condition.1 • Clinical diagnosis without investigation is unreliable2 - even gastroenterologists are correct in less than half their cases. Division of patients with dyspepsia into subgroups according to symptoms does not predict pathology at endoscopy.3 It is still, however, used to determine what symptomatic treatment should be offered to the patient who has not undergone endoscopy. • Early carcinomas of the stomach and oesophagus are potentially curable, but rare in younger patients. • H2-antagonists and proton pump inhibitors will improve symptoms in most serious cases of dyspepsia, including carcinoma. • Elderly patients with peptic ulceration, especially those on NSAIDs, are considerably more 131

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GASTROENTEROLOGICAL PROBLEMS

liable to complications than are younger, fitter patients. • Endoscopy resources are limited and must be reserved in the first instance for those most likely to have positive findings, e.g. carcinoma of the stomach or oesophagus, peptic ulcer, gastritis, duodenitis, oesophagitis or stricture.

Indications for endoscopy at first presentation Guidelines: Axon ATR et al. Guidelines on appropriate indications for upper gastrointestinal endoscopy. BMJ 1995; 310:853-6. Logan R, Delaney B. Implications of dyspepsia for the NHS. BMJ 2001; 323: 675-7. DoH. Guidelines for urgent referral of patients with suspected cancer. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/cancer/referral.htm

Refer patients: (a) aged 55 or over with a recent onset of dyspepsia/* with continuous symptoms^ or a change in symptoms; (b) of any age with risk factors for peptic ulcer, e.g. family history or NSAID use; (c) with severe or long-standing symptoms; (d) with weight loss;§ (e) with anorexia; (f) with vomiting,§ especially if there has ever been any blood in the vomit; (g) with anaemia,§ especially if faecal occult blood is present; or with overt GI bleeding; (h) with dysphagia§ or pain on swallowing. In dysphagia a barium swallow is necessary before endoscopy; (i) with an epigastric mass;§ (j) with a past history of gastric ulcer;§ (k) with a family history of upper GI cancer in >2 first degree relatives;§ (1) with a history of Barrett's oesophagus;§ (m) with pernicious anaemia;§ (n) with a history of surgery for peptic ulcer >20 years ago;§ (o) with known dysplasia,§ atrophic gastritis§ or intestinal metaplasia;§ or (p) jaundice.§

§ Referral is urgent (to be seen within 2 weeks) in these conditions.4

* Check Hb in all the above. * Give antacids while referral is proceeding. If the endoscopy is likely to be delayed for more than 4 weeks, consider giving a course of acid suppression for 2-4 weeks but complete the course at least 4 weeks before the endoscopy to avoid masking the pathology. * If endoscopy is negative and symptoms do not respond to acid suppression, consider alternative investigations: ultrasound of the gall bladder and pancreas, serum amylase or lipase, and LFTs during an exacerbation of pain. Consider other causes of abdominal pain.

Patients less likely to have positive endoscopic findings It is customary to treat these patients empirically, with endoscopy reserved for those who fail to respond or who relapse frequently. Against this approach is the fact that a response to acid suppression does not select those patients who will subsequently have a positive endoscopy. There is insufficient evidence to say that one approach is superior to the alternatives. The main options are therefore: (a) treat symptomatically with antacids, stepping up to an H2 antagonist then to a proton pump inhibitor (PPI) if necessary; or (b) test and treat; i.e. test all dyspeptics for H. pylori and eradicate it in those who are positive, without endoscopy; or (c) early endoscopy for all, which may be no more expensive than empirical treatment.5 Other options are: (d) test and endoscope if positive. This has been shown to increase endoscopy rates without any benefit to the patient.6 * In the absence of an authoritative current national guideline, the Primary Care Society for Gastroenterology7 recommends option (a), proceeding to option (b) or (c), according to local policy, in patients who relapse frequently or

DYSPEPSIA

whose symptoms are severe or do not respond to symptomatic treatment.

133

Patients in whom the diagnosis has been established Duodenal ulcer

Testing for H. pylori Use a breath test, or, if unavailable, a laboratory serology test enzyme-linked immunosorbent assay (ELISA).The bedside dipstick test on whole blood is unreliable.8 * Stop a proton pump inhibitor (PPI) 2 weeks before the breath test. Wait 4 weeks after eradication before repeating the test. * Serology cannot be used to assess response to eradication. It remains positive for 6-12 months.

Initial management * Explore the patient's fears and reassure as far as is justified. Worry about the seriousness of symptoms is the most significant factor in bringing patients with dyspepsia to their GP.9 * Advise the patient to stop smoking, to reduce coffee and alcohol intake and to avoid aspirin and NSAIDs. * Categorize the patient according to the Chicago Working Party report as follows:10 1. Peptic ulcer-like symptoms and any who are undefinable * Give antacids half-hourly if necessary. Avoid NSAIDs. * // there is no response after 1 week: check Hb and prescribe an H2-antagonist for 2-4 weeks. 2. Reflux-like symptoms * Advise the patient about general measures for reflux oesophagitis (see below). * Prescribe antacids ± alginates in adequate dosage, e.g. magnesium trisilicate or gaviscon 10-20 ml after meals + at night. * If the above fails, prescribe a PPI for 2-4 weeks. 3. Dysmotility-like symptoms Patients complain of bloating, nausea, retching and inability to eat a full meal. They may have symptoms of irritable bowel syndrome (IBS) as well. * Prescribe a prokinetic agent, e.g metoclopramide or, especially in the younger female, domperidone.

* Eradicate H. pylori without prior testing. At the time of writing the current regimen is to give 1 week of: (a) PPI at double the standard dose (in two divided doses); and (b) amoxycillin 1 g b.d.; and (c) clarithromycin 500 mg b. d. If the patient is intolerant of clarithromycin or amoxycillin, either can be changed to metronidazole 400 mg b.d. Alternatively, metronidazole may be used in place of amoxycillin first-line, provided the patient is not from an area of high metronidazole resistance (e.g. a developing country or a UK inner city with a large immigrant community). * If eradication is being given for an active ulcer, or for GI bleeding or to a patient on an NSAID, continue the proton pump inhibitor for a second week at the same dosage. * Only confirm that eradication is successful in complicated or refractory ulcers. Use the breath test, not serology, for confirmation, and delay testing until at least 4 weeks after treatment is completed. If the breath test is positive, give a second course of eradication therapy. * Patients who are still breath-test positive or who relapse after successful H. pylori eradication may have an antibiotic-resistant organism or may need longterm acid suppression. Refer to a gastroenterologist. Gastric ulcer * Test for H. pylori and eradicate if positive. Continue the PPI for 2 months, and ensure that follow-up endoscopy is performed until the ulcer is healed. NSAID-induced peptic ulcer * Stop the NSAID and treat the ulcer as above (but with H. pylori eradication only if testing for H. pylori is positive); or * Continue the NSAID in patients who cannot do without it, and prescribe a healing dose of a

134 GASTROENTEROLOGICAL PROBLEMS

PPI followed by a maintenance dose for as long as the patient continues to take the NSAID. H. pylori testing and eradication is unlikely to benefit the patient, who would anyway remain on long-term acid suppression.11 * Consider use of a COX II selective inhibitor or another newer NSAID, which is less toxic to gastric mucosa. The National Institute for Clinical Excellence (NICE) warns that even COXII-selective inhibitors should be used with caution in patients with a history of peptic ulcer, of GI bleeding or of perforation. NICE'S recommendations for the use of COX II selective inhibitors are summarized in the box below. Recommendations on the use of COX-ll-selective inhibitors12 Use them only in patients at high risk of serious adverse GI adverse effects: (a) Age 65 and over; (b) On concomitant medication which increases the risk of upper GI adverse effects (but not low-dose aspirin); (c) With serious comorbidity; or (d) When prolonged use at maximum dosage is contemplated.

* The benefit from acid suppression is modest.15 PPIs are slightly more effective than H2 antagonists.16 * The benefit from H. pylori eradication in those who are positive is small with a mere 9% (95% CI 40% to 4%) reduction in the number of patients still suffering after treatment.14 * Continue the general measures above, with extra attention to an explanation of the nature of the condition. * Explore whether psychological factors are playing a part. * Give antacids for acid-like symptoms or an prokinetic agent for dysmotility-like symptoms. * Control more severe symptoms with short courses of acid suppression. Before repeating or continuing acid suppression, remember that the placebo response in non-ulcer dyspepsia is marked.17 Consider an antidepressant, even in patients who are not clinically depressed. * Consider referral for counselling or psychotherapy for those in whom the psychological component appears to be large.18

They should not be combined with a gastroprotective agent.

Duodenitis * Erosive duodenitis is part of the spectrum of duodenal ulcer disease and should be treated in the same ways as duodenal ulcer. Non-erosive duodenitis and gastritis should be treated symptomatically without H. pylori eradication, even if they are H. pylori positive. Patients on long-term acid suppression for a proven peptic ulcer * Consider H. pylori eradication, without further investigation, as for newly diagnosed patients.13 Established diagnosis of non-ulcer dyspepsia • This is the final diagnosis in 90% of patients with dyspepsia under the age of 45 and 75% of such patients who are H. pylori positive.14

GASTRO-OESOPHAGEAL REFLUX DISEASE (GORD) • The diagnosis is made either by a classical history or by the finding of oesophagitis on endoscopy • A normal endoscopy does not exclude the diagnosis. As many as 50% of patients whose symptoms are due to reflux will have no visible oesophagitis. • Endoscopy is, however, essential in the diagnosis of Barrett's oesophagus and stricture. These patients need long-term acid suppression and regular endoscopy. Otherwise, the aim in patients with or without oesophagitis is to control symptoms. • Advise the patient to: (a) elevate the bed head by 6 inches; (b) stop smoking (two cigarettes in 20 minutes reduces lower oesophageal sphincter pressure by 50% and increases acid secretion);

COLORECTAL CANCER

(c) avoid fatty foods, alcohol, coffee, onions, peppermint, citrus fruits, tomatoes and other foods known to make symptoms worse. Weight loss in the obese may help symptoms; (d) avoid aspirin and other NSAIDs; (e) avoid lying down with a full stomach, and preferably eat 4 hours before going to bed. * Mild symptoms: 'step-up' as follows, moving to the next step if the previous one fails to control symptoms: 1. Antacids and/or alginates in adequate doses after meals and at night. Raft antacids cost about four times as much, but appear to give better relief of symptoms than antacids alone. 2. Acid suppression: a PPI daily for 4-8 weeks. This controls symptoms in 83% and heals oesophagitis in 78%, as opposed to 60% and 50% for H2 antagonists. Continuous use of a PPI gives the best results in heartburn19 but is not recommended in the UK for mild-moderate symptoms for reasons of cost (see box on NICE guidance below). 3. Prakinetic agents may be helpful in individual patients, either alone or in combination with a proton pump inhibitor. * Severe symptoms: 'step-down' treatment, starting with a PPI, and reducing to an H2 antagonist or antacid as symptoms resolve. If symptoms are only controlled by a PPI, continue it at the lowest dose that gives control. Patients who do not respond should be referred for endoscopy as below. * Surgery: consider referral of young patients (e.g. under age 50) uncontrolled by the above measures to a surgeon who specializes in fundoplication.20 Also consider referral in those in whom reflux has led to inhalation of gastric contents with respiratory symptoms, and in those in whom a hiatus hernia is causing mechanical problems. Fundoplication, however, frequently causes difficulty with swallowing, belching and dyspepsia.

Guidance on the use of PPIs from the National Institute for Clinical Excellence NICE. Guidance on the use of proton pump inhibitors in the treatment of dyspepsia. London: NICE, 2001. Online. Available: www.nice.org.uk

135

PPIs are appropriate: (a) When a patient on an NSAID must continue it and has a documented NSAID-induced ulcer. The dose should be stepped down for maintenance. (b) As part of an H. pylori eradication programme. (c) For GORD, either where symptoms are severe or with proven pathology (oesophageal ulceration or Barrett's oesophagus). The dose should be 'stepped down' except in cases of complicated oesphagitis (stricture, ulcer or haemorrhage). (d) In other situations, including undiagnosed dyspepsia, a short course may be indicated where other symptomatic treatment has failed.

COLORECTAL CANCER Guidelines: SIGN. Colorectal cancer: a national guideline recommended for use in Scotland by the Scottish Intercollegiate Guidelines Network. Scottish Intercollegiate Guidelines Network, 1997. Online. Available: www.sign.ac.uk Department of Health. Guidelines for the urgent referral of patients with suspected cancer. London: DoH, 2000. Online. Available: www.doh.gov.uk/ cancer/referral.htm

* Refer patients for screening if they have a sufficiently strong family history (see p. 136). * Refer patients of any age urgently (to be seen within 2 weeks21) who have a new occurrence of: (a) a right-sided abdominal mass; or (b) a rectal (not pelvic) mass; or (c) rectal bleeding and a change in bowel habit to looser stools and/or increased frequency of defecation persistent for 6 weeks; or (d) iron deficiency anaemia without an obvious cause (Hb <11 g/dl in men or <10g/dl in postmenopausal women). * Refer patients age >60 urgently who have a new occurrence of: (a) persistent rectal bleeding without anal symptoms; (b) or a change in bowel habit to looser stools and/or increased frequency of defecation persistent for 6 weeks without rectal bleeding. * Refer patients with other colorectal symptoms, but with less urgency.

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Note that it is not possible to decide on the need for urgent referral without performing abdominal and rectal examinations.

Screening for colorectal cancer Screening faeces for occult blood and/or endoscopy can detect colorectal cancer early and reduce mortality.22 Whole population screening is recommended in the USA for those age 50 and over but not in the UK, where the practice is to screen only those at high risk: (a) family history: - a first-degree relative with colorectal cancer before the age of 45 (lifetime risk = 1 in 10); -two first-degree relatives with colorectal cancer at any age (lifetime risk = 1 in 6); - a family history of hereditary non-polyposis colorectal cancer (HNPCC, see below) (lifetime risk = 1 in 3); - a first degree relative with familial adenomatous polyposis (FAP) (lifetime risk = 1 in 2). Screening should start 10 years before the age at which the relative developed the cancer. Total colonoscopy is required. (b) Personal history: -a previous colorectal cancer, successfully removed; - a previous adenomatous colonic polyp; - longstanding widespread inflammatory bowel disease. HNPCC exists when the family history meets the following criteria: Three or more relatives have colorectal cancer; and one of them is a first degree relative of one of the others; and at least two generations are affected; and one was diagnosed before the age of 50.

• Irritable bowel syndrome is a functional disorder of the whole GI tract characterized by abdominal pain with disturbed bowel function, and which may be associated with dysmotility of the upper GI tract. The GP can and should make a positive diagnosis of IBS, and so avoid the overinvestigation that tends to increase the patient's excessive concern about the symptoms. • Symptoms of IBS are present in up to 20% of adults, although up to 75% of them never consult. • IBS is often associated with symptoms from outside the GI tract: lethargy, backache, headache, dyspareunia and an irritable bladder. • Anecdotal evidence suggests that an episode of infective diarrhoea may trigger the onset of symptoms. • Anxiety and depression are more common in patients with IBS than in the normal population, although the symptoms are often masked. Even those without an affective disorder tend to show an excessive concern about their symptoms.

Patients who can be diagnosed from the history In the majority of patients, a positive diagnosis can be made from the history, using the Rome II criteria23 (see box below). The Rome II criteria for the diagnosis of IBS Abdominal discomfort or pain for 12 weeks or more in the last year with at least two of the following features: 1. relieved by defecation; 2. onset associated with a change in the frequency of the stool; 3. onset associated with a change in the form of the stool; especially if any of the following are present: (a) abnormal stool frequency (>3 a day or <3 a week); (b) abnormal stool form; (c) abnormal passage of stool (straining, urgency or a feeling of incomplete defecation); (d) mucus; (e) a feeling of abdominal distension.

IRRITABLE BOWEL SYNDROME (IBS) Jones J, Boorman J, Cann P et al for the British Society of Gastroenterology. Guidelines for the management of the irritable bowel syndrome. Gut 2000; 47 (Suppl. II) 1-9. Online. Available: www.bsg.org.uk

Patients in whom organic disease must be excluded * Refer those: (a) over 40 years of age with symptoms of recent onset;

CONSTIPATION

(b) with a change in symptoms; (c) passing blood; (d) with loss of appetite or loss of weight; (e) who have abdominal pain and altered bowel habit not fulfilling the Rome II criteria; (f) who have a family history of cancer of the colon, breast, ovary or uterus; (g) who feel unable to accept the diagnosis of IBS after full explanation; (h) who have severe symptoms despite the management outlined below.

Management of patients with a clear diagnosis of IBS * The GP's attitude at the first consultation may be critical. Show that you are taking the symptoms seriously, and examine the abdomen before reassuring the patient that there is no organic disease. * Ask about the patient's fears, and especially the fear of cancer. * If some investigation seems necessary, if only to assist in the reassurance, restrict it to FBC, ESR and stool for occult blood and pathogens. Consider hypothyroidism in the older patient. * Explain the nature of the condition, and explore with the patient whether stress or diet is playing a part. * Diet. Explain that in up to 50% of patients certain foods can precipitate attacks. Encourage patients to keep a diary to explore this. Alcohol, tea and coffee are common precipitants. A diet free of wheat, eggs and milk is occasionally helpful in those with diarrhoea. Other exclusion diets are unlikely to be helpful.24 Those with distension should avoid flatus-producing foods, e.g. onions, beans, celery, carrots, Brussels sprouts and wheat germ. * Smoking. Some patients report improvement after stopping smoking. * Constipation. For patients with constipation and only minor pain, recommend wheat bran. The patient should take increasing amounts of unprocessed bran, wholemeal bread or high-bran cereal until the motions are soft and regular. This has the advantage of not adding to the patient's conception of IBS as a disease. Bran may, however, make the pain of IBS worse while

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helping the constipation.25 If this happens, recommend the patient increase the intake of fibre with fruit, vegetables, wholemeal rice and pasta. Of the prescribable bulk laxatives, only ispaghula has been shown to be more effective than bran.26 * Diarrhoea. Use codeine phosphate, loperamide, or co-phenotrope for acute exacerbations of the diarrhoea. * Pain. Antispasmodics may help pain and diarrhoea. None is strikingly helpful, but mebeverine 1 tablet t.d.s. 20 minutes before meals is probably the best buy. Use for 4 weeks. If unhelpful, double the dose and only continue thereafter if symptoms improve.27 Consider using low doses of a tricyclic antidepressant for its anticholinergic effect, with full warning about the potential adverse effects. * Upper GI tract dysmotility symptoms. See dyspepsia, p. 133. * Recommend the self-help group, IBS Network, Centre for Human Nutrition, Northern General Hospital, Sheffield S5 7AU. Online. Available: www.ibsnetwork.org.uk Treatment of the psychological component * Explore the patient's mood and attitude to the condition in more detail. * Consider counselling or psychotherapy for those with an excessive concern with their symptoms. * Depression. Make a choice about counselling/ psychotherapy and antidepressant drugs (see page 310). If using an antidepressant, use a tricyclic for patients with diarrhoea and an SSRI for patients with constipation. * Hypnotherapy may help those still distressed by symptoms. 85% of patients unresponsive to other therapy may be helped.28 Contact the British Society of Medical and Dental Hypnosis, 4 Kirkwood Ave, Cookridge, Leeds LSI6 7JU www.bsmdh.org

CONSTIPATION NHS Centre for Reviews and Dissemination. Effectiveness of laxatives in adults. Effective Health Care 2001; 7(1).

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• Constipation is defined by the Rome II criteria as present when two or more of the following have been present for at least 12 weeks in the last year:29 (a) straining at stool at least a quarter of the time; (b) lumpy and/or hard stools at least a quarter of the time; (c) a sensation of incomplete evacuation at least a quarter of the time; (d) three or fewer bowel movements per week. • Ten per cent of the British population are regularly constipated (3% of young adults, 20% or more in the elderly). A further large number use laxatives regularly because of an unjustified expectation of regularity. • The frequency of constipation largely relates to the intake of dietary fibre, which has declined from about 40 g per day 100 years ago to less than 15-20 g per day today. Fibre reduces transit time, and increases stool bulk and softness. • Many patients who complain of constipation are suffering from irritable bowel syndrome (IBS) and have normal transit times. They can be distinguished by the fact that, however much difficulty they feel they have in opening their bowels, they do not pass the hard and often pellet-like motions which are diagnostic of constipation. High fibre is worth using (see above and p. 139), but these patients should not be given laxatives.

High fibre diet • Encourage the patient to increase the fibre content of the diet slowly. This will minimize the likely side-effects of bloating and flatulence, which will tend to subside anyway over 1-2 months. • Aim for 30 g of fibre and at least 2 litres of fluid per day. The fibre should be obtained from as many different sources as possible. 5 g is found in: (a) two wholewheat cereal biscuits; (b) two slices of wholemeal bread or three of brown; (c) two apples, oranges, pears or bananas; (d) one helping of wholemeal pasta; (e) one helping of baked beans, peas, swedes or sprouts; (f) two helpings of boiled or baked potatoes, in their skins; (g) two helpings of carrots or lentils;

(h) half a helping of kidney beans; or (i) one and a half sachets of Fybogel or Regulan. Insoluble particulate fibre is most effective (e.g. wheat bran); small particle or soluble fibre is less effective. Porridge and muesli contain oat bran, which is soluble, and so has less laxative value.

Acute constipation * Acute constipation most commonly occurs in patients recently confined to bed, those on opiates or other constipating drugs, during the immediate postnatal period or when defecation is painful. * Constipation as a new symptom where there is no obvious cause raises the possibility of colorectal carcinoma, and warrants abdominal and rectal examination and referral (see p. 135). * Anticipate if possible and give a stimulant laxative prophylactically, e.g. two senna tablets at night; the breast-feeding mother should be given bisacodyl instead. * Continue the stimulant and increase tablets to four or even eight at night if constipation does occur. * Disimpact if necessary. Disimpaction * Insert a suppository at the time of examination and prescribe a further supply. * Arrange for an enema to be administered or a manual removal to be performed. (a) If a rapid response is needed - use a sodium phosphate or sodium citrate enema; (b) If a more gentle response is needed - use an arachis oil enema; (c) For hard faeces in the rectum - use a 5ml enema containing a faecal softener, e.g. Fletcher's Enemette or Micolette.

CHRONIC CONSTIPATION Assessment * Confirm from the history that the patient is really constipated and not suffering from the abnormal non-propulsive contractions of IBS.

FAECAL INCONTINENCE

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* Ask whether there is soiling (see Faecal incontinence, below). Patients rarely volunteer this information. * Examine the abdomen and rectum. * Be alert for disorders that present as constipation, especially carcinoma of the large bowel and hypothyroidism. * Review the patient's medication to exclude iatrogenic causes.

(b) a stool softener. Docusate orally may be ineffective.32 Liquid paraffin is too dangerous alone, but seems safe as liquid paraffin and magnesium hydroxide emulsion. (c) an osmotic agent. Magnesium sulphate is effective in 2 hours, but should not be used more than once a week. Lactulose is more expensive, and has been shown to be less effective than a combination of senna and fibre.33

Treatment

* Consider referral of patients with intractable constipation for colorectal function studies. Surgery may be helpful in the most severe cases.

* Explain that regular evacuation depends on a regular stool habit, a diet high in fibre, regular exercise and regular meals. * Fluid intake. Encourage extra fluid intake in the elderly. * Time and privacy for defecation. Time must be set aside; colonic activity peaks after breakfast. * Fibre. Encourage increased dietary intake of fibre (see above). Systematic review of the literature does not show any fibre or laxative to be superior to any other.30 Where dietary fibre is not well tolerated, consider prescribing ispaghula, sterculia or methyl cellulose. These must be taken with a full glass of water or, in the case of methyl cellulose, two glasses. Do not add fibre in patients with constipation due to opiates, nor where it is associated with a hugely distended colon, nor in impaction. Warn patients that they should not have unrealistic expectations of the benefit that is likely from fibre. The mean increase is only 1.4 extra movements per week.31 * Laxatives. If there is no improvement or improvement is not maintained, start a stimulant laxative (e.g. senna 2-8 tablets or bisacodyl 2-4 tablets) at night or an osmotic laxative, or both. Find the dose that produces a motion the next morning. Prescribe it once or twice a week only, explaining the dangers of habituation if it is used more frequently.

Poor responders Poor responders may need a laxative from each group as well as fibre both dietary and prescribed: (a) a stimulant, e.g. senna or bisacodyl, or danthron in the elderly or terminally ill;

FAECAL INCONTINENCE Review: Kamm MA. Fortnightly review: Faecal incontinence. BMJ1998; 316: 528-32.

• Faecal incontinence is found in 25-35% of elderly people in institutions.34 Patients at home may have milder degrees of incontinence, for which they rarely seek help. Treatment should be related to the underlying cause: (a) Diarrhoea. Treat with an antidiarrhoeal agent once overflow diarrhoea has been excluded. (b) Sphincter weakness or damage. Make the stool firmer by reducing the fibre intake and stopping laxatives, and if necessary giving a constipating drug such as loperamide or codeine phosphate. Consider referral for surgical repair of the sphincter. Simple repair will cure 80% of women with a sphincter tear from obstetric trauma. (c) Anal sensory impairment. Keep the stool firm, and organize planned defecation with suppositories or enemas. (d) Impaction with overflow. See constipation. (e) Immobility. Reorganize the toiletting arrangements. (f) Loss of higher control, e.g. dementia or after stroke. Organize planned evacuation. (g) Rectal prolapse. Consider referral for surgery.

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(h) Loss of pelvic floor tone. Consider referral to physiotherapy for pelvic floor exercises and even surgery to restore the anorectal angle.

INFLAMMATORY BOWEL DISEASE (IBD) Guidelines: British Society of Gastroenterology. Inflammatory bowel disease. Guidelines in Gastroenterology. London: BSG, 1996. MeReC. Inflammatory Bowel Disease. MeReC Bulletin 1999; 10: 45-8. Online. Available: www.npc.co.uk

• Patients with IBD have made the following points:35 (a) GPs seem to underestimate the severity of their symptoms and delay the original referral; (b) many decisions about treatment require the patient to make a choice, and to do this they need information and time; (c) even if symptoms cannot be alleviated, patients appreciate the doctor who acknowledges their presence. • Training in self-management can reduce consultations and reduce the delay before relapses are managed effectively.36 • The role of the GP lies in the original referral for diagnosis, in the support of the patient with continuing symptoms and in the prompt treatment of relapses. Work-up for inflammatory bowel disease: (a) FBC; (b) ESR; (c) serum albumin; (d) LFTs; (e) stool culture.

* Refer more urgently anyone who is systemically unwell, or whose diarrhoea is heavily blood stained. * Refer anyone with blood and mucus per rectum and with urgency and bowel frequency, even if the stools are formed. * Consider the possibility of IBD in anyone with persistent abdominal pain, especially if associated with perianal disease, weight loss or systemic disturbance.

Management The psychological effects of chronic IBD may be worse than the physical symptoms, and it is easy for doctor and patient to focus mainly on the organic side of the condition. Broach the subject directly and recommend the patients' organisations in the box below. Patient organizations: The National Association for Colitis and Crohn's Disease, 4 Beaumont House, Sutton Road, St Albans, Herts AL1 5HH, tel. 01727 844296; information line 0845 130 2233; www.nacc.org.uk As well as information and support, the association can provide a 'Can't Wait' card, which will allow the patient to use the toilet in major shops. The British Colostomy Association, 15 Station Road, Reading, Berks RG1 1LG, tel. 0118 939 1537; helpline 0800 328 4257; www.bcass.org.uk

For the specific management of IBD see below.

ULCERATIVE COLITIS Management of the acute attack * Admit any patient who: (a) is systemically unwell, especially any who show signs of toxic megacolon; (b) fails to respond after 1 week of high dose prednisolone (see below).

Diagnosis

Home management

* Refer anyone with diarrhoea for 2 weeks which is not improving, provided there is no previous history to suggest irritable bowel syndrome and the stool culture is negative.

* Maintain fluid and carbohydrate intake. * Left-sided disease: If disease is confined to the rectum or rectosigmoid give corticosteroids, e.g. Colifoam, rectally twice a day until symptoms

INFLAMMATORY BOWEL DISEASE 141

have settled. If this fails, give daily rectal enemas of mesalazine for 2-4 weeks, or mesalazine suppositories if disease is confined to the rectum,37 or oral prednisolone, see below. * More generalized disease: Give prednisolone 40 mg daily for 2 weeks then tail off over 4 weeks if symptom-free. Avoid enteric-coated preparations, which may not be absorbed because of rapid transit. Refer urgently if there is no response after 1 week. * Patients on aminosalicylates: increase sulphasalazine or mesalazine to full dose for 1 month, then reduce to maintenance dose. * Diarrhoea may be controlled by codeine phosphate, but this should not be used in the ill patient for fear of precipitating toxic megacolon. * Nutrition: Patients unable to tolerate a simple diet will benefit from a prescribable food product, e.g. Enrich or Ensure. The prescription should be endorsed 'ACES'. Note: All aminosalicylates can cause marrow suppression. Check FBC at the start of treatment and warn patients to report bruising, bleeding, sore throat, fever or malaise.38

Maintenance therapy * Oral aminosalicylates reduce the annual relapse rate from 80% to 20%. They are less helpful in inducing remission. They are all equally effective. Give: (a) sulphasalazine at the minimum effective dose, usually 500 mg q.d.s. The commonest side-effects are nausea and headache; or (b) mesalazine 400 mg t.d.s. or olsalazine 500 mg b.d. for patients intolerant of sulphasalazine or men who do not wish to suffer oligospermia. They are considerably more expensive than sulphasalazine. * Rectal preparations. Rectal steroids or mesalazine may be useful, especially in patients with proctitis or distal colitis. The patient administers one dose each night during an acute attack, reducing to once or twice a week for maintenance. * Systemic corticosteroids are of little value in remission maintenance. * Azathioprine 2mg per kg is occasionally used for remission maintenance, but should only be

started by a specialist. Regular blood counts are needed. * Check the haemoglobin yearly. Anaemia can occur even in patients in remission.

The risk of carcinoma * Patients who have had pancolitis for more than 7-10 years should be reviewed by a specialist annually with a view to colonoscopy. The risk of carcinoma is 17 times that of the normal population. The risk of carcinoma in patients with localized disease is only fourfold, and the need for regular screening is less clear. * Re-refer patients in relapse who: (a) are failing to respond to a course of aminosalicylate and/or steroids; (b) are needing frequent courses of systemic steroids; (c) have symptoms suspicious of carcinoma or other cause for concern.

CROHN'S DISEASE Diagnosis and initiation of treatment is the province of the gastroenterologist, who should always be consulted in the management of exacerbations. The following are used, as in ulcerative colitis: (a) Oral corticosteroids: start with prednisolone 40 mg daily and aim to tail off within 3 months; (b) Rectal corticosteroids for distal disease; (c) Aminosalicylates, although they are less effective than in ulcerative colitis; (d) Azathioprine 100 mg daily, with monthly FBC and LFTs. Of these, probably only azathiaprine is useful in maintaining remission.

Additional measures (a) Diet. A high-fibre diet is of value while in remission, unless strictures are present. During exacerbations stop all other food and give an elemental diet, e.g. Triosorbon six packets a day, tailing off as symptoms settle, provided

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other measures to control the inflammation are in place. (b) Diarrhoea may be controlled by antidiarrhoeals, e.g. codeine phosphate. Diarrhoea caused by free bile acids in patients with ileal resection or dysfunction may be helped by a low fat diet and cholestyramine. (c) Perianal sepsis may respond to metronidazole 400mgb.d. for 7 days. (d) Surgery is likely to be needed by 70-80% of patients at some stage. This is likely to be for stricture, fistula, perianal disease and abdominal mass that does not respond to medical treatment. (e) Postileal resection. Check the vitamin B12 level annually. * Re-refer patients in relapse who: (a) are failing to respond to systemic steroids; (b) are needing frequent courses of systemic steroids; (c) have symptoms suggesting obstruction; (d) have severe weight loss; (e) have symptoms suspicious of carcinoma or other cause for concern.

ADULT COELIAC DISEASE Guidelines: British Society of Gastroenterology. Guidelines for the management of patients with coeliac disease. London: BSG, 1996. Online. Available: www.bsg.org.uk Primary Care Society for Gastroenterology. Decision points in the management of adult coeliac disease in primary care, 1999. Follow up care of adult coeliac disease, 2001. Online. Available: www.pcsg.org.uk

• Coeliac disease is common, with a prevalence in the UK of at least 1 in 300, of whom over twothirds are undiagnosed. The diagnosis in those who present as adults is often made years after the first presentation. • The endomysial antibody (EMA) test is a sensitive screening test for untreated coeliac disease; the sensitivity approaches 100%.39 Its lack of specificity, however, makes it a screening rather than a diagnostic test.

* Between 45% and 87% of patients with an established diagnosis fail to adhere to a glutenfree diet. * Suspect coeliac disease in an adult with diarrhoea and lassitude, especially if there is also anaemia. * Check the EMA. * Refer if the EMA is positive, for intestinal biopsy, without which a diagnosis cannot be made.

Diet * Explain that a gluten-free diet will be needed for life. The patient should not relax this even if symptoms do not return. Patients with coeliac disease who continue to take even small amounts of gluten have an increased risk of lymphoma of the small intestine, osteoporosis and infertility. The exception to this appears to be oats, which may not be harmful to adults, although they should not be recommended in children. * Prescribe adequate amounts of gluten-free products, marking the prescription 'ACES'. * Prescribe supplements of iron, folic acid, vitamin Bj2 and calcium at the time of diagnosis, and continue until the patient is well established on a gluten-free diet. Reintroduce supplements if a relapse occurs. * Women planning a pregnancy should receive folic acid 5mg daily until the 12th week.

Follow up * Check the following annually: (a) symptoms; (b) weight; (c) Hb, B12, folate, ferritin, albumin and calcium. * If compliance is in doubt, check the EMA. A positive result suggests that the patient is not adhering to the diet. * Perform a dual energy X-ray absorptiometry (DEXA) scan for osteoporosis at diagnosis and again at the menopause in women, at age 55 in men, and at any age if a fragility fracture occurs. * Offer immunization against pneumoccocal and H. influenzae type b infection once and against

GASTROENTERITIS 143

influenza annually. Most patients have some degree of splenic atrophy.

* Carcinoma of the stomach. The risk after 20 years is double, and after 45 years is sevenfold.

Uncontrolled symptoms * Re-refer to the dieticians: (a) if there is diarrhoea or weight loss or nutritional deficiency. Small amounts of gluten can be ingested in an unsuspected form, e.g. in commercial gluten-free wheat products; (b) if constipation is the problem; it may be due to the low fibre nature of the diet. * Re-refer to a specialist for reassessment of the diagnosis if dietary assessment fails to solve the problem or if there is abdominal pain or blood in the stool. Even if the original diagnosis was correct, the patient may have developed associated pathology: small bowel lymphoma or adenocarcinoma or ulcerative jejunoileitis.

Dermatitis herpetiformis (DH) DH occurs in 2-5% of patients with coeliac disease. It may take up to a year to respond to a gluten-free diet. It can be controlled in that time with dapsone or, in those intolerant to dapsone, with sulphapyridine. Patient information: Coeliac UK, PO Box 220, High Wycombe, Bucks HP11 2HY, tel. 01494 437278; www.coeliac.co.uk

POSTGASTRECTOMY In 1965, there were an estimated 500,000 patients in the UK who had a partial gastrectomy. All need life-long follow-up. * Check FBC yearly. * Iron deficiency. Fifty per cent become iron deficient. Give prophylactic oral iron for life. * B12 and folate deficiency. Iron deficiency may mask the typical macrocytosis. Check the B12 and folate in any patient whose anaemia does not respond totally to iron.

GASTROENTERITIS * Encourage the patient to eat and drink normally if they can; if not, encourage the use of fruit juices and soups. * Prescribe oral rehydration salts for infants, the frail elderly and any others at risk of dehydration. * Consider prescribing an antidiarrhoeal agent, e.g. loperamide, but not in a child nor in anyone with severe symptoms; it may precipitate ileus or toxic megacolon. * Send stool for culture if more than one member of the family or institution is affected or if there is blood in the stool or systemic upset. * Notify as suspected food poisoning if two or more people who have eaten the same food develop gastroenteritis at the same time. Otherwise, only notify if the stool sample is positive. * Exclude the patient from school or work until the diarrhoea has stopped. Food handlers and possibly healthcare workers should stay away from work until free from diarrhoea for 48 hours (unless typhoid or pathogenic E. coli are isolated, in which case the advice of the Consultant in Communicable Diseases should be sought). * Stress the importance of handwashing. * Antibiotics. Only prescribe antibiotics for the limited number of conditions in which they have been shown to be of benefit. Those endemic in the UK are: (a) campylobacter: use a macrolide or a quinolone; (b) shigella and salmonella: use trimethoprim or a quinolone and then only if there is systemic illness; (c) giardia: use metronidazole or tinidazole; (d) for any other patient with the clinical picture of acute dysentery and who is too unwell to wait for the stool result: use a quinolone. Admission would, however, usually be more appropriate for such a patient.

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GASTROINTESTINAL BLEEDING * Admit patients with current haematemesis or melaena. * Refer: (a) by telephone for urgent endoscopy if the stool has returned to normal colour, but the bleed was within the last 7 days. These patients are very likely to rebleed, and the risk of this happening can be determined at endoscopy; (b) urgently by letter if the bleed was over 7 days before; (c) less urgently if the patient is anaemic with positive occult bloods but without a history of overt bleeding. Stop NSAIDs if they are being taken, but do not assume that they were the cause of the blood loss until other pathology has been excluded. Start oral iron once iron deficiency is proved.

JAUNDICE

Urgent referral (a) Fever, especially if associated with right upper quadrant (RUQ) tenderness and rigors; this may be ascending cholangitis; (b) hepatic pre-coma, i.e. any alteration in mental state; (c) portal hypertension, especially if associated with GI bleeding; (d) an acute exacerbation of chronic liver disease; (e) suspected malignant obstructive jaundice; (f) pain or other evidence of biliary obstruction, or a palpable gall bladder; (g) jaundice in the pregnant patient. If acute and associated with abdominal pain plus malaise it may be acute fatty liver, which carries a 20% mortality rate.

The patient well enough to be kept at home * Base the diagnosis on: (a) the history, including the drug history (e.g. phenothiazines, sulphonylureas, combined oral contraceptive (COC), paracetamol, NSAIDs); (b) the examination;

(c) diagnostic tests: -first-line tests: LFTs, FBC, prothrombin time, hepatitis serology, urine for bilirubin and urobilinogen; -second-line tests: ultrasound scan (USS) or serology for glandular fever, CMV or leptospirosis, according to the results of the first-line tests. * Refer the patient who is not improving or in whom the diagnosis is not clear. A rising prothrombin time or a falling albumin are grounds for urgent referral.

Mild jaundice or a mildly raised bilirubin in a well patient This is likely to be Gilbert's disease (the commonest congenital failure of conjugation, affecting up to 7% in the UK). Diagnosis is on the basis of: (a) a mildly raised bilirubin with no bilirubin in the urine; (b) normal liver enzymes; (c) normal FBC, film and reticulocyte count. * Explain the nature of the condition and that recurrences are likely, especially during periods of relative starvation. * Encourage the patient to warn any other doctor who is performing blood tests that the bilirubin may be raised.

Other aspects of liver disease * Immunization. Consider immunization of contacts if hepatitis is confirmed. * Alcohol. Patients with jaundice should refrain from alcohol until the LFTs are normal. If the jaundice is due to alcohol they should abstain for life, although some hepatologists permit a return to mild drinking after 3 months of normal LFTs (including gamma-glutamyl transferase (GGT)) provided there is no fibrosis or cirrhosis on biopsy. Regular LFTs will be required. * Chronic liver disease. Patients with chronic liver disease should be under hospital surveillance. This permits patients with cirrhosis to be monitored for hepatocellular carcinoma, and all patients with chronic disease to be considered for liver transplantation, if appropriate.

REFERENCES 145

REFERENCES 1. Logan R, Delaney B. Implications of dyspepsia for the NHS. BMJ 2001; 323: 675-7. 2. The Danish Dyspepsia Study Group. Value of the unaided clinical diagnosis in dyspeptic patients in primary care. Am J Gastroenterology 2001; 96: 1417-21. 3. Agreus L, Talley N. Challenges in managing dyspepsia in general practice. BMJ 1997; 315: 1284-8. 4. DoH. Guidelines for urgent referral of patients with suspected cancer. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/cancer/referral.htm 5. Bytzer P, Hansen JM, de Muckadell OBS. Empirical H2-blocker therapy or prompt endoscopy in the management of dyspepsia. Lancet 1994; 343: 811-16. 6. Delaney BC, Wilson S, Roalfe A et al. Randomised controlled trial of Helicobacter pylori testing and endoscopy for dyspepsia in primary care. BMJ 2001; 322:898-901. 7. The Primary Care Society for Gastroenterology. Decision points for the management of H. pylori in primary care. January 1999. Available from: the PCSG, The Department of Primary Health Care, Institute of Health Sciences, Old Road, Headington, Oxford OX3 7LF. 8. Weijnen CF, de Wit NJ, Numans ME et al. Helicobacter pylori testing in the primary care setting: which diagnostic test should be used? Aliment Pharmacol Ther 2001; 15: 1205-10. 9. Lydeard S, Jones R. Factors affecting the decision to consult with dyspepsia: comparison of consulters and non-consulters. / R Coll Gen Pract 1989; 39: 495-8. 10. Colin-Jones et al. Management of dyspepsia: report of a working party. Lancet 1988; 1: 576-9. 11. Seager JM, Hawkey CJ. Indigestion and non-steroidal anti-inflammatory drugs. BMJ 2001; 323: 1236-8. 12. NICE. Guidance on the use of COX II selective inhibitors for osteoarthritis and rheumatoid arthritis. Technology Appraisal No. 27, 2001. Online. Available: www.nice.org.uk 13. Hippisley-Cox J, Pringle M. A pilot study of a randomized controlled trial of pragmatic eradication of H. pylori in primary care. Br J Gen Pract 1997; 47: 375-7. 14. Briggs AH et al. Cost-effectiveness of screening for and eradication of H. pylori in the management of dyspeptic patients under 45 years of age. BMJ 1996; 312: 1321-5. 15. Redstone HA, Barrowman N, Veldhuyzen Van Zanten SJO. H2 receptor antagonists in the treatment of functional (nonulcer) dyspepsia: a meta-analysis of randomised controlled clinical trials. Aliment Pharmacol Ther 2001; 15: 1291-9. 16. Talley NJ, Phung N, Kalantar JS. Indigestion; when is it functional? BMJ 2001; 323: 1294. 17. MeReC. Proton pump inhibitors: their role in dyspepsia. MeReC Bulletin 1998; 11. 18. Soo S, Moayyedi P, Deeks J et al. Psychological interventions for non-ulcer dyspepsia (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 19. Howden CW, Henning JM, Huang B et al. Management of heartburn in a large, randomized, community-based

study: comparison of four therapeutic strategies. Am J Gastroenterol 2001; 96: 1704-10. 20. Taylor TV, Holt S. Antireflux surgery. BMJ 1990; 300: 1603-4. 21. DoH. Guidelines for urgent referral of patients with suspected cancer. London: Department of Health 2000. Online. Available: www.doh.gov.uk/cancer/referral.htm 22. Rhodes JM. Colorectal cancer screening in the UK: joint position statement by the British Society of Gastroenterology, the Royal College of Physicians, and the Association of Coloproctology of Great Britain and Ireland. Gut 2000; 46: 746-8. 23. Thompson WG, Longstreth G, Drossman DA et al. Functional bowel disorders and functional abdominal pain. Gut 1999; 45 (Suppl. II): II43-7. 24. McKee AM, Prior A, Whorwell PJ. Exclusion diets in irritable bowel syndrome: are they worthwhile? / Clin Gastroenterology 1987; 9(5): 526-8. 25. Cann PA, Read NW, Holdsworth CD. What is the benefit of coarse wheat bran in patients with irritable bowel syndrome? Gut 1984; 25(2): 168-73. 26. DTB. Irritable bowel and its treatment. Drug and Therapeutics Bulletin 1983; 21: 37-9. 27. DTB. Some antispasmodic drugs for the irritable bowel syndrome. Drug and Therapeutics Bulletin 1986; 24: 93-5. 28. Whorwell PJ. Diagnosis and management of irritable bowel syndrome: discussion paper. / R Soc Med 1989; 82(10): 613-14. 29. Thompson WG, Longstreth G, Drossman DA et al. Functional bowel disorders and functional abdominal pain. Gut 1999; 45 (Suppl. II): II43-7. 30. Tramonte SM et al. The treatment of chronic constipation in adults. / Gen Intern Med 1997; 12: 15-24. 31. NHS Centre for Reviews and Dissemination. Effectiveness of laxatives in adults. Effective Health Care 2001; 7, No. 1. 32. Chapman R et al. Effect of oral dioctyl sodium sulphosuccinate on intake-output studies of human small and large intestine. Gastroenterology 1985; 89: 489-93. 33. Passmore AP et al. Chronic constipation in long-stay elderly patients: a comparison of lactulose and a senna-fibre combination. BMJ 1993; 307: 769-71. 34. Barrett JA. Colorectal disorders in elderly people. BMJ 1992; 305: 764-6. 35. British Society of Gastroenterology. Inflammatory bowel disease. Guidelines in Gastroenterology. London: BSG, 1996. 36. Robinson A, Thompson DG, Wilkin D, et al. Guided self-management and patient-directed follow-up of ulcerative colitis: a randomized trial. Lancet 2001; 358: 976-81. 37. DTB. A mesalazine enema for ulcerative colitis. Drug and Therapeutics Bulletin 1994; 32: 38-9. 38. Anonymous. Curr Prob Pharmacovigil 1995; 21: 5-6. 39. da Rosa Utiyama SR, da Silva Kotze LM, Nisihara RM et al. Spectrum of autoantibodies in celiac patients and relatives. Dig Dis Sci 2001; 46: 2624-30.

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8

CHAPTER CONTENTS Diabetes mellitus 147 Thyroid disease 154 Hyperthyroidism 155 Goitre in a euthyroid patient 155 After radiotherapy or partial thyroidectomy Adult hypothyroidism 156 Hirsutism References

Endocrine problems 156

157 159

DIABETES MELLITUS Guidelines: British Diabetic Association. Recommendations for the management of diabetes in primary care. London: BDA, 1997. www.diabetes.org.uk Scottish Intercollegiate Guidelines Network (SIGN). Online. Available: http://www.sign.ac.uk/ Department of Health. Diabetes National Service Framework. Online. Available: www.doh.gov.uk/nsf/diabetes/index.htm NHS Executive. Health Service Guidelines. Key features of a good diabetes service, HSG (97) 45. Leeds: NHS Executive, 1997.

Introduction Approximately 2% of patients have known diabetes and there are probably another 1-2% with undiagnosed diabetes. • The aim of treatment of both types 1 and 2 diabetes is to prevent the known complications occurring. • Risks of microvascular complications are reduced by maintaining blood glucose levels as close as possible to normal. Macrovascular complications are reduced by blood sugar control and by tackling known risk factors such as smoking, hypertension and hypercholesterolaemia. • General practitioners are expected to have a register of all their patients with diabetes and ensure that each patient has at least an annual review.1 147

148

ENDOCRINE PROBLEMS

• Structured care in general practice leads to outcomes that are at least as good as hospital care. This means a register of patients with diabetes, a recall system, a review at least annually and audit of the practice's performance.2

Aims • To prevent where possible new cases of type 2 diabetes. • To identify all cases of diabetes in the practice. • To provide appropriate health care for people with diabetes to prevent the development of complications. • To improve the outcome of pregnancies in women with diabetes. • To help people with diabetes lead a full and satisfying life.1

Prevention • Both type 1 and type 2 diabetes are becoming increasingly common. For type 2 diabetes this is because of increasing obesity, not only in older patients but also in children and young adults. • Obesity and reduced physical exercise are associated with an increased risk of developing type 2 diabetes and there is good reason to believe that by tackling these two issues the rising incidence of diabetes could be slowed.3 • Also at increased risk are women who have developed gestational diabetes, women with polycystic ovary syndrome (PCOS) and men or women with evidence of impaired glucose tolerance (IGT) or with impaired fasting glycaemia (IFG). • Monitor patients at high risk (as above) and explain how diet and exercise can reduce their risk of developing diabetes.

(b) a random or 2-hour plasma glucose 11.1 mmol/L or above. If the patient is asymptomatic then the diagnosis should be confirmed by a second test. An oral glucose tolerance test is not always necessary. • Impaired fasting glycaemia (IFG): a fasting plasma glucose of 6.1-6.9 mmol/L and a 2-hour glucose <7.8 mmol/L. • Impaired glucose tolerance (IGT): a fasting plasma glucose less than 7.0 and a 2-hour glucose of 7.8-11.0mmol/L (see p. 153). • Gestational diabetes: The WHO definition is a fasting sugar of 7mmol/L or above or a 2-hour sugar of 7.8 mmol/L or above (see p. 265), although UK practice varies from unit to unit.4

Undiagnosed diabetes • Studies have shown that it takes, on average, 7-10 years before a patient with type 2 diabetes is diagnosed. At any one time, 50% of patients with type 2 diabetes are undiagnosed. • Up to 35% of newly diagnosed patients will have already suffered complications of diabetes.5 It is therefore worthwhile identifying patients earlier and intervention has been shown to reduce the risk of subsequent complications. • General practices should consider screening those patients who are at high risk of diabetes. • Screen, with a fasting glucose, patients over 50 years of age who are obese, have a family history of diabetes, are from an ethnic minority (Chinese, African Caribbean, Indian, Pakistani and Bangladeshi), are women with a history of gestational diabetes or have a new diagnosis of macrovascular disease. • If the fasting value is greater than 7mmol/L repeat the test. • If the fasting value is between 6 and 7mmol/L, check the 2-hour glucose.

Diagnosis The WHO diagnostic criteria for diabetes have changed in the last few years. • Diabetes: (a) fasting venous plasma glucose 7 mmol / L or above; or

Management • Distinguish between type 1 and type 2 diabetes. Younger patients who present with acute illness, weight loss and ketonuria are type 1. However, there are increasing numbers of younger patients

DIABETES MELLITUS

who develop type 2 diabetes and are managed with diet only or with oral hypoglycaemics. Conversely, some patients with type 2 diabetes require insulin because of poor control with diet and oral agents but they retain the clinical features of a type 2 patient. • Check insulin autoantibodies and c-peptide status of patients in whom the type of diabetes is not clear. The presence of insulin auto antibodies supports the diagnosis of type 1, as does an absence of c-peptide.

Initial management • Newly diagnosed patients with ketosis or who appear to have type 1 diabetes should be referred immediately for assessment and usually admission to hospital for treatment with insulin. • Those with type 2 diabetes can be managed initially in general practice. Work-up in type 2 diabetes * Examination: weight and height, blood pressure, a cardiovascular assessment, examination for peripheral neuropathy and referral for retinal eye screening. * Blood tests: FBC, U&Es, glucose, HbA1c, serum creatinine, thyroid function, LFTs, fasting cholesterol, HDL and triglycerides. * Urine: glucose, ketones and proteinuria.

* Take a history, including family history, past serious illnesses, current medication and lifestyle questions including smoking, alcohol, exercise and employment. * Explain the nature of diabetes and give appropriate supporting literature. * Explain the importance of diet, exercise and attention to glycaemic control. * Refer every patient to a dietician.6 * Explain the problems of the complications of diabetes and the importance of regular eye checks, foot care, and, where appropriate, blood pressure and cholesterol management. Half of all patients with type 2 diabetes are hypertensive and require treatment to reduce their blood pressure to normal levels. * Attempt, in asymptomatic patients, to achieve control with the above measures for at least

149

Table 8.1 Target values to be achieved when treating patients with diabetes in primary care Good

Borderline

Poor

Fasting blood glucose (mmol/L)

4.4-6.1

6.2-7.8

>7.8

HbA1c

<7.0%

7.1-8.0

>8.0

Total HDL

<5.0 >1.1

Fasting triglycerides (mmol/L)

<1.7

5.0-6.5 0.9-1.1 1 .7-2.2

>6.5 <0.9 >2.2

BMI (kg/m2)

<25 < 140/80 <20 mg/L

Serum cholesterol (mmol/L)

Blood pressure Albuminuria

25-27

>27

140/80-160/90 20-200 mg/L

> 160/90 >200 mg/L

3 months before giving oral hypoglycaemic agents.6

Glycaemic control • Prolonged high blood sugar levels are associated with increased macrovascular and microvascular disease. Intensive treatment to reduce blood sugar levels and glycated haemoglobin (HbAlc) has been shown to be effective in reducing the risk of vascular disease in both type I7 and type 2 diabetes.8 Type 2 diabetes If good control is not achieved with diet and increasing physical activity add an oral agent. If the patient is obese (BMI >30) * Early use of metformin reduces the risk of death or developing diabetes related complications.9 * Do not use in patients with impairment of renal or liver function or in severe cardiac failure. * Start with 500 mg daily and increase up to a maximum of 2-3 g per day. * If good glycaemic control is not achieved by the maximum dose, add a sulphonylurea.

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ENDOCRINE PROBLEMS

If the patient is of normal weight

* Start with a sulphonylurea such as gliclazide. The longer-acting glibenclamide can be considered but should be avoided in those over 65 or those with impaired renal or hepatic function. * If good glycaemic control is not achieved with a sulphonylurea alone, add metformin. Alternative therapies

* If the combined therapies at maximum dosages fail then consider substitution of alternative oral treatments or switching the patient to insulin treatment. Alternative oral medications include rosiglitazone or pioglitazone, which act by improving insulin sensitivity and which can be used in combination with either a sulphonylurea or with metformin.10 • Glitazones. Randomized trials have not shown that the glitazones are any more effective than a sulphonylurea in combination with metformin but a trial is probably worthwhile in an individual who has failed on the usual therapy and who is otherwise likely to need insulin injections. • Rapaglinide. Another option is rapaglinide, which increases insulin release and can be used in combination with metformin. • Acarbose delays the absorption of starch in the gut and can be used alone or as an adjunctive to other oral therapies. A systematic review has estimated that acarbose will reduce HbAlc by between 0.5 and 0.9%.n It is more widely used in European countries such as Germany than it is in the UK.

Assessment of glycaemic control * Check HbAlc at least annually. It is the most reliable method of estimating glycaemic control. Aim for an HbAlc of less than 7%. * Make sure that attempts to reduce the HbAlc below 7% in patients treated with insulin or sulphonylureas does not lead to severe hypoglycaemia. * Encourage blood glucose monitoring in those with type 1 diabetes and patients with type 2 diabetes with poor control.

• Urine tests have poor sensitivity and specificity. However if the HbAlc is satisfactory and urine tests are negative then no other monitoring is necessary in type 2 diabetes.

Points about glycated haemoglobins (a) They represent an average of blood sugar control over the previous 6-8 weeks. They may be normal in patients with fluctuating sugar levels if the fluctuations are above and below the target, and should therefore be interpreted alongside the blood sugar tests. (b) A falsely high HbAlc occurs in alcohol abuse, uraemia and high levels of haemoglobin. HbAlc is specific for blood glucose, with fewer false positives. (c) Different laboratories may have different reference ranges although standardisation is occurring rapidly.

Blood pressure • Good control of blood pressure is important in reducing the risk of cardiovascular events.12,13 Thus a reduction of lOmmHg systolic blood pressure and SmmHg diastolic achieved a 24% reduction in diabetic related end points after 9 years - an NNT of 61. • Various studies have used different target blood pressures. There are therefore different suggested target blood pressures that are suggested by different guidelines. It is reasonable to aim for a diastolic below 80mmHg and a systolic blood pressure below 140mmHg (see p. 90). • There is little evidence that any particular agent is superior to another14 and a review has shown that ACE inhibitors, beta-blockers and diuretics are all equally effective. A combination of more than one drug is frequently required to control blood pressure.15

Lipids • There is good evidence that lipid lowering is worthwhile in reducing cardiovascular risk in high risk patients.16 Subgroup analyses of diabetic patients in large primary prevention studies17,18

DIABETES MELLITUS

and a small study in patients with type 2 diabetes19 also suggest that lipid lowering is worthwhile in diabetic patients without CHD. The NNT in primary prevention trials to prevent one episode of MI or cardiac death after 5 years treatment was between 14 and 27. • It is recommended that all patients with diabetes with occlusive arterial disease or a ten year risk of coronary heart disease greater than 30% (see p. 108) should be offered lipid-lowering therapy.20 • The tables used to calculate this risk involve measuring the total cholesterol/HDL ratio. Most trials involving secondary prevention have aimed to reduce the total cholesterol to below 5mmol/L. * Cholesterol of 4 mmol/L or over. Prescribe a statin to those at high risk with a baseline total cholesterol of 4 mmol/L or over; * Triglyceride of 5 mmol/L or over. Refer to an endocrine clinic anyone with a triglyceride of 5 mmol/L or over for assessment for suitability for a fibrate. This will lower the triglyceride and raise the HDL more effectively than a statin.

Smoking Although only around 15% of patients with diabetes are smokers it is important to identify these patients and make every effort to encourage them to give up.1 Consider nicotine replacement therapy or referral to specialist smoking cessation classes (see p. 331).

151

* Instruct patients on insulin or sulphonylureas to monitor their blood glucose before and after vigorous and prolonged exercise.

Secondary prevention, e.g. post-Mi People with diabetes who have already suffered a significant macrovascular event are at very high risk and require intensive treatment. * Follow the recommendations for secondary prevention on p. 109. * Intensive treatment with insulin for up to 3 months post-Mi significantly reduces the risk of a further event.

Renal disease * The commonest cause of end-stage renal failure in adults is diabetes. Persistent hyperglycaemia can lead to microvascular damage of the kidneys. Hypertension in patients with diabetes also causes renal damage. * Early identification and treatment can reduce the risks of end stage renal failure or cardiovascular mortality. * Test all patients for proteinuria annually. * If proteinuria is present check for infection with an MSU and repeat. * If proteinuria persists refer to a diabetologist for investigation.

Microalbuminuria Microalbuminuria Albuminuria

Definition

Roughly equivalent to

20-200 (jig/minute >200 |xg/minute

30-300 mg/24 hour >300 mg/24 hour

Exercise * Advise all patients with diabetes to increase their levels of exercise. They should be encouraged to take part in aerobic exercise such as walking, jogging or swimming for at least 4 hours per week.3 Exercise lowers blood sugar, improves control and reduces the risk of macrovascular events. * Give advice on footwear for those with peripheral neuropathy.

• Spot tests for microalbuminuria have poor sensitivity and specificity because they vary according to the patient's fluid output. The albumin/ creatinine (A/C) ratio and the 24-hour urine are more reliable. • Twenty five per cent of patients with type 2 diabetes have microalbuminuria and these patients have double the risk of death compared with those without and can benefit from treatment.

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Candidates for an annual check for microalbuminuria

(a) Those who have had type 1 diabetes for more than 5 years and who have no proteinuria. (b) All patients with type 2 diabetes who have no proteinuria.21 * Either test the urine with a stick, e.g. MicralTest II or Microbumintest, or send an early morning urine to the laboratory to test for albumin or an A/C ratio. * Repeat positive tests on at least two more occasions over the next 6 months. Repeat negative tests annually.21 * Control the blood pressure and blood sugar of patients with microalbuminuria even more intensively. * Treat those with microalbuminuria, good glycaemic control and normal blood pressure with an angiotensin converting enzyme (ACE) inhibitor.22 * Creatinine. Check the serum creatinine of patients with microalbuminuria at least annually. Refer to a consultant nephrologist if the creatinine is greater than 150 jimol/L.23

Foot care • Fifteen per cent of people with diabetes will develop foot ulcers associated with neuropathy and/or peripheral ischaemia. This can lead to joint involvement and may require amputation. • All patients at their annual review should have their feet checked for ulcers, their peripheral pulses checked and should have their sensation tested. This is best done with monofilaments plastic filaments of different thickness - which can be used to more accurately test sensation. • It is particularly important that those patients with sensory loss are given information about foot care as intensive management can reduce the risk of amputation by two-thirds. This intervention includes care from a podiatrist. • All patients with ulcers should be referred to a podiatrist for treatment.24

Eye care • The commonest cause of blindness in the working population is diabetes. This is aggravated by poor control of blood glucose.

• All patients with diabetes should have an annual review that includes an assessment of visual acuity and examination of the retina.25'26 • The best method of retinal screening is by retinal photography using a digital camera. An alternative is to use slit-lamp microscopy. Local arrangements to provide this service are usually available through diabetes clinics in acute trusts but alternative sources of the service may be available. Direct ophthalmoscopy alone, especially if carried out by a general practitioner, is not sensitive or specific enough for routine use.24

Overweight and obesity • Obesity is difficult to influence and indeed patients with type 2 diabetes who have been switched to insulin therapy often improve their glycaemic control while at the same time gaining weight. Despite this, it is important to encourage patients to exercise and eat a healthy diet and to try to lose weight. • Obese patients who have IFG or IGT need to lose weight to reduce their risk of developing diabetes. • The mainstay of treatment includes a weightreducing diet and an increase in physical activity. • For patients with a BMI over 30 the use of weight-reducing drugs can be considered.27

Erectile dysfunction Approximately 40% of males with diabetes suffer from impotence. Many patients are reluctant to discuss this with their doctor and discrete enquiries should be made. • Check for an underlying vascular cause. • Check that it is not the side-effect of medication, e.g. antihypertensives. • Consider treatment with sildenafil (see p. 248). It has made the treatment of impotence acceptable to many more patients and is effective in men with both type 1 and type 2 diabetes.28

Diabetes in pregnancy • Approximately 3 in 1000 pregnancies occur in women with type 1 diabetes. These patients should

DIABETES MELLITUS

be referred for specialist care as optimum glycaemic control is essential to ensure the best outcome for mother and child29 (see p. 255). • Depending on the criteria for diagnosis, between 1 and 4% of women will develop gestational diabetes, which is associated with increased complications for the mother and poorer fetal outcomes. Moreover, 30% of women with gestational diabetes will develop established diabetes later in life (see p. 265). • Women with gestational diabetes are at higher risk of developing eclampsia, having a caesarean section and having a large baby.

Impaired glucose tolerance • IGT is commonly identified in patients with macrovascular risk factors or in those with a past history of gestational diabetes, polycystic ovary syndrome, those from ethnic minorities or who are obese. • Although patients with IGT do not get the complications of diabetes, they are at risk of the macrovascular complications, such as ischaemic heart disease, stroke and peripheral vascular disease; 1 in 3 will develop diabetes within 10 years. • Screen, with a fasting glucose, all patients in the above high risk categories. Known patients with IGT * Follow up with intensive education, management of blood pressure, lipids, smoking, etc. and encourage them to lose weight and to exercise regularly. Check fasting glucose regularly.

The annual review All patients with diabetes should be on a register and have an annual review. The review should include an opportunity to offer education where this is lacking. Some practices offer all but the eye check on a 6-monthly rather than on an annual basis. An annual check should be regarded as the minimum monitoring that patients with uncomplicated diabetes receive.

153

Annual checks for patients with diabetes (a) Weight. (b) Blood pressure. (c) Urinalysis for protein and if negative microalbuminuria. If positive (i.e. more than a trace), check blood creatinine, and urine microscopy and culture. Repeat the urinalysis. If proteinuria persists refer for investigation. (d) Feet. Refer any problems to a chiropodist. (e) Eye check - visual acuity and fundi. (f) Random blood sugar, preferably obtaining an immediate result by using a reflectance meter. (9) HbA1c. (h) Total cholesterol, HDL and triglycerides. (i) Serum creatinine.

Education • Education is the mainstay of the management of diabetes. Patients who understand the disease are most likely to adhere to their management, although there is little evidence that they are less likely to suffer complications. • All new patients should receive education about the nature of diabetes, its day-to-day management, the handling of special problems such as hypoglycaemia and advice on living with diabetes.1 • This should be reinforced at least annually. Belonging to Diabetes UK gives patients an alternative source of information about their diabetes and may reduce some of the demands on the practice.

Hypoglycaemia * Patients on insulin or long-acting oral hypoglycaemics, if they are well controlled with an HbAlc below 7%, are likely to experience occasional hyoglycaemic attacks. * Teach patients how to prevent a hypoglycaemic attack. Explain the importance of always having a source of glucose with them and suggest they wear an alert bracelet (see p. 386). * Consider training a spouse or another family member to give glucagon parenterally, particularly with patients who have experienced severe hypoglycaemic episodes.30

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The management of a hypoglcaemic attack * Give oral glucose. * If the patient is unconscious, give glucagon 1 mg iv, im or sc. Once the patient is conscious, give oral glucose; the benefit of glucagon is shortlived. * Alternatively, give 50 ml of 20% glucose intravenously.

diabetes. The organization produces excellent advice for patients with diabetes, supports the formation and running of local support groups and runs regular educational events for people with diabetes. All newly diagnosed patients should be advised of its existence. The address is 10 Queen Anne Street, London WIG 9LH and www.diabetes.org.uk

Referral for specialist care

Practice approach to diabetes

Referral criteria for secondary care should be agreed with the local consultants. In general this should include:

All practices should consider the following matters:

(a) all newly diagnosed patients with type 1 diabetes or with ketosis; (b) all children; (c) all pregnant women; (d) all patients with serious complications such as ischaemic heart disease, renal impairment, retinopathy or neuropathy; (e) patients in whom optimum control of blood sugars or blood pressure cannot be achieved.1

(a) Does the practice have a diabetes register? (b) Do all patients have an annual review? (c) Are there agreed protocols for patient education, prescribing, referral, annual review? (d) Does the practice conduct regular audits of the care of their patients with diabetes? (e) Does the practice have a policy for screening for undiagnosed diabetes? (f) Does the practice have a policy on health promotion aimed at reducing the incidence of type 2 diabetes through encouraging exercise, reducing obesity and intensive care of high-risk patients? (g) Is there an active practice policy of patient participation in the development of practice protocols and the provision of services for diabetes? Is there a regular survey of patient's views on the care being offered?

Driving Insulin-treated patients are barred from driving HGV or PCV vehicles. They may drive a car but must notify the DVLA and their car insurance company and should stop driving if experiencing disabling hypoglycaemia. Patients on oral hypoglycaemics or with diabetic complications should also inform the DVLA. Those with no complications and controlled by diet alone do not need to inform the DVLA.31

THYROID DISEASE

Pneumococcal vaccine and flu vaccine All patients with diabetes should be offered an annual flu vaccine and a pneumococcal vaccine once.

Diabetes UK Diabetes UK is the largest organisation in the UK specifically aimed at supporting patients with

Reviews and guidelines: Weetman AR Management of thyroid disorders in primary care. Prescriber Feb 5 2001; 47-60. Weetman AR Hypothyroidism: screening and subclinical disease. BMJ1997; 314:1175-8. Vanderpump MPJ et al. Consensus statement for good practice and audit measures in the management of hypothyroidism and hyperthyroidism. BMJ 1996; 313: 539-44.

THYROID DISEASE

HYPERTHYROIDISM * Confirm the diagnosis by checking circulating free thyroxine (FT4), free tri-iodothyronine (FT3) and thyroid stimulating hormone (TSH) - either FT4 or FT3, or both, will be raised; the TSH is usually depressed.32 * Beware of diagnosing hyperthyroidism solely on a low TSH; it can be low in patients ill with other conditions. * Consider the diagnosis of pituitary adenoma if the FT4 is raised and the TSH is normal or raised.33 * Check the white blood cell (WBC) count before treatment is started. Agranulocytosis occurs in 0.3% of patients treated with antithyroid drugs. A baseline WBC allows the neutropenia associated with thyrotoxicosis to be distinguished from drug-induced agranulocytosis.33

Treatment * The definitive treatment of Graves' hyperthyroidism includes antithyroid drugs, radioactive iodine (I131) or thyroid surgery. * Repeat the FT4, FT3 and TSH before starting antithyroid drugs. Specific treatment should usually be withheld until a firm biochemical diagnosis has been made. * Prescribe a beta-blocker for those with tremor or tachycardia who need symptomatic relief (e.g. long-acting propranolol 160 mg per day or nadolol 80 mg per day). For the management of atrial fibrillation in thyrotoxicosis, see p. 105. * Refer all patients with thyrotoxicosis to a specialist. Usual antithyroid drugs include carbimazole and propylthiouracil. A policy should be agreed with the local hospital as to whether antithyroid treatment should be started by the general practitioner before the patient is seen by the specialist. * If treating in primary care, start with carbimazole 30-40 mg maintained for 4-8 weeks until the patient is euthyroid. Thereafter, titrate the dosage against the TSH. It can usually be reduced to a maintenance dose of 5-15 mg and should be continued for 12-18 months.34 * If the patient develops a rash or pruritus, change to propylthiouracil 300-600 mg; not all

155

patients will be sensitive to both drugs. Again, the dosage will usually be reduced to a maintenance dose; 50-150 mg is usually required. * Alternatively, give higher doses of antithyroid drugs with thyroxine: the 'block and replace regimen'. This is supposed to reduce the incidence of iatrogenic hypothyroidism. * Agranulocytosis. Warn all patients on carbimazole or propylthiouracil to seek attention promptly if they develop a sore throat, a rash or unusual itching. * Follow-up. See patients 4-weekly until euthyroid and then every 3 months. At follow-up appointments, check the weight, pulse and blood pressure and a blood test for FT4, FT3 and TSH. * Duration of treatment. Give antithyroid drugs for 12-18 months; even then there is a 50% relapse rate after stopping the drugs. Check the FT4 and TSH at 2 months and at 1 year after treatment has been stopped. Patients who relapse require definitive ablation of the gland either with I131 or with thyroid surgery. * Pregnancy. Patients who are being treated for thyrotoxicosis and become pregnant have an increased risk of miscarriage and should be referred back to a specialist.

Summary of laboratory tests in patients treated for hyperthyroidism Overtreated Adequate Undertreated

FT,

FT,

TSH

Low Normal High

Low Normal High

High Normal Low

GOITRE IN A EUTHYROID PATIENT * A solitary thyroid nodule may be malignant. Refer to medical or surgical outpatients urgently, according to the local expertise. * A generalized multinodular goitre should also be referred for a decision about management but not urgently, although certain features would make malignancy more likely: (a) onset under age 20 or over 60; (b) male sex; (c) a history of radiation;

156

ENDOCRINE PROBLEMS

(d) a short history; (e) lymphadenopathy. • A small diffuse goitre need not be investigated, especially if it occurs at puberty.

AFTER RADIOTHERAPY OR PARTIAL THYROIDECTOMY • Those who have had ablative radiotherapy will have been put on replacement thyroxine. This should be monitored clinically and a TSH should be measured yearly. • Patients treated with I131 or surgery should be placed on a practice register and have their TFTs checked annually. Fifty per cent become hypothyroid and will require thyroxine supplementation.35

ADULT HYPOTHYROIDISM • The diagnosis of hypothyroidism is notoriously difficult in the early stages. • In the elderly patient, consider hypothyroidism in patients with non-specific illness, e.g. falling or mental deterioration. Only one-third of elderly hypothyroid patients have classical signs 36

Diagnosis • Diagnosis depends on identifying low levels of FT4 with raised levels of TSH. • FT3 is an unreliable indicator of hypothyroidism.37 Five per cent of patients over 60 have a low FT3, but in as many as 80% of these it is due to non-thyroid conditions rather than hypothyroidism. If it occurs alone, a low FT3 is probably due to a partial failure of conversion of FT4 to FT3 in a euthyroid patient. This 'sick euthyroid' syndrome occurs in intercurrent illness and old age, or may be due to drugs: lithium, amiodarone, non-selective beta-blockers in high dosage and corticosteroids. Atypical tests

* If the TSH is not raised in hypothyroidism, consider hypopituitarism.

* Subdinical hypothyroidism. If the TSH is raised but the FT4 is normal and the patient is well, the patient has subclinical hypothyroidism. These patients can be considered for treatment if there is a goitre, if antithyroid antibodies are positive or if repeat TFTs after 6 months shows a deterioration. They may benefit from treatment if they are tired without being overtly hypothyroid, or if they have a low HDL or a high LDL cholesterol. Check TFTs yearly. If the TSH is raised and antithyroid antibodies are present, 5% will become overtly hypothyroid each year.38 * Look for thyroiditis by checking for antimicrosomal antibodies. Postpartum thyroiditis is common (estimates range from 4 to 17%). It occurs within 6 months of pregnancy. All pregnant and postpartum cases of hypothyroidism should be referred to a specialist. * Give thyroxine if the patient has symptoms, but prepare to be able to withdraw it after a few months. * If her antithyroid antibodies are positive, she will need annual TFTs for life. Refer

(a) Younger patients to outpatients. They merit an endocrinological assessment for a condition that will probably be life long. (b) Pregnant or postpartum women. (c) Patients with evidence of pituitary disease. (d) Patients with ischaemic heart disease. (e) Patients on amiodarone or lithium. (f) Admit anyone who is severely hypothyroid, e.g. who is unable to leave the house because of the hypothyroidism, who is at risk of hypothermia or who develops angina, dysrhythmias or heart failure on treatment.

Management Treat, without referral, older patients who are not severely ill. Check for: (a) autoantibodies (also be aware of the increased incidence of pernicious anaemia, hypoparathyroidism, Addison's disease and diabetes in those with antithyroid antibodies);

HIRSUTISM 157

(b) lipids; (c) coronary heart disease, including an ECG. Younger patients without evidence of heart disease

* Give thyroxine at a dose of 50-100|xg with increments of 50 ug every 4-6 weeks.35 * Review the patient regularly until the TSH is normal and the patient is clinically euthyroid. Monitoring can then be reduced to 6-monthly.33 * Warn all patients that they will not feel the benefits of treatment immediately. After 3 months they should notice the difference, but complete recovery can take up to a year. Patients over 60 or with IHD, with a long history, or more severely hypothyroid

1. Start thyroxine 25 fxg daily. 2. Increase by 25 ug each month until 100 ug a day is reached. Most patients are controlled by 100-150 ug. Children need more, and old people often less. 3. 6 weeks later: check TFTs. Aim for an FT4 that is normal or raised and an FT3 that is low/normal. The TSH should be suppressed into or below the normal range. If it is not, consider the possibility that the patient is not taking the tablets. 4. Increase by a further 25 ug daily to 150 ug daily if the TSH is still raised. Very few patients need higher doses than this. Wait 4-6 weeks after each dose change before checking the TSH; it takes this long to readjust.37 Thyroxine given twice a week is as effective as once a day. This can be helpful when someone else has to administer it. Ensure that the total dose per week is equivalent.39

General points

• UK guidelines35 recommend that all patients on thyroxine should have a yearly clinical assessment and check of thyroid function tests. • Some doubt the value of the biochemical check in monitoring the dose of thyroxine, as this remains constant for most patients and correlates poorly with TFTs anyway.40 • Thyroid function tests are useful when compliance is in doubt, during pregnancy and in concomitant drug use, e.g. amiodarone or anticonvulsants. • Studies have suggested that the dangers of overtreatment have been exaggerated and that undertreatment is the greater risk.41 • Patients who are taking thyroxine to suppress a thyroid carcinoma are usually on doses of 200 (jig or over. Their TSH should be undetectable. Patients who may no longer need thyroxine

• Up to 28%42 of patients may be taking thyroxine inappropriately. Some of these may have been prescribed it appropriately for autoimmune thyroiditis, but then recovered spontaneously. • Attempt to withdraw thyroxine in patients without clear evidence of irreversible hypothyroidism. Reduce the dose to half, and repeat the TSH after 2 months. If it is not raised, stop thyroxine and repeat the TSH after a further 2 months. If it is still not raised, the patient does not need thyroxine, but does need a yearly check.

HIRSUTISM Reviews: Rittmaster RS. Hirsutism. LanceM997; 349: 191-5.

Summary of tests in the treatment of hypothyroidism FT4

FT,

TSH

Adequate Overtreated

Normal/high High

Normal/low Low

Undertreated

Normal/low

Low/normal High or high/normal Low

High

de Berker D. The diagnosis and treatment of hirsutism. The Practitioner 1999; 243:493-9.

• Hirsutism in young women is a common problem, with up to 10% of young women having a significant degree of excess hair.43 • In many, the problem is genetically determined and is related to ethnic origin or family

158

ENDOCRINE PROBLEMS

history. In a proportion there will be an underlying endocrinological cause that will benefit from treatment.44'45 * Hirsutism is characterised by darker terminal hair in the male pattern and should be distinguished from hypertrichosis, where fine downy vellus hair occurs in a distribution unrelated to gender.46 * The commonest cause of hirsutism is polycystic ovary syndrome (PCOS) - other rare causes include congenital adrenal hyperplasia, Cushing's disease, a pituitary dysfunction or, more rarely an androgen-secreting tumour of the ovary or adrenal glands. * Ask about a family history, ethnic origin including the Mediterranean countries of Europe, and menstrual history. * Examine: (a) Is the excess hair all over (hypertrichosis)? This suggests a general endocrine disturbance, e.g. Cushing's disease, acromegaly or hypothyroidism, or a drug cause. (b) Is the excess hair in the male pattern (hirsutism) ? Look specifically for signs of virilism, e.g. male pattern hair growth, increased muscle bulk and clitoromegaly. This suggests an excess of androgenic hormones. (c) Is there ovarian enlargement on vaginal examination? * Exclude an iatrogenic cause, e.g. androgenic oral contraceptives, steroids, antiepileptic medication, diazoxide and minoxidil.47 Note: A woman with hirsutism since puberty and regular periods needs to be investigated no further, especially if she is predisposed to hirsutism by ethnicity or family origin. * Take blood for testosterone, luteinizing hormone (LH) and follicle stimulating hormone (FSH) on day 2 of the cycle. (a) A LH/FSH ratio of >2.5 is suggestive of PCOS (see p. 213) although a ratio <2.5 does not exclude it. A testosterone level up to twice that of normal (i.e. 2.6 to 4.8nmol/L) is consistent with a diagnosis of PCOS. Ultrasound of the ovaries may be of value if the blood picture is suggestive of PCOS, but is of little value as an initial investigation as up

to one third of normal women have polycystic ovaries.48 (b) Testosterone levels >4.8 nmol/L may indicate a tumour or adrenal hyperplasia, especially in those with a rapid history of excessive hair growth. * Menstrual irregularities. Consider checking prolactin and TSH, in addition to the above.48

Treatment * Advise on cosmetic methods: bleaching agents, depilatory creams, plucking, electrolysis or possibly laser treatment.47 * Recommend weight loss if appropriate. It can reduce androgen levels and may be helpful, although in women with insulin resistance and impaired adipocyte lipolysis, weight loss will be more difficult to achieve. * Attend to the emotional effect of being hirsute. Hirsute women often have high levels of anxiety and psychological support may be required.49 * Women with PCOS are at increased risk of developing diabetes and cardiovascular disease and may experience problems with fertility. Regular screening of such women is recommended.50,51 Careful consideration should be given to the impact such a diagnosis may have on a woman's sense of wellbeing.

Medical therapies Women with normal blood tests and no other abnormalities on history or examination can be treated in primary care. * Use a combined oral contraceptive (COC). Suppression of ovarian androgens is best achieved by administering exogenous oestrogens, e.g. as a COC. COCs with low androgenic progestogens are preferred (e.g. desogestrel-containing formulations such as Marvelon® and Mercilon®). * In severe cases or those with accompanying acne use Dianette® (a combination of the antiandrogen cyproterone acetate and ethinyloestradiol, and specifically licensed for the treatment of hirsutism and severe acne). Both desogestrel-containing COCs and Dianette® have demonstrated clinical

REFERENCES

benefit.52,53 Some women will be intolerant of COCs, but there is sparse evidence to support the use of cyproterone acetate alone.54 Its use without adequate contraceptive cover is strongly opposed because of the risk of fetal feminisation after exposure in utero. * In those who do not respond, finasteride and flutamide have been shown in randomised controlled trials to be useful54'55 whilst a systematic review of spironolactone has not demonstrated benefit.56 Refer on to a consultant any patients who have failed on other therapies and want a second opinion.

159

Referral Refer anyone with abnormal blood tests, rapid onset, virilism, galactorrhoea or menstrual disturbance: (a) to a gynaecologist if the clinical picture is predominantly that of a woman age 18-35 with menstrual disturbance, infertility or a raised LH/FSH ratio, or (at any age) a pelvic mass; or (b) to an endocrinologist if the picture is more one of rapid onset of hirsutism with virilism and a very high serum testosterone, especially if these occur outside the age range of 18-35.

REFERENCES 1. British Diabetic Association. Recommendations for the management of diabetes in primary care. London: BDA, 1997. 2. Griffin S, Kinmonth AL. Systems for routine surveillance for people with diabetes (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2002. 3. Tuomilheto J, Lindstrom J, Eriksson JG, Valle TT, Hamalainen H, Ilanne-Parikka P et al. Prevention of type 2 diabetes mellitus by changes in lifestyle among subjects with impaired glucose tolerance. New Engl J Med 2001; 344: 1343-9. 4. Report of the Expert Committee on the Diagnosis and Classification of Diabetes Mellitus. Diabetes Care 2000; 23: S4-19. 5. UKPDS Group. Complications in newly diagnosed type 2 diabetic patients and their association with different clinical and biochemical risk factors. Diabetes Res 1990; 13: 1-11. 6. British National Formulary. London: British Medical Association and the Royal Pharmaceutical Society of Great Britain, 2001, Section 6.1.2, p. 329. 7. Diabetes Control and Complications Trial Research Group. The effect of intensive treatment on the development and progression of long-term complications in insulin-dependent diabetes mellitus. Nezu Engl j Med 1993; 329: 977-86. 8. UKPDS Group. Intensive blood-glucose control with sulphonylureas or insulin compared with conventional treatment and risk of complications in patients with type 2 diabetes (UKPDS 33). Lancet 1998; 352: 837-53. 9. UKPDS Group. Effect of intensive blood-glucose control with metformin on complications in overweight patients with type 2 diabetes (UKPDS 34). Lancet 1998; 352: 854-65. 10. NICE. Guidance on rosiglitazone for type 2 diabetes mellitus. Technology Appraisal Guidance - No 9. London: NICE, 2000. 11. Campbell LK, White JR, Campbell RK. Acarbose: its role in the treatment of diabetes mellitus. Ann Pharmacother 1996; 30: 1255-62.

12. UKPDS Group. Tight blood pressure control and risk of macrovascular and microvascular complications in type 2 diabetes: UKPDS 38. BMJ 1998; 317: 703-13. 13. Hansson L, Zanchetti A, Carruthers SG et al. Effects of intensive blood pressure lowering and low dose aspirin in patients with hypertension: principal results of the Hypertension Optimal Treatment (HOT) randomised trial. Lancet 1998; 351: 1755-62. 14. UKPDS Group. Efficacy of atenolol and captopril in reducing risk of macrovascular and microvascular complications in type 2 diabetes: UKPDS 39. BMJ 1998; 317: 713-20. 15. Grossman E, Messerli FH, Goldbourt U. High blood pressure and diabetes mellitus: are all antihypertensive drugs created equal? Arch Intern Med 2000; 160: 2447-52. 16. Pyorala K, Pedersen TR, Kjekshus J et al. Cholesterol lowering with simvastatin improves prognosis of diabetic patients with coronary heart disease. A sub group analysis of the Scandanavian Simvastatin Survival Study (4S). Diabetes Care 1997; 20: 614-20. 17. Downs JR, Clearfield M, Weiss S et al. Primary prevention of acute coronary events with lovastatin in men and women with average cholesterol levels: results of AFCAPS/TexCAPS Prevention Study. JAMA 1998; 279: 1615-22. 18. Koskinen P, Manttari M, Manninen V et al. Coronary heart disease incidence in NIDDM patients in the Helsinki Heart Study. Diabetes Care 1992; 15: 820-5. 19. Elkeles RS, Diamond JR, Poulter C et al. Cardiovascular outcomes in type 2 diabetes. A double blind placebo controlled study of bezafibrate: the SENDCAP Study. Diabetes Care 1998; 21: 641-8. 20. National Service Framework for Coronary Heart Disease. London: DoH. Online. Available: www.doh.gov.uk/nsf/coronary.htm 21. NHS Centre for Reviews and Dissemination. Complications of diabetes: renal disease and promotion of self management. Effective Health Care 2000; 6 (1). 22. Heart Outcomes Prevention Evaluation (HOPE) Study Investigators. Effects of ramipril on cardiovascular and microvascular outcomes in people with diabetes

160 ENDOCRINE PROBLEMS

23. 24.

25. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35.

36. 37. 38. 39. 40.

mellitus: results of the HOPE study and MICRO-HOPE substudy. Lancet 2000; 355: 253-9. Scottish Intercollegiate Guidelines Network. Management of Diabetes. Edinburgh: SIGN, 2001, Section 5.5.6, p. 23. NHS Centre for Reviews and Dissemination. Complications of diabetes: Screening for retinopathy. Management of foot ulcers. Effective Health Care 1999; 5 (4). British Diabetic Association. Retinal photographic screening for diabetic eye disease. London: BDA, 1997. Royal College of Ophthalmologists. Guidelines for diabetic retinopathy. London: RCO, 1997. British National Formulary. London: British Medical Association and the Royal Pharmaceutical Society of Great Britain, 2001, Section 4.5, p. 198. Rendell MS. Sildenafil for the treatment of erectile dysfunction in men with diabetes. JAMA 1999; 281: 421-6. Scottish Intercollegiate Guidelines Network. Management of diabetes. Edinburgh, SIGN 2001, Section 8, pp. 34-5. British National Formulary. London: British Medical Association and the Royal Pharmaceutical Society of Great Britain, 2001, Section 6.1.4, p. 334. DVLA. Guide to the current medical standards of fitness to drive. Swansea: Drivers Medical Unit, DVLA, 2001. Weetman AP. Management of thyroid disorders in primary care. Prescriber Feb 5 2001; 47-60. Singer PA, Cooper DS, Levy EG et al. Treatment guidelines for patients with hyperthyroidism and hypothyroidism. JAMA 1995; 273: 808-12. British National Formulary. London: British Medical Association and the Royal Pharmaceutical Society of Great Britain, 2001, Section 6.2.2, p. 338. Vanderpump MPJ et al. Consensus statement for good practice and audit measures in the management of hypothyroidism and hyperthyroidism. BMJ 1996; 313: 539-44. Weetman AP. Hypothyroidism: screening and subclinical disease. BMJ 1997; 314: 1175. Lindsay RS, Toft AD. Hypothyroidism. Lancet 1997; 349: 413-17. Vanderpump MP et al. The incidence of thyroid disorders in the community: a 20-year follow-up of the Whickham survey. Clin Endocrinol 1995; 43: 55-68. Taylor J, Williams BO, Prater J et al. Twice-weekly dosing for thyroxine replacement in elderly patients with primary hypothyroidism. / Int Med Res 1994; 22: 273-7. Eraser WD, Biggart EM, O'Reilly DStJ et al. Are biochemical tests of thyroid function of any value in monitoring patients receiving thyroxine replacement? BMJ 1986; 293: 808-10.

41. Leese GP, Jung RT, Gutherie C et al. Morbidity in patients on L-thyroxine: a comparison of those with a normal TSH to those with a suppressed TSH. Clin Endocrinol 1992; 37: 500-3. 42. Anon. Evaluation of long-term thyroid replacement treatment. Swansea vocational training scheme. BMJ 1985; 291: 1476-8. 43. Rittmaster RS. Hirsutism. Lancet 1997; 349: 191-5. 44. de Berker D. The diagnosis and treatment of hirsutism. The Practitioner 1999; 243: 493-9. 45. Michelmore KF, Balen AH, Dunger DB, Vessey MP. Polycystic ovaries and associated clinical and biochemical features in young women. Clin Endocrinol 1999; 51: 779-86. 46. Al-Khawajah MM, Fouda Neel MA. Women with clinically significant hirsutism always have detectable endocrinological abnormalities. j Eur Acad Dermatol Venereol 1997; 9: 226-31. 47. Carmina E. Prevalence of idiopathic hirsutism. Eur J Endocrinol 1998; 139: 421-3. 48. DTB. Tackling polycystic ovary syndrome. Drug Ther Bull 2001; 39: No. 1. 49. Sonino N, Fava GA, Mani E et al. Quality of life of hirsute women. Postgrad Med J 1993; 69: 186-9. 50. Lobo RA, Carmina C. The importance of diagnosing the polycystic ovary syndrome. Ann Intern Med 2000; 132: 989-93. 51. Wild S, Pierpoint T, McKeigue P, Jacobs H. Cardiovascular disease in women with polycystic ovary syndrome at long term follow up: a retrospective cohort study. Clin Endocrinol 2000; 52: 595-600. 52. Porcile A, Gallardo E. Long term treatment of hirsutism: desogestrel compared with cyproterone acetate in oral contraceptives. Fertil Steril 1991; 55: 877-81. 53. Barth JH, Cherry CA, Wojnarowska F, Dawber RP. Cyproterone acetate for severe hirsutism: results of a double-blind dose-ranging study. Clin Endocrinol 1991; 35: 5-10. 54. Falsetti L, Gambera A. Comparison of finasteride and flutamide in the treatment of idiopathic hirsutism. Fertil Steril 1999; 72: 41-6. 55. Fruzzetti F, Bersi C, Parrini D et al. Treatment of hirsutism: comparisons between different antiandrogens with central and peripheral effects. Fertil Steril 1999; 71: 445-51. 56. Lee O, Farquhar C, Toomath R, Jepson R. Spironolactone versus placebo or in combination with steroids for hirsutism and/or acne. The Cochrane Library, Issue 2. Oxford: Update Software, 2001.

9

CHAPTER CONTENTS Osteoarthritis 161 Low back pain 164 The elderly with acute back pain Advice/exercises for LBP 167

167

Rheumatological problems

Neck pain 167 Mechanical pain 168 Regional problems Shoulder 169 Elbows 169 Wrist 169 Hips 169 Knees 169 Foot/ankle 170

168

Oral non-steroidal anti-inflammatory drugs 171 Role of NSAIDs in musculoskeletal disorders 171 Which oral NSAID? 171 Gout

OSTEOARTHRITIS

173 Clinical review article: Walker-Bone K, Kassim Javaid K, Arden N, Cooper C. Medical management of osteoarthritis. BMJ2000; 321: 936-40.

Rheumatoid arthritis 175 Pharmacological treatment 176 Spondyloarthropathies 178 Other spondyloarthropathies 178 Connective tissue diseases Osteoporosis

Guidelines: PCRS. The management of osteoarthritis patient-centred not disease-focused. Primary Care Rheumatology Society, PO Box 42, Northallerton, NorthYorksDL78YG.

179

Scott DL, Shipley M, Dawson A, Edwards S, Symmons DPM, Woolf AD. Strategies for improving clinical effectiveness. British Rheumatology Society. Online. Available: www.rheumatology.org.uk (choose 'clinical publications' then 'OA/RA management report').

180

Polymyalgia rheumatica/temporal arteritis 183 Polymyalgia rheumatica 183 Temporal arteritis 184 Fibromyalgia 185 References

186

Aims of management: • • • •

Empower patient in self-management. Control symptoms: pain and stiffness. Prevent progression of joint damage. Reduce disability and improve function.

Involve patient in self-management • Education of the patient is fundamental to self-management but while there are good reasons to support it, there is no proof that selfmanagement reduces pain.1 • Reassurance given without understanding the patient's concerns is likely to fail and raise the spectre of future disability.2 • Ask about the patient's concerns: what does the patient think is the cause and likely outcome? Dispel fears and myths: osteoarthritis is usually 161

162

RHEUMATOLOGICAL PROBLEMS

a mild disease and deterioration to the point of needing a joint replacement is the exception rather than the rule. * Be positive - emphasize what the patient can do for him-/herself (exercise, lifestyle). * Educate the patient about the course of the disease: although the 'wear and tear' is permanent, the symptoms are not, they will wax and wane. * Educate the patient in the self-management of analgesics so that he/she can step up or down the scale of treatment (see below). * Reinforce this with written material. The Arthritis and Rheumatism Council produces an informative booklet for patients. Patient organizations: Arthritis Research Campaign (ARC), Copeman House, St Mary's Court, St Mary's Gate, Chesterfield, Derbyshire S41 7TD, tel. 0870 850 5000, www.arc.org.uk (choose 'index' then 'O' for osteoarthritis). Arthritis Care, helpline 080 8800 4050, www.arthritiscare.org.uk

Controlling symptoms, especially pain * Most patients receive adequate pain relief with simple or compound analgesics. However, it is important to remember that osteoarthritis is not simply a degenerative disease. It is an umbrella term covering a collection of pathologies, including primary osteoarthritis and calcium pyrophosphate deposition disease. Furthermore, inflammation can occur in osteoarthritic joints (e.g. with crystal deposition) and in periarticular tissues, such as the medial ligament of the knee, so NSAIDs have a place beyond providing analgesia.3 Oral agents4,5 * First, review patient's self-medication - drug, dose, effectiveness. * Prescribe or advise over the counter (OTC) preparations progressively according to response: • Step 1. Start with paracetamol 1 g up to four times daily. Regular doses will be more effective for some patients than p.r.n. doses.

• Step 2. If inadequate, change to ibuprofen 400mgt.d.s. • Step 3. Either add paracetamol up to 4 g daily, or increase the dose of ibuprofen to 2.4g daily, or do both. • Step 4. Consider other NSAIDs (see section on NSAIDs) or codeine or compound analgesics, e.g. co-codamol or both. * Explain that paracetamol or co-codamol can be used with ibuprofen, but that co-codamol should never be taken with paracetamol because it contains the same drug. * Make sure the patient is not on more than one NSAID, including OTC preparations e.g. aspirin. * Teach the patient to 'step down' therapy if the pain permits. Whenever possible, try to get the patient to use NSAIDs only for short periods. * Consider adding a low dose tricyclic, e.g. amitriptyline 10-25 mg at night. Note: Glucosamine, sold as a supplement, has been promoted to the public as a safe and effective therapy. However, there are serious doubts about its effectiveness and the correct dose and route of administration, if any, are unknown.6

Topical agents • The evidence on the effectiveness of topical NSAIDs has received differing evaluations. One systematic review found that topical NSAIDs were more effective than placebo with an NNT of 3,7 but others argue that the evidence is weak.8 • The evidence for topical capsaicin is under evaluation.9 • Consider topical agents for single or limited joint disease. Both topical NSAIDs and capsaicin are available as OTC formulations.

Local injections • Corticosteroid injections are used because experience confirms short-term benefits but trials

OSTEOARTHRITIS

have not been good enough to provide stronger evidence.10 * Consider a steroid injection for: (a) inflammatory flare-ups within joints; (b) related soft-tissue problems, e.g. medial ligament knee; (c) single joint disease. * Limit steroid injection to a maximum of four per year for any one joint. * Consider referral for saline washouts and intraarticular hyaluronic acid injections for knees. They can provide relief for several months. They may have a place for patients not suitable for NSAIDs, intra-articular steroids or joint replacement.11

Lifestyle * Exercises to maintain muscle strength have been shown to reduce pain and disability. * For many patients, advice on exercise therapy given in the surgery may be sufficient (see Regional section). The ARC (see p. 162) booklets for neck, shoulder, knee and hip pain display suitable exercises. * The value of the physiotherapist is in teaching and monitoring exercise programmes. Many time-honoured electromagnetic tools have been shown either to be ineffective or of unproven effectiveness.12,13 * Provision of footwear and walking aids, other aids and appliances can increase function and decrease pain. * Recommend swimming, if appropriate, because it promotes mobility and strength without impact. * Advise on the need to balance the avoidance of activities that add to joint injury (e.g. heavy lifting) with the need to keep muscles strong and joints supple. * Give overweight patients a target weight and appropriate advice on reducing weight. * Discuss the possible benefits of impact cushioning shoes (air insole trainers, shoe inserts). * Demonstrate exercises or give advice sheets or (preferably) do both. * Consider referral to a physiotherapist.

163

Disability * Consider the patient's need for aids, e.g. bath aids and refer as appropriate to an occupational therapist. * Consider eligibility for benefits, e.g. attendance allowance, blue badge, and refer to appropriate agency for advice. * Consider need for support and rehabilitation. Refer as appropriate and according to local circumstances to social services or a rehabilitation team or to a hospital-based multidisciplinary team.

X-rays * Plain X-rays are not routinely indicated. They cannot confirm the diagnosis because degenerative changes in several joints, e.g. spine and knees, start in middle age and X-ray appearances correlate poorly with the symptoms.14 Diagnosis is mainly clinical in many syndromes and plain X-rays do not differentiate between disease and non-disease, e.g. shoulder impingement syndrome.14 * Refer for X-ray when: (a) soft tissue calcification is suspected, e.g. shoulder, and when the result might inform the choice to use NSAID or steroid injection; (b) referral for joint replacement is being considered; (c) there is knee pain with locking - X-ray may show radio-opaque loose bodies; (d) there is diagnostic doubt.

Referrals to a specialist15 * Refer when: (a) the diagnosis is uncertain; or (b) the patient fails to respond to therapy; or (c) you suspect septic arthritis.

Surgery Approximately 50,000 hip replacements and 30,000 knee replacements are performed each year in the UK. Joint replacement can reduce pain, increase mobility and decrease disability thereby

164

RHEUMATOLOGICAL PROBLEMS

dramatically improving quality of life. Hip and knee replacement, the longer established interventions, have been evaluated.16,17 After 10 years, 95% of hip and knee replacements are still functioning.18,19 Refer patients for consideration for joint replacement who:20 (a) have moderate to severe pain or disability despite conservative treatment; and (b) who are fit for surgery; and (c) whose X-rays back up the diagnosis of joint damage. Advanced age per se is not a contraindication; comorbidity is a more important consideration.

Summary of strength of the evidence for selected management options in osteoarthritis Area

Education and advice Physiotherapy Weight loss Occupational therapy Footwear Aids and appliances Surgical joint replacement Analgesics NSAIDs Local steroid injections Local NSAIDs and rubifacients

Evidence good

Evidence weak

Evidence insufficient

*

* *

*

*

*

Postoperative care Check that: (a) the patient has a raised toilet seat, a shower and a hand-grabbing device to pick things off the floor. If the patient lives alone, a dressing stick will be needed; (b) physiotherapy has been organized and that the patient has a walking stick; (c) the patient knows not to cross the legs or drive for 6 weeks. The danger of dislocation is greatest in the first 6 weeks and occurs when the hip is flexed, especially if internally rotated and adducted. After 6 weeks, patients may resume activities, including walking, swimming, bicycling and tennis, provided they take care not to fall. Contact sports and ladders are not advised. Recurrence of pain * Arrange for an X-ray, ESR and WBC in any patient with a hip prosthesis who develops pain. This may show loosening, infection, stress fracture or dislocation. Refer any of these to the surgeon urgently. Refer anyway if the pain does not settle with rest. Any of the above may be present despite a normal X-ray.

LOW BACK PAIN (LBP) Guidelines: Waddell G, Mclntosh A, Hutchinson A, Feder G, Lewis M. Guidelines for the management of acute low back pain. London: Royal College of General Practitioners, 1999. Online. Available: www.RCGP.org.uk/ rcgp/clinspec/guidelines/backpain/index.asp New Zealand Guidelines Group. New Zealand acute low back pain guide, 1998. Online. Available: www.nzgg.org.nz/library.cfm

Definitions of low back pain22 • Acute LBP: <6 weeks; • Subacute LBP: 6-12 weeks; • Chronic LBP: >3 months. LBP is extremely common. At any one time, 15% of adults have it and 60% will have had it some time in their lives.23 Back pain is the commonest cause of long term sickness: 52 million working days are lost each year from back pain. Mechanical LBP is self-limiting: 80-90% of patients recover spontaneously within 3 months.24

Antibiotic prophylaxis Patients with prosthetic joint implants including total hip replacements do not need antibiotic prophylaxis for dental treatment.21

Aims of management • Reduce pain and period of sickness in mechanical back pain.

LOW BACK PAIN

* Identify early the small minority with serious pathology needing immediate or urgent attention. * Prevent acute back pain becoming chronic back pain.

165

have fanatical supporters but there is no evidence that any specific exercises are best.26 Follow-up consultations (2-6 weeks)

* Triage patients into one of the three following groups on the basis of history and examination: (a) mechanical back pain (b) nerve root compression (c) possible serious spinal pathology - look for Red Flags (see p. 167) and act accordingly. * Note any psychological or social influences that might retard recovery - so-called Yellow Flags.

* If activity and function are improving, encourage return to normal activities even if pain is still present. Stress that activity will not damage the back. * If not improving, advise graded return to normal activity and set a return date to work.22 * If not improving, also consider referral to another health professional with expertise in LBP, usually a physiotherapist, or possibly a manipulative therapist. * Reassess for Red Flags (see p. 167). * Reassess for Yellow Flags. If present, be positive, schedule regular reviews, encourage a programme of activity. Consider early referral.

Managing acute mechanical LBP

Psychosocial blocks to recovery

Features of mechanical back pain

Yellow flags - patient has

(a) Presentation at age 20-55; (b) Pain felt over lumbosacral area, buttocks or thighs; (c) 'Mechanical' pain, i.e. alters with posture and activity; (d) The patient is well.

(a) A belief that back pain is harmful or potentially disabling. (b) Fear-avoidance behaviour (avoiding a movement or activity due to misplaced anticipation of pain) and reduced activity levels. (c) A tendency to low mood and withdrawal from social interaction. (d) An expectation that passive treatments rather than active participation will help.

Management of acute LBP At presentation:

At presentation * Take time to listen and examine, explain and reassure. This has a beneficial effect on pain and on anxiety.22 * Encourage continued activity, including work if appropriate. Bed rest worsens outcome and should only be taken if pain is severe, and then for no more than necessary.25 * Prescribe analgesics or NSAIDs. * Avoid prescribing muscle relaxants, e.g. diazepam. They control pain but the abuse potential and the availability of other analgesic options means that it should not be common practice. * Ensure the patient has a firm mattress. * Demonstrate stretching exercises or give written instructions (e.g. ARC booklet on www.arc.org.uk. 'choose 'index' then 'B' for backache'). Many exercises are available and some

After 6 weeks Failure to progress: * Continue and intensify current management; and * Consider referral to specialist services.

Management of chronic LBP The prevalence of LBP is not increasing but the level of disability and claims for long-term sickness benefits are.22 The management of chronic LBP therefore encompasses several approaches: (a) appropriate referral for physical treatments (see below);

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(b) principles of chronic pain management; (c) attention to psychosocial issues. * Consider prescribing a tricyclic antidepressant at low doses, even if depression is not present. * Recommend regular physical exercise.22 * Treat underlying anxiety or depression if present. * Explore work-related issues. If return to former employment is unlikely, advise the patient to see the Disability Employment Adviser at the job centre. * Consider referral to a multidisciplinary team; this can be effective in resistant cases.27

Nerve root compression * Mechanical back pain can be referred down the leg so not all sciatica arises from a prolapsed disc; nerve root compression is more likely if pain extends down to the foot. Conversely, not all prolapsed discs cause sciatica since they can be demonstrated in 50% of asymptomatic adults. * Sciatica arising from a prolapsed disc that causes significant disability has a life time prevalence of 5%. * Recognizing nerve root compression is important because surgery leads to quicker recovery than natural resolution, although there may be no difference in long-term outcomes.22 * Chymopapain discolysis is also effective but not widely used in the UK.22 * At presentation, test limitation of straight leg raising (see below). * Look for neurological deficit from L5/S1 compression: loss of sensation over the lateral border of the lower leg and foot, weakness of dorsi- and plantar flexion of foot, and impairment of the ankle reflex. In L3/4 compression the knee reflex may be impaired. * Manage sciatica as you would acute LBP. * Refer patients whose pain is not settling (the timescale will depend on local circumstances) or where there is increasing neurological deficit. 28

Diagnosing nerve root compression

The vast majority of cases involve the L5/S1 nerve roots.

* Symptoms: pain radiating below the knee. * Signs: limitation of SLR is a very sensitive finding; its absence makes nerve root compression very unlikely (sensitivity 0.88 to 1). Crossed SLR is a fairly specific test; its presence makes nerve root compression very likely (specificity 0.84 to 0.95).

Referrals Whom to refer to will depend on local availability and agreed care pathways: physiotherapist, pain clinic, multidisciplinary team, orthopaedic physicians or orthopaedic surgeons. * Failure to progress: all guidelines are consistent in recommending early referral (2 weeks or less) for physiotherapy or manipulation in order to prevent acute LBP becoming chronic. However, there is little evidence that this makes a difference to long-term outcome. * Be prepared29 to discuss manipulation; many patients take themselves to osteopaths or chiropractors and many physiotherapists practice a related treatment, mobilization. The evidence for manipulation is of low quality but it may lead to quicker recovery in the first few weeks without making any difference to long-term outcomes.30 * There is insufficient evidence for physical agents and passive modalities, e.g. ice, heat, short-wave diathermy, massage, ultrasound, transcutaneous electrical stimulation and acupuncture22 but they remain popular. * Cauda equina syndrome (see p. 167): refer immediately to an orthopaedic surgeon. * Other Red Flags - refer urgently where appropriate and if a malignancy is suspected refer urgently according to the recommendations of the Department of Health.31 * Sciatica - see above.

X-rays * Explain to patients with mechanical LBP why X-rays are unhelpful: degenerative changes are common, most disorders are of soft-tissue which cannot be shown on X-ray and the dose of radiation is high (60 times that of a chest X-ray).32

NECK PAIN

* Consider X-rays in the presence of Red Flags, alongside urgent referral.

Possible serious spinal pathology Red Flags (a) Cauda equina syndrome (features include urinary retention, bilateral neurological symptoms and signs, saddle anaesthesia - urgent referral indicated). (b) Significant trauma (risk of fracture). (c) Weight loss (suggestive of cancer). (d) History of cancer (suggestive of metastases). (e) Fever (suggestive of infection). (f) Intravenous drug use (suggestive of infection). (g) Steroid use (risk of osteoporotic collapse), (h) Patient aged over 50 years (cancer unlikely below this age). (i) Severe, unremitting night-time pain (suggestive of cancer). (j) Pain that gets worse when patient is lying down (suggestive of cancer).

THE ELDERLY WITH ACUTE BACK PAIN These patients are a special case because osteoporotic collapse and malignancy are more likely and they need an early X-ray. Half will have definite abnormality, and 10% will have a malignancy.33

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Muscle strengthening exercises - daily. You should start these as your pain improves and should keep them up long term. Take up aerobic exercise for general fitness, e.g. swimming. Suitable exercises (there are many others): Starting position: on all fours with hands shoulder width apart, arms and thighs vertical. 1. Arch the back and look down. Then lower the stomach towards the floor, hollowing the back. 2. Slowly walk the hands around to the one side, back to the starting position then around to the other side. 3. Raise one hand off the floor, reach underneath your body as far as you can. On the return, swing the arm out to the side as far as you can then return to the starting position. Follow the moving hand with the eyes. Repeat with the other arm. 4. Draw alternate knees to the opposite elbow. 5. Stretch one arm forward in front, at the same time stretching the opposite leg out behind. Repeat on the other side. 6. Swing the seat from side to side. All exercises should be repeated 10 times. Exercises that hurt should be set aside and returned to with fewer repetitions on another occasion.

Patient information: ARC. Back pain. Online. Available: www.arc.org.uk (choose 'index' then 'B' for backache). Organisation: BackCare 16 Elmtree Road, Teddington, Middlesex, TW11 8ST, tel. 020 8977 5474. Has several leaflets on aspects of neck and back pain, e.g. surgery, exercises, which are also available for download from www.backpain.org/care.html

NECK PAIN

ADVICE/EXERCISES FOR LBP The following is taken from the BackCare leaflet: Exercise for a better back; and from the ARC leaflet: Back pain, see below. Pay attention to sitting, lifting and bending to avoid aggravating pain and to prevent recurrence: Sitting: avoid prolonged sitting. A rolled towel in the small of the back may ease pain. Lifting: always lift with a straight back and bent knees. Bending: avoid spending too long at a job that requires bending, e.g. ironing. Exercise regularly Stretching exercises to maintain flexibility - daily. You may start these early on in an attack and should keep them up long term.

Guidelines: Prodigy. Neck pain. Online. Available: www.prodigy.nhs.uk/guidance/crs/Neck%20Pain.htm

The principles of management of low back pain apply to neck pain.34 • Triage neck pain into mechanical, nerve root compression (often termed radiculopathy) and potential serious pathology (Red Flags). • In mechanical neck pain, keeping active is more effective than immobilization. • Similar medication may be prescribed. • Psychosocial factors are important (see p. 165). • Plain X-rays are not routinely indicated.

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* Manipulation may be effective in the short term. There are many forms of therapy used but there is not enough evidence to comment on the effectiveness of most of them.35

MECHANICAL PAIN * Recognise by: (a) aged 18-55; (b) absence of signs of radiculopathy/Red Flags; (c) made worse by posture/activity. * Advise the patient to stay active.35 Collars should only be worn for a limited period. * Give regular analgesia. * Advise or refer for exercises. * Treat hyperextension injuries (including whiplash injuries) in the same way but avoid collar as this prolongs symptoms.

Red Flags They are as for LBP but note that in cervical myelopathy (equivalent of cauda equina syndrome) neck pain may be absent but should be suspected by the following features: (a) Sensory disturbances in upper and lower limbs; (b) Weakness in upper and lower limbs; (c) Clumsiness and gait disturbance; (d) Spasticity of lower limbs (upper limbs may be normal, spastic or flaccid); (e) Increased tendon reflexes; (f) Lhermitte's sign: paraesthesiae in limbs on neck flexion indicates neck instability and warrants immediate admission. Refer urgently for specialist opinion. Patient information: ARC. Neck pain. Online. Available: www.arc.org.uk (choose 'index' then 'N' for pain in the neck).

Daily stretching and strengthening exercises Hold neck for 10 seconds in each of the six positions (right and left lateral flexion and rotation, flexion and extension) within the pain free range. Repeat 10 times.

Radiculopathy * Distinguish radiculopathy from neck pain that is referred to the shoulder and arm. * Look for the following signs, though their sensitivity and specificity has not been adequately assessed: (a) diminished reflexes (triceps, biceps, supinator); (b) diminished power; (c) diminished sensation along dermatomes; (d) the axial compression test: extend neck and rotate head to side of pain, then apply pressure to head. Reproducing pain or paraesthesiae in arm signifies likely radiculopathy. * Treat as for mechanical neck pain but refer if there is no improvement after 4 weeks. Refer earlier if there is loss of power, and refer immediately if deterioration is rapid.

REGIONAL PROBLEMS Reviews: The Arthritis Research Campaign (ARC). Rheumatic disease in practice, practical advice on management of rheumatic diseases. Online. Available: www.arc.org.uk/ (choose 'about arthritis' then 'Publications for Medical Professionals'). Silver, T. Joint and soft tissue injection. Injecting with confidence, 2nd edition. London: Radcliffe Medical Press, 1999.

Therapies available (a) Analgesics and NSAIDs (see sections on Osteoarthritis and NSAIDs). (b) Flexibility and strengthening exercises. (c) Relative rest. (d) Application of heat or ice for pain relief. (e) Physical modalities - ultrasound, infrared, low-level laser are commonly used by physiotherapists, although evidence for them is lacking.36 (f) Manual therapies. The evidence for manipulation provided by osteopaths and chiropractors

REGIONAL PROBLEMS

is conflicting. Many physiotherapists use a similar technique called mobilization. (g) Intra- and periarticular steroid injections. The evidence for many of these is lacking but they appear to provide short term relief (see below). (h) Protection. (i) Surgery. • Demonstrate exercises in the surgery so that patients may start them as soon as indicated. Advise patients to perform them daily. • Consider referral to a physiotherapist for more advanced exercises and for supervision in selected cases. • Advise patients to reduce the strain on affected tendons and muscles where repetitive activity or work patterns play a significant part.37 This is not the same as immobilisation - flexibility and strengthening exercises should continue.

SHOULDER Exercises Flexibility Demonstrate pendular exercises: • Stand up and lean forward with arm of affected side hanging perpendicularly. • Sweep the arm around in a circle within the pain free range. Do this 20-30 times. • As time goes on, increase the range of the movement and length of time.

169

* Consider intra-articular injections for rotator cuff conditions. Beware direct injections near the long head of biceps because of the risk of tendon rupture.

ELBOWS Lateral and medial epicondylitis (tennis and golfer's elbow): * Consider steroid injection into the painful area for short term relief.39

WRIST Carpal tunnel syndrome * Refer for provision of splints to hold the wrist in neutral position to be worn at night. * Consider injection of steroid into the carpal tunnel for short term relief.40 * Refer resistant cases for surgical release. De Quervain's tenosynovitis * Consider injection of steroid into the tendon sheath, although accurate placing of the needle is difficult.

HIPS Injections * Consider injections for greater trochanteric bursitis. Protection

Strengthening the rotator cuff • Hold your arm outstretched at shoulder height and out to the side. • Bring your arm forward then up, push to the back then down to the starting position. • Repeat 10 times. As time goes on, build up to doing 3 sets of 10 repetitions.

Injections • Consider intra-articular saline and steroid injections for adhesive capsulitis.38

* Recommend that the patient wears impactcushioning soles, e.g. trainers, but if these are not acceptable there are several brands of sole inserts for shoes.

KNEES Exercises Flexibility Stretching the hamstrings: * Stand up with the affected leg slightly in front.

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• Place both palms over the knee cap and push towards the ground. Keep this position for 30 seconds. • Repeat four times. As time goes on, try to increase the stretch by pointing your toes upwards. Strengthening the quadriceps: • Lying down on your back, lift the leg with the knee straight to a position about 45 degrees off the horizontal. • Hold this position for a count of 10 seconds. • Lower the leg, and rest. • Repeat 10 times. As time goes on, build up to doing three sets of ten repetitions.

Injections • Consider injections for: (a) anserine bursitis; (b) the knee joint; (c) the medial ligament of the knee. Protection Recommend impact-cushioning soles.

FOOT/ANKLE Injections * Never inject the Achilles tendon because of the risk of rupture. * Consider steroid injection for plantar fasciitis if impact-cushioning soles prove ineffective.

Referrals * Refer patients for specialist advice when: (a) there is diagnostic doubt; (b) there is failure to improve with therapy; (c) where specialist skills are needed; (d) when the patient is willing to consider surgery and primary care therapy has failed. * Follow local referral protocols or patterns, which vary considerably.

Steroid injections Benefits The poor quality of many studies means that evidence of long-term benefit is lacking. However, it is used because clinical experience demonstrates at least short-term relief, for which there is evidence.41 Harms (a) Infection in one in 14,000-50,000 injections. (b) Tendon rupture in less than 1%. (c) Local scarring in less than 1%.38 * Discuss with the patient the benefits and harms. * Familiarise yourself with the common techniques.42 Indications (a) intensive use of other approaches for at least 2 months has failed; or (b) rehabilitation is inhibited by symptoms. Good practice: * Obtain the patient's consent. * Check that you can define the local anatomy. * Select the finest needle that will reach the lesion. * Clean your hands and the patient's skin. * Use a no-touch technique. * Use short- or medium-acting corticosteroid preparations in most cases, with local anaesthetic. * Injection should be peritendinous; avoid injection into tendon substance. * Minimum interval between injections should be 6 weeks. * Use a maximum of three injections at one site. * Soluble preparations may be useful in those patients who have had hypersensitivity/local reaction to previous injection. * Record details of the injection. * Do not repeat if two injections do not provide at least 4 weeks relief.

Postinjection advice Warn the patient of early postinjection local anaesthesia and to avoid initial overuse. Advise resting for at least 2 weeks after injection and avoid heavy loading for 6 weeks. The patient should

ORAL NON-STEROIDAL ANTI-INFLAMMATORY DRUGS 171

inform the doctor if there is any suggestion of infection or other serious adverse event. Contraindications to corticosteroid injection in soft tissue lesions (a) If pain relief and anti-inflammatory effects can be achieved by other methods; (b) Local or systemic infection; (c) Coagulopathy; (d) Tendon tear; (e) Young patients.

ORAL NON-STEROIDAL ANTI-INFLAMMATORY DRUGS Review article: Gotzsche PC. Non-steroidal antiinflammatory drugs. BMJ2000; 320: 1058-61. Guideline: Eccles M, Freemantle N, Mason J. North of England evidence based guideline development project: summary guideline for non-steroidal anti-inflammatory drugs versus basic analgesia in treating the pain of degenerative arthritis. BMJ 1998; 317: 526-30.

ROLE OF NSAIDs IN MUSCULOSKELETAL DISORDERS • NSAIDs have good analgesic action but many patients with acute or chronic pain can be managed with paracetamol or opioids such as codeine. • NSAIDs taken regularly at full dosage have an anti-inflammatory action which is beneficial in controlling swelling and stiffness, as well as pain, in inflammatory diseases such as rheumatoid arthritis (RA) and the spondyloarthropathies (SpA). • Unlike opioids, they do not cause constipation, drowsiness or dependence. This makes them attractive in treating chronic disease. Against this must be set serious risks, particularly in the elderly.

WHICH ORAL NSAID? • Clinical experience suggests that individuals respond differently to different classes of NSAID,

and sometimes several are tried before a suitable one is found, although systematic reviews have not confirmed this view.43 • The response to each NSAID is dose dependent. A failure to appreciate this has led to some being thought to be weaker than others. • The main difference between NSAIDs is their toxicity, with ibuprofen coming out as the safest. • Where anti-inflammatory action is crucial, clinical experience favours naproxen as a first choice, combining efficacy with intermediate risks.44 • COX II inhibitors selectively inhibit cyclooxygenase 2, which is involved in the inflammatory process, more than they do cyclo-oxygenase 1, which protects against peptic ulceration. Early results suggest that they do reduce the risk of serious GI events, hepatotoxicity and renal toxicity45 but several guidelines advise caution because experience with them is limited. The CSM warns that rofecoxib is contraindicated in the presence of peptic ulceration or GI bleeding and advises caution in presence of heart failure, left ventricular dysfunction, hypertension and oedema.

Adverse drug reactions (a) Upper GI disease: peptic ulceration, bleeding, non-ulcer dyspepsia. (b) Fluid retention, leading to aggravation of heart failure. (c) Renal failure, precipitated in those with preexisting renal impairment. (d) Hypersensitivity. (e) Worsening of hypertension control.46

Older patients The risk of toxicity rises with age and rises steeply over the age of 75. Renal failure and heart failure are probably uncovered rather than caused by NSAID use. The risks, expressed as number of expected hospital admissions, in a PCO with an average population of 100,000 are shown in Table 9.1.47

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Table 9.1 NSAID adverse effects in older people in an average primary care group (PCO) with a population of 100,000 Event

Cases per year

Upper GI bleed Acute renal failure Congestive heart failure

18 10 22

Table 9.2

Risk of bleed caused by NSAID by age

Age

Chance of bleed due to NSAID

<45

1 1 1 1

45-64 65-74 5=75

in in in in

2100 646 570 110

Gl toxicity Upper GI bleeds are dose dependent and toxicity varies between the NSAIDs. Taking ibuprofen as the reference standard (risk = 1), the risk of serious upper gastrointestinal complications rises by 1.6-9.2 times with piroxicam, ketoprofen, tolmetin and azapropazone coming out as those at highest risk.48 Diclofenac ranked second safest, after ibuprofen, with indomethacin, naproxen, sulindac and aspirin carrying an intermediate risk. The risk rises steeply in the elderly (Table 9.2).49 For the prevention of NSAID-related GI toxicity, see p. 133.50

Contraindications to NSAIDs Absolute contraindications: (a) Active peptic ulceration. (b) Hypersensitivity (rhinoconjunctivitis, bronchospasm, urticaria, angioedema, and laryngeal oedema) to aspirin or other NSAID. Avoidance is essential to prevent life-threatening reactions.51 (c) Pregnancy - third trimester.

Cautions (a) Breast feeding - largely due to manufacturers' warnings. The BNF states that the amounts

in breast milk for most NSAIDs are insignificant. See individual NSAIDs in Appendix 3 of BNF. (b) Renal, cardiac or liver failure - renal failure may worsen and U&Es need monitoring. (c) Asthma - worsening of asthma may be due to prescribed or OTC NSAIDs. (d) Coagulation defects - including anticoagulation therapy. (e) Elderly. (f) Previous peptic ulceration. (g) Concomitant steroid therapy (increases risk of upper GI disorder).

Prescribing practice * Before prescribing an NSAID, weigh-up the risks and benefits for the individual and involve the patient in the decision. * Start with low dose ibuprofen, 400 mg t.d.s., and titrate up to 800 mg t.d.s. if there is inadequate response. * However, if anti-inflammatory action is crucial and the patient is young, consider starting with naproxen 500 mg b.d. * If the patient has not responded after 3 weeks, consider changing to a NSAID from another class (see below). * Advise patients to take their tablets with meals and to report dyspepsia immediately. * Consider prescribing COX II inhibitors to patients at high risk of developing serious GI complications. NICE recommends that COX II inhibitors in OA and RA be restricted to those:52 (a) aged over 65 years; (b) on medications likely to increase GI complications; (c) with serious co-morbidity; or (d) requiring the prolonged use of maximum recommended doses of standard NSAIDs. Do not prescribe gastroprotective agents with COX II inhibitors. * Exercise particular caution with patients with a previous history of peptic ulcer or of GI bleeding. * Review the patient's continued need for NSAIDs regularly.

GOUT

What to do53 with NSAID-associated dyspepsia * Establish the accuracy of the diagnosis of NSAID-associated dyspepsia. * Review the need for any drug treatment. * Consider substituting paracetamol. * If paracetamol provides insufficient analgesia, consider substituting co-codamol. * Consider substituting low dose ibuprofen (1.2 g daily) for co-codamol. * Consider lowering the dose of the currently used NSAID. * If sufficient analgesia is achieved only with NSAIDs and the patient has dyspeptic symptoms, consider using acid suppression as adjunctive therapy (see p. 133).

Classes of NSAIDs • Salicylates: aspirin; diflunisal; benorylate. • Acetic acids: diclofenac; etodolac; indomethacin; sulindac; tolmetin. • Propionic acids: fenbufen; fenoprofen; flurbiprofen; ketoprofen; ibuprofen; naproxen; tiaprofenic acid. • Fenamic ac/'cte:flufenamic; mefenamic. • Enolic acids: piroxicam; tenoxicam. • Non-acidic acids: nabumetone. • Selective COX II inhibitors: celecoxib; rofecoxib.

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* Patient education. Advise the patient to start treatment as soon as possible after the onset of any future attacks and to have a standby supply of NSAID. Patients not responding or unable to take NSAIDs

Give: (a) Colchicine 500 ug, two tablets stat followed by one tablet 2-hourly to a maximum total dose of 12 tablets. The drug should be stopped when the attack abates or if side-effects, namely vomiting, diarrhoea or abdominal cramps, outweigh the benefit. Warn the patient that some diarrhoea is likely. Caution in the elderly, in hepatic and renal impairment and in GI and cardiac disease.55 (b) Oral prednisolone 40 mg daily until the attack is settling, then reduce to zero so that the total course is 7-10 days. (c) Methylprednisolone 40 mg intra-articularly (10 mg for a small joint) as a single dose. * Reconsider the diagnosis and investigate compliance if the patient is still not responding. Investigations

GOUT Review: Sturrock RD. Gout, easy to misdiagnose. BMJ 2000; 320:132-3. Guideline: Prodigy Guideline. Gout. Online. Available: http://www.prodigy.nhs.uk/guidance/crs/gout.htm

Treatment of the acute attack * Rest the joint. * Give a NSAID in full dose until symptoms subside, e.g. indomethacin 50 mg t.d.s. or diclofenac 50 mg t.d.s. The CSM has restricted azapropazone for use only when other NSAIDs have failed.54 Do not advise aspirin (which reduces uric acid secretion). * Tail-off the NSAID over 5-10 days.

(a) Uric acid - do this after the attack has settled because it may be lowered during an attack. A raised level does not prove the diagnosis nor does a low level exclude it. The main value is in determining and monitoring prevention. (b) U&Es - may have renal impairment. (c) FBC - may have myeloproliferative disease. (d) Consider joint aspiration when the diagnosis is in doubt, e.g. knee monoarthritis when the differential diagnosis may be gout or pseudogout (urate crystals and calcium pyrophosphate dihydrate crystals seen respectively on microscopy). (e) X-rays are rarely helpful but can sometimes confirm pseudogout (chondrocalcinosis seen on plain films) or demonstrate joint damage from chronic gout, which is more likely to happen as an insidious process in the elderly. (f) Other cardiovascular risk factors. Check BP, cholesterol, weight and smoking habit, which form a cluster that includes hyperuricaemia.

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Prevention Lifestyle

* Advise patients to lose weight if obese but to avoid rapid weight loss, which can precipitate an attack. * Advise the patient to avoid alcohol excess. * Advise the patient to avoid excessive amounts of purine-rich foods, e.g. offal, red meat, oily fish and shellfish, pulses and whole grain. Review medication * If possible, stop drugs which reduce uric acid excretion, otherwise reduce to the lowest effective dose. They are aspirin, thiazides and loop diuretics. Drug prophylaxis

Indications for drug prophylaxis: (a) recurrent attacks of gouty arthritis despite attempts to reduce risk factors; (b) polyarticular gout; (c) the presence of tophi; (d) clinical or radiological signs of chronic gouty arthritis; (e) prophylaxis when cytotoxic therapy is given for haematological malignancy; (f) recurrent uric acid renal stones. Asymptomatic hyperuricaemia is not considered an indication for drug prophylaxis.55 Note: Wait until the acute attack has subsided for at least 2 weeks before starring prophylaxis, otherwise the attack may be prolonged.

in severe conditions. Doses above 300 mg daily should be divided. * Check the uric acid level 4-weekly until it reaches the normal range, and then check uric acid and creatinine yearly. * If minor side-effects occur, e.g. rash, stop allopurinol and then reintroduce it at as low a dose as the patient can cut from a 100 mg tablet, e.g. 10 mg. If this is tolerated, increase the dose slowly. * Do not restart if the rash was exfoliative dermatitis or if it was associated with fever or involvement of another organ. * Give colchicine or an NSAID for the first 3-6 months; attacks may be precipitated in the early stages of allopurinol treatment. Use colchicine 500 |xg b.d. or a standard dose of an NSAID. * If an attack occurs during prophylaxis, continue to use allopurinol at the same dose and treat the attack in its own right. Check alcohol consumption and compliance since this is likely to be cause of failure.56 Uricosuric drugs

* Consider uricosuric drugs if the patient does not have nephropathy, renal calculi or a primary overproduction of uric acid. * Advise the patient to have a good fluid intake and take: (a) probenecid 500 mg daily, increasing to 1 g b.d. if necessary; or (b) sulphinpyrazone l00 mg daily, increasing over 4 weeks to 600 mg daily. * Prescribe colchicine or an NSAID for the first 3-6 months.

Prophylactic drugs Allopurinol

Indications for referral55

* Allopurinol is the first choice because it is so effective and because uricosurics increase the risk of uric acid stone formation. * Prescribe allopurinol starring at 100 mg daily, gradually increasing over 2-3 months according to response of uric acid levels. Lower doses are needed in the elderly and those with renal impairment. The usual maintenance dose is 100-200 mg in mild, 300-600 mg in moderate and 700-900 mg

* If sepsis is suspected. * If the diagnosis in doubt. * If oral medication is not possible or effective, and intra-articular steroid is needed (and facilities for this are unavailable in primary care). Also refer: * Young men or premenopausal women, as the disorder can be secondary.

RHEUMATOID ARTHRITIS

RHEUMATOID ARTHRITIS Review: Madhok R, Kerr H, Hilary A, et al. Recent advances. Rheumatology BMJ 2000; 321: 882-5. Guidelines: Scott DL, Shipley M, Dawson A, et al. The clinical management of rheumatoid arthritis and osteoarthritis: strategies for improving clinical effectiveness. Br J Rheumatol 1998; 37: 546-54. Abridged version Online. Available: www.rheumatology.org.uk/clinical/oara.htm Prodigy. Rheumatoid arthritis. Online. Available: www.prodigy.nhs.uk/guidance/crs/Rheumatoid

Aims of management (a) (b) (c) (d) (e)

To control symptoms: pain and stiffness. To minimize disease progression. To preserve joint function. To minimise drug side-effects. To promote independence and quality of life.

Shared care Shared care between the rheumatology department and the general practitioner is the ideal.

175

• Disease-modifying antirheumatic drugs (DMARDs) slow down disease progression and reduce the degree of joint damage in rheumatoid arthritis (RA).57 Early diagnosis is vital: they must be started within 2 years of onset to achieve most benefit. • Some of the diagnostic criteria set by the American College of Rheumatology (Table 9.3)58 do not appear until late, so waiting for a definite diagnosis would delay referral. • The rheumatoid factor is not a diagnostic test; it can neither confirm nor rule-out the diagnosis, since it is present in many other rheumatological conditions and 30% of RA patients are seronegative. Therefore, referral must be on clinical suspicion. • The choice of DMARD should be made by the rheumatologist and an informed patient.59 • Referral gives the patient access to the multidisciplinary team. • Refer all patients with a suspected diagnosis to a rheumatologist unless there are strong grounds not to, e.g. mild disease in the elderly adequately managed without DMARDs. • Suspect the diagnosis when the following symptoms are combined and persist beyond

Table 9.3 1987 Criteria for the classification of acute arthritis or rheumatoid arthritis (American College of Rheumatology). The presence of four criteria is necessary for diagnosis Criterion

Definition

1. Morning stiffness

Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement

2. Arthritis of three or more joint areas

At least three joint areas simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) observed by a physician. The 14 possible areas are right or left PIP, MCR wrist, elbow, knee, ankle and MTP joints At least 1 area swollen (as defined above) in a wrist, MCR or PIP joint Simultaneous involvement of the same joint areas (as defined in 2) on both sides of the body (bilateral involvement of PIPs, MCPs or MTPs is acceptable without absolute symmetry)

3. Arthritis of hand joints 4. Symmetric arthritis

5. Rheumatoid nodules 6. Serum rheumatoid factor 7. Radiographic changes

Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxta-articular regions, observed by a physician Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in <5% of normal control subjects Radiographic changes typical of rheumatoid arthritis on PA hand and wrist radiographs, which must include erosions or unequivocal bony decalcification localized in or most marked adjacent to the involved joints (osteoarthritis changes alone do not qualify)

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RHEUMATOLOGICAL PROBLEMS

6 weeks (this differentiates RA from viral arthritides): • morning stiffness, • arthritis of three or more joint areas, • synovitis (joint swelling, not bony swelling) of hand joints, • symmetrical arthritis. * Also assess the patient for • extra-articular manifestations including constitutional upset, • psychosocial effects, • disability and the need for physiotherapy or occupational therapy. Prereferral work-up for rheumatoid arthritis59 (a) plasma viscosity (PV) or ESR and c-reactive protein (CRP), as an indication of disease activity. (b) Rheumatoid factor - do this because a positive result early in the disease has prognostic significance (see Table 9.3). (c) FBC, for anaemia and as baseline because some DMARDs affect the blood count. (d) LFTs - some DMARDs affect the liver. (e) U & Es and dip testing of urine because some DMARDs affect renal function. (f) Consider viral serology to exclude reactive viral arthritis. (g) Consider X-ray of affected joints and of the hands and feet because rheumatologists usually request them as a baseline for future progression. However, X-rays are unlikely to be helpful in diagnosis because erosive changes are not usually apparent early in the disease, although X-rays of the feet can sometimes be diagnostic.60

NSAIDs NSAIDs are effective in controlling stiffness, pain, and swelling.62 The incidence of RA rises with age and peaks in the sixties so that those most at risk of NSAID toxicity are those most likely to need them. * Prescribe the most effective NSAID at the lowest effective dose (see section on NSAIDs). Although there is no good evidence of the superiority of one NSAID over another, or that some patients are better suited to certain types, it remains customary to try a patient on one NSAID for a period of several weeks and then to switch to an alternative if appropriate.

DMARDs See Table 9.4.

Patients with poor prognosis

* Support patients during the initiation of therapy. There may be no benefit for 4-6 months. * Ensure that prescriptions for methotrexate state clearly that the dose is to be taken weekly the CSM reports fatal errors in prescribing and dispensing.63 * Ensure patients know about the dangers to conception: several DMARDs are contraindicated in pregnancy and both male and female patients should avoid conception until 6 months after stopping cytotoxics. * Follow local protocols for monitoring adverse effect of these drugs, as protocols vary between institutions.64 See Appendix 22 for a proposed protocol.

The following carry a poor prognosis if present early in the disease:

Steroids

(a) high level of disability; (b) high ESR or c-reactive protein; (c) positive rheumatoid factor.61

PHARMACOLOGICAL TREATMENT Analgesics * Advise regular paracetamol as a first line. If necessary, a weak opioid such as codeine phosphate or dihydrocodeine may be added.

High-dose steroids rapidly improve symptoms and slow disease progression but the side-effects are unacceptable.59 Low-dose steroids slow progression but the effect stops after cessation of therapy. * Consider steroids in the following situations: (a) exacerbations or at the start of using DMARDs: short-term oral steroid therapy; (b) localized flare-ups: use local steroid injections;

RHEUMATOID ARTHRITIS

Table 9.4

177

DMARDs, usual dose and selected toxicity66

Drug

Usual maintenance dose

Toxicity Infrequent rash, diarrhoea, rare retinal toxicity Rash, infrequent myelosuppression, Gl intolerance

Hydroxychloroquine

200 mg twice daily

Sulfasalazine Methotrexate

1000mg twice or 3 times daily 7.5-1 5 mg per week

Injectable gold salts

25-50 mg im every 2-4 weeks

Gl symptoms, stomatitis, rash, alopecia, infrequent myelosuppression, hepatotoxicity, rare but serious (even life threatening) pulmonary toxicity Rash, stomatitis, myelosuppression, thrombocytopenia, proteinuria

Oral gold

3mg daily or twice daily

Same as injectable gold but less frequent, plus frequent diarrhoea

Azathioprine

50-1 50 mg daily

Penicillamine

250-750 mg daily

Myelosuppression, infrequent hepatotoxicity, early flu-like illness with fever, Gl symptoms, elevated LFTs Rash, stomatitis, loss of taste, proteinuria, myelosuppression, infrequent but serious autoimmune disease

(c) when DMARDs are contraindicated or are not acceptable and when NSAIDs pose particular risk (e.g. the elderly): use long-term lowdose oral corticosteroids (7.5 mg per day or less of prednisolone).65 Note: cautions in long-term steroids: See steroids in section Polymyalgia/Temporal arteritis and section on Osteroporosis. Newer DMARDs63

Although effective, the long-term safety of these drugs has not been established: • Leflunomide is a newer immunosuppressant whose toxicity includes myelosuppression, increased risk of infection and cancer. Persistence of the active metabolite means that wash out procedures are needed to treat adverse effects and before starting other DMARDs or before conception. • Tumour necrosis factor inhibitors: etanercept and infliximab are licensed for use when other DMARDs have failed.

Non-drug management Research evidence is lacking for the effectiveness of many non-drug treatments59 but this constitutes an absence of evidence rather than evidence of absence of effect. Hence, education, physiotherapy and related interventions remain at the centre of care.

* Constantly review patients not under the care of the multidisciplinary hospital team for the need for referral to any of the following: (a) physiotherapists - for exercise programmes and splints; (b) occupational therapists - for mobility and aids of daily living; (c) nurse specialists; (d) orthotic and prosthetic departments; (e) chiropodists.

Surgery Surgery can be highly effective in selected cases59 and covers tendon transfers, arthroplasties (including upper limb and small joints), and arthrodeses.

Alternative therapies/diet Alternative therapies and diets are popular with patients.67 * Be prepared to discuss alternative strategies and recommend ARC booklets (see p. 178). * Encourage increased intake of oily fish.68

Disability Over 80% of patients will be at least moderately disabled after 20 years. * Management; See under Disability p. 403.

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PROBLEMS

Patient information: ARC. Rheumatoid arthritis. Online. Available: www.arc.org.uk (choose 'index' then 'R' for rheumatoid arthritis).

• Positive answers (shown in italics) to a combination of five questions (see box below) are highly predictive for the disease.71 Screening questions for the detection of ankylosing spondylitis:

SPONDYLOARTHROPATHIES

onset - insidious; age - less than 40; duration - for longer than 3 months; morning stiffness - present, effect of exercise - relieves pain.

Review: Keat A. Spondyloarthropathies. BMJ 1995; 310:1321-4. Guideline: Prodigy. Guideline 1999. Online. Available: www.prodigy.nhs.uk (choose 'clinical guidance'/'released guidance' then follow links to A.S.).

The Spondyloarthropathies (SpA) are a group of conditions with symptoms that overlap with ankylosing spondylitis. They are associated with the HLA-B27 tissue type. The distinguishing feature is disproportionate inflammation at the entheses (the sites of attachment of tendons and ligaments to bone), and any associated synovitis is considered secondary to the enthesitis.69 The category includes: • ankylosing spondylitis (AS); • reactive arthritis (following an infection, usually bowel or genitourinary, the latter often termed Reiter's syndrome); • psoriatic arthropathy; • enteropathic arthritis (associated with inflammatory bowel disease).

Aims of management • Control symptoms: pain, stiffness and disability, • Prevent deformity and disability, • Manage non-skeletal complications: fatigue, anterior uveitis, pulmonary and thoracic restriction (AS), aortic incompetence (AS), and inflammatory bowel disease.

Diagnosis of AS • Early diagnosis is important to establish an exercise program to prevent deformity. • Back pain and AS: physical examination is unreliable; the history is much more important.70

OTHER SPONDYLOARTHROPATHIES * Search for clues in personal and family histories and on examination for diseases of bowel, skin and sexually transmitted disease.

Investigations (a) CRP and ESR or PV are frequently raised but may be normal. (b) HLA-B27 does not confirm the diagnosis since the background incidence is 6-8% in the general population. However, a negative result makes the diagnosis unlikely because 95% of AS sufferers are positive. (c) X-rays of the sacroiliac joints (SIJs) give a high dose of radiation with little result, because diagnostic changes take years to develop. MRIs are more accurate than X-rays. * Ask for CRP and ESR or PV to assess inflammation. * Check FBC for the anaemia of chronic disease. * Explain why a plain X-ray is unlikely to help early in the disease. Consider X-ray of the sacroiliac joints if symptoms have been present for several years. Consider referral for diagnosis.

Management * Prescribe an NSAID. Choose one with a long half-life to ease morning stiffness, e.g. naproxen. * Prescribe or advise OTC analgesics for p.r.n. use if needed. * Explain the need for regular, daily exercise: it helps to control pain and prevents increasing stiffness and prevents deformity.72 Swimming is a

CONNECTIVE TISSUE DISEASES

good all-round exercise but specific daily exercises have been developed for maintenance of posture. * Refer to physiotherapy for exercises or to the local NASS group (see box below), or both. * During flare-ups, advise patients to continue exercises within the limits of pain. * Advise patient to lie on floor prone or supine for 20 minutes each day. * Prescribe low-dose antidepressants for nocturnal pain and fatigue.73 * Advise the patient to stop smoking. * Prescribe or advise hypromellose eye drops for conjunctival disease (dry gritty eyes). * Watch out for and warn patient of acute iritis or uveitis. * Consider local steroid injections for peripheral joint disease or enthesitis for short-term relief during flare-ups.74

Referrals To a rheumatologist (Prodigy)

* Strongly consider referring all new or suspected cases of ankylosing spondylitis. * Refer if flare-ups fail to settle. * Refer in the presence of peripheral joint involvement. DMARDs may relieve peripheral arthritis.75,76 To other specialities

* Refer immediately for an ophthalmological opinion if you suspect acute iritis. * Refer to a gastroenterologist in presence of symptoms of inflammatory bowel disease. Booklets and information: National Ankylosing Spondylitis Society (NASS), PO Box 179, Mayfield, East Sussex, TN20 6ZL, tel. 01435 873527; fax 01435 873027; website http://nass.co.uk

CONNECTIVE TISSUE DISEASES Review: Ahmad Y, Bruce I. Connective tissue disease and the role of the general practitioner. Arthritis

179

Research Campaign (ARC), 2000. Rheumatic disease in practice, Number 3. Online. Available: www.arc.org.uk (choose 'about arthritis' then 'Publications for Medical Professionals').

• The term connective tissue diseases (CTDs) covers a group of inflammatory diseases affecting multiple systems, which have a variable presentation and course, and whose features overlap. They are strongly associated with several autoantibodies. • Complications can be serious and fatal, e.g. lung fibrosis, renal failure, gangrene. • Many features, especially early in the disease, are non-specific. • A group practice with 8000 patients may have 50-80 patients with Sjogren's syndrome, 4 with systemic lupus erythematosus (SLE), and 4-8 with other CTDs. They are uncommon but the GP has a special role in both diagnosis and treatment because of the multisystem and chronic nature of CTDs. • Evidence-based guidelines are not available for CTDs in general. However, many of the management issues overlap with those of other inflammatory rheumatic conditions already mentioned.

Diagnosis • Suspect CTD when symptoms suggesting multiple system involvement are present. The more suggestive features there are, the greater is the likelihood of disease being present, especially if combined with non-specific features. • Review the medical history for salient features which may have been overlooked in the past, e.g. miscarriages. • Order the following tests: (a) FBC - leucopenia, lymphopenia, thrombocytopenia or haemolysis suggest CTD; (b) Urine dipstick and U&Es screening for renal disease; (c) Creatine kinase; (d) ESR/PV - usually raised (note CRP is often normal in SLE in the absence of infection); (e) Serum ANA - is positive in over 90% of many CTDs. However, it should not be relied on as a diagnostic test in the absence of clinical

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features as it is positive in 5% of the general population and >30% of rheumatoid arthritis sufferers.

Management * Refer suspected cases early to a rheumatologist for diagnosis and a management plan. * Share care for monitoring progress and therapy with the hospital specialist (as for RA). * Screen for and manage other cardiovascular risk factors, e.g. BP (especially when renal disease is present or high dose steroid therapy used). * Women's health: consider the increased risk of thromboses and drug therapy (e.g. cytotoxics) for CTD in preconception counselling, contraception, pregnancy and hormone replacement therapy (HRT). * Treat infections promptly in SLE as they may lead to flare-ups. Consider possibility of atypical organisms in the immunocompromised. Patient information: The British Sjogren's Syndrome Association, Unit 1 Manor Workshops, Nailsea Wall Lane, West End, Nailsea, Bristol BS19 2RA, tel. 01275 854 215. Lupus UK, St James House, Eastern Road, Romford, Essex RM1 3NH, tel. 01708 731 251. The Raynaud's and Scleroderma Association, 112 Crewe Road, Alsager, Cheshire ST7 2JA, tel. 01270 872 776.

OSTEOPOROSIS Guidelines: Royal College of Physicians. Osteoporosis: clinical guidelines for prevention and treatment. London: RCP, 1999. Online. Available: www.doh.gov.uk/osteorep.htm Royal College of Physicians and The Bone and Tooth Society of Great Britain. Osteoporosis: clinical guidelines for prevention and treatment. Update on pharmacological interventions and an algorithm for management. London: RCP, 2000. Online. Available: www.rcplondon.ac.uk National Osteoporosis Society. The prevention and management of corticosteroid induced osteoporosis. NOS: Bath, 1998. Online. Available: www.nos.org.uk (choose 'information for professionals').

• Osteoporosis is a disorder of diminished bone density and degenerate microarchitecture leading to increased fragility and risk of fracture. • It causes over 200,000 fractures each year in the UK. • Over one-third of women and one in twelve men will suffer an osteoporotic fracture in their lifetime. • Little therapeutic research has been performed on men but there is no reason to suppose they respond differently to women, so management is similar. • The terms prevention and treatment refer to the prevention and treatment of osteoporosis, whether or not a fracture has occurred. Definitions DEXA (dual energy X-ray absorptiometry) measures bone mineral density (BMD). Osteoporosis = BMD T score >2.5 standard deviations below the young adult mean. Osteopenia = BMD T score between 1 and 2.5 standard deviations below the young adult mean. AT score above -1 is normal.

Patients at high risk (a) The frail, with increased risk of falls, whether housebound or not. (b) Those with major risk factors: • untreated hypogonadism, i.e. premature menopause, secondary amenorrhoea, primary hypogonadism in women, primary or secondary hypogonadism in men; • prolonged corticosteroid therapy (see below); • diseases associated with osteoporosis, e.g. Crohn's disease; • radiological osteopenia (both vertebral fractures and apparent loss of bone density). Other risk factors include family history of osteoporosis, low body weight, and smoking. (c) Those with a previous fragility fracture.

Work-up when osteoporosis is diagnosed * Order the following: FBC, ESR, calcium, phosphate, LFTs, U&Es and TFTs (hyperthyroidism causes secondary osteoporosis).

OSTEOPOROSIS

* Consider ordering: a serum FSH if the hormonal status of a woman is unclear; or serum testosterone, LH and sex hormone binding globulin (SHBG) in men. Serum paraprotein and urine Bence-Jones proteins. * Consider ordering: lateral thoracic and lumbar spine X-rays and an isotope bone scan.

181

site is taken as the end point, the relative risk of fracture on pharmacological therapy over 1 to 3 years is between 0.33 to 0.4 (compiled from data in RCP guidelines) compared to no treatment. For the frail elderly with or without osteoporosis

Management Lifestyle advice for all categories * Exercise - take regular weight-bearing exercise, e.g. aerobics, running. * Smoking - stop smoking. * Alcohol - keep to recommended limits (see p. 321). * Diet - include calcium 700 mg and vitamin D 400 iu intake. (See Table 9.5 for calcium contents of selected foods.) Pharmacological therapies * Only alendronate and risedronate have been shown to reduce bone loss and fracture rate at all sites (spine, hip, and elsewhere).77 However, the RCP does not recommend any one form of treatment over another. * The RCP recommends HRT for at risk postmenopausal women without established osteoporosis. However, HRT for >5-10 years is associated with a 50% increase in breast cancer and the beneficial effects of HRT may be lost a few years after cessation (see p. 219). * Depending on the drug used, the population studied, length of follow-up and which fracture Table 9.5

* Consider prescribing calcium 0.5-1 g daily and vitamin D 400 iu daily. * Consider falls risk assessment, advice and referral, e.g. to physiotherapy (walking aids, hip protectors) or occupational therapy.78 For those at high risk, or with established osteoporosis with or without fracture * Consider referral to specialist for DEXA scan or refer directly to imaging unit. Only refer if the result is likely to alter your management. * Treat according to the result as follows: -T score above —1: Reassure and give lifestyle advice.79 -T score —1 to —2.5: Lifestyle advice and recommend pharmacological therapy if previous fracture. -T score below —2.5: Lifestyle advice and recommend pharmacological therapy. Postmenopausal women * Use HRT or a bisphosphonate, or calcitonin second line. * If using a bisphosphonate for prevention, use alendronate 5mg daily or, for treatment, 10 mg

Estimating dietary intake of calcium

Food source

Quantity

Calcium content mg

Milk

Glass, 200 ml

Cheddar cheese Low-fat fruit yoghurt White or brown bread Wholemeal bread Baked beans Canned sardines in tomato sauce with bones Broccoli

30 g Pot(150g) 2 slices of large loaf 31 g 2 slices of large loaf 31 g 1 small can, 150g 100g

237-249 (depending on whether full fat, semi-skimmed or skimmed) 216 225 72 39 80 460

Cooked, 85 g

34

Source: The Calcium Counter, National Dairy Council, 5-7 John Princes Street, London.

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RHEUMATOLOGICAL PROBLEMS

daily or 70 mg once a week; or use cyclical etidronate with calcium; or risedronate 5 mg daily. Steroid-induced osteoporosis * Prescribe alendronate 5mg daily for prevention or treatment but 10 mg if postmenopausal and not on HRT; or use cyclical etidronate with calcium; or risedronate 5 mg daily. Premenopausal women or men <65 with osteoporosis * Refer; they are more likely to have an underlying cause. Older men * Treat as for postmenopausal women but without the HRT. When prescribing biphosphonates Advise the patient to swallow the tablets whole, half an hour before breakfast and to remain upright for half an hour afterwards. Nausea is a common side-effect, which can be reduced by the once-weekly formulation.

Alternative treatments • Raloxifene prevents bone loss but may cause hot flushes. It is associated with a decreased risk of breast cancer and so may be indicated in women for whom the breast cancer risk with HRT is unacceptable. • Calcitonin is only available as parenteral therapy and so only likely to be considered when neither biphosphonates nor HRT is suitable. • Calcium and vitamin D are only suitable as supplements to other forms of therapy in the treatment of osteoporosis. • Calcitriol is a vitamin D derivative licensed for the treatment of postmenopausal osteoporosis. The evidence is less robust than for the treatments above. Corticosteroid use80 The greatest bone loss occurs in the first 6 months of therapy.

* Review frequently the need for continued therapy or dose. * Consider an alternative route of administration, e.g. inhaled steroids for asthma. * Consider alternative drugs, e.g. azathioprine. * Give lifestyle advice to reduce the osteoporosis risk. * Assess the need for prophylactic drugs if starting steroid therapy with prednisolone 7.5 mg daily or more which is likely to continue for 6 months or has already been given for 6 months. Triage according to dose and other risk factors. * The following should receive pharmacological prevention: (a) those on prednisolone 15 mg daily or more; (b) those on prednisolone 7.5 to 15 mg daily plus one or more risk factors (see above). * Those on prednisolone 7.5 to 15 mg daily but who have no other risk factors need referral for DEXA scanning to quantify their risk. Treat if T is below —1.5; otherwise repeat in a year and treat if there has been a loss >4% at the spine or >7% at the hip. Then recheck the DEXA scan 1-3 yearly according to the risk. * Intermittent steroids. Manage the patient as above if the total dose over 6 months is equivalent to the doses given above. This means considering prophylactic drugs if intermittent steroid use equates to 7.5 mg daily over 6 months.

Management of fracture In vertebral collapse, exercises are contraindicated and recovery is likely to take 3 months. * Refer to domiciliary physiotherapy and occupational therapy to keep the patient mobile and independent. * Prescribe analgesics. * If analgesia is insufficient, prescribe calcitonin 50-100 iu sc or im injection, three times a week or daily. Reduce the frequency of dosing as the pain is controlled. The maximum duration of treatment is 3 months.81 It should be combined with calcium (at least 600 mg/day) and vitamin D (400 iu daily). Its analgesic action is independent of its effect on the osteoporosis.

POLYMYALGIA RHEUMATICA/TEMPORAL ARTERITIS 183

Patient information: ARC. www.arc.org.uk (choose 'index' then 'O' for osteoporosis). The National Osteoporosis Society, Camerton, Bath BA2 OPJ, tel. 01761 471771; Helpline 01761 472721; www.nos.org.uk

POLYMYALGIA RHEUMATICA/ TEMPORAL ARTERITIS Review: Swannell AJ. Polymyalgia rheumatica and temporal arteritis: diagnosis and management. BMJ 1997; 314:1329. Guideline: Polymyalgia, clinical recommendation from prodigy, 1999. Online. Available: www.prodigy.nhs.uk/ guidance/crs/Polymyalgia%20Rheumatica.htm

Background (a) About half of patients with giant cell arteritis (GCA) have symptoms of polymyalgia rheumatica (PMR), and 15-50% of patients with PMR have GCA.82 (b) In PMR, the inflammation affects mainly the shoulder and pelvic girdle muscles. (c) They are both conditions predominantly of the elderly, rarely occurring under the age of 50.

POLYMYALGIA RHEUMATICA Management objectives • To control symptoms of stiffness and pain (plus associated constitutional symptoms, e.g. fatigue, weight loss). • To reduce the risk of the complication of temporal arteritis. • To minimize the risks of steroid therapy.

Diagnosis • The patient is being committed to at least 2 years of steroid therapy. As no single test reliably confirms the diagnosis, the history is important. • The typical history in PMR is of pain, stiffness, or both, affecting the upper arms and upper legs

with diurnal variation, that is, say, worse in the mornings. This history is present in 95% of cases.83 • Confidently diagnose PMR if three or more of the diagnostic criteria (see box below) are present or if one criterion is associated with a clinical or pathological abnormality of the temporal artery. The sensitivity for this criterion-based test is 92% (i.e. it will correctly identify 92% of all cases) and the specificity is 80% (i.e. it will correctly exclude 80% of normals).84 Features of polymyalgia rheumatica (a) Bilateral shoulder pain or stiffness. (b) Onset of illness <2 weeks duration. (c) Initial ESR >40mm/h. (d) Age 65 years or more. (e) Depression and/or weight loss. (f) Bilateral tenderness in the upper arms.

Investigations * Check ESR or plasma viscosity. Note that the ESR will be normal in 20% of cases. * Check FBC; a normocytic normochromic anaemia is common.

Initial management * Measure weight, BP and test urine for sugar before starting steroids. * Prescribe prednisolone 15 mg daily. Higher starting doses are rarely needed and are more likely to cause adverse effects.85 * Review within a fortnight. Expect a dramatic response within days. * Consider these alternative diagnoses if there is no response to steroids: (a) cervical spondylosis (the commonest differential diagnosis); (b) rheumatoid arthritis; (c) malignancy especially lung cancer; (d) connective tissue disease - polymyositis; (e) myeloma; (f) hypothyroidism. * Therefore, retake history, re-examine and consider the following investigations: (a) CXR;

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RHEUMATOLOGICAL PROBLEMS

(b) FBC; (c) biochemistry screen: U&E, calcium, LFTs, TFTs; (d) protein electrophoresis - to exclude myeloma; (e) creatine kinase - to exclude polymyositis. * Raise the dose of prednisolone to 30 mg daily if you have confidently excluded other diagnoses.

Maintenance steroid therapy * The risk of relapse is highest early on in treatment and at doses below 10 mg. Temporal arteritis is more likely to develop at doses below 10 mg daily. * Treatment needs to be maintained for at least 2 years. Even then, only a quarter of patients can stop steroids after this time.86 * Over half of patients with PMR (and GCA) will develop complications of steroids, the commonest being weight gain.87 * Reduce prednisolone dose to the lowest possible to maintain control. A suggested routine is as follows:88 * Prescribe 15 mg daily for the first month. Check inflammatory markers after 2-6 weeks. * Reduce by 2.5 mg each fortnight until 10 mg daily is reached, providing there is no relapse of symptoms or rise in inflammatory markers. * Review the patient 1 week after each dose reduction. * Reduce the daily dose by 1 mg every 6 weeks until 5-7 mg daily is reached. * Maintenance dose: 5-7 mg for 12 months providing there is no relapse of symptoms or inflammatory markers. * Final reduction: Reduce the daily dose by 1 mg every 6 to 8 weeks, providing symptoms and (less importantly) inflammatory markers remain controlled. * If symptoms suggest continuing disease in presence of normal inflammatory markers, give more weight to the former. * Warn patients that symptoms may return and advise early consultation. A return of symptoms is a more reliable early indication of relapse than inflammatory markers.

Referral * Refer patients when: (a) Symptom control requires a high maintenance dose of steroids or the patient develops significant adverse steroid effects on lower doses (steroid sparing drugs may be considered). (b) There is doubt about the diagnosis, particularly when there is synovitis and anaemia suggestive of rheumatoid arthritis.

Patient education * Advise patients to seek urgent attention if they develop a unilateral headache, tenderness in the scalp or jaw claudication (facial pain on chewing). * Advise patients about the risks of steroids (see below) and precautions to take. * Offer the ARC booklet, available: www.arc. org.uk (choose 'index' then 'P' for polymyalgia rheumatica).

TEMPORAL ARTERITIS • Temporal arteritis is the commonest form of GCA but other arteries may be involved. After headache or pain in the temple, the next most common syndrome is jaw claudication. • It is almost exclusively a disease of white people. • A normal ESR, PV or negative temporal artery biopsy do not rule out the disease. The chance of obtaining a positive biopsy after 1 week of steroid therapy falls to 10%.82 • Steroid therapy should not be delayed pending the results of investigations because untreated GCA carries a risk of visual loss in 40% of patients.89

Initial management82 * Visual loss: If vision is impaired, give prednisolone up to 80 mg immediately and make an urgent referral to an ophthalmologist. If there is likely to be a delay, continue that dose daily. * Clear clinical diagnosis without visual loss: (a) Take blood for an ESR and FBC; (b) Start prednisolone 40 mg daily;

POLYMYALGIA RHEUMATICAfi'EMPORAL ARTERITIS

(c) See the patient after 48 hours: If the symptoms have virtually gone, the diagnosis is confirmed (see below for maintenance doses). If the symptoms are still present, discuss the patient with a rheumatologist/ophthalmologist urgently. Temporal artery biopsy is likely to be needed, and should be performed as soon as possible after starting steroids. * Suspected diagnosis: Check ESR or PV, start steroids 40 mg daily and arrange for the patient to be seen within 48 hours by a rheumatologist/ ophthalmologist.

Maintenance treatment * Reduce the dose by 5 mg every 4 weeks down to 10 mg daily, then as for polymyalgia rheumatica. * If long-term maintenance is needed, keep the dose as low as possible, e.g. about 3 mg daily.

Steroids Adverse effects will develop in up to 50% of patients. The risk is positively associated with the dose.82 * Educate the patient about the risks. (a) weight gain; (b) hypertension; (c) osteoporosis; (d) development of diabetes/worsening of diabetic control; (e) infection risk, especially chicken pox; (f) risk of GI perforation in combination with NSAIDs; (g) adrenal suppression. * Ensure the patient has an up-to-date steroid card. * Advise the patient not to stop steroids suddenly. * Advise the patient that it may be necessary to increase the dose of steroids during intercurrent illness and to seek medical advice. * Warn the patient to avoid contagious contacts with chicken pox and shingles (there is a risk of fatal disseminated chickenpox if not immune). Advise them to seek urgent medical advice if they are exposed. Obtain specialist advice if such exposure occurs (see p. 31).

185

* Consider testing immune status for chickenpox when commencing steroid therapy. * Check weight, BP and urine (or blood) for glucose every 3 months. * Patients are likely to be on 7.5 mg or more prednisolone daily for 6 months so should be started on prophylaxis against osteoporosis (see Osteoporosis, above). * Advise flu vaccination if on high doses of steroid over the winter months.

FIBROMYALGIA Review: Doherty M, Jones A. ABC of rheumatology: Fibromyalgia syndrome. BMJ1995; 310: 386-9.

* This is a distinctive syndrome90 with the following features: (a) diffuse pain; (b) fatigue and non-restorative sleep; (c) multiple points of extreme tenderness, usually neck and low back, trapezius, chest wall, lateral humeral epicondyle, upper outer quadrant of buttock, greater trochanter and knee. * Some deny its existence as a syndrome.91 Its preponderance in women (90% of sufferers), the presence of fatigue and poor sleep, and its association with anxiety and depression have led to the view that it is a form of somatisation. However, these facts do not de facto prove it to be a psychosomatic disease, since they apply to several rheumatic problems, e.g. rheumatoid arthritis.

Management * Find out the patient's concerns and worries and answer appropriately. * Explain that there is no serious pathology but do not imply that the symptoms are not genuine.92 * Advise gradual increasing exercise.92 * Explain the value of tricyclics in raising the pain threshold and improving sleep. Prescribe low dose amitriptyline 25 mg o.n. and increase according to the response.93 * In severe unremitting cases, consider referral to a multidisciplinary team.

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RHEUMATOLOGICAL PROBLEMS

Patient information: ARC leaflet. Online. Available: www.arc.org.uk (choose 'index' then 'F' for fibromyalgia).

Patient support group: Fibromyalgia Association UK, PO Box 206, Stourbridge, DY9 8YL, tel. 01384 820052; www.fmsni.freeserve.co.uk

REFERENCES 1. Superio-Cabuslay E, Ward MM, Lorig KR. Patient education interventions in osteoarthritis and rheumatoid arthritis: a meta-analytic comparison with nonsteroidal anti-inflammatory drug treatment. Arthritis Care Res 1996; 9: 292-301. 2. Donovan JL, Blake DR. Qualitative study of interpretation of reassurance among patients attending rheumatology clinics: 'just a touch of arthritis, doctor?' BMJ 2000; 320: 541-4. 3. Bird HA. When are NSAIDs appropriate in osteoarthritis? Drugs Aging 1998; 12: 87-95. 4. Eccles M, Freemantle N, Mason J et al. North of England evidence based guideline development project: summary guideline for non-steroidal anti-inflammatory drugs versus basic analgesia in treating the pain of degenerative arthritis. BMJ 1998; 317: 526-30. 5. PCRS. The management of osteoarthritis - patient-centred not disease-focused. Primary Care Rheumatology Society. Online. Available: www.liv.ac.uk/HumanAnatomy/phd/pcr/ 6. Chard J, Dieppe P. Glucosamine for osteoarthritis: magic, hype, or confusion? BMJ 2001; 322: 1439^0. 7. Moore RA, Tramer MR, Carroll D et al. Quantitative systematic review of topically applied non-steroidal anti-inflammatory drugs. BMJ 1998; 316: 333-8. 8. Eccles M, Freemantle N, Mason J et al. North of England evidence based guideline development project: summary guidelines for non-steroidal anti-inflammatory drugs versus basic analgesia in treating the pain of degenerative arthritis. BMJ 1998; 317: 526-30. 9. Zhang WY, Li Wan Po A. The effectiveness of topically applied capsaicin: a meta-analysis. Eur J Clin Pharmacol 1994; 46: 517-22. 10. Creamer P. Intra-articular corticosteroid injections in osteoarthritis: do they work and if so, how? Ann Rheum Dis 1997; 56: 634-6. 11. DTB. Hyaluronan for knee osteoarthritis. Drug Ther Bull 1999, 37. 12. Gam AN, Johannsen F. Ultrasound therapy in musculoskeletal disorders: a meta-analysis. Pain 1995; 63: 85-91. 13. Puett DW, Griffin MR. Published trials of non-medicinal and non-invasive therapies for hip and knee osteoarthritis. Ann Intern Med 1994; 121: 133^0. 14. Royal College of Radiologists. Making the best use of a department of clinical radiology. London: RCR, 1998. 15. Scott DL, Shipley M, Dawson A et al. Strategies for improving clinical effectiveness. London: British Rheumatology Society. Online. Available: www.rheumatology.org.uk 16. Towheed TE, Hochberg MC. Health-related quality of life after total hip replacement. Semin Arthritis Rheumatism 1996, 26: 483-91.

17. Callahan CM, Drake BG, Heck DA et al. Patient outcomes following tricompartmental total knee replacement: a meta-analysis. JAMA 1994, 271: 349-57. 18. Garellick G et al. Survival of hip replacements. Clin Orthop Rel Res 2000; 375: 157-67. 19. Moran CG, Horton TC. Total knee replacement: the joint of the decade. BMJ 2000; 320: 820. 20. National Institutes for Health. Total hip replacement. MH Consensus Statement 1994; 12: 1-31. 21. Simmons N, Ball A, Cawson R et al. The case against antibiotic prophylaxis for dental treatment of patients with joint prostheses. Lancet 1992; 339: 310. 22. Nachemson A, Jonsson E (Eds). Neck and back pain. Philadelphia: Lippincott Williams & Wilkins, 2000. 23. Mason V. The prevalence of back pain in Great Britain. London: OPCS HMSO, 1994. 24. Garvey T, Marks M, Wiesel S. A prospective, randomized, double-blind evaluation of trigger-point injection therapy for low-back pain. Spine 1989; 14: 962-4. 25. Waddell G, Feder G, Lewis M. Systematic reviews of bed rest and advice to stay active for acute low back pain. Br J Gen Pract 1997; 47: 647-52. 26. Tulder MV, Malmivaara A, Esmail R, Koes B. Exercise therapy for low back pain (Cochrane Review). The Cochrane Library, Issue 2. Oxford: Update Software, 2000. 27. Guzman J, Esmail R, Karjalainen K et al. Multidisciplinary rehabilitation for chronic low back pain: systematic review. BMJ 2001; 322: 1511-16. 28. Vroomen P, de Krom M, Knotterus J. Diagnostic value of history and physical examination in patients suspected of sciatica due to disc herniation: a systematic review. / Neurol 1999; 246: 899-906. 29. Hazard R, Haugh L, Reid S et al. Early physician notification of patient disability risk and clinical guidelines after low back injury: a randomized, controlled trial. Spine 1997; 22: 2951-8. 30. Koes BW, Assendelft WJ, van der Heijden GJ et al. Spinal manipulation for low back pain: an updated systematic review of randomized clinical trials. Spine 1996; 21: 2860-71. 31. DoH. Referral guidelines for suspected cancer. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/cancer/referral.htm 32. Royal College of Radiologists. Making the best use of a department of clinical radiology. London: RCR, 1998. 33. Frank A. Low back pain. BMJ 1993; 306: 90. 34. Nachemson A, Jonsson E (Eds). Neck and back pain. Philadelphia: Lippincott Williams & Wilkins, 2000. 35. Aker P, Gross A, Goldsmith C et al. Conservative management of mechanical neck pain: systematic overview and meta-analysis. BMJ 1996; 313: 1291-6.

REFERENCES

36. Gam AN, Johannsen F. Ultrasound therapy in musculoskeletal disorders: a meta-analysis. Pain 1995; 63: 85-91. 37. Yassi A. Repetitive strain injuries. Lancet 1997; 349: 943-7. 38. Speed C, Hazelman B. Shoulder pain. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. 39. Assendelft W, Hay E, Adshead R et al. Corticosteroid injections for lateral epicondylitis: a systematic overview. BJGP 1996; 46: 209-16. 40. Marshall S, Tardif G, Ashworth N. Local corticosteroid injection for carpal tunnel syndrome (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 41. Speed C. Corticosteroid injections in tendon lesions. BMJ 2001; 323: 382-6. 42. Silver T. Joint and soft tissue injection. Injecting with confidence, 2nd edition. London: Radcliffe Medical Press, 1999. 43. Gotzsche PC. Non-steroidal anti-inflammatory drugs. BMJ 2000; 320: 1058-61. 44. British National Formulary. Non-steroidal antiinflammatory drugs. 2001, 41: Section 10.1.1. Online. Available: http://BNF.org 45. Silverstein FE, Faich G, Goldstein JL et al. Gastrointestinal toxicity with celecoxib vs. nonsteroidal anti-inflammatory drugs for osteoarthritis and rheumatoid arthritis. The CLASS study: a randomised controlled trial. JAMA 2000; 284: 1247-55. 46. Johnson AG, Nguyen TV, Day RO. Do nonsteroidal anti-inflammatory drugs affect blood pressure: a meta-analysis. Ann Intern Med 1994,121: 289-300. 47. Anon. GI, renal and cardiac AEs of NSAIDs. Bandolier 2000; 79: 6. Online. Available: www.jr2.ox.ac.uk/bandolier/index.html 48. Henry D, Lim LL, Garcia Rodriguez LA et al. Variability in risk of gastrointestinal complications with individual non-steroidal anti-inflammatory drugs: results of a collaborative meta-analysis. BMJ 1996; 312:1563-6. 49. Anon. Cox-2 roundup. Bandolier 2000; 75. 50. Rostom A, Wells G, Tugwell P et al. Prevention of NSAID-induced gastroduodenal ulcers (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 51. The diagnosis and management of anaphylaxis. / Allergy Clin Immunol 1998; 101 (6 Pt 2): S465-S528. 52. NICE. Cox II selective inhibitors, celecoxib, rofecoxib, meloxicam, and etodolac for osteoarthritis and rheumatoid arthritis. Technology Appraisal Guidance No. 27, London: NICE, July 2001. 53. Eccles M, Freemantle N, Mason J. North of England evidence based guideline development project: summary guideline for non-steroidal anti-inflammatory drugs versus basic analgesia in treating the pain of degenerative arthritis. BMJ 1998; 317: 526-30. 54. British National Formulary. Non-steroidal anti-inflammatory drugs. 2001, 42: Section 10.1.1. Online. Available: http://BNF.org 55. PRODIGY. Gout, clinical recommendations: 1999. Available on www.prodigy.nhs.uk 56. Ralston S, Capell H, Sturrock R. Alcohol and response to treatment of gout. BMJ 1988; 296: 1641-2. 57. Egsmose C, Lund B, Borg G et al. Patients with rheumatoid arthritis benefit from early second line

58. 59.

60. 61.

62. 63. 64. 65. 66.

67. 68. 69. 70.

71. 72.

73.

74. 75.

76.

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therapy: 5 year follow up of a prospective double blind placebo controlled study. / Rheumatol 1995; 22: 2208-13. Arnett FEA. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988; 31: 315-24. Scott D, Shipley M, Dawson A et al. The clinical management of rheumatoid arthritis and osteoarthritis: strategies for improving clinical effectiveness. Br J Rheumatol 1998; 37: 546-54. Royal College of Radiologists. Making the best use of a department of clinical radiology. London: RCP, 1998. Calin A, Elswood J, Klouda P. Destructive arthritis, rheumatoid factor HLA DR4: susceptibility versus severity: a case-control study. Arthritis Rheum 1989; 32: 1221-5. Brooks P, Day R. Non-steroidal anti-inflammatory drugs: differences and similarities. New Engl J Med 1991; 324: 1716-25. British National Formulary. Antimetabolites. 2001; 42: Section 8.1.3. Online. Available: http://BNF.org Thurtle O. Whose guidelines should you follow? Musculoskel Med 1993; 3: 4. PRODIGY. Rheumatoid arthritis, clinical recommendations: 1999. Available on www.prodigy.nhs.uk American College of Rheumatology. Guidelines for the management of rheumatoid arthritis 1996. Online. Available: www.rheumatology.org (choose 'publications' then 'treatment guidelines'). Palinkas L, Kabongo M. The use of complementary and alternative medicine by primary care patients. Practice 2000; 49: 1121-30. Fortin P, Lew R, Liang M et al. Validation of a meta-analysis: the effects of fish oil in rheumatoid arthritis. / Clin Epidemiol 1995; 48: 1379-90. Jacobs JC. Spondyloarthritis and enthesopathy. Current concepts in rheumatology. Arch Intern Med 1983; 143: 103-7. Van den Hoogen HMM, Koes BW, Van Eijk JTM, Bouter LM. On the accuracy of history, physical examination, and erythrocyte sedimentation rate in diagnosing low back pain in general practice: a criteria-based review of the literature. Spine. 1995; 20: 318-27. Calin A, Porta J, Fries JF, Schurman DJ. Clinical history as a screening test for ankylosing spondylitis. JAMA 1977; 237: 2613^. Kraag G et al. The effects of comprehensive home physiotherapy and supervision on patients with ankylosing spondylitis. A randomised controlled trial. Rheumatol 1990; 17: 228-33. Koh WH, Pande I, Samuels A et al. Low dose amitriptyline in ankylosing spondylitis: a short term, double blind, placebo controlled study. / Rheumatol 1997; 24: 2158-61. Maugars Y et al. Assessment of the efficacy of sacroiliac corticosteroid injections in spondyloarthropathies: a double blind study. Br J Rheumatol 1996; 35: 767-70. Clegg DO, Reda DJ et al. Comparison of sulfasalazine and placebo in the treatment of ankylosing spondylitis. A Department of Veterans Affairs Cooperative Study. Arthritis Rheum 1996; 39: 2004-12. Creemers MC, Franssen MJ, Van de Putte LB et al. Methotrexate in ankylosing spondylitis: an open study. / Rheumatol 1996; 22: 1104-7.

188

RHEUMATOLOGICAL PROBLEMS

77. Royal College of Physicians and The Bone and Tooth Society of Great Britain. Osteoporosis: clinical guidelines for prevention and treatment. Update on pharmacological interventions and an algorithm for management. London: RCP, 2000. Online. Available: www.rcplondon.ac.uk 78. Close ], Ellis M, Hooper REA. Prevention of falls in the elderly trial (PROFET: a randomised controlled trial). Lancet 1999; 353: 93-7. 79. Ernst E. Exercise for female osteoporosis. A systematic review of randomised clinical trials. Sports Medicine 1998; 25: 359-68. 80. National Osteoporosis Society. The prevention and management of corticosteroid induced osteoporosis. Bath: NOS, 1998. 81. British National Formulary. Drugs affecting bone metabolism. 2001, 42: Section 6.6. Online. Available: http://BNF.org 82. Fountain G, Hazleman B. Polymyalgia rheumatica and giant cell arteritis. BMJ 1995; 310: 1057-9. 83. Bahlas A, Ramos-Remos C, Davis P. Clinical outcome of 149 patients with polymyalgia rheumatica and giant cell arteritis. / Rheumatol 1998; 25: 99-104. 84. Bird H, Esselinckx W, Dixon ASJ et al. An evaluation of criteria for polymyalgia rheumatica. Ann Rheum Dis 1979; 38: 434-9. 85. Kyle V, Hazleman B. Treatment of polymyalgia rheumatica and giant cell arteritis. I. Steroid regimens in the first two months. Ann Rheum Dis 1989; 48: 658-61.

86. Kyle V, Hazleman B. Clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment. Ann Rheum Dis 1993; 52: 847-50. 87. Kyle V, Hazleman B. Treatment of polymyalgia rheumatica and giant cell arteritis. II. Relation between steroid dose and steroid-associated side effects. Ann Rheum Dis 1989; 48: 662-6. 88. PRODIGY. Polymyalgia, clinical recommendations: 1999. Available on www.prodigy.nhs.uk 89. Birkhead N, Wagener H, Shick R. Treatment of temporal arteritis with adrenal corticosteroids: results in 55 cases in which the lesion was proven at biopsy. / Am Med Assoc 1957; 163: 821-7. 90. Wolfe F. The relation between tender points and fibromyalgia symptom variables: evidence that fibromyalgia is not a discrete disorder in the clinic. Ann Rheum Dis 1997; 56: 268-71. 91. Makela M. Is fibromyalgia a distinct clinical entity? The epidemiologist's evidence. Bailliere's Best Pract Res Clin Rheumatol 1999; 13: 415-19. 92. Gowans S, deHueck A, Voss S et al. A randomized, controlled trial of exercise and education for individuals with fibromyalgia. Arthritis Care Res 1999; 12: 120-8. 93. O'Malley P, Balden E, Tomkins G et al. Treatment of fibromyalgia with antidepressants: a meta-analysis. / Gen Intern Med 2000; 15: 659-66.

10

CHAPTER CONTENTS Migraine 189 Menstrual migraine 191 Cluster headaches 191

Neurological problems

Non-migrainous headache 191 Tension-type headache 191 Chronic paroxysmal hemicrania 192 Exertional and coital headache 192 Thunder-clap' headache 192 Raised intracranial pressure 192 Epilepsy

192

Parkinson's disease 196 Stroke 198 TIA and minor stroke

200

Motor neurone disease Multiple sclerosis

202

202

MIGRAINE

Paraplegia 204 Essential tremor References 205

205

Guidelines: British Association for the Study of Headache. Guidelines for all doctors in the diagnosis and management of migraine and tension-type headache. March 1999. Available from the British Association for the Study of Headache, Wood Dene, Stanton Lees, Mr Matlock, Derbyshire DE4 2LQ www.bash.org.uk William EM et al. Guidelines for the diagnosis and management of migraine in clinical practice. Can Med Assoc J1997; 156: 1273-87.

Treatment of the acute attack Standard advice for every attack

(a) Advise patients to remove themselves to a quiet place and sleep if possible. Consider giving a benzodiazepine if sleeping has helped in the past. (b) Give: dispersible aspirin 900 mg or soluble paracetamol 1 g, or ibuprofen 400 mg, at the first sign of the attack; and metoclopramide lOmg or domperidone 10 mg orally or a 30 mg suppository. This will reduce nausea and lead to quicker absorption of analgesics. Do not use codeine or other opioids: they tend to worsen nausea and are more prone to lead to analgesic-overuse headache. Severe attacks

* Give diclofenac lOOmg suppositories and domperidone 30 mg suppositories. 189

190

NEUROLOGICAL PROBLEMS

* Consider a 5-HTj agonist, whether by tablet, wafer, nasal spray or self-administered injection. The tablets or spray will abort 70% of attacks at whatever stage they are given,1 the injection 85%.2 They may be less effective during an aura.3 If one 5-HTx agonist fails or causes side-effects, consider a trial of another. * Hold ergotamine in reserve for use only if the above fail and observe the restrictions on its use listed in the BNF. Oral ergotamine is ineffective, being almost entirely removed by first-pass metabolism. The benefits of ergotamine are usually outweighed by its problems: side-effects are common (20%), especially nausea and abdominal pain; overuse leads to headache, which is only relieved by taking further ergotamine; it should not be used in those with vascular disease, nor in those on propranolol or methysergide, nor if sumatriptan has been taken in the previous 6 hours. If the doctor is called * Administer either a 5-HTi agonist (e.g. sumatriptan sc) unless two injections have already been given in the last 24 hours or one dose in the last hour; or diclofenac 75 mg im plus metoclopramide 10 mg im or prochlorperazine 12.5 mg im for vomiting. * Admit the patient with a prolonged attack who is becoming dehydrated.

Prevention * Encourage the patient to discover the triggers of an attack and see how they can be altered, e.g. stress, excitement, certain foods, hunger, fatigue, exertion, oversleeping and coitus. * Stop combined oral contraceptives (COCs) in a woman whose migraine starts or worsens when taking them, especially if focal symptoms develop. Their use is associated with an increased risk of cerebral thrombosis. If stopping the COC is unacceptable, give low-dose aspirin daily, and use the lowest oestrogen pill acceptable. * Consider prophylaxis in anyone whose life is sufficiently disturbed by attacks.

Self-help group: Migraine Action Association, Unit 6, Oakley Hay Lodge Business Park, Great Folds Road, Great Oakley, Northants NN18 9AS, tel. 01536 461333; www.migraine.org.uk

Prophylaxis Three or more attacks per month is the usual criterion for prophylaxis, but a patient with severe attacks may choose prophylaxis with far fewer than that. * Explain that most of the prophylactic drugs have the same efficacy, reducing the frequency of severity of attacks by about 50%, but that individual patients respond to some and not others so a trial of drugs will be necessary. * Continue a drug that is effective for at least 6 months, and tail off slowly (except methysergide, see below). Try each for 2 months before abandoning it. Ask the patient to monitor attacks with a diary. * Use only one prophylactic drug at a time, except as an extreme measure. (a) Beta-blockers without intrinsic sympathomimetic activity (ISA). Propranolol, atenolol, metoprolol, nadolol and timolol. Start at a low dose, e.g. propranolol 10 mg t.d.s., and increase monthly up to 160 mg daily. A patient may fail to respond to one beta-blocker but respond to another. (b) Pizotifen helps 60% of patients, but side-effects of sleepiness and weight gain are common. Start at 1.5mg at night. If tolerance develops, increase the dose. (c) NSAIDs. Aspirin 75-500 mg daily helps up to 30% of patients.4 Ibuprofen, naproxen and flurbiprofen may help about 50%,5 but are not appropriate for long-term use; the risk of side-effects does not abate with time. (d) Calcium channel blockers. Nifedipine, verapamil and diltiazem are effective in conventional doses but not licensed for use in migraine. (e) Tricyclic antidepressants, e.g. amitriptyline 10-75mg at night, may be helpful and are especially useful combined with propranolol in patients with a combination of migraine and tension headaches.

NON-MIGRAINOUS HEADACHE

(f) Sodium valproate may be helpful although unlicensed for use in migraine. Start at 200 mg b.d. (g) Methysergide still has a role where all other prophylaxis has failed, but it should be given under specialist supervision for 4 months at a time with 1 month between courses to reduce the risk of retroperitoneal fibrosis. (h) Feverfew 200 mg daily may help,6 although not prescribable. Benefit only occurs after 6 weeks of use. Once started, patients should always buy the same product, because the constituents are not standardized. (i) Acupuncture but may help, although the evidence is not convincing.7

MENSTRUAL MIGRAINE * NSAIDs, e.g. naproxen 250-500 mg b.d., starting at the onset of bleeding. * Percutaneous oestrogen, e.g. Estraderm TTS patch (an unlicensed use) applied 2 days before the migraine is likely to start and left on for 4 days. Start with the 50 juig patch and adjust the strength up or down according to the response. Do not use oestrogens in women with focal migraine. * Women already on the COC. Consider the tricycle regimen (see p. 230).

CLUSTER HEADACHES (MIGRAINOUS NEURALGIA) * 5-HT^ agonists will abort three-quarters of all attacks within 15 minutes.8 * Ergotamine (see p. 190) taken half an hour before an attack is due will prevent some attacks. * Verapamil 20 mg t.d.s. will stop two-thirds of episodes once attacks begin. If it fails, use a betablocker or pizotifen. * Methysergide may be used more readily than in migraine because it will be needed for 3 months or less. * Lithium carbonate is probably the most effective prophylactic, and should be used as for manicdepressive illness (see p. 308). * Prednisolone 40 mg daily for 5 days then reducing to the lowest dose that will control attacks is justified if other prophylactic treatments fail.

191

NON-MIGRAINOUS HEADACHE • A history and simple examination (BP, fundi and a search for focal neurological signs) permit a clinical diagnosis in most patients. An ESR may be needed in patients over 50 to exclude giant cell arteritis. Cervical spondylosis, sinusitis, intracranial haemorrhage and other conditions of which headache is a symptom will need treatment in their own right. • Analgesic headache may complicate management, and is most likely to occur in patients taking analgesics combined with benzodiazepines or opioids such as dextropropoxyphene. Analgesics for headaches should not be taken on more than 15 days per month.9

TENSION-TYPE HEADACHE Guideline: DTB. Management of tension-type headache. Drug Ther Bull 1999; 37: 41-4.

* Exclude conditions which can cause secondary tension headache, e.g. cervical spondylosis, poor neck posture, temporomandibular (TM) joint dysfunction, sinus pain or eye muscle disorders. * Explain that the condition is real and benign. * Explore the tensions in the patient's life. * Advise relaxation techniques: yoga, massage, 'time-out' from a stressful day or more formal stress management.10 * Assess whether depression is present. * Check whether medication overuse is contributing to the headache. * Recommend paracetamol or aspirin over the counter. * Prescribe: (a) an NSAID, e.g. ibuprofen 400 mg t.d.s. if needed, but for no more than 3 days per week; (b) a tricyclic antidepressant, e.g. amitriptyline 10-75mg at night; or (c) propranolol 10^0 mg t.d.s.

192

NEUROLOGICAL PROBLEMS

CHRONIC PAROXYSMAL HEMICRANIA These are repeated attacks of unilateral headache lasting less than 45 minutes. * NSAIDs, e.g. indomethacin 25 mg t.d.s. reducing to 25 mg daily, will prevent most attacks.

EXERTIONAL AND COITAL HEADACHE * Give an NSAID or propranolol before attacks once a pattern is established.

THUNDER-CLAP' HEADACHE * Refer urgently if the first attack is sufficiently severe to present acutely. It may be a subarachnoid haemorrhage, presaging a more severe bleed.

RAISED INTRACRANIAL PRESSURE This is rarely the cause of headache in a patient without vomiting, papilloedema or neurological signs. However, exceptions occur. * Refer patients with a short history of headache (less than 4 months), especially if it is localized, worse on waking, worse on coughing or straining, and especially in the older patient.11

2. Prevent onlookers from restraining the fitting patient. 3. Do not give drugs initially - the fit is likely to have stopped before they can act. Give drugs if the fit is continuing without signs of abating after 5 minutes. 4. Admit any patient with a fit if there is suspicion that the fit is secondary to other illness; if the patient fails to recover completely after the fit (other than feeling sleepy); or if there is status epilepticus. Status epilepticus exists if more than one seizure occurs without the patient regaining consciousness, or if the fitting continues for over 30 minutes; 5 minutes of fitting is a more reasonable guide to when drugs should be given. 1. Give lorazepam 4mg iv slowly, and repeat every 15 minutes until the fits are controlled. Diazepam lOmg may be given iv or rectally, but is probably less effective.12 iv administration by paramedics (using half those doses) can reduce respiratory or circulatory complications from 20%, with placebo, to 10% with the benzodiazepine. 2. Check the blood sugar with a test strip. 3. Arrange immediate admission even if the fits are controlled - the patient may need other measures to prevent cerebral oedema.

Subsequent management EPILEPSY Guidelines: British Epilepsy Association. Principles of epilepsy management 1999. Online. Available: www.epilepsy.org.uk Crawford P, Appleton R, Betts T et al. Best practice guidelines for the management of women with epilepsy. Se/zt/re1999;8:201-17. MeReC. The treatment of epilepsy parts 1 and 2. MeReC Bull 1995; 6:17-20, 25-8.

Management of a major fit 1. Turn the patient into the coma position and ensure that the airway is not obstructed.

• A single fit is not necessarily epilepsy. It is only followed by recurrence in 40% of patients over 15. If the EEG is normal, the chance of further fits is only about 10% and does not warrant anticonvulsants.13 • Refer for neurological assessment, to be seen within 4 weeks, because of the need to exclude an underlying cause and because of the implications for work and driving. Refer more urgently if there are multiple seizures or focal neurological signs. The only patient who need not be referred is a child aged 18 months to 5 years who has recovered promptly from a febrile convulsion. • Discuss with the specialist the need to start an antiepileptic drug before the patient has been seen if there has been a previous fit in the

EPILEPSY

preceding 12 months or if the waiting list is longer than 4 weeks. * Once the diagnosis of epilepsy has been made and a decision taken about drug treatment, the GP is best placed to take over management, calling on a specialist for advice if in difficulties. Follow-up must, however, be structured, and defaulters sought out. Repeat prescriptions of drugs and a policy of waiting until a patient complains of problems are inadequate.14 * Re-referral is needed where: (a) control is poor or the drugs are causing side-effects; (b) seizures have continued for 5 years; (c) there are pointers to a previously unsuspected cause for the fits; (d) concurrent illness complicates management; or (e) the patient needs preconceptual advice.

Initial management * Find out how much the patient and his or her family understand about epilepsy, and answer their questions. Issues to discuss are: (a) Heredity. If one parent has epilepsy the risk for a child is increased from 0.5-1% to 4%. If both parents have epilepsy the risk increases to 15-20%.15 (b) Underlying pathology. It is not usually evidence of a brain tumour. Only 5% of those having their first fit over 21 years old have cerebral pathology. For those aged 45-55 it is still only 10%. (c) Prognosis. In 80% of patients with epilepsy, fits can be controlled by drug treatment and most of those patients need drug treatment for less than 5 years.16 * Acknowledge the distress and anger the patient and family feel at the disruption this diagnosis has brought to their lives. * Driving. Advise the patient of the need to notify the DVLA and the insurance company of any seizure, however minor. Document this advice. A licence is likely to be withdrawn until: (a) free from fits for 1 year; or (b) if the patient's fits in the last year have been while asleep and the patient has had fits

193

while asleep for >3 years with no fits while awake in that time (see p. 8). Fits at the time of waking or falling asleep count as 'daytime' fits. * Employment. Advise the patient not to work at heights or near dangerous machinery. An HGV or PSV licence will be lost until fit free for 10 years. * Lifestyle. Stress the positive side of how few changes there need to be. Swimming is possible provided someone else is present who could lifesave if necessary. Cycling in traffic is probably unwise. Counsel the patient about disclosing the diagnosis to friends and employers. It appears that more than half do not tell their fiancees.

Self-help groups: Epilepsy Action, New Anstey House, Gate Way Drive, Yeadon, Leeds LS19 7XY www.epilepsy.org.uk, helpline 0808 800 5050 The National Society for Epilepsy, Chalfont St Peter, Bucks SL9 ORJ, tel. 01494 601400 for details of local groups and also as an excellent source of information, www.epilepsynse.org.uk

Drug treatment • The British Epilepsy Association recommends prescribing by brand name because of evidence that generic prescribing leads to patients being switched between brands, and this carries a 10% risk of a worsening of seizure control.17 • Most patients are treated with one of three firstline drugs: sodium valproate, carbamazepine or phenytoin. In most types of seizure they are equally effective and the choice is made on their side-effect profile. Sodium valproate is first choice in primary generalized, absence and myoclonic seizures, while it is first equal with carbamazepine in partial seizures. • Phenytoin is very much third choice because of its narrow therapeutic window and the frequency of side-effects.18 Phenobarbitone is rarely used because adverse effects are greater.19 • If control is not achieved with a single firstline drug, a newer add-on drug is given as well.

194

NEUROLOGICAL PROBLEMS

Procedure 1. Start at a low dose as follows, and increase until fits are controlled or side-effects occur: (a) Carbamazepine 100 mg daily, increasing every week by lOOmg daily to a maximum of 600 mg b.d. Doses up to 2000 mg a day can be used with consultant advice. (b) Sodium valproate 300 mg b.d. increasing by 200 mg daily every 3 days to 1 g b.d. Doses up to 2500 mg daily can be used with consultant advice. (c) Phenytoin 200 mg daily, increasing every week by 100 mg daily to 600 mg a day. A blood level should be performed 1 week after each change of dose once 300 mg a day has been reached because of the narrow therapeutic window. 2. If the control of fits is unsatisfactory despite the prescription of adequate doses: check compliance; if using carbamazepine, check the blood level. If it is below the therapeutic range, check compliance again. If it seems satisfactory, continue to increase the dose with repeat blood levels; if using sodium valproate, do not check blood levels; they are not a guide to efficacy. 3. Abandon a drug if control is poor despite maximum dosage, but only after a sufficiently long trial (e.g. 2 months at full dosage), or, if fits are infrequent, the time in which three to five fits would be expected to occur.20 4. Tail-off one drug only when the patient is established on the next. 5. Only if some, but not enough, benefit has been obtained from the first drug should it be continued as well as the second. Even then a single firstline drug plus a newer add-on drug is probably better. Practical points * Phenytoin can be given once daily, and sodium valproate and carbamazepine (unless given in the slow-release form) twice a day. * Monotherapy. Use one drug at a time if possible. The addition of a second drug may only give a further 10% of control. * Compliance. Explain the importance of not missing doses and, especially, of not stopping

treatment abruptly. Poor compliance may be a sign that the patient does not fully accept the diagnosis. * Alcohol. Explain that moderate drinking of 1 to 3.5 units per day twice a week has no effect on seizure control.21 1 to 2 units a day is probably safe. Heavier drinking may induce a fit as well as interact with antiepileptic drugs. * Aspirin. Warn patients taking sodium valproate against taking intermittant aspirin. It displaces valproate from protein-binding sites and so potentiates it. If regular aspirin is needed, the dose of valproate may need to be reduced to allow for this. * Pregnancy. (a) Warn women of child-bearing age to seek advice about the use of their drugs before trying to become pregnant. No drug seems any safer than any other.22 The principle is to maintain a woman who needs drugs on a single drug at the lowest effective dose. Dose adjustment may be needed; absorption may be poor and the drug will be diluted by the increased blood volume (see p. 267). If a woman gets pregnant unintentionally, she should not stop treatment. Any damage will already have been done, and she merely risks losing her baby if she subsequently fits. (b) Women taking sodium valproate or carbamazepine should be offered mid-trimester screening for neural tube defects. (c) Advise women to take folic acid 5 mg daily before trying to conceive and throughout pregnancy. (d) Give vitamin K in the last month of pregnancy and to the newborn. Antiepileptic drugs can increase the risk of haemorrhagic disease of the newborn. * Contraception. Remember that women taking phenytoin, phenobarbitone or carbamazepine need, if requiring COC, either an increased oestrogen dosage (see p. 230) or a change to sodium valproate. Measure the blood progesterone on day 21 to confirm that ovulation has been suppressed. Doses of oestrogen of up to 100 |jig may be needed. Three packs may be run into one, without a break. If the patient is using the progestogen-only pill, the dose should be doubled or the method changed.

EPILEPSY

* Short-lived exacerbations of epilepsy. Clobazam is useful to tide patients over times when fits are more likely, e.g. menstruation and tiredness. It can be used to control fits occurring in clusters. Tolerance prohibits longer-term use. * Add-on drugs. Vigabatrin, lamotrigine, gabapentin, tiagabine, topiramate and zonisamide may be initiated by a specialist. They seem to be equally effective.23 The GP needs to know that: (a) they do not interfere with oral contraceptives; (b) their half-life may be affected by other anticonvulsants, e.g. sodium valproate prolongs the half-life of lamotrigine. Conversely, lamotrigine can precipitate carbamazepine toxicity, topiramate can precipitate phenytoin toxicity, while vigabatrin can lower phenytoin concentrations.

Follow-up (a) Review any fits (preferably recorded on a chart) and their precipitating causes. (b) Review the patient's attitude to the epilepsy and any social problems that have arisen. Encourage the family to attend, whether the patient is an adult or a child. The epilepsy may put other members of the family under considerable stress. (c) Review drug compliance, by checking the frequency of repeat prescriptions. (d) Review side-effects. (e) Do not check anticonvulsant serum levels routinely except with doses of phenytoin above 300 mg a day. The serum level should not determine the anticonvulsant dose; the correct dose is the smallest dose that controls the seizures. With the exception of phenytoin, only check serum levels when control is poor, there is concern about toxicity, higher than usual doses are being considered, more than one drug is being given, compliance is in doubt, or in renal or hepatic impairment.24 (f) Blood tests. It is traditional to recommend yearly checks of FBC, calcium, phosphate and alkaline phosphatase, advice which is traditionally ignored. Abnormalities are uncommon and minor and only worth testing for inpatients

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on high doses. The one potentially fatal sideeffect, hepatotoxity due to sodium valproate, occurs in only 1 in 10,000, mainly in mentally handicapped children under 3 years old, and routine testing would not predict it anyway. Tests of serum electrolytes in patients on carbamazepine in order to detect hyponatraemia may be worthwhile.25

Stopping treatment • Specialist advice is wise before withdrawing drugs in a patient who has been free from fits for a number of years. Ultimately it is the patient who must make the decision, weighing the problems of drug-taking against the upset of a further fit with its implications for driving, employment and family distress. Patients who stop their drugs and remain fit-free have higher morale than those who are fit-free on drugs.26 • Withdrawal may be considered in grand mal when the patient has been free of fits for 2 years, although there is no evidence on which to base this.27 It is likely to be followed by relapse in half of adults and 20% of children in the first year, against a recurrence rate of 33% for adults who remained on medication. Recurrence does not worsen the overall prognosis.28 • Withdrawal is most likely to be successful in patients with: (a) primary generalized epilepsy; (b) epilepsy controlled on a single drug with no seizures on treatment; (c) a normal EEC; (d) a few seizures over a short space of time; (e) childhood or teenage onset; (f) no family history of mental retardation or cerebral pathology.29 • Maximum dose reduction. In adults the maximum reduction each time should be: carbamazepine 200 mg; phenytoin 50 mg; sodium valproate 200 mg; phenobarbitone 30 mg; primidone 125 mg. • Speed of withdrawal. Withdrawal should take at least 6 months. If a drug is being withdrawn and being replaced by another it can be withdrawn faster, but in steps not shorter than one every 3 weeks.

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PARKINSON'S DISEASE Guideline: The Primary Care Task Force of the Parkinson's Disease Society (UK). Parkinson's aware in primary care. 1999. Online. Available: www.parkinsons.org.uk

• The disease should be confirmed by a specialist in all cases; 25% of referrals to a Parkinson's disease clinic were found not to have Parkinson's disease.30 • 'Parkinsonism', whether due to drugs, multiple small strokes or other causes, does not respond to treatment with levodopa and must not be confused with Parkinson's disease.

Management The diagnosis of Parkinson's disease has huge medical, psychological and social implications for the patient and family. The key worker, who may be a GP, specialist nurse or consultant, is needed to coordinate the professionals involved. The medical aspects of care will be shared between specialist and GP. While the specialist will initiate treatment, the GP is often responsible for coping with adverse effects or altering drug doses. * Find out what specific problems the patient has. Treatment should be individually tailored to these problems. * Explain the diagnosis and the management plan. Stress that almost everybody enjoys prolonged benefit from treatment. * Explain the problem that lies at the heart of drug treatment: that levodopa is the most effective drug for the relief of disability but that prolonged use leads to the problems described below. Delaying its use and keeping its dose low may mean that it will remain effective later in the disease: hence the use of other agents early, and of levodopa-sparing agents later. * Depression. Use antidepressants as you would in other depressed patients. Depression occurs in

almost half of patients and is part of the disease, not merely a reaction to the disability. * Dementia occurs in 10%, and is likely to be missed unless specifically tested for. Its discovery has important implications in planning treatment. * Constipation should be treated by encouraging mobility, a high-fibre diet if the patient can swallow it, and laxatives. * Physiotherapy may be helpful though the evidence is sparse.31 One meta-analysis found that physiotherapy could improve walking speed and the activities of daily living.32 * Occupational therapy can help with difficulties with feeding and dressing, and provide alterations to the bed, chair and bath although, again, the evidence is sparse.33 Difficulties with speech and swallowing. Referral to speech therapy may help to make the voice more forceful.34 There is no evidence either way on benefit for dysphagia. * Financial benefits. Many patients are eligible for benefits for the disabled (see p. 4). * Carers. Assess, and reassess, the ability of the carer to cope. * Driving. Advise drivers to notify the DVLA and the insurance company. * Surgery. A few patients will be candidates for destructive surgery. They are likely to be those under 70, with no other cerebral disease, who have severe unilateral tremor or dyskinesia. Self-help group: The Parkinson's Disease Society, 215 Vauxhall Bridge Road, London SW1V 1EJ, tel. 020 7931 8080 has information for patients and carers and organizes local groups. Helpline: 0808 800 0303; www.parkinsons.org.uk

Drug treatment Choice and timing of drug therapy depends on the age of the patient, because of the limited time for which levodopa is likely to be effective. The approach for patients under 50 and over 70 is fairly clear. Patients between the ages of 50 and 70 are in a borderline area where individual circumstances will influence the decision.

PARKINSON'S DISEASE

The younger patient Start drugs only when they are needed for symptom control. Options are: (a) Anticholinergics, which may be given especially for tremor. They are very prone to cause confusion in the elderly and in these patients the only strong indication for them is excessive salivation. A convenient choice is benzhexol 2mg daily increasing up to a maximum of 5mg t.d.s., or orphenadrine 50-100 mg t.d.s. If the main side-effect is a dry mouth, take before food; If the main side-effect is nausea, take after food. (b) Amantadine at a dose of 100 mg b.d. is of modest benefit but has few side-effects. (c) Dopamine agonists: bromocriptine, pergolide, lysuride or one of the newer drugs can all relieve symptoms. Initial treatment with bromocriptine rather than levodopa appears to be slightly less effective in reducing disability but with fewer motor complications.35 Dopamine agonists are very likely to cause initial nausea or vomiting and domperidone may be needed. (d) Selegiline postpones the time when levodopa is needed.36 Earlier hopes that this was due to a neuroprotective effect are probably unfounded,37 and one trial has raised the possibility that it causes an increase in mortality.38 (e) Levodopa (see below) should be started when symptoms interfere sufficiently with the patient's life, despite the above. The older patient * Levodopa. Start levodopa when symptoms are interfering significantly with the patient's life. Not only is there less need to 'save' levodopa for later, but also the levodopa-sparing drugs are more likely to give rise to adverse effects in the elderly. (a) Warn patients about the immediate sideeffects, e.g. nausea and postural hypotension. Give the tablets after food to avoid nausea. Be aware, however, that a protein meal can compete with levodopa for absorption. If an antiemetic is needed, use domperidone. Check

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the standing blood pressure before and after starting the drug. (b) Use a preparation that combines levodopa with a decarboxylase inhibitor. (c) Agree on a specific goal that treatment should achieve, e.g. being able to walk to the shops. (d) Start with a low dose, containing levodopa 50 mg t.d.s., and increase by 50 mg a week until the goal has been achieved. (e) Monitor the response. As well as asking about the benefit, set the patient a task (e.g. unbuttoning a coat or walking to the door) and time it for an objective assessment. Stop increasing the dose as soon as sufficient benefit has been achieved. * Add amantadine, dopamine agonists or selegeline (see above) but not anticholinergics when levodopa alone is not sufficient. Later problems with levodopa These may not begin for some years after the drug is started: (a) end-of-dose deterioration; (b) dyskinesia, usually at peaks of levodopa levels but also as the dose wears off; (c) on-off motor fluctuations unrelated to levodopa dose; (d) nausea and vomiting; (e) psychiatric side-effects and acute confusion (see Psychiatric problems, below). * Management of motor complications: (a) Give a smaller amount more often to avoid the peaks and troughs of drug levels. In extreme cases, 2-hourly doses may be needed. Alternatively, change to a controlled-release preparation. (b) Add selegiline, which will improve end-ofdose deterioration in 50% of patients and permit a reduction in levodopa dose, which will help any dyskinesia. (c) Add a dopamine agonist, e.g. bromocriptine 1.25mg daily, increasing slowly as detailed in the BNF. Reduce the dose of levodopa by one-third. Confusion and postural hypotension

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often limit the value of this drug. One of the newer dopamine agonists may suit an individual patient better. (d) Arrange for admission for initiation of apomorphine injections which the patient or carer can then give at home. Apomorphine is useful in patients with fluctuating motor responses, on-off oscillations and dyskinesias. It works within 20 minutes, but benefit only lasts for about an hour.39 * Nausea and vomiting: (a) ensure that doses are taken after meals; (b) decrease the dose of levodopa while increasing the dose of decarboxylase inhibitor. The intention is to lower the extracerebral level of levodopa without lowering the intracerebral level; (c) add domperidone.

Psychiatric problems In addition to the difficulty that a patient is likely to have in adjusting to the condition, Parkinson's disease and its treatment cause a whole range of psychiatric disorders. • Levodopa can cause anxiety, agitation, elation, depression, insomnia, paranoia, hallucinations and delusions. Dose reduction is more effective than trying to treat the reaction with further drugs. • Depression may be part of the disease rather than a reaction to the disease. It responds to antidepressants. Choose a first generation tricyclic if anticholinergic action is required as well, e.g. in excessive salivation. If constipation is already a problem or the patient is already on an anticholinergic drug, choose one with less anticholinergic activity such as lofepramine or a selective serotonin reuptake inhibitor (SSRI). • Psychosis may be due to Parkinson's disease or to the medication. Withdraw the latter in the manner outlined below. If part of the disease, neuroleptics may be needed despite the detrimental effect they are likely to have on the parkinsonism. • Organic confusion is likely to be due to the medication. Stop the drug most recently added. If there has been no recent change, stop drugs in the following order: anticholinergics, selegiline,

amantadine and dopamine agonists. Levodopa is the drug least likely to cause confusion, but its dose may need to be altered. Palliative care of the terminal patient A time will come when no further alteration in drugs will be helpful, and the patient and family will need an increased amount of support. Adequate analgesia and sedation of the patient will be needed, as in any palliative situation.

STROKE Guidelines: The Intercollegiate Working Party on Stroke. National Clinical Guidelines for Stroke. London: The Royal College of Physicians, 2000. Available from the RCR 11 Saint Andrews Place, Regent's Park, London NW1 4LE; www.rcplondon.ac.uk/ceeu_stroke_ concise.htm Scottish Intercollegiate Guidelines Network (SIGN). Management of patients with stroke; Parts 1-4; Online. Available: www.sign.ac.uk/guidelines/ The National Service Framework for Older People. London: Department of Health, 2001. Online. Available: www.doh.gov.uk/nsf/olderpeople.htm

• Management of stroke patients in a stroke unit is associated with a sustained reduction in mortality40 and improvement in the quality of life.41 • Rehabilitation, physiotherapy, occupational therapy and psychological support are all part of well-organized stroke care. This can dramatically reduce mortality and disability.42 • Brain imaging by CT or MRI scanning should be undertaken to detect intracerebral or subarachnoid haemorrhage, and to exclude other causes of the stroke syndrome, in all patients within 48 hours of onset unless there are good clinical reasons for not doing so.43 • Thrombolysis in selected patients reduces the risk of death or deterioration after a stroke with an NNT of approximately 20.44 It is only suitable for patients who can be admitted to a specialist unit with experience of thrombolysis within 3, or at the outside, 6 hours of the onset of the stroke.45

STROKE

Acute stroke Admit all patients unless:^3 (a) the diagnosis is clear and examination allows the localization of the damage to one cerebral area; and (b) brain imaging can be arranged within 48 hours unless there is a good clinical reason not to do so; and (c) the diagnosis can be reviewed by an experienced clinician; and (d) cardiovascular status can be reviewed; and (e) care services can provide support within 24 hours; and (f) a specialist stroke service is part of those services. If managing the patient at home, the key action points (in liaison with the specialist stroke services) are: * Maintain the airway. * Ensure that hydration is maintained. * Explain the situation to the patient and carers and give support. * Give aspirin (300 mg) as soon as possible after the onset of stroke symptoms if a diagnosis of haemorrhage is considered unlikely (NNT = 77).45 * Assess the patient's cardiovascular state, including heart, carotid arteries and blood pressure. Do not lower blood pressure in the acute phase.46 * Assess the patient's neurological state including motor and sensory function regularly until stable. * Check swallowing. Can the patient swallow saliva? If so, can he or she swallow a teaspoon of water? Clinically detectable dysphagia occurs in about half of patients with first stroke47 and is often unrecognized.45 Patients with dysphagia need urgent assessment by a speech therapist. A nasogastric tube or gastrostomy may be needed to avoid aspiration pneumonia. * Control the blood glucose in diabetics. * Control pyrexia with paracetamol, fan and treat any underlying cause. * Monitor pressure areas if there is immobility. * Check urinary function and continence, and catheterize if necessary.

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* Apply a full length compression stocking to a weak or paralysed leg to prevent venous thrombosis.

Longer-term management * Early discharge is only appropriate if a specialist community stroke rehabilitation team is available. If so, early discharge may improve outcomes48 but at the risk of worsening the mental health of the carers.49 * Depression has been found in as many as half of patients 3 months after a stroke and at 12 months. The prevalence in carers is almost as high.50 A further 20% suffer from poststroke emotional lability.51 * Refer to a specialist rehabilitation team, if not already involved. This team will manage the rehabilitation process but may need support from the GP. * Check that a multidisciplinary assessment has been performed, including assessment of consciousness level, swallowing, pressure sore risk, nutritional status, cognitive impairment, movement and handling needs. * Check that carers and family have been involved closely with the planning of discharge from inpatient care. * Muscular spasm. Use antispasmodics as needed to prevent or treat spasticity, e.g. baclofen 10 mg up to four times daily or diazepam 2-1 Omg three times daily, but with caution because of the risk of sedation. * Depression. Monitor for depression with a screening questionnaire (see p. 309) giving information and advice, and considering antidepressant drug treatment (see p. 311). Tricyclic antidepressants and SSRIs are effective in major depression52 and emotional lability.51

Secondary prevention The risk of recurrence is 8% per year.53 * Lifestyle changes. Assist smokers to stop (see p. 331) and urge weight reduction, dietary change, and exercise where appropriate (see p. 108).

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* Aspirin. Prescribe 75 mg daily unless the stroke is likely to have been haemorrhagic or aspirin is contraindicated. In patients with a previous ischaemic stroke, it will prevent 39 events per 10,000 patients over a mean of 37 months but at the expense of 12 haemorrhagic strokes.54 * Blood pressure. Once the initial phase of the stroke is over (usually about 2 weeks), control any hypertension to < 140/85 or < 140/80 in diabetics (see p. 90). * Atrial fibrillation. Arrange anticoagulation with warfarin whether valvular disease is present or not. * ACE inhibition. Give ramipril (increasing to lOmg daily) in all in whom it is not contraindicated. It can reduce the risk of future stroke by 32% independent of its effect on BP (The HOPE Study 2002). * Co-incidental cardiac disease. Arrange echocardiography in patients with stroke and cardiac disease (other than atrial fibrillation). Almost 20% will be found to have an intracardiac source of an embolus.55 The commonest cardiac pathologies are mitral valve disease, prosthetic valves, left atrial enlargement and myocardial infarction in the last 3 months.43 * Raised cholesterol. Use a statin and diet to lower cholesterol as recommended in the prevention of coronary heart disease (see p. 109). Other routine matters * Explain the prognosis if requested (65% are likely to achieve independence). Those with a better prognosis are those who are continent, who have regained power on the affected side within 1 month and who are progressing towards walking within 6 weeks. Improvement is likely to continue for some months. * Driving. Explain that the patient should not drive for at least 1 month after a stroke or transient ischaemic attack (TIA) and that the DVLA and the insurance company should be informed. * Arrange relief admissions and other support for the carers (e.g. attendance allowance) in consultation with the stroke team.

Primary prevention Prevention should be targeted towards patients with one or more of these factors. * Hypertension. Control blood pressure to systolic blood pressure <140mmHg and diastolic blood pressure < 85 mmHg; the minimum accepted level of control recommended for audit purposes is <150/<90mmHg (see p. 90). A meta-analysis of 17 trials, in which the mean reduction in diastolic BP was 5-6 mmHg, gave an NNT of 20 to prevent one major vascular event in 5 years among older patients and an NNT of 60 for younger patients.56 * Atrial fibrillation. Advise the patient about anticoagulation or antiplatelet treatment according to risk57 (see p. 104). * Aspirin. Recommend aspirin in primary prevention only in high-risk patients (see p. 109).58 * Cholesterol. Assess the patient's risk from all occlusive arterial disease. The combined data suggest that treatment of raised cholesterol levels prevents stroke, in middle-aged persons.59 However, the benefit is small. Even in patients at high risk the NNT is 143 over 4 years.60 The patient is likely to benefit more from the reduction in coronary heart disease risk (see p. 108). * Alcohol. Advise the patient to keep to the national guidelines for alcohol consumption of <21 units per week for a male and <14 units per week for a female. Regular light-to-moderate consumption of alcohol seems to decrease the risk for ischaemic stroke by reducing atherothrombotic events.61 Heavy drinking or binge drinking can increase the risk of stroke.61 * Smoking. Encourage the patient to stop (see p. 331). Studies have found a dose-response relationship between the amount of cigarettes smoked per day and the relative risk of stroke.62 * Encourage lifestyle and behaviour modifications (including exercise) that are effective in the prevention of cardiovascular disease and stroke.63

TIA AND MINOR STROKE Review: Hennessy MJ, Britton TC. Transient ischaemic attacks: evaluation and management. Int J Clin Prac 2000; 54: 432-6.

STROKE

* A TIA is a sudden focal neurological disturbance lasting less than 24 hours. Minor strokes are events which last over 24 hours, but which carry the same prognostic significance as TIAs. The risk of stroke following either in the carotid artery territory is 11% in the first year and then 5% per year. * Investigation is urgent in the case of minor strokes, where CT scanning is needed to distinguish minor ischaemia from haemorrhagic stroke. Only in ischaemic strokes are further investigations useful. A CT scan must be done within 1 week of the event to distinguish ischaemia from haemorrhage in small strokes, although an MRI scan will provide useful information later than that.53 * Check that the symptoms are due to a TIA. Differentiate TIA from: (a) Transient cerebral symptoms caused by hypoperfusion due to cardiac disease. One survey suggests that nine out of ten TIAs diagnosed in general practice are not TIAs at all. (b) Cerebral tumour. Patients with sensory TIAs, jerking TIAs, loss of consciousness or speech arrest should be assumed to have a tumour until proved otherwise. (c) Epilepsy. Careful history of the attacks from an observer is important. Investigations include EEC and CT/MRI scanning. * Differentiate from the history between: (a) Vertebrobasilar attacks, characterized by homonymous hemianopia, bilateral transient blindness, diplopia, bilateral motor or sensory loss, or vertigo. (b) Carotid artery attacks, characterized by dysphasia, visual loss in one eye only, hemiparesis or hemianaesthesia. Ataxia, dysarthria and unilateral motor or sensory loss may be carotid or vertebrobasilar in origin. * Look for cardiovascular causes: (a) arrhythmia. This may require a 24 hour ECG; (b) valvular disease; (c) hyper- or hypotension; (d) carotid artery stenosis. Auscultate the neck for a bruit. The absence of a bruit does not, however, exclude carotid artery stenosis. The

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presence of a bruit on the other side should not lead to action. Treatment of asymptomatic bruit has not been shown to be worthwhile. There is insufficient evidence of benefit from surgery to asymptomatic stenoses to recommend it.64

Work-up for patients with TIA or minor stroke (a) Hb for polycythaemia; (b) ESR for giant cell arteritis; (c) blood sugar for hypoglycaemia and diabetes; (d) serum lipids; (e) U & Es; (f) CXR and ECG; (g) syphilitic serology, in those for whom it might be relevant.

* Refer urgently: (a) To an ophthalmologist in the first instance anyone with unilateral visual symptoms, unless the history is of classical amaurosis fugax. Glaucoma and other ocular pathology can mimic a TIA. (b) To a cardiologist if an arrhythmia or cardiac lesion may be the source of emboli. Anticoagulants are indicated in those in whom they are indicated anyway (see p. 104) but not otherwise. (c) To a neurologist if a carotid TIA or minor stroke occurs in someone fit enough to be considered for carotid artery surgery if a symptomatic stenosis is found. Over half will have a significant stenosis, as will almost all those with a bruit on the side of the TIA. Endarterectomy is indicated if the stenosis is over 70%, but should only be performed in an experienced unit with a perioperative complication rate of stroke or death of under 6%.65 Referral is worthwhile up to 6 months after the event. * Aspirin: give 75 mg daily to all unless contraindicated. This will reduce the subsequent incidence of stroke by 15%.66 One possible contraindication is a patient with a minor stroke who has not had a haemorrhage excluded by CT scan. In these patients, wait 3 weeks from the event before starting aspirin. * Prevention: Treat as for stroke. * Driving. Advise as for stroke.

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MOTOR NEURONE DISEASE * Refer every patient for confirmation of the diagnosis. * Explain what little is known about the disease. * Support the patient and family as you would in any terminal illness. The median survival is only 2-3 years, with older patients having the worse prognosis. * Discuss at an early stage with the patient and the family the fact that tracheostomy or ventilation may become necessary. There is often no time to discuss it with them during a crisis.67 * Refer for physiotherapy, occupational therapy, speech therapy for help with eating as well as speech, district nursing, health visitor and social work assistance as appropriate.

patient from respiratory failure with iv diazepam or morphine. Advice: The Motor Neurone Disease Association, PO Box 246, Northampton NN1 2PR, tel. 01604 250505; Helpline 08457 626262; www.mndassociation.org

MULTIPLE SCLEROSIS Guideline: The Multiple Sclerosis (MS) Society. Basics of best practice in the management of multiple sclerosis, July 1999. Available from the MS Society, 372 Edgware Road, London NW2 6ND.

The above guideline recommends the following among the 'basics of best practice':

Palliative care Reference: O'Brien T et al. ABC of palliative care: nonmalignant conditions. BMJ 1998; 316: 286-9.

* Musculoskeletal pain. Consider physiotherapy to relieve the stiffness associated with prolonged immobility. Treat with NSAIDs and opioids. Use opioids early rather than late in patients with pain, as well as to ease the distress of terminal respiratory failure.68 Treat muscle spasms with baclofen, dantrolene or diazepam. * Dribbling. Treat dribbling with anticholinergics, e.g. sublingual hyoscine 0.3 mg t.d.s. or hyoscine hydrobromide transdermal patches. * Consider referral for tracheostomy for sputum retention or stridor, or for recurrent aspiration of food or drink. * Nutrition. Consider referral for percutaneous endoscopic gastrostomy (PEG) tube feeding for patients who feel hungry, thirsty or suffer with dysphagia. * Respiratory failure. Consider referral for ventilatory support if the quality of life is otherwise sufficiently good. Be prepared to ease the death of a

(a) the diagnosis should be made by a neurologist; (b) an MS specialist nurse or other liaison professional should be available to provide additional support and advice; (c) a key worker should be identified who would coordinate services; (d) patients should have access to a multidisciplinary team whose composition would depend on the patient's particular needs. It may include physiotherapist, speech and occupational therapists, psychologist, continence adviser, dietician, orthotist, social worker and local MS support group and carers group.

A better prognosis is associated with: (a) Young age at onset; (b) Female gender; (c) A relapsing and remitting course; (d) Initial symptoms: sensory or optic neuritis; (e) First manifestations affecting one CNS region; (f) High degree of recovery from initial bout; (g) Longer interval between first relapses; (h) Low number of relapses in the first 2 years; (i) Less disability at 5 years after onset.

MULTIPLE SCLEROSIS

* Prognosis. Explain what is known about the disease and how good the prognosis is; 76% of MS patients are alive 25 years after the onset, which is 85% of that seen in age- and sex-matched controls. Point out the positive prognostic features (see box above) if they apply. * Relapses. For disabling attacks give prednisolone 60 mg daily for 1 week, tapered for 3 weeks. An alternative is methylprednisolone 500-1000 mg iv per day for 3 days, with or without a short tapering dose of oral corticosteroids. Steroids can hasten recovery, but there is no evidence that the long-term course is altered.69 Only oral steroids are feasible in primary care because the high-dose intravenous preparation should be given over 30 minutes. * Beta-interferon in patients with relapsing remitting MS, reduces the occurrence of exacerbations by 20% (RR = 0.8 (95% CI 0.73 to 0.88) and progression of the disease by 31% (RR = 0.69 (95% CI 0.55 to 0.87) 2 years after randomization.70 However, the non-inclusion of dropouts in the studies puts even this modest benefit in doubt; and evidence of benefit after 2 years is currently lacking. Evidence for glatiramer is less compelling.71 In the UK, under the 'risk-sharing scheme' set out in Health Service Circular 2002/004, the following patients are entitled to disease-modifying treatment (beta-interferon or glatiramer) at NHS expense as part of a costeffectiveness study: (a) those with relapse-remitting MS; and (b) those with secondary progressive MS in which relapses are the dominant feature, provided they meet the criteria of the Association of British Neurologists. Treatment is to be initiated at a specialist MS treatment centre. Those already on treatment may continue until they and their consultant agree it is time to stop. Clinicians may also prescribe the drugs at NHS expense if they deem it clinically necessary. Both drugs must be given by injection. * Mitoxantrone and other immunomodulatory drugs show some evidence of benefit but there are few trials of each drug, side-effects are frequent and no cost-effectiveness studies have been conducted.72

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* Spasticity. The evidence of benefit from therapy is poor.73 Only persevere with a treatment if that patient seems to be helped: 1. Begin with a stretching programme. 2. Add an evening dose of a benzodiazepine if extensor spasms and clonus interfere with sleep. 3. Use a skeletal muscle relaxant, e.g. baclofen, if daytime spasticity is troublesome, increasing the dose slowly to reduce side-effects. Up to lOOmg/day of baclofen may be required. Abrupt withdrawal of baclofen may result in hallucinations or seizures, making it necessary to taper doses. In paraplegic patients with severe spasticity and intolerance to the required oral dose, intrathecal baclofen delivered by a subcutaneously implanted pump allows a much smaller dose and is often effective in alleviating intractable spasticity and may lessen urinary urgency. * Fatigue. The treatment of fatigue is far from optimal, and medications are only partially effective. Amantadine at 100 mg twice per day is the standard initial treatment. * Paroxsymal symptoms: give carbamazepine starting with 100 mg b.d. * A hyperreflexic bladder: give an anticholinergic, e.g. oxybutynin (5mg b.d-q.d.s). * A flaccid bladder with symptoms suggesting retention of urine: perform a urinalysis, serum creatinine and a post-void residual urine measurement. Residuals in excess of 15 ml are abnormal. If they are above 50 ml, consider urological referral. Try oral distigmine but reassess bladder function periodically and recheck the residual urine if bladder function seems to change. Intermittent catheterization should be considered when the residual urine reaches 100ml. Avoid a chronic indwelling urinary catheter if at all possible. * Urinary infection: it is usually not necessary to use antibotics prophylactically in the prevention of urinary tract infections. Urinary calculi may be prevented by acidification of the urine with cranberry juice. * Constipation: can usually be managed with bulk laxatives and stool softeners. More severe constipation may require osmotic agents, bowel

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stimulants, anal stimulation, suppositories, or enemas. Bedridden patients may develop fecal impaction unresponsive to these measures and require manual disimpaction. Fecal incontinence may be minimized by adherence to a schedule for bowel movements. * Sexual problems: the precise nature of the sexual dysfunction will determine the treatment. Physical difficulty from spasticity may be alleviated by premedication with baclofen, and a fastacting anticholinergic such as oxybutynin may calm urinary urgency. Sexual dysfunction should not be automatically attributed to MS. It may be necessary to investigate hormonal levels and to obtain urological or gynaecological consultation. Manual lubrication with gel is a ready solution to vaginal dryness. Erectile dysfunction may be treated by sildenafil at NHS expense in the UK. If it fails, older treatments may succeed (vacuum devices, intracavernous injections or a penile implant; see p. 248). Self-help group: The Multiple Sclerosis (MS) Society, 372 Edgware Road, London NW2 6ND, tel. 020 8438 0700; National helpline 0808 800 8000; www. mssociety.org. uk

PARAPLEGIA Every patient with paraplegia will be under the care of a consultant. The GP, however, is likely to be the doctor to whom certain problems present. A proactive approach can make a difference to the patient's quality of life.

Spasticity Use established drugs (baclofen, dantrolene sodium, diazepam, clonidine) singly or in combination on a trial basis. The place of newer drugs (tizanidine, cannabinoids) is not yet clear. Attempts to control troublesome spasticity should not be abandoned without referral for consideration of intrathecal baclofen.74

Urinary tract infection * Always confirm suspected urinary tract infections with an MSU. * Do not give antibiotics if the patient is catheterised unless there are systemic symptoms or Proteus is grown (see p. 281).

Autonomic dysreflexia This is reflex sympathetic overactivity, giving rise to flushing and severe hypertension. It only occurs in patients with lesions above T6. The patient may present with pounding headache and profuse sweating and flushing or mottling above the level of the lesion. (a) Sit the patient up. (b) Remove the cause (e.g. distended bladder, UTI, loaded colon or anal fissure). (c) Give nifedipine 5-10 mg sublingually, or glyceryl trinitrate (GTN) 300 |JLg sublingually. (d) Admit urgently to hospital if not settling.

Pressure ulcers * Warn patients to make contact if a red patch does not improve within 24 hours after relieving the pressure on the area. * Treat sores or ulcers intensively, and admit early if not resolving.

Psychological aspects * Be aware of the fact that the patient may be suffering: (a) a severe grief response to the loss of function and independence; (b) a feeling of fear and vulnerability; (c) difficulties with their changing relationships with those around them. * Be prepared to raise the issue of sexuality. Avoid the temptation to think of the individual as asexual. Sexual function and fertility are possible with appropriate support. Recommend publications from the Spinal Injuries Association (see box below).

REFERENCES

Advice for patients and professionals: The Spinal Injuries Association, 76 St James' Lane, London N10 3DF, tel. 020 8444 2121; Information line 0800 980 0501; www.spinal.co.uk

ESSENTIAL TREMOR * Mild cases: patients may choose not to take any medication. Check that they are not exacerbating the tremor with, for instance, caffeine. * More severe cases: use: (a) propanolol, 30mg/day up to 160-320 mg/ day;75

205

(b) primidone, 62.5mg/day, up to Ig/day.76'77 It may be more effective than propranolol78 and can be combined with it; (c) gabapentin, 300mg/day, up to 1.2-3.6 g/ day;79 (d) alprazolam, initial dosing: 0.75mg/day, up to 2.75mg/day.80 Long-term use carries the risk of dependence. Note: Some of the higher doses quoted above are beyond the maximums for which the drugs are licensed in the UK. * Severe cases uncontrolled by medication: consider referral for neurosurgery, either electrode implantation or thalamotomy.81

REFERENCES 1. Ryan R et al. Sumatriptan nasal spray for the acute treatment of migraine. Neurology 1997; 49: 1225-30. 2. DTB. Sumatriptan: a new approach to migraine. Drug Ther Bull 1992; 30: 85-7. 3. Bates D et al. Subcutaneous Sumatriptan during the migrainous aura. Neurology 1994; 44: 1587-92. 4. Nelson-Piercy C, De Swiet M. Low-dose aspirin may be used in prophylaxis (letter). BMJ 1996; 313: 691. 5. Diamond S et al. Fenoprofen in the prophylaxis of migraine: a double-blind, placebo-controlled study. Headache 1987; 27(5): 246-9. 6. Pittler MH, Vogler BK, Ernst E. Feverfew for preventing migraine (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 7. Melchart D, Linde K, Fischer P et al. Acupuncture for idiopathic headache (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 8. The Sumatriptan Cluster Headache Study Group. Treatment of acute cluster headache with sumatriptan. New Engl J Med 1991; 325: 322-6. 9. Olesen J. Analgesic headache. BMJ 1995; 310: 479-80. 10. Holroyd KA, O'Donnell FJ, Stensland M et al. Management of chronic tension-type headache with tricyclic antidepressant medication, stress management therapy and their combination. JAMA 2001; 285: 2208-15. 11. Clough C. Non-migrainous headaches. BMJ 1989; 299: 70-2. 12. Alldredge BK, Gelb AM, Isaacs SM et al. A comparison of lorazepam, diazepam, and placebo for the treatment of out-of-hospital status epilepticus. New Eng J Med 2001; 345: 631-7. 13. van Donselaar CA et al. Idiopathic first seizure in adult life: who should be treated. BMJ 1991; 302: 620-3. 14. Specification for Epilepsy Services. Epilepsy Task Force, 1995. 15. O'Brien MD, Gilmour-White S. Epilepsy and pregnancy. BMJ 1993; 307: 492-5.

16. Anon. Changing view of prognosis of epilepsy. BMJ 1990; 301:1112-14. 17. Crawford P et al. Generic prescribing for epilepsy: is it safe? Seizure 1996; 5: 1-5. 18. DTB. Drug treatment of epilepsy. Drug Ther Bull 1994; 32: 45-8. 19. Taylor S, Tudur Smith C, Williamson PR et al. Phenobarbitone versus phenytoin monotherapy for partial onset seizures and generalised onset tonic-clonic seizures (Cochrane review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 20. Shorvon SD. Medical assessment and treatment of chronic epilepsy. BMJ 1991; 302: 363-6. 21. DTB. Drugs and alcohol: harmful cocktails? Epilepsy. Drug Ther Bull 1996; 34: 36-8. 22. DTB. Epilepsy and pregnancy. Drug Ther Bull 1994; 32: 49-51. 23. Marson AG et al. New anti-epileptic drugs: a systematic review of their efficacy and tolerability. BMJ 1996; 313: 1169-74. 24. Chadwick DW. Overuse of monitoring of blood concentrations of antiepileptic drugs. BMJ 1987; 294: 723^1. 25. Gandelman MS. Review of carbamazepine-induced hyponatraemia. Prog Neuro-Psychopharm Biol Psych 1994; 18 (2): 211-33. 26. Jacoby A, Johnson A, Chadwick D. Psychosocial outcomes of anti-epileptic drug discontinuation. The Medical Research Council Antiepileptic Drug Withdrawal Study Group. Epilepsia 1992; 33(6): 1123-31. 27. Sirven JI, Sperling M, Wingerchuk DM et al. Early versus late antiepileptic drug withdrawal for people with epilepsy in remission (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. 28. Chadwick D et al. Outcomes after seizure recurrence in people with well-controlled epilepsy and the factors that influence it. Epilepsia 1996; 37: 1043-50.

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29. Medical Research Council Antiepileptic Drug Withdrawal Study Group. Prognostic index for recurrence of seizures after remission of epilepsy. BMJ 1993; 306: 1374-8. 30. Care of the Elderly 1990; 2: 4-8. 31. Deane KHO, Jones D, Playford ED et al. Physiotherapy for patients with Parkinson's disease (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford; Update Software, 2001. 32. de Goede CJT, Keus SHI, Kwakkel G et al. The effects of physical therapy in Parkinson's disease: a research synthesis. Arch Phys Med Rehab 2001; 82: 509-15. 33. Deane KHO, Ellis-Hill C, Playford ED et al. Occupational therapy for patients with Parkinson's disease (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford; Update Software, 2001. 34. Deane KHO, Whurr R, Playford ED et al. Speech and language therapy versus placebo or no intervention for dysarthria in Parkinson's disease (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 35. Lees AJ, Katzenschlager R, Head J et al. Ten-year followup of three different initial treatments in de-novo PD. Neurology 2001; 57: 1687-94. 36. The Parkinson's Disease Study Group. Effects of tocopherol and Deprenyl on the progression of disability in early Parkinson's disease. Neiv Eng ] Med 1993; 328: 176-83. 37. Calne DB. Selegiline in Parkinson's disease. BMJ 1995; 311: 1583-4. 38. Lees AJ. Comparison of therapeutic effects and mortality data of levodopa and levodopa combined with selegiline in patients with early, mild Parkinson's disease. BMJ 1995; 311: 1602-7. 39. Choudhury KR, Clough C. Subcutaneous apomorphine in Parkinson's disease. BMJ 1998; 316: 641. 40. Langhorne P, et al. Do stroke units save lives? Lancet 1993; 342: 383-4. 41. Indredavik B, et al. Stroke unit treatment improves longterm quality of life: a randomised controlled trial. Stroke 1998; 29: 895-9. 42. Harper G, Treatment of stroke in older patients. A state of the art review. Drugs & Aging 1995; 6: 29^44. 43. The Intercollegiate Working Party on Stroke. National Clinical Guidelines for Stroke. London: The Royal College of Physicians, 2000. 44. Wardlaw JM, Warlow CP. Thrombolysis in acute ischemic stroke: does it work? Stroke 1992; 23: 1826-39. 45. Bath PMW, Lees KR. Acute stroke. BMJ 2000; 320: 920-3. 46. Gubitz G, Sandercock P. Stroke management: blood pressure reduction. Clinical Evidence, Issue 4, 2000. London: BMJ Publishing Group, available on www.clinicalevidence.org 47. Mann G, Hankey GJ, Cameron D. Swallowing function after stroke. Stroke 1999; 30: 744-8. 48. Mayo NE et al. There's no place like home: an evaluation of early supported discharge for stroke. Stroke 2000; 31: 1016-23. 49. Anderson C et al. Home or hospital for stroke rehabilitation? Results of a randomised controlled trial. Stroke 2000; 31: 1024-31. 50. Kotila M et al. Depression after stroke: results of the FINNSTROKE Study. Stroke 1998; 29: 368-72.

51. Brown KW, Sloan RL, Pentland B. Fluoxetine as a treatment for post-stroke emotionalism. Acta Psychiatr Scand 1998; 98: 455-58. 52. Wiart L et al. Fluoxetine in early post-stroke depression: a double-blind placebo-controlled study. Stroke 2000; 31: 1829-32. 53. Lees KR, Bath PMW, Naylor AR. Secondary prevention of transient ischaemic attack and stroke. BMJ 2000; 320: 991^. 54. He J et al. Aspirin and risk of hemorrhagic stroke: a meta-analysis of randomised controlled trials. JAMA 1998; 280: 1930-5. 55. Kapral MK, Silver FL and the Canadian Task Force on Preventive Health Care. Preventive health care 1999 update 2. Echocardiography for the detection of a cardiac source of embolus in patients with stroke. CMAJ 1999; 161: 989-96. 56. MacMahon S, Neal B, Rodgers A. Blood pressure lowering for the primary and secondary prevention of coronary and cerebrovascular disease. Schweizerische Medizinische Wochenschrift; Journal Suisse de Medecine 1995; 125: 2479-86. 57. Hart RG et al. Prevention of stroke in patients with nonvalvular atrial fibrillation. Neurology 1998; 51: 674-81. 58. Hart RG et al. Aspirin for the primary prevention of stroke and other major vascular events. Arch Neural 2000; 57: 326-32. 59. Blauw GJ et al. Stroke, statins, and cholesterol. A metaanalysis of randomized, placebo-controlled, doubleblind trials with HMG-CoA reductase inhibitors. Stroke 1997; 28: 946-50. 60. Warshafsky S et al. Efficacy of 3-hydroxy-3 methylglutaryl coenzyme A reductase inhibitors for prevention of stroke. / Gen Intern Med 1999; 14: 763-74. 61. Hillbom M. Alcohol consumption and stroke: benefits and risks. Alcoholism: Clin Exper Res 1998; 22 (7 Suppl): 352S-358S. 62. Higa M, Davanipour Z. Smoking and stroke. Neuroepidemiology 1991; 10: 211-22. 63. Fletcher GF. Exercise in the prevention of stroke. Health Reports 1994; 6: 106-10 64. Gubitz G, Sandercock P. Prevention of ischaemic stroke. BMJ 2000; 321: 1455-9. 65. Cina CS, Clase CM, Haynes RB. Carotid endarterectomy for symptomatic carotid stenosis (Cochrane Review). In: The Cochrane Library Oxford: Update Software, 1999. 66. Johnson ES et al. A metaregression analysis of the doseresponse effect of aspirin on stroke. Arch Intern Med 1999; 159: 1248-53. 67. Shneerson JM. Motor neurone disease. BMJ 1996; 313: 244-5. 68. Norris FH. Motor neurone disease. BMJ 1992; 304: 459-60. 69. Filippini G, Brusaferri F, Sibley WA et al. Corticosteroids or ACTH for acute exacerbations in multiple sclerosis (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 70. Rice GPA, Incorvaia B, Munari L et al. Interferon in relapsing-remitting multiple sclerosis (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 71. DTB. Glatiramer acetate for multiple sclerosis. Drug Ther Bull 2001; 39: 41-3.

REFERENCES 207

72. Bryant J, Clegg A, Milne R. Systematic review of immunomodulatory drugs for the treatment of people with multiple sclerosis: is there good quality evidence on effectiveness and cost? / Neural Neurosurg Psychiatry 2001; 70: 574-9. 73. Shakespeare DT, Boggild M, Young C. Anti-spasticity agents for multiple sclerosis (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 74. Taricco M, Adone R, Pagliacci S et al. Pharmacological interventions for spasticity following spinal cord injury (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 75. Cleeves L, Findley LJ. Propranolol and propranolol-LA in essential tremor: a double blind comparative study. / Neural Neurosurg Psychiatry 1988; 51: 379-84. 76. Sasso E, Perucca E, Fava R, Calzetti S. Primidone in the long-term treatment of essential tremor: a prospective study with computerized quantitative analysis. Clin Neuropharmacol 1990; 13: 67-76.

77. Koller WC, Vetere-Overfield B. Acute and chronic effects of propranolol and primidone in essential tremor. Neurology 1989; 39: 1587-8. 78. Gorman WP, Cooper R, Pocock P, Campbell MJ. A comparison of primidone, propranolol, and placebo in essential tremor, using quantitative analysis. / Neurol Neurosurg Psychiatry 1986; 49: 64-8. 79. Ondo W, Hunter C, Vuong KD, Schwartz K, Jankovic J. Gabapentin for essential tremor: a multiple-dose, double-blind, placebo-controlled trial. Mov Disord 2000; 15: 678-82. 80. Huber SJ, Paulson GW. Efficacy of alprazolam for essential tremor. Neurology 1988; 38: 241-3. 81. Schuurman PR, Bosch DA, Bossuyt PMM et al. A comparison of continuous thalamic stimulation and thalamotomy for suppression of severe tremor. New Engl J Med 2000; 342: 461-8.

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11

CHAPTER CONTENTS Dysmenorrhoea 209 Primary dysmenorrhoea 209 Secondary dysmenorrhoea 210 Menorrhagia

210

Gynaecological problems

Irregular periods 211 Intermenstrual bleeding 212 Amenorrhoea 212 Primary amenorrhoea 212 Secondary amenorrhoea 212 Polycystic ovary syndrome 213 Vaginal discharge 213 Candida 213 Recurrent candidiasis

DYSMENORRHOEA

214

Bacterial vaginosis 214 Pelvic inflammatory disease 214 Premenstrual syndrome

216

Review: Wilson M, Farquhar C. Dysmenorrhoea. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Online. Available: www.clinicalevidence.com

Gestational trophoblastic disease: hydatidiform mole and choriocarcinoma 217 The menopause 217 Hormone replacement therapy Cervical screening 223 References

224

218

PRIMARY DYSMENORRHOEA * Reassure the patient that this is not a sign of disease. An examination of the abdomen is worthwhile as part of that reassurance as well as to exclude gross pelvic pathology. The need for a vaginal examination will depend on the individual circumstances. * Give aspirin, paracetamol or compound analgesics.1 * Give an NSAID, alone or in addition to an analgesic. If taken from just before or at the onset of menstruation until pain subsides, they are effective in about 80%. Examples are ibuprofen 1200 mg daily, mefenamic acid 750-1500 mg daily or naproxen, although adverse effects may be more common with the latter.2 * Consider a trial of the combined oral contraceptive (COC). It is commonly used despite the lack of evidence either way about its benefit. * Consider a trial of thiamine 100 mg daily. In one study it was more effective than placebo.3 * Magnesium. Results suggest magnesium may be a promising treatment for dysmenorrhoea. It is unclear what dose or regime of treatment should be used, therefore no strong recommendation 209

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can be made until further evaluation is carried out.4 * Women who are still in pain. Consider referral for all those not responding.

SECONDARY DYSMENORRHOEA * Examine vaginally. Refer women in whom there is suspicion of pelvic pathology for laparoscopy. * Refer women who also have dyspareunia and low-grade pain throughout the cycle; they may have subclinical endometriosis, adenomyosis or low-grade pelvic inflammatory disease despite a normal pelvic examination. * Otherwise, treat symptomatically as for primary dysmenorrhoea. * IUD. Consider removing an intrauterine contraceptive device, if present.

Endometriosis Farquhar C. Endometriosis. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Online. Available: www.clinicalevidence.com Royal College of Obstetricians and Gynaecologists. The investigation and management of endometriosis. 'Green top'clinical guidelines. London: RCOG, 2000. www.rcog.org.uk

* Refer for laparoscopy patients in whom you suspect the diagnosis. The interpretation of the finding of endometriosis can, however, be difficult. It is found in about half of women presenting with dysmenorrhoea. In some of these it may be co-incidental, in that it is present in 2-22% of women who have no symptoms. * Where endometriosis has been diagnosed. Treat as follows. The effectiveness of the different medical treatments are similar although the adverse effects differ.5 (a) Patients not wishing to become pregnant: consider: -A low-dose oral contraceptive. Give three packets of a combined oral contraceptive back to back, followed by a 1 week break. Continue for 9 months in the first instance.6

-Progestogens continuously for 6 months, e.g. norethisterone 10-20mg daily. Dydrogesterone appears to be no better than placebo.7'8 - Danazol 400 mg daily for 2 months decreasing to 200 mg daily then 100 mg daily, at 2-monthly intervals. Maintain the patient on the lowest effective dose for 6 months. Occasionally patients need 600-800 mg daily. Warn the patient about the possible side-effects of acne, weight gain, muscle cramps, oedema and irreversible voice changes.9'10 Non-hormonal contraception is essential if the patient is sexually active. -Gonadorelin analogues. Refer for complete ovarian suppression for up to 6 months. Nonhormonal contraception is essential if the patient is sexually active.9 (b) Patients wishing to become pregnant: consider using NSAIDs, as in primary dysmenorrhoea. Patient advice: The National Endometriosis Society, 50 Westminster Palace Gardens, Artillery Row, London SW1P 1RL, tel. 020 7222 2781; helpline 0808 808 2227; www.endo.org.uk

MENORRHAGIA Review: Duckitt K. Menorrhagia. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Online. Available: www.clinicalevidence.com RCOG. The initial management of menorrhagia. Evidence-based clinical guideline. London: RCOG, 1999. Online. Available: www.rcog.org.uk/guidelines/ menorrhagia.html

• Menorrhagia is defined as regular bleeding with a loss of more than 80 ml of blood per month. • Confirmation of true menorrhagia can be obtained by a history of bleeding that cannot be controlled with tampons alone, and by having to get up during the night to change. • 50% of women referred for menorrhagia have depression or anxiety as their primary problem.11 • One in 20 women aged 30-55 years consults her GP each year with menorrhagia.12

IRREGULAR PERIODS 211

* Menstrual diary. Advise women to keep a record of bleeding in order to spot any irregularities and to record the amount of the loss. * Perform a pelvic and abdominal examination. * Check Hb; two-thirds will be anaemic. * Exclude hypothyroidism only if there are signs or symptoms. * Refer to a gynaecologist for assessment if: (a) the patient is over 45; (b) there is suspicion of an organic cause (fibroids, pelvic pain, dyspareunia); (c) there is any postcoital, intermenstrual, or irregular bleeding, or any sudden change in blood loss; (d) medical treatment is unsuccessful.

Treatment * Antifibrinolytics. Start on the first day and continue until heavy bleeding has ceased. Tranexamic acid 1 g q.d.s decreases bleeding by up to 70%.13 * NSAIDs. Give mefenamic acid 500 mg t.d.s or naproxen 500 mg b.d. Taken shortly before or at the start of menstruation and continued during heavy loss, they can decrease bleeding by 20-50%. They are especially useful if there is dysmenorrhoea. * Progestogen-containing intrauterine device (IUD). This appears to be the most effective non-surgical treatment,14'15 and is now licensed for the treatment of menorrhagia. * Combined oral contraceptive (COC) is commonly used but there is insufficient evidence at present to adequately assess its effectiveness.16 It is especially useful if there also dysmenorrhoea and if contraception is required. * Danazol is effective but is limited by sideeffects (acne, weight gain, muscle cramps, oedema, irreversible voice changes) and the need to avoid conception. 100-200 mg daily can reduce blood loss by 30-70%. * Progestogens. Give them for 21 days of each cycle. Giving them for the second half of each cycle only is no better than placebo.17 * IUD. If an IUD is in situ either give an NSAID or an antifibrinolytic or change to a progestogenreleasing IUD. * Women not responding. Consider referring for endometrial ablation or hysterectomy.

Endometrial ablation has a shorter hospital stay, and less time off work but a proportion of women will have further bleeding and will require further surgery.18

Flooding * Give norethisterone 15 mg b.d. for 2 days, then lOmg b.d. for 2 days, then 15mg once daily for 2 days, followed by 10 mg once daily for 14 days. The patient should expect a bleed 2-3 days after stopping the norethisterone.

Delaying a period * Give norethisterone 5mg b.d. or t.d.s 3 days prior to expected period. The next period can be expected 2-3 days after stopping norethisterone.

IRREGULAR PERIODS Teenagers * Reassure the patient that irregular periods are common after the menarche and that a regular cycle will probably establish itself without treatment. * If the patient is sufficiently bothered. Give a progestogen cyclically, from day 1 to day 21, for 3 months. There is, however, no reason to think that this will speed up the establishment of normal periods. * COC can be considered especially if contraception is required or they have dysmenorrhoea. * Very infrequent periods. These girls need assessment (see p. 212).

Women in their reproductive years * Check whether irregular periods or amenorrhoea have always been a feature. They may have PCOS (see p. 213). * Exclude pregnancy. * Check whether the irregularity is due to the progestogen-only pill (POP) (see p. 235). * Look for weight loss, recent stress, hirsutism, galactorrhoea or infertility (see p. 157).

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* Take blood for TFTs, prolactin, LH and FSH on day 2 of the cycle. * If results are normal and the patient wishes to become pregnant consider referral for clomiphene (see p. 247). * If the results are normal and pregnancy is not desired, COC or cyclical progestogens can be considered.

* Women on the COC: check compliance. If the examination is normal, and bleeding persists consider changing the pill (see p. 233). * Women with an IUD: (a) Take endocervical swabs and a high vaginal swab (HVS). (b) Remove the IUD if there is no infection but remember to discuss alternative contraception. (c) Reassess in 3 months.

Older women * Distinguish the irregularity due to ovarian failure from the pathological pattern of intermenstrual bleeding. * Consider cyclical progestogens if the patient is sufficiently bothered. * COC will regulate bleeding and provide contraception. The known risks of thrombosis and breast carcinoma should be discussed with the patient and documented. * HRT can be considered if there are other symptoms of the menopause and the bleeding is not pathological.

Postmenopausal women * Refer if bleeding occurs after 6 months amenorrhoea.

AMENORRHOEA Review: Crosignani PG, Vegetti W. A practical guide to the diagnosis and management of amenorrhoea. Drugs 1996; 52: 671 -81.

PRIMARY AMENORRHOEA Girls aged 14-16 years * Refer any girl aged 14 who has no breast development. * Refer any girl whose breast development began more than 2 years ago and who has not yet menstruated. Girls aged 16 and over

INTERMENSTRUAL BLEEDING (1MB) * Examine: (a) for anaemia; (b) the pelvis for abnormalities with a bimanual examination; (c) the cervix for abnormalities, and take a smear; (d) the vulva and vagina. * Take a pregnancy test if appropriate. * Refer any patient with gynaecological pathology or with persistent 1MB. * Premenstrual spotting. Reassure the patient if the loss is light and premenstrual. It is probably due to failure of the corpus luteum, and should settle within a few cycles. Give cyclical progestogens to women sufficiently troubled by the symptoms.

* Examine her for evidence of endocrine disorders and weight disturbance. * Look at the external genitalia and secondary sexual characteristics, and check for an imperforate hymen (associated with cyclical pain). * Refer to a gynaecologist.

SECONDARY AMENORRHOEA Secondary amenorrhoea exists when a woman has not menstruated for 6 months, having had a previously established cycle. * In the history, note particularly: (a) any weight loss (if the patient's weight is less than 45 kg and she is of average height she is unlikely to menstruate). (b) excessive exercise.

CANDIDA

(c) recent stress. (d) the character of the cycles prior to the amenorrhoea. * Exclude pregnancy. * Take blood for LH and FSH, prolactin, oestradiol and TFTs. * Refer to a gynaecologist if: (a) the FSH is high (>30iu/L) and the patient is under the age of 45. This indicates premature ovarian failure. (b) the LH/FSH ratio is more than 2.5. This suggests polycystic ovary syndrome, (see below). (c) the prolactin is over 800miu/L. This suggests a pituitary adenoma. Repeat the test if the level is between 400 and 800miu/L. (d) the oestradiol is low.

POLYCYSTIC OVARY SYNDROME (PCOS) Review: DTB. Tackling polycystic ovary syndrome. Drug TherBull 2001; 39(1): 1-5.

• Polycystic ovary syndrome is a common problem affecting approximately 5% of women of reproductive age.19 • The characteristic features include: (a) hirsutism; (b) acne; (c) oligomenorrhoea/ amenorrhoea; (d) obesity; (e) raised serum testosterone; (f) raised luteinising hormone. • Patients with PCOS are twice as likely to develop diabetes.20 • Take blood for testosterone, LH and FSH on day 2 of the cycle. A LH/FSH ratio >2.5 is suggestive of PCOS. • Treatment is aimed at managing current symptoms and includes: (a) Weight loss advice. (b) Hirsutism. Consider: -the COC or cyproterone acetate and ethinylestradiol (Dianette).

213

- Spironolactone or finasteride, although they are not licensed for this purpose. Contraception is required as they carry the theoretical risk of feminising a male fetus.21 (c) Oligo-/amenorrhoea. The COC or cyclical progestogens can provide a regular withdrawal bleed and prevent the chronic unopposed oestrogen secretion which may put them at risk of endometrial carcinoma. (d) Subfertility. Refer for a specialist opinion. The use of metformin is a relatively new development that followed the discovery of a link between PCOS and insulin resistance. Preliminary evidence suggests metformin reduces serum insulin and testosterone and may improve menstrual irregularity.22 Self help group: Verity. The polycystic self-help group, 52-54 Featherstone Street, London EC1Y 8RT; www.verity-pcos.org.uk

VAGINAL DISCHARGE * Perform the following in patients with a vaginal discharge: (a) An HVS. Check that the patient has not recently used an over the counter anti-candida medication. (b) Endocervical and urethral swabs if there is any reason to suspect gonococcus or chlamydia (e.g. the patient is aged less than 25 or has changed her sexual partner in the last year). (c) Examine and check for a cervical erosion or a foreign body. * Instruct patients to return if discharge remains after initial treatment.

CANDIDA Guideline: Clinical Effectiveness Group (Association for Genitourinary Medicine and Medical Society for the study of Venereal Disease). National Guidelines on the Management of Vulvovaginal Candidiasis, 2001. Online. Available: www.mssvd.org.uk/CEG/ceguidelines.htm

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GYNAECOLOGICAL PROBLEMS

* Give general advice to avoid local irritants, and avoid tight fitting synthetic clothing.23 * Treat only if symptomatic. * Prescibe a topical imidazole. Cure rates are 80-95%. Give it either as a single dose or as a short course. Oral imidazoles are no more effective24 but they have more side-effects. In addition, they are contraindicated in pregnancy. * Male partners. There is no evidence to support the treatment of asymptomatic male sexual partners.25 * COC. Do not stop the COC. Its use is not associated with candidiasis.

RECURRENT CANDIDIASIS * Check the urine for glucose approximately 2 hours after a meal or glucose load. * Exclude other risk factors; iron deficiency anaemia, thyroid disease, frequent antibiotic use, corticosteroid use, immunodeficiency. * Screen for other vaginal infections. * Give an imidazole pessary daily for 2 weeks then weekly for 6 months; or use a pessary only when an infection is most likely, e.g. after intercourse or when taking a broad-spectrum antibiotic. These treatment regimes are empirical and are not based on randomised controlled trials. * Candida of the gut. There is no evidence to suggest that eradication of Candida from the gut is helpful.

BACTERIAL VAGINOSIS Guideline: Clinical Effectiveness Group of the Association for Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases. National guideline for the management of bacterial vaginosis, 2001. Online. Available: www.mssvd.org.uk/CEG/ceguidelines.htm Systematic review: Joesoef M, Schmid G. Bacterial vaginosis. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001.

• Bacterial vaginosis is characterized by a reduction in lactobacilli and an overgrowth of predominantly anaerobic organisms (Gardnerella vaginalis, Mycoplasma hominis, Prevotella species, Mobiluncus species).

• Approximately 50% of women with the condition are asymptomatic. • It is not regarded as sexually transmitted. It can arise and remit spontaneously in women regardless of sexual activity. However, it is more common in women with sexually transmitted infections. • No evidence has been found supporting the treatment of partners of women affected by bacterial vaginosis.26 • Treatment is indicated for: (a) symptomatic women; (b) pregnant women with a history of recurrent miscarriage; (c) women undergoing termination of pregnancy. They are at greater risk of pelvic inflammatory disease if the bacterial vaginosis is untreated. • Suspect it in women who have a profuse vaginal discharge which smells fishy. The HVS will show 'clue cells' on microscopy with no pathogens. A more rigorous diagnosis would use Amstel's criteria (see box below). • Take endocervical swabs for gonococcus and chlamydia. • Give metronidazole 400-500 mg b.d. for 5-7 days or 2 g stat (avoid single dose in pregnancy); or intravaginal metronidazole gel (0.75%) once daily for 5 days; or intravaginal clindamycin cream (2%) once daily for 7 days. Amstel's criteria for bacterial vaginosis (three out of four should be positive) (a) Thin, grey/white homogenous discharge. (b) Clue cells on microscopy (available on most high vaginal swabs). (c) pH of vaginal fluid >4.5 (pH paper is cheap and readily available). (d) Release of fishy odour on adding alkali (10% KOH).

PELVIC INFLAMMATORY DISEASE (PID) Guideline: Clinical Effectiveness Group of the Association for Genitourinary Medicine and the Medical Society for the study of Venereal Diseases. Guidelines

PELVIC INFLAMMATORY DISEASE

for the management of pelvic infection, 2001. Online. Available: www.mssvd.org.uk/CEG/ceguidelines.htm Systematic review: Ross J. Pelvic Inflammatory disease. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org

• PID is usually the result of ascending infection from the endocervix. Neisseria gonorrhoeae and Chlamydia trachomatis have been identified as causative agents, whilst Gardnerella vaginalis, anaerobes and other organisms commonly found in the vagina may also be implicated. • PID has a high morbidity; about 20% of affected women become infertile, 20% develop chronic pelvic pain, and 10% of those who conceive have an ectopic pregnancy.27 • Repeated episodes of PID are associated with a four- to sixfold increase in the risk of permanent tubal damage.28 • PID may be asymptomatic. • Symptoms can include lower abdominal pain, dyspareunia, abnormal bleeding, abnormal vaginal or cervical discharge. • 10-20% of women with PID will develop perihepatitis. • It is likely that delaying treatment increases the risk of long-term sequelae such as ectopic pregnancy, infertility and pelvic pain. Because of this, and the lack of definitive diagnostic criteria, a low threshold for the empiric treatment of PID is recommended.

Diagnosis Clinical diagnosis is correct in only 65% of cases compared to laparoscopy. Even laparoscopy can miss mild cases but it remains the most reliable investigation. In the UK it is not recommended in all cases because of the cost implications. • If not referring perform the following: (a) Endocervical swabs for gonococcus and chlamydia. Negative microbiological tests do not exclude a diagnosis of PID; in at least 50% of cases no specific organism is identified; (b) Urinanalysis, pregnancy test, and MSU specimen to look for other causes of lower abdominal pain;

215

(c) Blood for ESR or C-reactive protein. A raised level would support the diagnosis.29 * Recommend rest and provide adequate analgesia. * Recommend that unprotected intercourse be avoided until the woman and her partner(s) have completed treatment and follow up. * If an IUD is in situ, and this is the preferred method of contraception, leave it in place. Remove it only if the condition fails to respond to treatment and other causes of pain have been excluded.30'31'32 * Drug treatment. (a) ofloxacin 400 mg b.d for 14 days plus metronidazole 400 mg b.d. for 14 days; or (b) doxycycline 100 mg b.d for 14 days plus metronidazole for 14 days (add in ciprofloxacin 500 mg stat dose if the infection was acquired abroad or there is a high incidence of gonococcal infection in the local population); or (c) erythromycin 500 mg b.d for 14 days plus metronidazole 400 mg b.d. for 14 days. * Review daily in case admission becomes necessary.

Admission Admit patients if (a) the illness is severe; (b) not responding after 3 days of oral therapy; (c) unable to tolerate oral drugs; (d) a pelvic mass is present; (e) the patient is pregnant; (f) there is diagnostic uncertainty; (g) the patient suffers from immunodeficiency; (h) the patient is above the normal age for PID. An alternative diagnosis, e.g. ovarian carcinoma, is more likely.

Follow-up for all patients * Trace, investigate and treat the woman's partner^) if an STD is diagnosed.33 One study of women with acute salpingitis found that 30 out of 34 male contacts had urethritis.34 Another study found that 60% of contacts had relevant infections, and that in most of these it was asymptomatic.35 * Discuss with the patient her need for safer sex.

216

GYNAECOLOGICAL PROBLEMS

* Advise the patient to ask for antibiotic cover (e.g. doxycycline 200 mg stat) if she ever needs a termination of pregnancy (TOP) or a dilatation and curettage (D&C). * Advise the patient to seek advice promptly if the symptoms return.

(b) Stress management. Explain that although it is not the cause of the syndrome, stress is harder to cope with during the premenstrual period. Relaxation methods, yoga, meditation and exercise can be of benefit.

Therapeutic options Patient information: www.patient.co.uk. (choose 'women's health' then 'pelvic inflammatory disease').

PREMENSTRUAL SYNDROME Systematic review: Premenstrual syndrome. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. Online. Available: www.clinicalevidence.com Guideline: National Association for Premenstrual Syndrome. Treatment guidelines for premenstrual syndrome. Guidelines 2001,15. Online. Available: www.eGuidelines.co.uk

* 95% of women have symptoms related to their menstrual cycle; in 5% they are disabling. More than 150 symptoms have been reported. * Diagnosis depends not on the type of symptom, but on the timing of the symptoms and their cyclicity. Symptoms present 1-14 days prior to menstruation and disappear at the onset or on the day of the heaviest flow. If behavioural symptoms persist throughout the cycle then consider a psychological/psychiatric disorder. * Ask the patient to keep a menstrual and symptom diary for at least 2 months. She can score symptoms according to their severity. * Listen sympathetically. It is a real entity. This alone can be therapeutic. * Give simple advice: (a) Dietary advice. Explain that some women seem more sensitive to blood sugar changes at this time of the cycle, and this may be the cause of food cravings, panic reactions, irritability and aggression. Advise patients to reduce their refined sugar intake as well as their caffeine intake. Small frequent carbohydrate snacks seem effective in about 30% of women.

* Oestrogens may improve or in some cases worsen the situation. The COC can prove helpful, especially in those women requiring contraception. Women who experience symptoms during their pill-free week can take three consecutive packets back-to-back. A lower dose of oestrogen, as HRT, may help some women but cyclical progestogens must be used in women with an intact uterus. * Prostaglandin inhibitors, e.g mefenamic acid, may be of value for breast and pelvic pain, joint pains and headache. They may be taken from day 14, or from the start of symptoms, until symptoms abate. * Antidepressants may help even if depressive symptoms are absent. SSRIs, e.g fluoxetine 20^40 mg show improvements in both physical and psychological symptoms.36 * Vitamin B6 50-100 mg daily.37 This recommendation is based on limited information from poor quality trials, but looks as if B6 may be of benefit.38 * Breast tenderness. Treatment options include: (a) bromocriptine 1.25mgb.d. (b) danazol 200 mg daily. Note: Progestogens have been found unhelpful in the treatment of PMS.39 Note: Evening primrose oil. There is no evidence from high quality studies that it is helpful in PMS.40

Patients not responding * Refer to a gynaecologist for the consideration of suppression of ovarian function. Treatment options include high-dose danazol, high-dose oestrogen, gonadotrophin-releasing hormone analogues with add-back HRT. Patient support: The National Association for Premenstrual Syndrome (NAPS), 41 Old Road,

THE MENOPAUSE 217

East Peckham, KentTN12 SAP, helpline 0870 7772177; www.pms.org.uk Premenstrual Society, PO Box 429, Addlestone, Surrey KT15 1DZ, helpline 01932 872560 (11 a.m.-6 p.m., weekdays only).

GESTATIONAL TROPHOBLASTIC DISEASE: HYDATIDIFORM MOLE AND CHORIOCARCINOMA RCOG. The management of gestational trophoblastic disease. Guideline No. 18. London: Royal College of Obstetricians and Gynaecologists, 1999.

• Gestational trophoblastic disease is a rare event in the UK, with a calculated incidence of 1.54 per 1000 live births.41 • In the UK there is an effective registration and treatment programme with high cure rates. • Once the diagnosis of hydatidiform mole has been made, follow-up will be needed for between 6 months and 2 years. The aim is to detect the occurrence of choriocarcinoma. • Serum estimations of levels of human chorionic gonadotrophin (HCG) levels should be performed according to protocols from the regional expert units (London, Sheffield and Dundee). • The combined oral contraceptive pill and hormone replacement are safe to use after HCG levels have reverted to normal. • Women should be advised not to conceive until the HCG level has been normal for 6 months or follow-up has been completed (whichever is the sooner). Patient information: The Hydatidiform Mole and Choriocarcinoma UK information service, www.hmolechorio.org.uk

Screening centres Trophoblastic Tumour Screening and Treatment Centre Department of Oncology, Charing Cross Hospital, Fulham Palace Road, London W6 8RF

Tel. 020 8846 1409, fax 020 8748 5665 Trophoblastic Tumour Screening and Treatment Centre Weston Park Hospital, Whitham Road, Sheffield. S10 2SJ Tel. 0114 226 5202. Fax. 0114 226 5511 Hydatiform Mole Follow-up (Scotland) Department of Obstetrics and Gynaecology, Ninewells Hospital, Dundee DD1 9SY Tel. 01382 632748. Fax. 01382 632096

THE MENOPAUSE Information: Prodigy guidance. Menopausal management (HRT), June 2001. Online. Available: www.prodigy.nhs.uk/guidance National Prescribing Centre. The benefits and risks of HRT. MeReC Bulletin 2001; No. 16, www.npc.co.uk Rymer J, Morris E. Menopausal symptoms. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001, pp. 1303-10. Online. Available: www.clinicalevidence.com Rees M, Purdie DW. Management of the menopause. Handbook of the BMS. London: BMS Publications, 1999.

Establishing the diagnosis • FSH levels fluctuate markedly during the perimenopause and so are of limited value in symptomatic women, in whom a clinical history can form the basis of diagnosis. • There is little place for LH, oestradiol and progesterone levels in clinical practice.42 • No investigations are routinely indicated before starting HRT. • Consider taking serial FSH measurements in the following circumstances: (a) Women with symptoms under the age of 40 because of the implications of premature ovarian failure. (b) Women with symptoms who have had a hysterectomy with ovarian conservation. They are at risk of an early menopause. (c) Women over the age of 45 who are still on hormonal contraception who wish to establish

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GYNAECOLOGICAL PROBLEMS

whether they are menopausal. The hormonal contraception can mask the menopause (see p. 222 contraception in the perimenopause). * Take FSH levels 4-8 weeks apart when the woman is not on HRT or hormonal contraception. FSH levels of greater than 30 iu/L are generally considered to be in the postmenopausal range. * Abnormal bleeding. Refer women, without starting HRT, if they give a history of abnormal bleeding, e.g. sudden change in menstrual pattern, intermenstrual bleeding, postcoital bleeding or a postmenopausal bleed.42'43

Initial management * Counsel women about the menopause. Ideally, all perimenopausal women should be given the opportunity to discuss the menopause, with particular reference to the symptomatology, common misconceptions and treatment options. * Take the opportunity to discuss lifestyle issues (smoking cessation, diet and exercise). * Osteoporosis. Identify those patients at high risk of osteoporosis and assess whether HRT is indicated (see p. 180 Osteoporosis). * Vasomotor symptoms. (a) HRT is extremely effective in controlling these symptoms.44 (b) Consider clonidine for those unable to take HRT.45-46 (c) Diet. Discuss the limited evidence that suggests that phytoestrogens may be helpful in relieving vasomotor symptoms.47'48 They are plant substances similar to oestrogen, found in soya beans, chickpeas and cereals. * Vaginal dryness. Treatment options include: (a) HRT (see below). (b) Local oestrogens. These can be given per vaginum daily until symptoms have ceased, then twice weekly for 3 to 6 months. There is some systemic absorption of the oestrogens and therefore if using long-term oral progestogen should be taken for 10 days of each month.49 (c) Vaginal lubricants. These include KY jelly, Replens (a non-hormonal aqueous moisturizer) and Senselle (a water-based lubricant), although the latter two are not available on the NHS.

* Urinary symptoms. (a) Symptoms related to urogenital atrophy will respond to oestrogen by any route. Maximum benefit takes between 1 and 3 months of therapy.50 (b) Stress incontinence is unlikely to respond to oestrogen replacement therapy alone. Refer patients who are sufficiently troubled to a gynaecologist. * Psychological symptoms. Psychological symptoms may relate to a woman's hormonal status but, equally, the menopause may become a 'scapegoat' for patients with underlying emotional problems.51 Counselling may be helpful, but many will improve when their physical symptoms improve.52 Depression may need treatment in its own right.

HORMONE REPLACEMENT THERAPY (HRT) Information: National Prescribing Centre. The benefits and risks of HRT. MeReC Bulletin 2001; 16. Online. Available: www.npc.co.uk

• Relatively few eligible women use HRT, and those that do often stop it within the first few months. The main reasons for this are the fear of breast cancer and the return of withdrawal bleeding.53 Also many women express concerns around weight gain on HRT (see below). • There is evidence from randomized trials that the use of unopposed oestrogens or combined oestrogen/progestogen therapy does not cause extra weight gain in addition to that normally gained at the time of the menopause.54 • HRT can be considered for all peri and postmenopausal women, especially in those: (a) who have had a premature menopause; (b) who have marked symptoms and want symptom relief; (c) at high risk of osteoporosis (see p. 180).

Contraindications Oestrogen replacement therapy is absolutely contraindicated in very few patients. Even in women in whom treatment appears contraindicated,

THE MENOPAUSE

oestrogen therapy may be prescribed under supervision of a specialist menopause clinic if the women's symptoms are particularly severe.55 • Absolute contraindications include: (a) acute-phase myocardial infarction, pulmonary embolism or deep vein thrombosis (DVT); (b) active endometrial or breast cancer; (c) pregnancy; (d) undiagnosed breast mass; (e) uninvestigated abnormal vaginal bleeding; (f) severe active liver disease. Note: Many contraindications given in prescribing data sheets are derived from high-dose combined oral contraceptives, and are, in the view of most experts, not applicable to HRT.55 HRT use in women with comorbidity (relative contraindications) (a) Thromboembolic disease (personal or family history) Women with a personal or family history suggestive of thrombophilia (see p. 418) should be offered thromboembolic screening before starting. (b) Past history of breast or endometrial cancer. Refer women who want to consider HRT to the appropriate specialist. Although conventional advice is that oestrogens are contraindicated, small studies of breast cancer survivors have not shown an adverse effect on survival. (c) Diabetes and gall bladder disease. Use a transdermal preparation. (d) Liver disease. Refer to a specialist clinic. (e) Endometriosis. Refer to a specialist clinic. (f) Fibroids. HRT may enlarge fibroids, causing heavy or painful withdrawal bleeds. Warn patients to report pain or pressure effects on the bladder or bowel.

Benefits and risks Benefits (a) Symptom relief. Improvement in vasomotor symptoms occur in a few weeks, and vaginal symptoms in 3 months. (b) Osteoporosis prevention (Table 11.1). Oestrogen therapy at sufficient doses is effective for the

219

Table 11.1 Minimum doses of oestrogen needed for bone conservation Drug

Dosage

Frequency

Estradiol Oral conjugated equine oestrogens

1-2mg

daily*

0.625 mg

daily

50 (jig

daily

1.5g (two measures)

daily 6 monthly

Transdermal estradiol patch Estradiol gel Estradiol implants

50 mg

* Although 1 mg or 2mg oral estradiol can be used for prevention of osteoporosis, the bone protective effect is dose related. Some products are licensed for osteoporosis prevention at 2 mg only, while others are licenced at 1 mg and 2mg.

prevention of osteoporosis.56 This bone protection lasts as long as HRT is taken, but bone loss begins as soon as HRT is stopped. Evidence that HRT prevents fractures is less strong. Observational studies show a reduction in fracture risk of65%.57 Unproven benefits (a) Primary prevention of cardiovascular disease. The majority of the evidence comes from observational studies and so firm recommendations cannot be made until the results of ongoing randomized, controlled trials (RCTs) are known. There is insufficient data to suggest that HRT should be initiated solely for the purpose of primary prevention of cardiovascular disease (CVD). (b) Secondary prevention of cardiovascular disease. HRT does not appear to prevent the progression of cardiovascular disease.58 At present it is recommended that: -HRT should not be initiated for the secondary prevention of CHD; -the decision to continue long-term HRT in women with CVD should be based on noncoronary benefits and risks and on patient preference; and -if a woman has an acute cardiovascular event while taking HRT it is prudent to consider stopping HRT therapy. Reinstitution should be based on non-coronary benefits and risks and patient preference.59

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GYNAECOLOGICAL PROBLEMS

(c) Dementia and cognitive function. Evidence on the effect of HRT on the onset of dementia is conflicting and HRT should not be given for the prevention of dementia at the present time. Risks (a) Breast cancer -Short-term use (less than 5 years) does not appear to increase the risk of breast cancer.60 - Long-term use (more than 5 years) is associated with a small increase in the cases of breast cancer.61 -How big is the risk? The 20-year risk is as follows:62 (i) women aged 50 not using HRT: 45 per 1000; (ii) women using HRT for 5 years: 47 per 1000; (iii) women using HRT for 10 years: 51 per 1000; (iv) women using HRT for 15 years: 57 per 1000. - Once a woman has stopped HRT for 5 years or more she loses the excess risk. - Breast cancer in HRT users appears to have a better prognosis than in non-users.63 (b) Endometrial cancer. Unopposed oestrogen therapy enhances the development of endometrial hyperplasia and increases the risk of endometrial cancer. Addition of progestogens reduces the risk. (c) Venous thromboembolism. The excess risk is 16-23 per 100,000 HRT users per year, compared to a base-line risk of 8-11 per 100,000 per year. This increased risk disappears once HRT is stopped.64

Initial assessment * Take a history and assess menopausal status. * Check for contraindications to HRT therapy. * Give lifestyle advice, as above. * Check BP. * Advise the woman about breast awareness. Check that mammography screening is in place if over 50 years. Mammography is not needed before commencing HRT unless the woman is at high risk of breast cancer.65

* Check that regular cervical screening is taking place.

Treatment General principles Oestrogens: * Start at the lowest possible dose of oestrogen (especially in the older women who tend to get more oestrogenic side-effects) and increase at 3-monthly intervals if necessary to achieve optimum symptom control. * Give oestrogens continuously, and only give them without progestogens if the woman has had a hysterectomy. Progestogens: These must be added for endometrial protection in women with a uterus. Most products contain either C-19 derivatives (norethisterone/levonorgestrel) which are more androgenic, or C-21 derivatives (medroxyprogesterone acetate/dydrogesterone) which are less androgenic. * Change to a less androgenic preparation if the patient is troubled by progestogenic side-effects. Perimenopausal women with an intact uterus:

* Use a cyclical regimen (monthly or 3-monthly). The majority of women will have a bleed towards the end of the progestogen phase. Postmenopausal women with a uterus:

* Use: (a) a cyclical regimen; or (b) a continuous combined regimen. Continuous regimens induce an atrophic endometrium and so do not produce a withdrawal bleed, although irregular bleeding can occur in the first 4-6 months. Bleeding should be investigated if it persists for longer than 6 months, becomes heavier rather than less, or if it occurs after amenorrhoea; or (c) tibolone. This combines oestrogenic and progestogenic activities, and weak androgenic activity. It is indicated for the treatment of vasomotor symptoms and osteoporosis prophylaxis. It must only be started at least 1 year after the menopause. There is evidence that it improves libido.66'67

THE MENOPAUSE

Alternative modes of delivery (a) Oestrogen implants. Repeat every 4-8 months. Occasionally there can be a recurrence of vasomotor symptoms with supraphysiological plasma concentrations of oestradiol (tachyphylaxis). Check plasma estradiol levels have returned to normal (less than lOOOpmol/L) before inserting a new implant. (b) Transdermal patches and gels. These avoid the first-pass metabolism in the liver and deliver a more constant level of hormone. Patches come as either reservoir or matrix patches. Skin reactions are less common with the matrix patches. (c) Intrauterine system. The levonorgestrel releasing system (LNG-IUS) is not licensed for HRT, but would be an alternative route for the delivery of progestogen to protect the endometrium. A recent 5-year follow-up concludes that the LNGIUS effectively protects against endometrial hyperplasia.68 It provides contraception, and it is the only way a non-bleed regime may be achieved in the perimenopause.

221

5% of women, but pregnancy needs to be excluded in perimenopausal women. * Refer if there is: (a) a change in the pattern of withdrawal bleeds and breakthrough bleeding that persists for more than 3 months; (b) unexpected or prolonged bleeding that persists for more than 4 weeks after stopping HRT: refer urgently. Bleeding on continuous combined therapy or tibolone * Explain to patients that the risk of bleeding is 40% in the first 4-6 months. * Make sure the patient was at least 1 year postmenopausal before she started the regimen. * Bleeding beyond 6 months requires further investigation. * Bleeding that occurs after a period of amenorrhoea requires further investigation. Oestrogen-related side-effects

Managing the side-effects of HRT Bleeding on cyclical combined therapy * Check when the bleeding occurs. These regimes should produce regular predictable bleeds starting towards or soon after the end of the progestogenic phase. * Consider poor compliance, drug interactions or gastrointestinal upset. * Try stopping HRT to see if it is the cause of the bleeding. * If bleeding problems are due to HRT, alter the progestogen: (a) heavy or prolonged bleeding: increase the dose or duration of progestogen, or change type of progestogen to a more androgenic type (see p. 220). (b) bleeding early in the progestogenic phase: increase dose or change type of progestogen. (c) painful bleeding: change type of progestogen. (d) Irregular bleeding: change regime or increase dose of progestogen. * No bleeding whilst taking a cyclical regime reflects an atrophic endometrium and occurs in

* Oestrogenic side effects include fluid retention, bloating, breast tenderness and enlargement, nausea, headache, leg cramps and dyspepsia. * Encourage the patient to persist with therapy for 12 weeks, as most side-effects resolve with time. * For persistent side-effects consider (a) reducing the dose; or (b) changing the oestrogen type (swap between the two main forms of oestrogen: estradiol and conjugated equine oestrogens); or (c) changing the route of delivery. * Nausea and gastric upset. Change the timing of the oestrogen dose, e.g. try taking it with food or at bedtime. Progestogen-related side-effects Progestogen-related side-effects tend to occur in a cyclical pattern during the progestogenic phase of cyclical HRT. Continuous combined products contain lower doses of progestogens and sideeffects are less likely. Side-effects include fluid retention, breast tenderness, headaches, mood

222

GYNAECOLOGICAL PROBLEMS

swings, depression, acne, lower abdominal pain and backache. * Encourage the patient to persist with therapy for about 12 weeks, as some side-effects will resolve. * Various changes in the progestogens may be helpful, but remember not to reduce the dose or duration below that which protects the endometrium. Options: (a) reduce the duration but not below 10 days; (b) reduce the dose of progestogen; or (c) change the progestogen type (either C-19 or C-21 derivatives; see p. 220); or (d) change the route of progestogen (oral, transdermal, vaginal or intrauterine); or (e) reduce the frequency of how often the progestogen is taken by switching to a longcycling regime. Progestogens are administered for 14 days every 3 months (but only suitable for women with scant periods or postmenopausal); or (f) change to a continuous combined therapy which often reduces progestogenic side-effects with established use, but it is only suitable for postmenopausal women.

Follow-up of women on HRT * See after 3 months, then 6 monthly. * Check for compliance, bleeding patterns and side-effects. * Check that the patient is up to date with her cervical smears and mammograms. Note: There is no need to check the BP except as good practice in any well-person screening.

How long to continue? * Symptomatic relief. Give HRT until there is a reasonable chance that the symptoms will not return. If troublesome symptoms recur on withdrawal HRT can be restarted for 6-12 months at a time. * Osteoporosis prevention. Five years treatment is the minimum, and 10 years is believed to give the optimum risk/benefit ratio. The fact that the benefit rapidly drops-off after stopping HRT must be

set against the risk of breast cancer associated with longer-term use.

Stopping treatment * HRT may be stopped abruptly or gradually. For gradual reduction: (a) cyclical therapy. Reduce the oestrogen dose progressively over 3 months but maintain the progestogen as before; (b) continuous therapy. Reduce both the oestrogen and progestogen dose by 50% for a few weeks then stop. * Review after a few months. If troublesome symptoms have recurred consider restarting therapy at the lowest possible dose and titrating up as necessary. If only local symptoms persist use a vaginal preparation.

HRT and contraception * Perimenopausal women cannot be assumed to be infertile. * Routine HRT preparations do not suppress ovulation and do not provide contraceptive cover. * Contraception should be continued for 1 year after the last menstrual period (LMP) for women over 50 years, or for 2 years after the LMP for women under the age of 50 years. * Perimenopausal women may use: (a) barrier methods, IUD or LND-IUS, as well as HRT; or (b) the COC instead of HRT; or (c) the progestogen-only pill (POP), possibly combined with HRT, although there are theoretical concerns that the oestrogen component of HRT may interfere with the action of the POP on the cervical mucus.69

When has the menopause occurred when masked by HRT or COC? It is important to know this so a realistic idea of how long the women needs to remain on contraception can be calculated (see above); 80% of women are postmenopausal by the age of 54 years.70

CERVICAL SCREENING 223

* Discontinue HRT or COC for 6-8 weeks then check FSH and repeat after a further 4-8 weeks.71 If both FSH levels are above 30 iu/L, stop contraception after a further year. If a spontaneous period occurs, or FSH less than 30 iu/L, continue contraception and repeat the test in 1 year. * Women on the POP. Check the FSH without stopping the POP. Patient organisations: British Menopause Society. 36, West Street, Marlow, Bucks SL7 2NB, tel. 01628 890199; www.the-bms.org The Amarant Trust. 11-13 Charterhouse Buildings, London EC1M 7AN, helpline 01293 413000. Women's Health Information Service. 52 Featherstone Street, London EC1Y 8RT, tei. 020 7251 6333; helpline 0845 125 5254; www.womenshealthlondon.org.uk

CERVICAL SCREENING Guidance: NHSCSR Publication No 8. Guidelines for clinical practice and programme management, 2nd edition. Sheffield: NHSCSP Publications, 1997. NHS Cancer Screening Programme. Online. Available: www.cancerscreening.nhs.uk/cervical

number of false negatives and inadequate samples. The National Institute for Clinical Excellence (NICE) has considered the evidence about LBC and has recommended pilot studies to evaluate controlled introduction of LBC into the NHS cervical screening programme.

Taking a smear • In England in 1991-2, 6.3% of smears were inadequate.74 • Extended-tip spatulas appear to be better for collection of cytology samples than the Ayre spatula.75 * Repeat an inadequate smear within 3 months. If there are two further inadequate smears, refer for colposcopy. * If the smear result is inflammatory and the laboratory cannot comment on whether the smear is positive or negative: (a) perform HVS and endocervical swabs; (b) treat patient and partner for the specific organism; (c) repeat the smear.

Terminology Table 11.2 Terminology used during cervical screening

• The NHS screening programme requires that all women between the age of 20 and 64 are offered a smear every 3 years. • Women reaching the age of 65, who have had at least two normal smears need not have further smears. The last smear should be no more than 5 years before her 65th birthday. • There were 2740 new cases of invasive cervical cancer in England and Wales in 1997. This is a 26% fall in incidence over the previous 5 years.72 • According to the Imperial Cancer Research Fund, cervical screening prevents between 1,100 and 3,900 cases of cervical cancer each year.73 • Coverage of the target age group by the NHS cervical screening programme has increased from 45% in 1988-89 to 83.7% in 1999-2000. • New developments to improve the accuracy of cervical cytology are in the pilot stage. It is likely that liquid-based cytology (LBC) will reduce the

Cytological terms

Histological equivalent*

Mild dyskaryosis Moderate dyskaryosis Severe dyskaryosis

CIN 1 (mild dysplasia) CIN 2 (moderate dysplasia) CIN 3 (severe dysplasia or carcinoma in situ)

CIN, cervical intraepithelial neoplasia. The correlation between status on smear and on histology is not close.

Follow-up Borderline or mild dyskaryosis ±'human papilloma virus In the absence of signs or symptoms: (a) treat vulval warts if present (see p. 44); (b) repeat the smear in 6 months. If unchanged, refer for colposcopy. If negative; then

224 GYNAECOLOGICAL PROBLEMS

(c) repeat in a further 6 months. If this is still negative the woman can revert to 3-yearly smears. Note: An alternative view is that referral of all women with mild dyskaryosis would be more cost-effective.76 The possibility that a woman is likely to default from follow-up would sway the balance in favour of immediate referral. Moderate or severe dyskaryosis

* Refer for early colposcopy. Stress that this is part of the screening process and does not mean that the patient has cancer. * Advise the woman to continue her contraception, including the COC; pregnancy makes management more difficult.

After colposcopy and treatment

* Follow-up at 6 and 12 months (by gynaecologist). * Then smear yearly for 5 years (GP or gynaecologist). * If normal, then revert to 3-yearly smears. After hysterectomy with a CIN history

* Perform vault smears at 6 and 12 months. Further smears every 3 years are only needed if there is a suspicion that the condition was not completely removed.

REFERENCES 1. Zhang WY, Po AL. Efficacy of minor analgesics in primary dysmenorrhoea: a systematic review. Br J Obst Gyn 1998; 105(7): 780-89. 2. Wilson M, Farquhar C. Dysmenorrhoea. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org 3. Gokhale LB. Curative treatment of primary (spasmodic) dysmenorrhoea. Indian J Med Res 1996; 103: 227-31. 4. Wilson ML, Murphy PA. Herbal and dietary therapies for primary and secondary dysmenorrhoea. Cochrane Review. In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. 5. Farquhar C, Sutton C. The evidence for the management of endometriosis. Curr Opin Obst Gynaecol 1998; 10 (4): 321-32. 6. Moore J, Kennedy S, Prentice A. Modern combined oral contraceptives for pain associated with endometriosis (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. 7. Farquhar C. Endometriosis. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org 8. Vercellini P, Cortesi I, Crosignani PG. Progestogens for symptomatic endometriosis - a critical analysis of the evidence. Fertil Steril 1997; 68(3): 393-401. 9. DTB. Managing endometriosis. Drug Ther Bull 1999; 37: 25-9. 10. Pattie MA, Murdoch BE, Theodoras D, Forbes K. Voice changes in women treated for endometriosis & related conditions: the need for comprehensive vocal assessment. / Voice (US) 1998; 12: 366-71. 11. DTB. Surgical management of menorrhagia. Drug Ther Bull 1994; 32: 70-2. 12. RCOG. The initial management of menorrhagia. Evidence-based clinical guidelines, No. 1.

13. 14. 15. 16. 17.

18. 19. 20.

21.

22.

London: Royal College of Obstetricians and Gynaecologists, 1999. Lethaby A, Farquhar C, Cooke I. Antifibrinolytics for heavy menstrual bleeding (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. Centre for Reviews and Dissemination. The management of menorrhorragia. Effective Health Care, 1995. DTB. Levonorgestrel intra-uterine system for menorrhagia. Drug Ther Bull 2001; 39 (11). Iyer V, Farquhar C, Jepson R. Oral contraceptive pills for heavy menstrual bleeding (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. Lethaby A, Irvine G, Cameron I. Cyclical progestogens for heavy menstrual bleeding (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. Gannon MJ et al. A randomised trial comparing endometrial resection and abdominal hysterectomy for the treatment of menorrhagia. BMJ 1991; 303: 1362-4. Soloman CG. The epidemiology of polycystic ovary syndrome. Prevalence and associated disease risk. Endocrinol Metab Clin N Am 1999; 28 (2): 247-63. Wild S, Pierpoint T, Mckeigue P, Jacobs H. Cardiovascular disease in women with PCOS at long term follow up: a retrospective cohort study. Clin Endocrinol 2000; 52: 595-600. Farquhar C, Lee O, Toomath R, Jepson R. Spironolactone versus placebo or in combination with steroids for hirsutism and/or acne (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000. Moghetti P, Castello R, Negri C et al. Metformin effects on clinical features, endocrine and metabolic profiles and insulin sensitivity in polycystic ovary syndrome: a randomised, double blind, placebo-controlled 6-month trial, followed by open, long-term clinical evaluation. / Clin Endocrinol Metab 2000; 85: 139-46.

REFERENCES 225

23. Bingham JS. What to do with patients with recurrent vulvovaginal candidiasis. Sex Transm In/1999; 75: 225-7. 24. Watson MC, Grimshaw JM, Bond CM et al. Oral versus intra-vaginal imidazole and triazole anti-fungal treatment of uncomplicated vulvovaginal candidiasis (thrush) (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. 25. Bisschop MP, Merkus JM, Scheyground H, van Clusen J. Co-treatment of the male partner in vaginal candidosis: a double blind randomised controlled study. Br } Obstet Gynaecol 1986: 93: 79-81. 26. Potter J. Should sexual partners of women with bacterial vaginosis receive treatment? Br J Gen Pract 1999; 49: 913-18. 27. Metters JS, Catchpole M, Smith C et al. Chlamydia trachomatis; summary and conclusions of CMO's expert advisory group. London: Department of Health,1998. 28. Hills SD, Owens LM, Marchbanks PA et al. Recurrent chlamydial infections increase the risks of hospitalisation for ectopic pregnancy and pelvic inflammatory disease. Am f Obstet Gynecol 1997; 176: 103-7. 29. Miettinen AK, Heinonen PK, Laippala P, Paavonen J. Test performance of ESR & C-reactive protein in assessing the severity of acute PID. Am J Obstet Gynecol 1993; 169: 1143-9. 30. Teisala K. Removal of an IUD and the treatment of acute PID. Ann Med 1989; 21: 63-5. 31. Larsson B, Wennergren M. Investigation of Cu-IUD for possible effect on frequency and healing of PID. Contraception 1981; 24: 137-49. 32. Soderberg G, Lingren S. Influence of an IUD on the course of acute salpingitis. Contraception 1981; 24: 137-43. 33. Robinson AJ, Kell P. Male partners of women with pelvic infection should be traced (letter). BMJ 1995; 311: 630. 34. Kinghorn GR et al. Clinical and microbiological investigation of women with acute salpingitis and their consorts. Br } Obst Gyn 1986; 93: 869-80. 35. Kamwendo F et al. Gonorrhoea, genital chlamydia infection and non-specific urethritis in male partners of hospitalized women treated for acute pelvic inflammatory disease. Sex Trans Dis 1993; 20: 143-6. 36. Dimmock PW, Wyatt KM, Jones PW, O'Brien PM. Efficacy of selective serotonin re-uptake inhibitors in premenstrual syndrome: a systematic review. Lancet 2000; 356: 1311-36. 37. Wyatt KM, Dimmock PW, Jones PW, O'Brien SP. Efficacy of vitamin B-6 in the treatment of premenstrual syndrome: systematic review. BMJ 1999; 318: 1375-81. 38. Anon. Vitamin B6 for premenstrual syndrome. Bandolier Jan 2000. 39. Wyatt KM, Dimmock PW, Ones P et al. Efficacy of progesterone and progestogens in the management of PMS. BMJ 2001; 323 (7316): 776-80. 40. Anon. Evening primrose oil for premenstrual syndrome. Bandolier Aug 2000. 41. Bagshawe KD, Dent J, Webb J. Hydatiform mole in England and Wales 1973-1983. Lancet 1986; 2: 673-7. 42. Hope S, Rees M, Brockie J 1999. Hormone replacement therapy - a guide for primary care. Oxford: Oxford University Press, 1999. 43. Korhonen MO, Symons JP, Hyde BM et al. Histological classification and pathological findings for endometrial biopsy specimens obtained from 2964 perimenopausal

and postmenopausal women undergoing screening for continuous hormone replacement therapy. Am J Obstet Gynaecol 1997; 176: 377-80. 44. MacLennan A, Lester S, Moore V. Oral oestrogen replacement therapy versus placebo for hot flushes (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. 45. Edington RF, Chagnon JP. Clonidine for menopausal flushing. Can Med Assoc J 1980; 123: 23-6. 46. Nagamani M, Kelver ME, Smith ER. Treatment of menopausal flashes with transdermal administration of clonidine. Am J Obstet Gynecol 1987; 156: 561-5. 47. Scambia G, Mango D, Signorile PG et al. Clinical effects of a standardised soy extract in postmenopausal women: a pilot study. Menopause 2000; 7: 105-11. 48. Anon. Alternatives for the menopause. Bandolier Oct 1998; 56-9. 49. Weiderpass E, Baron JA, Adami HO et al. Low potency oestrogen and risk of endometrial cancer: a case control study. Lancet 1999; 353: 1824-8. 50. Cardozo L, Bachmann G, McClish D et al. Meta-analysis of estrogen therapy in the management of urogenital atrophy in postmenopausal women: second report of the Hormones and Urogenital Therapy Committee. Obstet Gynecol 1998; 92: 722-7. 51. Hunter MS. Depression and the menopause. BMJ 1996; 313: 350-1. 52. Gath D et al. Depression and the menopause. BMJ 1990; 300: 1287-8. 53. MeReC. Hormone replacement therapy 2: new preparations. MeReC Bulletin 1997; 8: 17-20. 54. Norman RJ, Flight IHK, Rees MCP. Oestrogen and progestogen hormone replacement therapy for perimenopausal and post-menopausal women: weight and body fat distribution (Cochrane Review). In: The Cochrane Library, Issue 3. Oxford: Update Software, 2001. 55. Rees M, Purdie DW. Management of the menopause. The handbook of the BMS. London: BMS Publications, 1999. 56. Writing group for the PEPI trial. Effects of hormone therapy on bone mineral density. Results from the postmenopausal estrogen/progestin interventions (PEPI) trial. JAMA 1996; 276: 1389-95. 57. Royal College of Physicians and The Bone and Tooth Society of Great Britain. Osteoporosis: clinical guidelines for prevention and treatment. Update on pharmacological interventions and an algorithm for management. London: RCP, 2000. Online. Available: www.rcplondon.ac.uk 58. Hulley S, Grady D, Bush T et al. Randomized trial of estrogen plus progestin for secondary prevention of coronary heart disease in postmenopausal women. Heart and estrogen/progestin replacement study (HERS) research group. JAMA 1998: 280(7): 605-13. 59. Mosca L, Collins P et al. Hormone replacement therapy and cardiovascular disease. A statement for healthcare professionals from the American Heart Association. Circulation 2001; 104: 499-503. 60. New Zealand Guidelines Group. Best practice evidencebased guidelines for the appropriate prescribing of hormone replacement therapy. Online. Available: www.nzgg.org.uk 61. Collaborative Group on Hormonal Factors in Breast Cancer (1997). Breast cancer and HRT: collaborative re-analysis of data from 51 epidemiological studies of 52,705 women with breast cancer and 108,411 women without breast cancer. Lancet 1997; 350: 1047-59.

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62. Committee on Safety of Medicines. EPINET message from the chairman 9.10.97. 63. Gapstur SM, Morrow M, Sellers TA. Hormone replacement therapy and risk of breast cancer with a favorable histology: results of the Iowa Women's Health Study. JAMA 1999; 28: 2091-7. 64. CSM. Risk of venous thromboembolism with hormone replacement therapy. Curr Prob Pharmacovigil 1996; 22. Online. Available: www.mca.gov.uk 65. Hope S, Rees M, Brockie J. Hormone replacement therapy a guide for primary care. Oxford: Oxford University Press, 1999. 66. Nathorst-Boos }, Hammer M. Effect on sexual life a comparison between tibolone and a continuous estradiol-norethisterone acetate regimen. Maturitas 1997; 26:15-20. 67. Kokcu A, Cetinkaya MB, Yanik F et al. The comparison of effects of tibolone and conjugated estrogenmedroxyprogesterone acetate therapy on sexual performance in postmenopausal women. Maturitas 2000; 36: 75-80. 68. Varila E, Wahlstrom T, Rauramo I. A 5-year study of the use of a levonorgestrol intra-uterine system in women receiving hormone replacement therapy. Fertil Steril 2001; 76: 969-73.

69. Pitkin J. Contraception and the menopause. Maturitas 2000; 1: S29-S36. 70. DTB. Hormone replacement therapy. Drug Ther Bull 1994; 34: 81-4. 71. Gebbie AE. Contraception for women over 40. The management of the menopause, annual review (Ed. John Studd). London: Parthenon Publishing, 1998, pp. 67-80. 72. NHS Cancer Screening Programmes. Cervical cancer - incidence, mortality and risk factors, 2002. Available: www.cancerscreening.nhs.uk/cervical/ risks.html 73. Sasieni PD, Cuzick J, Lynch-Farmery E et al. Estimating the efficacy of screening by auditing smear histories of women with and without cervical cancer. Br J Cancer 1996; 73: 1001-5. 74. Austoker J. Screening for cervical cancer. BMJ 1994; 309: 241-8. 75. Martin-Hirsch P, Jarvis G, Kitchener H, Lilford R. Collection devices for obtaining cervical cytology samples (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 76. Hammond R. Management of women with smears showing mild dyskaryosis. BMJ 1994; 308: 1383-4.

12

CHAPTER CONTENTS Hormonal contraception 228 The combined oral contraceptive pill 228 The progestogen-only pill 234 Injectable progestogens 236 Etonogestrel-releasing implant: Implanon 238 Non-hormonal methods of contraception Intrauterine devices 238 Barrier methods 241 Emergency contraception

Contraception and sexual problems

238

242

Contraception in special cases 243 Contraception and young people 243 Contraception and older women 244 Contraception and learning disability 244 Sterilization 244 Termination of pregnancy Infertility

246

Adoption 247 Sexual problems 247 References

250

245 Advice: Glasier A, Gebbie A. Handbook of family planning and reproductive healthcare, London: Churchill Livingstone, 2000. Rowlands S. Managing family planning in general practice. London: Radcliffe Medical Press, 1997. Faculty of Family Planning & Reproductive Health Care. Online. Available: www.ffprhc.org.uk.

When giving contraceptive advice it is good practice if this is backed up with appropriate and accessible written information. The Family Planning Association (fpa) provides a range of leaflets covering all methods, which is regularly revised and is available through the local Health Promotion Unit. These leaflets should be used when a patient is deciding on a method, initiating a method and from time to time as a refresher. Recording that a patient has been given the relevant leaflet, has read it and discussed it will help to reduce litigation risk.1 Patients wanting to go to a family planning clinic or who are being referred from general practice can get details of a convenient clinic from:

fpa, 2-12 Pentonville Road, London N1 9FP, tel. 020 7837 4044; www. (the web site allows a search on a map with details right down to street level).

227

228

CONTRACEPTION AND SEXUAL PROBLEMS

HORMONAL CONTRACEPTION THE COMBINED ORAL CONTRACEPTIVE (COC) PILL Assessment of the patient

The following are the essential components of the assessment before initiating the COC: (a) menstrual and obstetric history; (b) history of migraine - presence of aura/focal nature; (c) past or current illnesses which might represent contraindications; (d) family history of venous thromboembolism (VTE), MI, cerebrovascular accident (CVA), hypertension and breast cancer; (e) current drug therapy (prescribed and OTC); (f) allergies; (g) BP measurement (defer starring the COC until 8 weeks after delivery in women who have had pre-eclampsia, even if the BP is normal); (h) baseline height/weight: BMI (important, as it is both an arterial and a venous risk factor).

Contraindications The World Health Organization gives four categories of condition in which use of the COC is (or is not) contraindicated:2 • WHO1 is a condition for which there is no restriction on use of the method. • WHO2 is when the advantages of the method generally outweigh the theoretical or proven risks. Table 12.1

• WHO3 is when the theoretical or proven risks usually outweigh the advantages but the method can be used with caution and additional care as a 'method of last choice'. • WHO4 is a condition which represents an unacceptable health risk and the method should not be used.

Absolute contraindications (WHO4) Cardiovascular

(a) Any past arterial or venous thrombosis. (b) Ischaemic heart disease and cardiomyopathies. (c) Severe or combined risk factors for venous or arterial disease (see Tables 12.1 and 12.2). (d) Migraine, if severe or focal.3 The following suggest transient ischaemia and preclude use of the COC: loss of a part of the visual field; unilateral weakness/parasthesiae; disturbance of speech; first ever migraine attack after starting COC; status migrainosus. Migraine without aura is safe (this includes blurred vision, photophobia, phonophobia and flashing lights affecting the whole visual field). (e) Transient ischaemic attacks (TIAs). (f) Hyperlipidaemia - avoid COC if total cholesterol >8mmol/L or triglyceride >4.5mmol/L. (g) Thrombophilias - inherited, e.g. factor V Leiden mutation, or acquired, e.g. lupus anticoagulant. (h) Post-splenectomy if platelet count >500 X 109/L.

Risk factors for venous thromboembolism4

Risk factor

Absolute contraindication

Relative contraindication

Family history of VTE in 1 st degree relative age <45

Known coagulation abnormality, e.g. factor V Leiden or antiphospholipid antibodies

Overweight Immobility

BMI >39 Confined to bed Leg in plaster Past thrombosis Sclerosing treatment >50

Normal coagulation factors including factor V Leiden, protein C, protein S, antithrombin III BMI 30-39 Confined to wheelchair

Varicose veins Age

Extensive varicose veins >35

Adapted from Handbook of family planning and reproductive healthcare, Glasier A, Gebbie A (Eds), 2000, by permission of the publisher Churchill Livingstone.

HORMONAL CONTRACEPTION

(i) Other conditions predisposing to thrombosis: blood dyscrasias; autoimmune disorders, e.g. SLE; Klippel-Trenaunay syndrome; severe polycythaemia; elective major or leg surgery (see p. 232); during leg immobilization; before and after all varicose vein treatments (see Table 12.1 and p. 232); travel to above 4500 m; inflammatory bowel disease (ulcerative colitis and Crohn's disease, both are associated with hypercoagulability during exacerbations and so the COC should not be used during such exacerbations or in those with severe disease). (j) Past cerebral haemorrhage, (k) Vascular malformations of the brain. (1) Structural heart disease, e.g. septal defects and valvular pathology because of the risk of systemic emboli in the presence of arrythmias or shunts, (m) Pulmonary hypertension. Liver (a) Active liver disease with abnormal LFTs (safe to start COC 3 months after LFTs have returned to normal). (b) Cholestatic jaundice if induced by COC or history of this condition in pregnancy. (c) Dubin-Johnson or Rotor syndrome. (d) Liver adenoma, carcinoma or focal nodular hyperplasia. (e) Gallstones. (f) The acute hepatic porphyrias.

229

Sex-steroid-COC-induced conditions (a) Chorea. (b) COC-induced hypertension. (c) Acute pancreatitis linked with hypertriglyceridaemia. (d) Pemphigoid gestationis. (e) Haemolytic uraemic syndrome or thrombotic thrombocytopenic purpura. (f) Otosclerosis. (g) Stevens-Johnson syndrome. (h) Trophoblastic disease - until HCG levels are undetectable. (i) SLE (also because of thrombotic risk). Pregnancy Undiagnosed genital tract bleeding Oestrogen-dependent neoplasms These (e.g. breast cancer) are absolute contraindications. Anxiety about COC safety If not relieved by counselling.

Relative contraindications (WHO2 and WHO3) (a) Risk factors for venous and arterial disease (Tables 12.1 and 12.2). The presence of a single risk

Table 12.2 Risk factors for arterial disease4 Risk factor

Absolute contraindication

Relative contraindication

Family history of arterial CVS disease in 1 st degree relative age <45 Cigarette smoking Overweight Migraine

Abnormal lipid profile 30+ per day BMI >39 Migraine with focal aura Severe migraine Rx Ergot derivative

Normal lipid profile 1st attack in relative age 45 or more 5-30 per day BMI 30-39 Migraine without aura Migraine with non-focal aura Rx 5-HTi agonist

Age

>50

>35

Hypertension Diabetes

BP > 160/95 Retinopathy, nephropathy or neuropathy Duration of diabetes mellitus >20 years Age 30+

BP 140-159/90-95 No complications Age <30

Adapted from Handbook of family planning and reproductive healthcare, Glasier A, Gebbie A (Eds), 2000, by permission of the publisher Churchill Livingstone.

230

CONTRACEPTION AND SEXUAL PROBLEMS

factor is WHO2, provided it is not serious enough to make it an absolute contraindication. (b) Superficial thrombophlebitis (WHO2). (c) Breast cancer in remission - sometimes with specialist advice. (d) Cervical intraepithelial neoplasia. (e) Hyperprolactinaemia under specialist supervision (WHO2). (f) Severe depression (WHO2). (g) Sickle-cell disease (homozygous) (WHO2). Arterial and venous thrombosis The risk factors for VTE and arterial disease should be assessed separately. Obesity and age are risk factors common to both conditions. More than one risk factor in either the VTE or arterial disease boxes means that all COC formulations should be avoided. Many of the risk factors can be viewed as being on a sliding scale, for instance there is not suddenly a problem with age on the 35th birthday. Smoking status, weight and immobility are the only factors that may be changed in the future and the suitability for the COC reviewed.

Choosing a combined pill • The initial dose of oestrogen should normally be in the range 20-35 jxg combined with a low or standard dose of progestogen (Appendix 13). • Since June 1999, desogestrel- and gestodenecontaining pills have been recommended as firstline COCs.5 However, in view of the apparent increased risk of VTE with these preparations6 (odds ratio 1.7), the slightly increased risk of VTE should be explained to the patient and these pills should not be used in those with a risk factor for VTE. There is insufficient evidence to comment on norgestimate in relation to VTE.7 • Desogestrel- and gestodene-containing pills are probably best avoided for young first-time users. Their risk of VTE is 3.1 times the risk they would run with a second-generation preparation.6 These brands are useful, however, for those who have side-effects, for acne sufferers or those with cycle control problems. • Women react individually to the pill; if sideeffects are experienced, it is well worth trying

at least one other brand before abandoning the method.

Recommendations The first time user Guidelines on first prescription of the COC were produced in 2000:8 (a) BP should be measured and recorded before first prescription of a COC. (b) No blood tests are necessary before first prescription of a COC without specific clinical indication. (c) In asymptomatic women breast and pelvic examinations are not necessary before first prescription of a COC.9 Higher doses of oestrogen Formulations containing 50|xg of ethinylestradiol should not be used unless specific individual circumstances warrant a higher dose: (a) long-term use of an enzyme-inducing drug; (b) persistent breakthrough bleeding (BTB) on a standard strength COC, provided no other cause is found; (c) past true COC method failure, suggesting unusually rapid metabolism or malabsorption (an alternative is tricycling and shortening the pill-free interval (PFI) to 4 days - see below). Special cases * Recommend an alternative method when there has been a previous failure of COC or where pill efficacy may be reduced because the patient: (a) is on long-term hepatic enzyme-inducing drugs, e.g. rifampicin, griseofulvin, phenytoin, carbamazepine or St John's wort; or (b) has severe malabsorption. * If an alternative method is unacceptable, consider: (a) starting a high-dose pill, or two lowdose pills, to give at least 50 |xg of ethinylestradiol; or (b) running three packs of pills together (the 'tricycle' regimen) plus reducing the 3-monthly PFI to 4 days; and

HORMONAL CONTRACEPTION

(c) checking the serum progesterone on day 21 to be sure that ovulation has been suppressed.10 * If breakthrough bleeding still occurs, or the 21-day progesterone is not suppressed, try 60 juig per day. It would be wise to obtain specialist advice before increasing the dose above 60 |xg of oestrogen.

231

Changing the pill (a) Same or higher strength oestrogen but the same progestogen. Start it after the 7-day break. No extra precautions are necessary. (b) Lower strength oestrogen or a different progestogen. Omit the 7-day break. If the break is not omitted, extra precautions are needed for 7 days. Postponing a period

Phased preparations

This is possible (see below).

Phased preparations:

(a) give a better bleeding pattern for a lower monthly dose (has been shown for levonorgestrel preparations11); (b) are more expensive than fixed-dose preparations, not least because these products attract two dispensing fees (biphasics) or three dispensing fees (triphasics) in the UK; (c) have a reduced margin for error, especially early in the packet; (d) may cause premenstrual tension-like symptoms towards the end of the packet; (e) are less flexible when a patient wants to postpone a period.

Taking the pill: procedure and advice

Stopping the COC Alternative methods of contraception are needed from the day of stopping the COC, not the end of the PFI.

Advice Mode of action The main action of the pill is to suppress the normal cycle so that ovulation does not occur; the 'periods' while on the pill are withdrawal bleeds. Within 7 days of use the ovaries are fully suppressed. Conversely, during the PFI there is no follicular activity, unless the PFI is lengthened beyond 7 days.

Starting the pill

Risks

(a) Days 1-3 of the cycle. If started on or before day 3, then no other precautions are necessary. (b) After day 3, other precautions should be used for the first 7 days. (c) Changing from the POP. If the COC is started on day 1 of the cycle, no other precautions are necessary. (d) After childbirth. If starting at the end of the third week postpartum, no other precautions are needed. A later start necessitates extra precautions for 7 days. Note that the earliest recorded ovulation after delivery is day 30,12 so waiting until a postnatal examination is not an option unless a woman is exclusively breast-feeding. (e) After miscarriage or TOP. Start within 24 hours. If starting later, extra precautions are needed for 7 days. The earliest recorded ovulation after TOP is day 16.12

(a) Venous thromboembolism. The risk of VTE while using any COC is less than the risk of VTE during pregnancy. (b) Myocardial infarction. The risk of MI on a COC is confined to smokers and those with arterial risk factors. Women who do not smoke, who have their BP checked and who do not have hypertension or diabetes are at no increased risk of MI on a COC, regardless of their age.4 (c) Ischaemic stroke. Among women who do not smoke, have their BP checked and who do not have hypertension, the risk is increased about 1.5-fold.4 (d) Gallstones. COCs may accelerate the presentation of cholelithiasis in those who are predisposed.4 Their use should be avoided in those with known gall bladder disease. They can be used after cholecystecomy but not after medical treatment for gallstones;

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CONTRACEPTION AND SEXUAL PROBLEMS

(e) Hypertension. COCs can induce hypertension, particularly in the early months of use.13 About 1% of COC users become clinically hypertensive with modern formulations. Pill-induced hypertension should not be treated with antihypertensive drugs, but the pill stopped and observation continued. (f) Cancer. There is a possible increased risk of cancer of the breast and cervix, although this is mitigated by a 2-3-fold reduction in risk of cancer of the ovary and the endometrium4 and by a reduction in the risk of cancer of the colon.14 The excess breast cancer risk is related to how recently the woman was using the COC, provided she was on it for at least a year. Current users have a relative risk of 1.24, which falls to 1.16 1^4 years after stopping and to 1.07 5-9 years after stopping. After that the excess risk disappears. Because the baseline risk in young women is low, a relative risk of 1.24 is not a major concern (1.5 extra cancers per 10,000). By the age of 40-44, however, COC use is associated with 30 extra cancers per 100,000 women.15 Side-effects There are side-effects, but most wear off after the first few cycles, especially weight gain, nausea and breast tenderness. Nausea may be reduced by taking the pill at night. Depression, if it occurs, tends to continue; it may respond to pyridoxine 50 mg daily. Periods These may be lightened and BTB may occur until the most appropriate pill for the woman is found. BTB is not a reason for stopping the pill in midpacket. Missed pills The most risky time for pills to be forgotten is on either side of the pill-free week. Forgetting in the middle of a packet is less likely to give rise to breakthrough ovulation or pregnancy. (a) If less than 12 hours late, the woman should: - take the missed pill; - take the next one on time; - assume no loss in contraception.

(b) If more than 12 hours late, she should: -take the (last) missed pill; - take the next pill at the normal time; -take extra precautions for 7 days. (c) If more than 12 hours late and the 7 days of precautions run into the PFI, she should, in addition: -omit the PFI and start a new pack immediately; - disregard any bleeding; - report if the next withdrawal bleed fails to occur. (d) If two or more pills are missed, consider emergency contraception (see p. 242) if: -four or more pills are missed mid-pack; or -two or more pills are missed from the first seven pills in a packet (if two or more pills are missed from the last seven pills in a packet, emergency contraception is not necessary provided that the PFI is omitted). Diarrhoea or vomiting She should take extra precautions for the period of illness and for 7 days afterwards, as above. However, it is known that diarrhoea has to be of dysenteric proportions to reduce pill absorption. Antibiotics Certain antibiotics may reduce the effectiveness of the COC. Ampicillin, amoxicillin, coamoxiclav, broad-spectrum cephalosporins and tetracyclines can possibly alter the reabsorption of oestrogen (this does not occur with narrow-spectrum antibiotics, nor with trimethoprim or erythromycin). Rifampicin, griseofulvin and antiretroviral drugs used for HIV infection induce liver enzymes. Oral antifungal agents have also been associated with anecdotal reports of COC failure. * Extra precautions should be taken during the treatment and for 7 days thereafter. If this runs into the PFI the next packet should be started without a break. Rifampicin is such a powerful enzyme-inducer that extra precautions should be taken for 4 weeks even after a 2-day course and elimination of PFIs during this time.8 * Long-term antibiotics: If starting the patient on long-term treatment, extra precautions need to be

HORMONAL CONTRACEPTION

taken for the first 2 weeks. Thereafter, if the bleeding pattern is normal, adequate contraception can be presumed. Surgery The COC should be stopped from 4 weeks before until 2 weeks after any major surgery, varicose vein surgery or sclerotherapy, or any operation likely to be followed by immobilization (e.g leg surgery).4 It is otherwise not necessary to stop the COC.

Postponing a period Monophasic pills Patients on a fixed-dose combined pill can postpone the next withdrawal bleed by starting the next packet immediately, omitting the PFI. Phased preparations * If on Synphase, another packet can be taken immediately following the first. * If taking other phased preparations there would be an abrupt drop in progestogen levels if the above regimen were followed, leading to a risk of bleeding. Advise: (a) tablets from the last phase of a spare packet can be taken, to give 7 (TriNovum) or 10 (Logynon, Trinordiol, Tri-Minulet, Triadene) or 14 (Binovum) days' postponement; or (b) a packet can be started of the next higher fixed-dose pill (up the ladder; see Appendix 13) omitting the PFI. If postponement by a few days is needed, e.g. to avoid a bleed at weekends, the necessary number of pills from a fresh pack should be taken, and the rest of that pack thrown away. If on phased preparations, they should follow the principles above. Note: ED preparations. In all the above advice, the seven inactive pills in ED preparations should be discarded.

Follow-up * The patient should be seen again at 3 months, or earlier if side-effects occur. Do not give repeat

233

prescriptions without someone seeing the patient and at least checking the blood pressure. * Once established on the pill, see the patient 6-monthly and: (a) assess whether there are any new risk factors, including migraine; (b) check whether the patient is smoking; (c) ask about side-effects; (d) check BP (discontinue if BP increases to and remains at 160/95mmHg). If the BP is satisfactory 2 years after commencement of the COC, BP checks can be extended to annually in women without risk factors or relevant diseases.

Changing the pill because of side-effects The pill ladders (see Appendix 13) provide an easy guide to the hormone dose and the progestogen type in each pill. When changing a pill because of side-effects, the choice lies between going up or down one ladder, or changing to a pill with a different progestogen. Breakthrough bleeding (BTB) BTB occurs in up to 30% of users in the first few cycles, but tends to settle from the third cycle onwards. Unless prior warning of this is given, discontinuation rates will be high, especially in the young. * If BTB occurs in the first 3 months, ask whether pills have been missed; encourage the patient to persevere. * If BTB continues beyond 3 months, exclude lesions of the cervix and problems with taking the pill, e.g. vomiting. Also encourage smokers to quit, as they are more likely to have BTB.16 Give a pill with a higher progestogen dose, i.e. move up the ladder, change to a progestogen with better cycle control, e.g. gestodene or change to a triphasic formulation of the same progestogen. If this fails consider increasing the oestrogen content as well. * If BTB occurs when previous control had been good, exclude lesions of the cervix, chlamydia infection, interacting drugs, gastrointestinal disorder and a change to a vegetarian diet.

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CONTRACEPTION AND SEXUAL PROBLEMS

Absent withdrawal bleeds (WB)

* Explain that this is not unsafe and does not signify overdosage. * Missing one WB does not need any action. If two are missed, exclude pregnancy. * If the patient is concerned despite reassurance, consider a switch to a triphasic pill. Oestrogen withdrawal headache during pill-free interval

* Consider advising the patient to use three packets consecutively without a break, followed by a 7-day break (tricycle regimen). Relative oestrogen excess As characterized by:

(a) (b) (c) (d) (e) (f) (g)

nausea; dizziness; premenstrual tension; cyclical weight gain; 'bloating'; vaginal discharge without infection; breast tenderness.

* Change to 20 |ag pill (Femodette, Mercilon or Loestrin 20) or to a more progestogenic pill, e.g. Loestrin 30 or Norimin. Relative progestogen excess As characterized by:

(a) (b) (c) (d) (e) (f) (g)

dry vagina; sustained weight gain (increased appetite); depression; loss of sexual drive; lassitude; acne; hirsutism.

* Change to a more oestrogenic pill, e.g. Ovysmen/Brevinor, Trinordiol/Logynon, Loestrin 20 or Cilest; or to Dianette; or to a less progestogendominant formulation, e.g. gestodene or desogestrel.17 Those with weight gain may be better on Yasmin.

THE PROGESTOGEN-ONLY PILL (POP)

Patients for whom the POP is particularly indicated (a) Older women. Those over 45 years old without risk factors, and those over 35 years old who smoke. (b) Those with medical contraindications to oestrogen, including a personal history of VTE. (c) Those with risk factors for arterial disease, including hypertension, diabetes mellitus, focal migraine and smokers aged >35. (d) Lactating mothers - progestogen in the breast milk has no adverse effect on the baby.18 (e) Those who choose it, especially those aged over 25 or 30 years, and who accept that it is less reliable than the COC.

Absolute contraindications (WHO4) (a) Severe arterial disease or at very high risk. (b) Serious side-effect on COC not clearly due to oestrogenic component. (c) Undiagnosed genital tract bleeding. (d) Actual or possible pregnancy. (e) Recent trophoblastic disease - until HCG levels are undetectable. (f) Acute porphyria - history of an actual attack. (g) Anxiety about POP safety - if not relieved by counselling.

Relative contraindications (WHO2 and WHO3) (a) Risk factors for arterial disease, e.g. hypertension and hyperlipidaemia. (b) Past subarachnoid haemorrhage (WHO2). (c) Breast cancer (WHO3). (d) Current liver disorders (WHO2). (e) Acute porphyria - latent without a past attack. (f) Previous ectopic pregnancy (WHO2). (g) Functional ovarian cysts. (h) The young - because of the strict adherence necessary and its lower efficacy than the COC.

HORMONAL CONTRACEPTION 235

Taking the POP: procedure and advice Starting the POP: (a) Day 1. If starting on day 1 (i.e. the first day of the period) then no other precautions need be taken. (b) Later in the cycle. Extra precautions should be taken for the first 7 days. (c) Switch from COC. If changing from a COC, go straight on to the POP without a 7-day break. Extra precautions are not necessary but it may be necessary to take the pill at a different time of day. (d) Postpartum. Start at 3 weeks postpartum. Extra precautions are only needed for the next 7 days if starting later than that. Note: Time of day. The pill must be taken regularly at the same time each day (ideally at least 4 hours before the most frequent time of intercourse to ensure maximal mucus-thickening effect). Advice (a) Efficacy. Explain that, in women aged 40 or over, the POP has a similar efficacy to the COC in women aged 25. The efficacy is lowest in women with no alteration in cycle activity and is greatest in those with complete suppression of cycles and consequent amenorrhoea (roughly 40% of women continue to ovulate, in 40% there is variable interference with the follicular and luteal phase, and in 20% ovulation is inhibited completely). (b) Bleeding pattern. From (a) it follows that 40% have similar cycles to their normal pattern, 40% have shorter cycles (which may gradually lengthen towards normal over time) with episodes of spotting or breakthrough bleeding and 20% have long cycles or amenorrhoea. (c) Safety. There is no evidence of an increased risk of thrombosis. However, there is some evidence of an increased risk of breast cancer for use in the previous 5 years; relative risk 1.17 (SD = 0.09, p = 0.06), but no increase 10 or more years after stopping.15 (d) Antibiotics. The POP is not affected by antibiotics, except rifampicin and griseofulvin (see (h) below).

(e) Missed pills. If a pill is missed or delayed for more than 3 hours then alternative precautions need to be taken for 7 days. (f) Diarrhoea or vomiting. If vomiting or diarrhoea occur, continue to take the pill regularly but take extra precautions during the attack and for the next 7 days. (g) Obesity. Give patients weighing over 70 kg two pills per day. (h) Enzyme-inducing drugs. Epileptics and others on enzyme-inducing drugs are probably best advised not to rely on POPs, even if more than one pill daily is given.

Side-effects Non-menstrual These are the usual progestogenic ones such as headaches, tender breasts, acne, depression, weight gain and loss of sexual drive. But because the dose of progestogen is so low, these sideeffects are not that common and often not severe. Irregular bleeding * Examine to exclude a pathological cause. * Do not change to another POP. There is no suggestion that with the current range of POPs changing brand will improve menstrual or nonmenstrual side-effects. This situation will change when the POP containing 75 jag of desogestrel is launched as this inhibits ovulation to a greater degree.19 * Suggest a change to another method. Abdominal pain of gynaecological origin * Consider ectopic pregnancy. If it can be excluded, then refer for ultrasound. It may be due to a functional ovarian cyst. Such a cyst usually resolves without treatment. Amenorrhoea when cycles have been present * Exclude pregnancy before assuming that it is due to the POP.

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CONTRACEPTION AND SEXUAL PROBLEMS

* Encourage the woman to persevere with the POP. She is in the group least likely to become pregnant with it.

Relative contraindications (WHO2 and WHO3)

* Depot medroxyprogesterone acetate (DMPA) Depo-Provera: 150 mg in 1 ml. Shake the preloaded syringe thoroughly before administration. * Norethisterone enantate (NE) - Noristerat: 200 mg in 1ml. Warm the vial close to body temperature before administration.

(a) Risk factors for arterial disease, e.g. hypertension and hyperlipidaemia. (b) Breast cancer (WHO3). (c) Current liver disorders (WHO3). (d) Past severe depression. (e) Women planning a pregnancy in the reasonably near future. (f) Women with risk factors for osteoporosis (WHO3). (g) Women aged over 45 - to ensure optimal bone mineral density on entering the menopause (see below).

Note: NE is not licensed for long-term use. When used for longer than 16 weeks, this use outside the licence must be discussed with the patient.

Before starting

INJECTABLE PROGESTOGENS Preparations

Discuss with the patient the following:

Indications The indications are similar to those for the POP. Patients for whom injectable progestogens are especially indicated are women:

Advantages

(a) likely to forget (or to worry about forgetting) to take daily pills; (b) for whom higher dose long-term progestogens are beneficial, e.g. those with fibroids or endometriosis; (c) with sickle-cell disease: DMPA improves the blood picture and reduces the number of crises.20

Injectables are effective and independent of coitus, with no oestrogenic side-effects. They are 'invisible', which may be important to some women, e.g. those in controlling or abusive relationships. They may enhance lactation and relieve premenstrual and menstrual symptoms. Seizure control has been reported to be improved in some epileptics. In addition, they afford some protection against pelvic infection and may improve endometriosis and sickle-cell disease.

Absolute contraindications (WHO4)

Efficacy

(a) Severe arterial disease or at very high risk. (b) Serious side-effect on COC not clearly due to oestrogenic component. (c) Liver adenoma, severe liver impairment, severe past steroid-associated cholestatic jaundice. (c) Undiagnosed genital tract bleeding. (d) Actual or possible pregnancy. (e) Recent trophoblastic disease - until HCG levels are undetectable. (f) Acute porphyria. (g) hypoestrogenism - not amenorrhoea alone, (h) Anxiety about safety of injectables - if not relieved by counselling.

Injectables are a very effective means of contraception: DMPA failure rates are <0.7 per 100 woman years and NE <1.0 per 100 woman years, i.e. as good as female sterilization. Blood levels are not affected by drugs including enzyme-inducers; there is no justification for increasing the dose or reducing injection intervals for this reason.21 Side-effects Erratic bleeding. This will occur in most women initially. Prolonged episodes of bleeding may occur, but are rarely heavy and decrease over time.22

HORMONAL CONTRACEPTION 237

* Examine (especially the cervix). * Give either: (a) the next injection early (but not earlier than 4 weeks after the last one); or (b) a 21-day course of oestrogens, e.g. the COC, if no contraindication. * Refer if the above do not control the bleeding. * Alternatively the patient may prefer to discontinue the method.

5 years is 1.17 (SD = 0.13), which is not statistically significant. However, it is 0.94 (SD = 0.13), which is also not statistically significant, 10 or more years after stopping use.15 There is a fivefold reduction in the risk of endometrial cancer.20

Amenorrhoea. This is likely, more so the longer the method is used. The amenorrhoea rate with DMPA is around 50% at 12 months.22 With NE, episodes of bleeding are the more usual pattern and the amenorrhoea rate at 12 months is 25%. Explain that these methods make the lining of the womb so thin that there is no monthly shedding. Many women find the amenorrhoea a very acceptable side-effect. Exclude pregnancy (beware of weight gain at successive visits with nausea or breast symptoms). There is concern that injectables can lower ovarian estradiol production. Some cross-sectional studies have shown loss of bone mineral density, while others have not. Prospective studies are reassuring so far.23'24 There is a lack of consensus on any action to be taken with continuous amenorrhoea for over 5 years. Some authorities have recommended checking serum estradiol but this is not a useful proxy indicator for bone mineral density. Bone densitometry is the test of choice but the expense cannot be justified unless the risk is proven. Weight gain. This is possible, through appetite stimulation, although some studies show no effect on weight.21 The weight gain is not generally the result of fluid retention. Modification of diet and behaviour will tend to counteract the weight gain. Any weight gain is less likely with NE. Return of fertility. This may be delayed, with a mean time to ovulation of 18 weeks from the expiry of the last dose of DMPA (maximum 49 weeks). Return of fertility is much faster with NE: mean of 4 weeks and maximum of 26 weeks.25

* Give the first injection within 5 days of onset of menstruation, 6 weeks after delivery, or 1-5 days after miscarriage or termination. The patient should use additional precautions for 7 days after the first injection unless it was given as above. * Use the upper outer quadrant of the buttock or the lateral thigh. The deltoid is used when the patient is obese, as it is important the drug reaches muscle. Warn the patient not to massage the site as this may speed up drug release. * Give subsequent injections: Depo-Provera 150mg 12-weekly; Noristerat 200 mg 8-weekly.

Risks Cancer. There is some evidence of an increased risk of breast cancer in current users but numbers are small. Relative risk for use in the previous

Initiating treatment and follow-up Schedule for the injections

If the patient returns late for the next injection of DMPA or NE, proceed as follows: Up to 5 days from date injection was due§

* Give injection * No extra precautions needed

From 6 days to 10 days from due date

* Consider giving emergency contraception if any episodes of sexual intercourse without use of barriers after 5 days overdue * Give injection * Extra precautions next 7 days * Check pregnancy test in 3 weeks

After 10 days from due date

* Do a pregnancy test -> negative * Depending on timing of last sexual intercourse without barrier method use: (a) if <2 weeks ago or patient unsure; advise extra precautions or abstinence. Repeat pregnancy test 2 weeks after last sex. If negative, give injection. Extra precautions for a further 7 days. Repeat pregnancy test after a further 3 weeks (b) if >2 weeks ago; give injection. Extra precautions for 7 days

§ Assumes patient receiving injections at full intervals, i.e. 12 weeks for DMPA and 8 weeks for NE. Note: If a pregnancy test is positive on the injectable, reassure no convincing evidence of any adverse effect on fetus20 and counsel according to woman's wishes.

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ETONOGESTREL-RELEASING IMPLANT: IMPLANON Etonogestrel-releasing subdermal implant Implanon: 68 mg. (Marketing of Norplant was discontinued in 1999; some women will have Norplant in situ until 2004.) This has the advantage that it lasts for 3 years but is reversible should the need arise. It has a high efficacy, on a par with injectables but there is insufficient experience so far to give an exact figure. The return of fertility after removal of Implanon is much faster than after stopping injectables; 94% have ovulated within 3 months of removal.26 It is about four times faster than Norplant to insert and remove.27 Implanon has a beneficial effect on dysmenorrhoea.28 Disadvantages are the expense and the discomfort of insertion and removal. Otherwise, the advice given to the patient should be similar to that given for injectable progestogens - except women using Implanon are more likely to have persistent bleeding, even if not heavy, than with injectables. If it is implanted other than on the first day of the menstrual cycle, extra precautions are needed for the first 7 days, if there is no previous method providing cover.

Absolute contraindications (WHO4) (a) Severe liver disease - with markedly abnormal LFTs. (b) Progestogen-dependent tumours - liver adenoma, active trophoblastic disease. (c) Acute porphyria. (d) Known or suspected pregnancy. (e) Undiagnosed vaginal bleeding.

Relative contraindications (WHO2 and WHO3) (a) Breast cancer (WHO3).

Before administering the implant * Check weight and blood pressure.

Problems with the implant Menstrual Bleeding patterns are variable even in an individual over time. Frequent irregular bleeding, spotting and prolonged bleeding are all possible - heavy bleeding is rare.28 Amenorrhoea occurs in 20% of users. Non-menstrual29

Acne, mastalgia, headache, weight gain, abdominal pain and decrease in sexual desire. Mood changes are less common than with Norplant and are not usually a reason for a request for removal.

NON-HORMONAL METHODS OF CONTRACEPTION INTRAUTERINE DEVICES (IUD) • IUDs should only be fitted if specific practical training has been obtained and a minimum number of devices are fitted to keep up the necessary skills. • All IUDs with frames have copper wire wound round the stem. GyneFix is a frameless device with six copper cylinders on a thread. Modern coppercontaining IUDs are clinically effective and safe for at least 5 years.30 The T380S device had a licensed lifespan of 10 years - marketing was discontinued in the UK in 2000. • IUDs do not offer any protection against STIs but previous studies that purported to show an increased risk of PID have now been shown to be flawed.31 There is no evidence that prophylactic use of antibiotics for IUD insertion is of significant benefit in healthy women.32 • High-load IUDs protect against ectopic pregnancy. • The second generation IUDs, e.g. Nova-T, Novagard, Multiload Cu 250, Ortho Gyne-T, are no longer first choice as they carry a smaller copper load. The third generation IUDs, e.g. Multiload Cu 375, Ortho Gyne-T 380 Slimline (now discontinued), are more effective, with pregnancy rates

NON-HORMONAL METHODS OF CONTRACEPTION 239

of <1.0 per 100 woman years and a duration of action of 8 years or more. • Cumulative expulsion rates at 5 years for copper lUDs are <10 per 100 women. Expulsion may occur at any time after insertion, but is most likely in the early cycles, especially during menstruation. Patients who are older and of higher parity are less likely to expel their device.

* Examine for infection or malposition of the device. * Prescribe NSAIDs, which may reduce pain and bleeding (see p. 209). * Remove the IUD if it appears to be associated with a change in bleeding pattern. If the pattern does not return to normal, refer for gynaecological assessment.

Absolute contraindications (WHO4)

Pelvic infection

(a) Pregnancy. (b) Unexplained vaginal bleeding. (c) PID - current or in the last 3 months. (d) STI with purulent cervicitis - current or within the last 3 months. (e) Distortion of the uterine cavity - congenital malformation, submucous fibroids. (f) Copper allergy. (g) Wilson's disease. (h) Cavity length <5.5cm - does not apply to frameless devices. (i) Trophoblastic disease.

* Symptoms of infection require examination and endocervical swabs for STIs. * Treat with antibiotics (see p. 215). * If the symptoms settle, the IUD can be left in place. * Actinomyces-like organisms (ALOs) may be found on a cervical smear. (a) If the patient is asymptomatic, the IUD may be left in place.33 Smears should be repeated at 6 months and then yearly. An alternative is to remove the IUD and insert a new one at the same session. This usually results in disappearance of the organisms. Repeat smears as above. If the ALOs persist, either change the IUD again or change to a different method. (b) If the patient has symptoms, i.e. pain or discharge, remove the IUD and send it for microscopy and culture. If ALOs are found, treatment with amoxicillin 3g orally will be needed daily for 3 months.

Relative contraindications (WHO2 and WHO3) (a) Menorrhagia. (b) Anaemia - Hb <9 g/dl. (c) Increased risk of STIs - multiple sexual partners or partner who has multiple partners. (d) Age <20 - as a surrogate of STI risk. (e) AIDS/HIV-positive/high risk of HIV infection. (f) Previous ectopic pregnancy - not for highload copper devices. (g) Nulliparity - increased risk of expulsion. (h) Risk of bacteraemia - valvular heart disease and septal defects (antibiotic cover needed at time of insertion), renal disease/dialysis/transplant and immunosuppressive therapy (not steroids), (i) Severe dysmenorrhoea.

Adverse effects Heavier periods, intermenstrual bleeding, dysmenorrhoea * Warn the patient that these are common in the first few cycles after insertion.

Pregnancy * If symptoms suggest an ectopic pregnancy, admit to hospital; 4% of pregnancies that occur with an IUD in place are ectopic. * If a scan indicates that the pregnancy is intrauterine, gently remove the IUD if the threads are visible and the pregnancy is less than 12 weeks. This will halve the miscarriage rate. If the pregnancy is >12 weeks or no threads are seen, refer early for antenatal assessment. Lost threads * Teach the patient to feel for the threads after each period, or on the first of the month if there is amenorrhoea with the IUS (see p. 240).

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* If the threads are not palpable, warn the patient temporarily to use other precautions, and arrange for USS. * If USS confirms that the device is in situ, leave in place until it is due to be changed. Repeat vaginal examination yearly, and only repeat USS if there is reason to suspect expulsion.

Follow-up * Review after the next period and then annually. * Ask about menstrual blood loss, pelvic pain, vaginal discharge and discomfort to the partner. * Do a pelvic examination and check the presence of the threads.

Timing of insertion

Intrauterine system (Mirena)

* Insertion should be after the end of menstruation and before the calculated time of implantation, unless another method of contraception has been used throughout that cycle. * Do not insert before the final days of menstruation, or before 4 weeks postpartum; insertion at these times carries a higher expulsion rate. * Do not insert within 6 weeks of a caesarian section. * An IUD can be safely fitted immediately after a first trimester abortion (spontaneous or therapeutic), although this carries an increased risk of expulsion.34

The steroid reservoir contains 52 mg levonorgestrel. Lifespan is 5 years. It is licensed for both contraception and menorrhagia.

Removal or refit * Women aged 40 and over. An IUD fitted after the age of 40 can be left as the only means of contraception until the menopause.30 When there has been 6 months of amenorrhoea it should be removed; later removal may be difficult because of cervical stenosis. If a woman presents more than 6 months after the menopause with a device in situ, attempt to remove it. If difficulty is encountered try again after a short course of treatment with topical oestrogen.

Timing of removal * If pregnancy is desired, the IUD may be removed at any time. * If pregnancy is not desired, remove the IUD when the patient is established on a hormonal method or when barrier methods have been used carefully since the last period. * Emergency contraception may be necessary if intercourse has occurred within the last 72 hours and removal of the IUD is urgent (see p. 242).

Advantages of the intrauterine system (IUS) over lUDs (a) It reduces blood loss instead of increasing it and is a form of therapy for menorrhagia. (b) It can reduce dysmenorrhoea. (c) It is more reliable as a contraceptive, with a failure rate of <0.3 per 100 woman years against up to 1.0 per 100 woman years for high load copper lUDs.30 (d) It has a very low rate of ectopic pregnancy. Disadvantages of the IUS (a) Initial cost is much more than lUDs. (b) It is slightly more difficult to insert than a copper IUD because of a wider insertion diameter. (c) The patient may have irregular slight bleeding in the first 3 months of use. (d) Progestogenic side-effects (headache, breast tenderness, nausea, mood changes, acne) are possible, especially to begin with, as there is some systemic absorption. Patients for whom the IUS is particularly indicated * As an alternative to sterilization when family is complete. * Those with menorrhagia. * Those in whom the COC is contraindicated. * Those with learning disabilities or physical disabilities needing long-term contraception.

NON-HORMONAL METHODS OF CONTRACEPTION 241

BARRIER METHODS These have the advantage of protecting against STIs as well as pregnancy.35 However, efficacy is utterly dependent on consistent use before/during intercourse; there is considerable potential for user failure, especially among young people. Condoms and diaphragms made of latex rubber can be damaged by oil-based products such as: (a) baby oil/bath oil/body oil/Vaseline; (b) suntan oil/massage oil; (c) cream/ice cream/salad cream; (d) lipstick/hair conditioner; (e) many antithrush preparations; (f) progesterone pessaries.

Male condoms36

(b) A strip of spermicide about 5 cm long should be placed on the upper side of the diaphragm before insertion. If intercourse takes place more than 3 hours later, more spermicide should be inserted, either as a pessary or as cream with an applicator. (c) The diaphragm must be washed in warm soapy water, dried after use and stored in a cool place in its container to maintain its shape. * Check the fit and comfort after 1 week and discuss again the routine for its use. * See after 3 months and then annually, but more frequently if there are difficulties or if there is a weight change of more than 3 kg. Prescribe a new diaphragm annually.

Cervical caps

Male condoms give a pregnancy rate of 2-15 per 100 woman years. Polyurethane condoms (Avanti and eZ-on) are now available in addition to latex rubber products; they cannot be damaged by oilbased products. * Ensure the couple knows to use kitemarked condoms (British Standard EN 600 for latex condoms). * Advise the couple about use of emergency contraception in the event of a mishap.

These had become rather out of fashion, but a revival is under way with the Oves cap37 made of silicone now available over the counter. Before starting, sizing is needed by a doctor or nurse. The Oves cap comes in three sizes, is disposable and is used with one application of spermicide only. It can be left in situ for up to 72 hours. There are insufficient data yet to give a precise failure rate.

Female condom

Natural family planning

Femidom is a loose-fitting polyurethane sheath with two flexible rings which is inserted into the vagina. It lines the vagina and covers some of the vulva. It is disposable and comes in one size. Its failure rate is 5-15 per 100 woman years.

Natural family planning (NFP; or fertility awareness) relies on avoidance of intercourse around the time of ovulation. Failure rates can be as low as 2.6 per 100 woman years when multiple indicators are used to define the beginning and the end of the fertile period38 - but this high efficacy is only achieved by highly motivated and welltrained couples. Expert training is essential; advise the patient to contact the following for information about local classes.

Diaphragms Diaphragms are more effective than the condom, and have the advantage that they do not need to be inserted and removed at the time of intercourse. They are not, however, as effective a protector as condoms against STIs. They have a failure rate of 4-8 per 100 woman years. They come in sizes rising in steps of 5 mm. Correct size and type is determined on vaginal examination.

Recognized indicators are:

(a) The diaphragm should be inserted before intercourse and left in place for at least 6 hours after the last act of intercourse.

(a) waking body temperature; (b) cervical mucus; (c) fertility monitors.

Information: FertilityUK, Clitherow House, 1 Blythe Mews, Blythe Road, London W14 ONW, tel. 020 7371 1341; www.fertilityuk.org

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Combining indicators is best; secondary indicators such as changes in position and softness of the cervix, width of the os, Mittelschmerz, discharge, breast sensitivity or mood changes can be added to the primary indicators above. Persona is a fertility monitor that measures estrone-3-glucuronide and LH in the urine and predicts fertile days for an individual by storing information on the woman's biochemistry and cycle length. It requires eight urine tests per cycle. A red light indicates fertile days and a green light non-fertile days. A yellow light indicates that a urine test is required. It must be realized that when this device is used by the non-NFP fraternity without combining indicators, failure rates will be higher. Nevertheless, a failure rate of 6.2% is predicted in such a population.39

mid-packet or two or more pills around the pill-free interval (see p. 232); (b) POP. Either method can be considered for a patient who misses two or more pills.

Regimen for hormonal emergency contraception According to the licence, this consists of one tablet of Levonelle-2 within 72 hours of (the first episode of) unprotected intercourse and one tablet 12 hours later. However, there is now some evidence that the efficacy of hormonal treatment is fair between 72 and 120 hours,41 significantly reducing the chance of pregnancy. So give Levonelle-2 in this window, even if IUD insertion is planned, as insertion can be delayed and may fail, especially in teenagers.

Contraindications EMERGENCY CONTRACEPTION Review: Glasier A. Emergency postcoital contraception. New Engl J Med 1997; 337: 1059-64.

Guidelines were last updated in 2000.40 The Yuzpe regimen (Schering PC4) has been superseded by the progestogen-only method, which is the most effective method currently available.41 • Hormonal methods: the progestogen-only method is available as Levonelle-2 on prescription and as Levonelle as a pharmacy item. It is more effective than the Yuzpe regimen and is much less likely to cause vomiting.42 • IUD insertion can be considered if: (a) the patient presents within 5 days of unprotected intercourse; or (b) within 5 days of the calculated time of ovulation if intercourse took place more than 5 days before presentation, whether or not further intercourse has taken place in the last 5 days. • Patients who have failed to take oral contraception correctly: (a) COC. Either method can be considered for a patient who misses four or more pills in

(a) Suspected pregnancy. (b) IUD. Contraindications to the insertion of an IUD apply except that, in an emergency, an IUD can be inserted with antibiotic cover in a patient at risk of PID. Contraindications to the long-term use of an IUD do not apply because the IUD can be removed after the next period.

Special situations (a) Enzyme-inducing drugs. These may be prescribed or OTC. Patients taking enzyme-inducing drugs should take two Levonelle-2 pills stat and then one 12 hours later. (b) Previous ectopic pregnancy. Patients should understand that neither method will protect against a further ectopic pregnancy. (c) The woman who wants the most reliable method or in whom there has been multiple exposure; use an IUD.

Management * Establish from the consultation: (a) dateofLMP; (b) whether the LMP was normal; (c) the normal menstrual cycle length and probable date of ovulation; (d) all the occasions of unprotected sexual intercourse (UPSI) in the last cycle;

CONTRACEPTION IN SPECIAL CASES

(e) number of hours since first episode of UPSI; (f) the method of contraception normally used. * Warn the couple that the maximum risk of pregnancy after midcycle UPSI is about 9%/44 but depends on the inherent fertility of the couple. * Explain to the patient that: (a) the method is not guaranteed. The average risk of pregnancy from a single episode of coitus is 3%.44 The progestogen-only method reduces this risk by around 85% (95% if given in the first 24 hours).42 The IUD is more effective, reducing the risk by over 95%;45 (b) if the hormonal method is given, barrier methods must be used until the next period, which may be a little earlier or later than expected. The COC may then be started; (c) she should re-attend if the next period does not come, or she has any other worries; (d) if vomiting occurs within 3 hours of taking the dose she should re-attend the following day.

Follow-up * If a normal period has not occurred, exclude pregnancy and bear in mind the possibility of ectopic pregnancy. * Discuss the long-term need for contraception. Use the opportunity to counsel the young and inexperienced.

CONTRACEPTION IN SPECIAL CASES CONTRACEPTION AND YOUNG PEOPLE Information and advice: Brook Advisory Centres Helpline. Free confidential counselling service for young people. Tel. 0800 0185023; www.brook.org.uk Sexwise for 12-18-year-olds with problems with sex, puberty, relationships and contraception. Helpline, 0800 282930; www.ruthinking.co.uk

In England and Wales, provision of contraceptive advice and treatment by health professionals to those aged under 16 is governed by health circular HC(86)1, issued in accordance with the Gillick

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case (Eraser guidelines). Advice and treatment may be given without parental knowledge when the young person: (a) has sufficient maturity to understand the moral, social and emotional implications of the treatment; (b) cannot be persuaded to inform the parents or allow them to be informed; (c) is very likely to begin, or to continue, sexual intercourse with or without contraception; (d) would be likely to suffer in terms of physical or mental health if no contraceptive advice or treatment were given; (e) has their best interests served by being given contraceptive advice or treatment without parental consent. Young people continue to have concerns about the possibility of breaches of confidentiality. Improving young people's trust in the confidentiality of their practice should help remove one of the main obstacles that deter some teenagers from seeking early sexual health advice.46 Every practice should develop its own confidentiality policy. * Legal aspects. Inform the patient (and, if she gives her consent, her parents) of the legal situation - it is an offence for a man aged 14 or over to have sexual intercourse with a girl under the age of 16, even if the girl consents (Sexual Offences Act 1956). * Smoking. Discuss the importance of not smoking, especially if the COC is to be used. * Discuss the risks of contracting STIs, especially chlamydia, and how condoms protect against transmission. * Raise the advantages, both psychological and physical, of not having sexual intercourse at a young age.

Combined oral contraceptive * Warn the patient that BTB can occur in the first few packets, is not harmful, and to come in and discuss the problem rather than stop the pill. * Stress the value of using the condom as well to reduce the risk of STIs including HIV (dual protection). Young people should be shown how to use condoms.

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CONTRACEPTION AND OLDER WOMEN * Women over 40 are more likely to have irregular cycles, but ovulation still occurs in around 35% of these cycles and there is still need for contraception. * Current advice is for contraception to be continued in women aged 40-50 until there have been 2 years' amenorrhoea, and in women over 50 until there has been 1 year's amenorrhoea.47

Women on the COC * Age. Healthy non-smokers without risk factors may continue until the age of 50. * Advise women who have stopped the COC at 50 to use the POP, barrier methods or spermicide until they have had 1 year's amenorrhoea. If menstruating on the POP, follow the advice below.

Women on the POP * When amenorrhoea occurs, stop the POP but use other precautions for 1 year if aged over 50, for 2 years if aged 50 or less. * Alternatively, check the FSH: (a) If the FSH level is raised, stop the POP and advise barrier methods. Recheck FSH after 1 month; if it is still high, the patient has probably reached the menopause, although ovulation has been known to occur in women with a high FSH. Contraception should be continued for a further year. (b) If the FSH level is low, continue with the POP. Recheck FSH levels once a year. * Those women who have always had amenorrhoea on the POP can have their FSH checked once a year after age 45.

Hormone replacement therapy (HRT) HRT is not contraceptive as the natural oestrogens do not necessarily inhibit ovulation. * Women started on HRT before the menopause should continue contraception (barrier or IUD, possibly the POP) until there is reason to think that they have reached the menopause, e.g. at age 50. It is impossible to assess the timing of the natural

menopause in women using HRT. If a woman is unwilling to discontinue HRT for 3-4 months to allow accurate assessment of FSH levels, then barrier contraception, an IUD or the POP can be used until the age of 55, at which time loss of natural fertility can be assumed. * Women on the COC do not need HRT until the COC is stopped.

CONTRACEPTION AND LEARNING DISABILITY48 Contraception is most likely to be considered in women with learning disability who: (a) are entering a sexual relationship. There is a 40% chance of learning disability in children born to a couple where both parents have learning disability; (b) are in danger of being exploited. * Consent. Consult the consultant psychiatrist if there is doubt about the ability to give informed consent. The majority of patients with moderate and mild learning disability are able to give informed consent. * Sex education. Refer to the local community learning disability team, Social Services department, Education department or voluntary organization. * Epilepsy. If patients are on enzyme-inducing antiepileptics, the dose of the COC needs to be higher (see pp. 230,232). Those on the POP should probably change method.

STERILIZATION When counselling for sterilization the following should be borne in mind: (a) Both partners should ideally be seen together. (b) Patients should be made aware of the high efficacy of alternative long-acting reversible contraceptive methods. (c) The possibility of death of partner or child should be covered. (d) The possibility of relationship breakdown should be discussed. (e) The pros and cons of male versus female sterilization should be explained.

TERMINATION OF PREGNANCY 245

Which partner should be sterilized? Sometimes it is not the partner who presents her-/ himself who is subsequently sterilized. Points to be raised here are: (a) Vasectomy is more effective than tubal occlusion: 1 in 2000 life-time pregnancy risk compared with 1 in 200 for tubal occlusion.49 (b) Vasectomy is a more minor operation. (c) Vasectomy is safer as it is usually performed under local anaesthesia. (d) A man has a longer reproductive span to lose. (e) A man is more likely to feel threatened (fear of loss of sexual drive and sexual prowess).

Reversal If a patient wants a reversal of sterilization, patency rates of around 90% can be achieved with microsurgical techniques but fertility is lower: 44-92% in females and 17-82% in males (rates tend to be at the low end of this range when the vasectomy was performed more than 10 years previously).49 When counselling a patient, be aware that risk factors for subsequent regret about sterilisation include:49 (a) age <25; (b) no children; (c) not in a relationship; (d) association in time with pregnancy (full term or abortion); (e) crisis in relationship; (f) coercion (or at least pressure from a partner in making decision).

TERMINATION OF PREGNANCY The Abortion Act 1967 as amended by the Human Fertilization and Embryology Act 1990 allows termination of pregnancy (TOP) if two doctors agree that: (a) The continuance of the pregnancy would involve risk to the life of the pregnant woman greater than if the pregnancy were terminated; (b) The termination is necessary to prevent grave permanent injury to the physical or mental health of the pregnant woman;

(c) The pregnancy has not exceeded its 24th week and the continuance of the pregnancy would involve risk, greater than if the pregnancy were terminated, of injury to the physical or mental health of the pregnant woman; (d) The pregnancy has not exceeded its 24th week and the continuance of the pregnancy would involve risk, greater than if the pregnancy were terminated, of injury to the physical or mental health of any existing child (ren) of the family of the pregnant woman; (e) There is a substantial risk that if the child were born it would suffer from such physical or mental abnormalities as to be seriously handicapped. Note: There is no time limit for grounds (a), (b) and (e). * Mortality for a first trimester TOP is about 1 per 100,000 compared to about 10 per 100,000 for death in childbirth. * The risk increases with advancing gestation and when the woman has had previous TOPs. * The risk of infertility after vaginal TOP is probably less than after a normal delivery.50 * The majority of women make a good subsequent mental adjustment.51 * Counsel the woman to help her come to her own decision about TOP and to come to terms with any subsequent regret, guilt or anger she may feel. * Ensure that she is aware of the alternative of continuing the pregnancy and keeping the baby or giving it up for adoption. * Check she is not under pressure from partner or parent. * Discuss the need for and if possible plan future contraception, including information about emergency contraception if not already known about. * Arrange a follow-up appointment 2 weeks after the termination to check for medical and psychological sequelae. * If the patient wishes to pursue the possibility of medical TOP with mifepristone and gemeprost or misoprostol, refer her to a unit offering it, and check that she will be seen quickly enough. The abortion should be performed by the 63rd day from the first day of the last period. Check that she does not have any contraindications, of which the commonest is smoking over the age of 35.

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The patient under 16 years old Involve the parents or guardians, with the patient's consent. They will be needed to sign the consent form as well as to support the patient before and after the termination. If the patient refuses to inform a parent or guardian, she has the right, under the Family Law Reform Act 1969, to consent to treatment herself, provided she understands the nature of the procedure including its risks and complications. If a competent child consents to treatment, a parent cannot override that consent.

INFERTILITY Guideline: Royal College of Obstetricians and Gynaecologists. The initial investigation and management of the infertile couple. London: RCOG 1998; www.rcog.org.uk (choose 'Good Medical Practice').

* Reassure the couple that the average time to conception is 8 months, and that 90% are pregnant within 18 months. * Start investigations as soon as the couple voice their anxiety about conceiving. Explain that in 30% of cases the problem lies with the male alone, in 30% with the female alone, and that in the remainder it is mixed or unexplained. * Aim to consider referral after 1 year, or sooner if there is good reason to predict difficulties, e.g. oligo- or amenorrhoea, abnormal sperm count or maternal age over 35. Refer the couple direct to a tertiary centre holding the contract for the treatment of subfertility, not to a general gynaecology clinic.

(b) Check that the woman is rubella immune and taking folic acid. (c) Examine for evidence of pelvic pathology. (d) Progesterone level. Blood should be taken 7 days before menstruation is due, for progesterone levels. A level of >16nmol/L suggests ovulation, and a level of >30nmol/L confirms it. Borderline levels may be due to a deficient luteal phase, or to mistimed sampling. Levels <16nmol/L confirm that the cycle was anovulatory. Note: There is no value in measuring TFTs or prolactin in women with regular menses in the absence of galactorrhoea or symptoms of thyroid disease. Assessment of the man

(a) Medical history should include operations on or infections of the testes and operations on the prostate. (b) Drug history - sulfasalazine, tetracyclines, allopurinol, cannabis and cocaine have all been shown to interfere with male reproductive function. (c) Examine him, including genitals and secondary sexual characteristics. (d) Arrange for two semen analyses. These should be produced by masturbation 2-3 days after the last ejaculation, and examined within an hour. (e) If the count is low, refer to a specialist fertility clinic. (f) Warn the patient that even a 'normal' sperm count does not mean that there is not some sperm dysfunction which can only be detected on more sophisticated tests. Normal values for semen (WHO) (a) volume: ^2 ml; (b) concentration: ^20 million per ml; (c) motility: ^50%; (d) normal morphology: >30%; (e) white blood cells: <1 million per ml.

Investigations Assessment of the woman

(a) History should include details of menstrual cycle, previous pregnancies, pelvic infections or operations. Check that the couple are having intercourse at the woman's most fertile time.

Advice Advice to the couple should routinely be: (a) The woman should stop smoking. (b) The man should stop smoking.

SEXUAL PROBLEMS

(c) The woman should drink no more than one or two units of alcohol once or twice weekly while trying to conceive. (d) Men who drink heavily should cut their drinking to no more than 3-4i units in any day. (e) Women with a BMI >30 should lose weight whether ovulatory or not. (f) Men should avoid soaking in hot baths, wearing tight underwear and remaining seated for many hours at a time. (g) Couples should be advised to have regular intercourse throughout the cycle; there is no evidence that use of temperature charts and LH detection tests to time intercourse improves pregnancy rates. Use of clomifene in general cannot be justified in general practice in view of the increased risk of multiple pregnancy and ovarian cancer. An exception to this is if a woman, who previously conceived using it, presents again with anovulatory cycles. The role of the GP after referral * Administer the drugs for assisted conception according to a protocol agreed with the specialist clinic. * Support the couple throughout the drawn-out process of investigation and treatment. Self-help organizations who supply literature and run support networks: ISSUE, 114 Lichfield Street, Walsall, WS1 1SZ, tel. 01922 722888; www.issue.co.uk CHILD, 3 St Leonard's Road, Bexhill-on-Sea TN40 1JA, tel. 01424 732361; www.child.org.uk Books and videos about assisted conception are also available from The Human Fertilization and Embryology Authority, Paxton House, 30 Artillery Lane, London E1 7LS, tel. 020 7377 5077; www.hfea.gov.uk

ADOPTION Patient contact: The Director, British Agencies for Adoption and Fostering, Skyline House, 200 Union Street, London SE1 OLX, tel. 020 7593 2000; www.baaf.org.uk

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SEXUAL PROBLEMS Review: Tomlinson J (Ed). ABC of sexual health. London: BMJ Books, 1999.

• Sexual problems may have a physical or psychological origin, but often they are mixed. • All patients with sexual problems develop 'performance anxiety', where they are not relaxed and spontaneous in love making, are alert to further failure and become a spectator of their own sexual performance. • In about 30% of patients, the partner also has a problem. • In any consultation about sexual problems it is important to ascertain: (a) what is the real complaint? (b) why is the problem being presented now? (c) is the desire for change real? (d) who is really complaining, the patient or the partner? (e) what are the expectations of the patient? • Consider the following psychological aspects in any patient presenting with a problem: (a) Ignorance and misunderstanding. This includes faulty sex education and technique, inability to communicate sexual needs, and unrealistic expectations due to stereotyped views of expected behaviour and performance. (b) Anger and resentment. This often remains unresolved, with the couple unable to communicate their feelings to each other clearly. It often arises where the partners are unable to express their feelings as children because of excessive unresolved anger in their parents. It may occur in response to relationship difficulties, financial difficulties, children, in-laws or stress at work. (c) Shame, embarrassment and guilt. This may be due to a negative sexual attitude laid down in childhood, where the parents looked upon sexuality as 'bad'. Traumatic sexual experiences may add to these fears. (d) Anxiety/fear about sex. Fear of closeness, vulnerability, letting go and failure may lead to a self-perpetuating cycle of anxiety.

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(e) Poor self-image may contibute to lack of interest in sex and sexual response. Changes in women may occur after operations, especially mastectomy or hysterectomy postmenopausally and after childbirth. Both sexes commonly suffer after redundancy and when depressed. Information for patients: Litvinoff S. The Relate guide to sex in loving relationships. Vermilion, 1999. Self-help material on the internet: the 'Sex and relationships' section at www.netdoctor.co.uk Advice for disabled people: SPOD, 286 Camden Road, London N7 OBJ, tel. 020 7607 8851. www.spod-uk.org Referrals for sex therapy: via local family planning clinic or Relate.

Lack of sexual desire This is the commonest presenting female sexual dysfunction, is often the hardest to treat and usually needs specialist help. Underlying psychological difficulties are frequent. These may relate specifically to sex, e.g. previous sexual abuse, or they may relate to a more widespread psychological disorder. Physical illness and drugs/ substances are also possible causes. Women specifically lose sexual desire: (a) postpartum; (b) because of pain; (c) where their partner's performance repeatedly leads to frustration, as in premature ejaculation.

Erectile dysfunction Guideline: Ralph D, McNicholas T. UK management guidelines for erectile dysfunction. BMJ200Q; 321:499-503.

Erectile dysfunction occurs in 10-15% of men but varies with age, with some degree of dysfunction being experienced by 40% of men at age 40 and by 70% at age 70. Patients who have the courage to mention it should be told that much can be

done to help. Although at least 50% of the patients referred to specialist clinics have an organic component, anxiety about the situation will make things worse. * From the history, assess particularly whether: (a) the impotence has arisen suddenly, with a precipitating cause, and is variable, with erections occurring in the early morning but not during intercourse. These patients are likely to have a psychological cause for the problem. (b) the onset was gradual, is constant, with partial or poorly sustained erections and no full early morning erections. These patients are likely to have a physical cause, although a psychological component is frequently superimposed. * Be alert to psychiatric problems including generalized anxiety states (excessive adrenergic constrictor tone), depression, psychosis, body dysmorphic disorder, gender identity problems and alcoholism. * Check for the cause being an adverse drug reaction to beta-blockers, thiazides, spironolactone, cimetidine, antidepressants, phenothiazine antipsychotics and, especially, alcohol. * Examine: (a) blood pressure. Check blood sugar. (b) genitalia (includes testicular size, fibrosis in the shaft of the penis and retractibility of the foreskin). Further examination may sometimes be indicated by age or findings in the history especially cardiovascular, neurological, endocrine and urinary systems.

Work-up (a) blood glucose; (b) testosterone (if history or examination suggest possible hypogonadism or if required to reassure patient).

Treatment in general practice52 (a) Oral sildenafil. The number of men needed to be treated with sildenafil for one additional man to experience an improvement in his erections is 1.86.53 Disadvantages are that it is a facilitator

SEXUAL PROBLEMS 249

rather than an initiator of erections and that it has a slower onset of action than injected or transurethral alprostadil (see below). Prescribe sildenafil at NHS expense to men with any of the conditions approved by the Department of Health, marking the prescription SLS, and giving enough (usually) for one treatment a week. A private prescription can be issued to any patient who does not come within the DoH guidelines (Health Services Circular HSC 1999/148 available at www.doh.gov.uk/publications/coinh.html). (b) Sublingual apomorphine - takes only 20 minutes to produce an erection. Like sildenafil, it is on the NHS 'grey list' (Schedule 11). Counselling may be successful in psychogenic impotence, and is appropriate for couples who do not wish to be referred: * See the couple together. * Recommend a manual, e.g. The Relate guide to sex in loving relationships (see box above). * Instruct the couple on the sensate focus programme. Instructions can be downloaded from the web, e.g. atwww.personalmd.com/healthtopics/ crs/touch.htm * Set 'homework' assignments for the couple; these can be tailored to meet specific needs.

Referral * Refer those not responding for sex therapy via the local family planning clinic or Relate. The outcome is successful in 50-80% of cases. * Refer for relationship counselling those whom you sense have larger relationship or personality problems. * Refer to a urologist those with physical causes, those who have not responded to sildenafil/ apomorphine or who have contraindications to it and those with psychological causes that are proving intractable. Possible treatments are: (a) hormone replacement therapy in those with hypogonadism; (b) alprostadil - as injections into the corpus cavernosum or transurethrally; (c) vacuum devices; (d) penile prostheses.

Details of therapists are available from: British Association for Sexual and Relationship Therapy, PO Box 13686, London SW20 9ZH, www.basrt.org.uk Institute of Psychosexual Medicine, 11 Chandos Street, London W1M 9DE, tel. 020 7580 0631; www.ipm.org.uk

Dyspareunia Eight per cent of women aged 35-59 admit that sexual intercourse usually causes discomfort.54 The functional problems may be primary, or secondary to a physical cause, and considerable skill may be needed to unravel the problem. * Distinguish from the history and examination between: (a) lack of lubrication due to lack of interest; (b) vaginismus, which usually becomes apparent at vaginal examination (see below); (c) vulval or vaginal causes: infections, vulvar vestibulitis syndrome, lichen sclerosus, lichen planus, urethral caruncle, postmenopausal vaginitis, postepisiotomy syndrome. Those for whom there is no specific treatment may benefit from 5% lignocaine ointment applied 20 minutes before sexual intercourse; (d) pelvic causes with tenderness on rocking the cervix or palpating the fornices, e.g. endometriosis, pelvic infection, ovarian pathology; (e) psychogenic causes, where vulval burning or pain is due to somatization of other difficulties. Vaginismus This consists of a phobia of penetration and involuntary spasm of the pubococcygeal and associated muscles surrounding the lower third of the vagina. * Explore the root cause. It may be: (a) fear of the unknown or the patient's ignorance of her own anatomy; (b) a past history of unpleasant experiences such as rape, sexual abuse or severe emotional trauma; or previous dyspareunia; (c) a defence mechanism against growing up and becoming a woman. (A common pattern is

250 CONTRACEPTION AND SEXUAL PROBLEMS

for the patient to live close to her parents, remaining the daughter and marrying an unassertive man.) * Desensitize simple cases by encouraging the women to examine herself, and also encourage her partner to be confident enough to insert a finger into the vagina. Some women may prefer to use vaginal dilators. * Refer to a sex therapist if this simple approach fails. * Give the patient details of Resolve - the Vaginismus Support Group, PO Box 820, London N10 SAW.

the glans between finger and thumb during relaxation phases. * Clomipramine 25 mg daily or paroxetine 20 mg each evening are effective in delaying ejaculation.52 Retarded ejaculation

This is usually a sign of long-standing sexual inhibition. Often the patient can ejaculate by masturbation, but not intravaginally. * Explore any feelings of anxiety and guilt. * Start a sensate focus programme (see above). * If 'home therapy' fails, refer for psychosexual counselling.

Disorders of orgasm Anxiety tends to delay a woman's orgasm, but accelerates a man's. Premature ejaculation

This is present when ejaculation occurs sooner than either partner would wish, usually before penetration or soon after. Interest in sex may be reduced in both partners. * Advise the patient that, with practice, he can learn to delay ejaculation. The stop/start technique can be taught by therapists or learnt from the book cited above. In essence, when during caressing or during intercourse a man feels he is close to climax he should stop being stimulated and relax for 30 seconds. Stimulation can then recommence until he is close to climax again, when the relaxation is repeated. If this fails, the woman should squeeze the penis at the base of

Orgasmic problems in women

* A woman who has never achieved an orgasm may have deep-seated psychological reasons for being afraid to let go. She may need 'permission' to investigate her body's own responses further, either by masturbation or vibrator. When she has learnt how to relax, she should be encouraged to tell her partner and incorporate caressing into their usual lovemaking. * Women who have lost the ability to achieve orgasm may need counselling, especially about their current relationship or a recent loss of selfimage. * Check that she is not taking drugs that inhibit orgasm, e.g. clonidine, and that her failure to achieve orgasm is not due to neurological disease or pelvic surgery. Contrary to the results of some poorly executed studies, total hysterectomy does not impair ability to achieve orgasm.55

REFERENCES 1. Brown ADG. Legal aspects of family planning. In Glasier A, Gebbie A (Eds). Handbook of family planning and reproductive healthcare. London: Churchill Livingstone, 2000, pp. 231-48. 2. Anonymous. Improving access to quality care in family planning: medical eligibility criteria for contraceptive use. Geneva: World Health Organization, 2000. 3. MacGregor EA, Guillebaud J. Combined oral contraceptives, migraine and ischaemic stroke. Br J Fam Planning 1998; 24: 55-60.

4. Guillebaud J. Combined oral contraception. In Glasier A, Gebbie A (Eds). Handbook of family planning and reproductive healthcare. London: Churchill Livingstone, 2000, pp. 29-76. 5. Anonymous. Combined oral contraceptives containing desogestrel or gestodene and the risk of venous thromboembolism. Curr Prob Pharmacovigil 1999; 25: 12. 6. Kemmeren JM, Algra A, Grobbee DE. Third generation oral contraceptives and risk of venous thrombosis: meta-analysis. BMJ 2001; 323: 131-4.

REFERENCES

7. Walker AM. Newer oral contraceptives and the risk of venous thromboembolism. Contraception 1998; 57: 169-81. 8. Anonymous. First prescription of combined oral contraception: recommendations for clinical practice. Br } Fam Planning 2000; 26: 27-38. 9. Stewart FH, Harper CC, Ellertson CE, Grimes DA, Sawaya GF, Trussell J. Clinical breast and pelvic examination requirements for hormonal contraception. JAMA 2001; 285: 2232-9. 10. O'Brien MD, Gilmour-White S. Epilepsy and pregnancy. BMJ 1993; 307: 492-5. 11. Rosenberg MJ, Long SC. Oral contraceptives and cycle control: a critical review of the literature. Adv Contracep 1992; 8 (Suppl. 1): 35-45. 12. Guillebaud J. Contraception and sterilization. In Turnbull A, Chamberlain G (Eds). Obstetrics. Edinburgh: Churchill Livingstone, 1989, pp. 1135-52. 13. Poulter NR. Oral contraceptives and blood pressure. In Hannaford PC, Webb AMC (Eds). Evidence-guided prescribing of the pill. Carnforth: Parthenon, 1996, pp. 77-88. 14. Fernandez E, La Vecchia C, Balducci A, Chatenoud L, Franceschi S, Negri E. Oral contraceptives and colorectal cancer risk: a meta-analysis. Br J Cancer 2001; 84: 722-7. 15. Collaborative Group on Hormonal Factors in Breast Cancer. Breast cancer and hormonal contraceptives: collaborative reanalysis of individual data on 53,297 women with breast cancer and 100 239 women without breast cancer from 54 epidemiological studies. Lancet 1996; 347: 1713-27. 16. Rosenberg MJ, Waugh MS, Stevens CM. Smoking and cycle control among oral contraceptive users. Am J Obstet Gynecol 1996; 174: 628-32. 17. Hampton N. The role of hormonal contraceptives in acne. Br J Sex Med 1995; May/June: 11-14. 18. Fotherby K. The progestogen-only pill. In Filshie M, Guillebaud J (Eds). Contraception: science and practice. London: Butterworths, 1989, pp. 94-108. 19. Rice C, Killick S, Hickling D, Coelingh Bennink H. Ovarian activity and vaginal bleeding patterns with a desogestrel-only preparation at three different doses. Hum Repro 1996; 11: 737-40. 20. Bhathena RK. The long-acting progestogen-only contraceptive injections: an update. Br J Obstet Gynaecol 2001; 108: 3-8. 21. Kaunitz AM. Injectable contraception. Obst Gynecology Clin N Am 2000; 27: 741-80. 22. WHO. A multicentred phase III comparative trial of depot-medroxyprogesterone acetate given three-monthly at doses of lOOmg or 150mg: II. The comparison of bleeding patterns. Contraception 1987; 35: 591-610. 23. Merki-Feld GS, Neff M, Keller PJ. A prospective study on the effects of depot medroxyprogesterone acetate on trabecular and cortical bone after attainment of peak bone mass. Br J Obstet Gynaecol 2000; 107: 863-9. 24. Tang OS, Tang G, Yip PSF, Li B. Further evaluation on long-term depot-medroxyprogesterone acetate use and bone mineral density: a longitudinal cohort study. Contraception 2000; 62: 161-4. 25. Fotherby K, Howard G. Return of fertility in women discontinuing injectable contraceptives. / Obstet Gynaecol 1986; 6: S110-S15.

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26. Croxatto HB, Makarainen L. The pharmacodynamics and efficacy of Implanon. Contraception 1998; 58: 91S-7S. 27. Mascarenhas L. Insertion and removal of Implanon. Contraception 1998; 58: 79S-83S. 28. Affandi B. An integrated analysis of vaginal bleeding patterns in clinical trials of Implanon. Contraception 1998; 58: 99S-107S. 29. Urbancsek J. An integrated analysis of nonmenstrual adverse events with Implanon. Contraception 1998; 58: 109S-15S. 30. Rowlands S, Hampton N. Intrauterine contraception. In Kubba A, Sanfilippo J, Hampton N, eds. Contraception and office gynecology: choices in reproductive healthcare. London: WB Saunders, 1999, pp. 93-112. 31. Grimes D. Intrauterine device and upper-genital-tract infection. Lancet 2000; 356: 1013-19. 32. Grimes D, Schulz KF. Antibiotic prophylaxis for intrauterine contraceptive device insertion (Cochrane Review). The Cochrane Library, Issue 4. Oxford: Update Software, 2000. 33. The Clinical and Scientific Committee. Recommendations for clinical practice: actinomyces like organisms and intrauterine contraceptives. Br J Fam Planning 1998; 23: 137-8. 34. Grimes D, Schulz K, Stanwood N. Immediate postabortal insertion of intrauterine devices (Cochrane Review). The Cochrane Library, Issue 3, Oxford: Update Software, 2000. 35. Hicks D. The risks and benefits of contraceptive method regarding sexually transmitted infections. Br J Fam Planning 1996; 22: 34-6. 36. Mindel A. Condoms. London: BMJ Books, 2000. 37. Longworth JCD, Marable E. Recent advances in barrier contraception: review and report of clinical trial of the Oves® cervical cap. Rev Gijnaecolog Pract 2001; 1: 89-99. 38. European Natural Family Planning Study Groups. Prospective European multi-center study of natural family planning (1989-1992): interim results. Adv Contracep 1993; 9: 269-83. 39. Bonnar J, Flynn A, Freundl G, Kirkman R, Royston P, Snowden R. Personal hormone monitoring for contraception. Br J Fam Planning 1999; 24: 128-34. 40. Anonymous. Emergency contraception: recommendations for clinical practice. Br J Fam Planning 2000; 26: 93-6. 41. Cheng L, Giilmezoglu AM, Ezcurra E, Van Look PFA. Interventions for emergency contraception (Cochrane Review). The Cochrane Library, Oxford: Update Software, 2000. 42. Task Force on Postovulatory Methods of Fertility Regulation. Randomised controlled trial of levonorgestrel versus the Yuzpe regimen of combined oral contraceptives for emergency contraception. Lancet 1998; 352: 428-33. 43. Rodrigues I, Grou F, Joly J. Effectiveness of emergency contraceptive pills between 72 and 120 hours after unprotected intercourse. Am J Obstet Gynecol 2001; 184: 531-7. 44. Wilcox AJ, Dunson DB, Weinberg CR, Trussell J, Baird DD. Likelihood of conception with a single act of intercourse: providing benchmark rates for assessment of post-coital contraceptives. Contraception 2001; 63: 211-15. 45. Trussell J, Ellertson C. Efficacy of emergency contraception. Fertil Control Rev 1995; 4: 8-11.

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46. Donovan C, Hadley A, Jones M, et al. Confidentiality and young people. London: Royal College of General Practitioners and Brook, 2000. 47. Gebbie A. The menopause. In Glasier A, Gebbie A (Eds). Handbook of family planning and reproductive healthcare. London: Churchill Livingstone, 2000, pp. 371-94. 48. Cooper E. Couples with learning disabilities. In Killick S (Ed). Contraception in practice. London: Martin Dunitz, 2000, pp. 229-40. 49. Royal College of Obstetricians and Gynaecologists. Male and female sterilisation: evidence-based clinical guidelines, No. 4. London: RCOG Press, 1999. 50. Frank P, McNamee R, Hannaford PC, Kay CR, Hirsch S. The effect of induced abortion on

51. 52. 53.

54.

55.

subsequent fertility. Br ] Obstet Gynaecol 1993; 100: 575-80. Gilchrist AC. Psychological sequelae of abortion. Trends in urology, gynaecology and sexual health 1998; March: 9-10. Holmes S. Treatment of male sexual dysfunction. Br Med Bull 2000; 56: 798-808. Burls A, Gold L, Clark W. Systematic review of randomised controlled trials of sildenafil (Viagra®) in the treatment of male erectile dysfunction. Br J Gen Pract 2001; 51: 1004-12. Osborn M, Hawton K, Gath D. Sexual dysfunction among middle aged women in the community. BMJ 1988; 296: 959-62. Farrell SA,. Kieser K. Sexuality after hysterectomy. Obstet Gynecol 2000; 95: 1045-51.

CHAPTER CONTENTS Prepregnancy care 253 Genetic disorders 253 Previous obstetric history 254 Medical history 255 Lifestyle 256 Infection 256 Nutrition 256

13 Obstetric problems

Routine antenatal care 256 Prenatal diagnosis and screening 258 Problems in pregnancy 259 Vaginal bleeding and miscarriage 259 Abdominal pain 261 Infection or contact with infectious disease 261 Vomiting and reflux 263 Pruritus 263 Excessive weight gain 263 Fundal height 263 Hypertension 264 Anaemia 265 Rhesus-negative mothers 266 Abnormal lie 266 High head 266 Post-maturity 266 Specific medical conditions and pregnancy 267 Drug misuse 268 Intrapartum care Postnatal care References

268

268

271

Policy statement: The role of general practice in maternity care. Occasional paper 72. London: Royal College of General Practitioners, 1995.

PREPREGNANCY CARE The GP has an important role in identifying risk factors before conception. This can be done either at a specific consultation or when the opportunity arises, e.g. as part of contraceptive care, or when seeing patients with diabetes or hypertension. GENETIC DISORDERS All couples should be referred for genetic counselling if they request it, or there are risk factors which need further investigation. The list of identifiable conditions is increasing, and recent studies in the UK suggest clinicians across specialties are poor at appreciating which conditions have identifiable genetic components.1 If there is any doubt, contact the nearest department of clinical genetics for advice. Children or adults with a major abnormality or disease, even if not at present thought to be genetic in origin, should have blood stored at a regional genetics laboratory for later analysis. Those at higher risk are: (a) Couples with a personal or family history of an abnormality that is presumed to be genetic in origin. They should be referred for genetic 253

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counselling or have blood taken by the GP on the advice of the geneticist. Important examples are: - cystic fibrosis; - Huntington's chorea; - Duchenne and other muscular dystrophies; -polycystic kidneys; -mental retardation, which may be due to fragile-X syndrome. (b) Couples belonging to a high risk ethnic group (see below). (c) Older women. The risk of having a baby with Down syndrome is 1:400 at age 35, rising to 1:100 at age 40 and 1:30 at age 45. After the age of 37 the risk of chromosomal abnormality is greater than the risk of miscarriage after amniocentesis. Apart from this, neonatal outcome is not affected by maternal age, although women having their first pregnancy over age 35 are more likely to have hypertension, diabetes or placental abruption. (d) Consanguinous couples. First-degree cousins have a slight but significant increase in congenital malformations. This is likely to be higher if there is a family history of congenital disease. Caucasian first-degree cousins should be offered screening for cystic fibrosis, even where there is no family history.

High-risk ethnic groups African-Caribbean: Sickle-cell and thalassaemia * Exclude sickle-cell trait in the parents by checking their Hb and asking for sickle solubility test. If positive, request Hb electrophoresis. * Refer all couples who are both heterozygous, or where the mother is and the father has thalassaemia minor or is unknown. Patient leaflets: Sickle Cell Society, 54 Station Road, London NW10 4UA, tel. 020 8961 7795; www.sicklecellsociety.org

Indian subcontinent, Far East and Southern Europe: Thalassaemia * Check Hb and, if the mean cell haemoglobin is below 25 pg, arrange Hb electrophoresis; or * Screen for haemoglobinopathy regardless of the MCH, if that is local policy.

Note: If both partners have thalassaemia trait, refer for specialist genetic advice. Patient leaflets: UK Thalassaemia Society, 19 The Broadway, Southgate Circus, London N14 6PH, tel. 020 8882 0011; www.ukts.org

Jewish population: Tay Sachs * Consider referral for the exclusion of Tay Sachs trait. One in 30 of Ashkenazi Jewish descent is positive. Telephone the Tay Sachs carrier testing centre at Guy's Hospital, tel. 020 7955 4648, or the Regional Genetics Centre.

PREVIOUS OBSTETRIC HISTORY (a) Down syndrome: make certain that all women who have had a previous Down syndrome baby have received genetic counselling, regardless of the age at which they conceived. (b) Previous miscarriages (see also p. 260): inform all patients, if asked, that the overall incidence of miscarriage is around 15%. However, women whose last pregnancy was normal have a 5% chance of miscarriage, whereas women whose last pregnancy ended in miscarriage have a 20-25% chance of a miscarriage.2 Women with three consecutive miscarriages have a 40% chance of the next pregnancy ending in miscarriage. (c) Recurrent miscarriages: refer to a gynaecologist for assessment all women who have had three consecutive miscarriages. Reassure them, however, that they still have a 60% chance of a normal pregnancy. Consider early referral if: - there is a history of cycle irregularity; - there is a family history of miscarriage or of congenital disease; - there is a relevant medical problem; - the miscarriage was mid trimester; or - there is an urgency to achieve a live birth, as in the older woman. Hospital work-up for recurrent miscarriage: (a) Blood sugar; (b) TFTs; (c) LH on day 7; (d) Blood group and antibodies;

PREPREGNANCY CARE 255

(e) (f) (g) (h) (i)

Antiphospholipid antibodies; Factor V Leiden; Karyotyping of both partners; Pelvic ultrasound; Hysterosalpingography if miscarriages are late.

(d) Family history or previous infant with neural tube defects: All women in this category should receive folic acid prior to conception. Give 5mg daily, starting 1 month prior to stopping contraception, and continue throughout the first trimester. This will reduce the incidence of further neural tube defects by 70% (DoH letter, 12 August 1991). Women should also receive advice about antenatal diagnosis (see below). (e) Size, gestation and maturity of previous infants: -previously large babies: consider screening for type 2 diabetes. -previous intrauterine growth retardation: stress the importance of not smoking and drinking alcohol. The woman should be referred early in pregnancy for consideration of uterine artery Doppler as a screening test, and for serial growth scans from 24 weeks gestation. MEDICAL HISTORY Hypertension (see p. 264) * This should be assessed before a woman gets pregnant, as it may be masked by the fall in blood pressure in the first half of pregnancy. * Discuss the most appropriate drug with the relevant specialist before conception. Diabetes (see p. 267) * Explain that diabetes is associated with an increase in congenital abnormalities (about double), but that this can be decreased significantly by good control of the blood sugar prior to and during pregnancy.3

multiplied by two for every further drug taken.4 Sodium valproate increases the risk of neural tube defects 50 times, to about 1.5%. With carbamazepine the rate is 1%. Otherwise abnormalities are not specific to individual drugs. There is little data on the safety of newer drugs. There is no convincing evidence that any one drug is safer than another.5 * Discuss with the neurologist: (a) whether to stop anticonvulsants in any woman who has been free of convulsions for 2 years; but counsel the patient about the risks of untreated epilepsy in pregnancy. Convulsions may lead to stillbirth; or (b) whether to change anticonvulsants or reduce their dose in those in whom they cannot be stopped. * Make it clear that even on anticonvulsants there is a 90% chance of a normal outcome, that prenatal diagnosis is effective in picking up neural tube defects (see below) and that many defects can be corrected after birth. * Ensure that all epileptics are offered: (a) Folic acid. Those continuing antiepileptic medication should take folic acid 5mg daily from 3 months before conception until the end of the 12th week. Those stopping their drugs need only take 400 |xg daily from 1 month before conception; (b) an anomaly ultrasound examination at 18/19 weeks; and (c) Vitamin K. Give vitamin K 20 mg orally daily for the last 4 weeks of pregnancy to women on enzyme-inducing antiepileptic treatment. * Educate a woman's partner about what to do should she fit, as epilepsy may be less predictable.

Other medication A decision should be taken about the need to stop other drugs which are associated with fetal abnormalities, e.g. warfarin and lithium.

Epilepsy The risk of fetal abnormality in births to epileptic mothers not on treatment is probably the same as that of the general population (2-3%). This rises to 4-9% if one antiepileptic drug is taken and is

Psychiatric history The Confidential Enquiry into Maternal Deaths in the UK for 1997-9 recognized that the second commonest indirect cause of death was associated

256 OBSTETRIC PROBLEMS

with psychiatric disease (30 deaths over the 3-year period). They recommend seeking a history early in pregnancy and ensuring effective follow up in late pregnancy and the early postnatal period.

(f) Genital herpes and warts (see p. 262). Management to protect the neonate is only an issue in the last weeks of pregnancy.

NUTRITION LIFESTYLE

Anaemia

* Alcohol/smoking. Counsel the couple to stop smoking and women to reduce their alcohol consumption to a maximum of 1 unit a day.

* Check Hb in all women with a previous history or at high risk of anaemia, e.g. vegans, multiple pregnancies, and those with a short interval between pregnancies. * Check the serum ferritin in those women who have a history of iron-deficiency anaemia, even if the haemoglobin is normal.

INFECTION (a) Rubella. All women should have rubella antibodies tested before each pregnancy; immunity can be lost between one pregnancy and the next. In 1986 in the UK, 2% of pregnant women are still not immune.6 (b) Hepatitis B. Women from high-risk countries (especially Vietnam and other areas of South East Asia) or those at risk by virtue of occupation or lifestyle should be screened for hepatitis B. The case for nationwide screening in pregnancy is strong/ and many units now offer this routinely. (c) Listeria. Advise women to avoid soft cheeses, 'cook-chill' foods (unless thoroughly reheated) and pates prior to and during pregnancy. They should avoid contact with sheep at lambing time, and with silage. In 1990 there were 24 cases of listeriosis in pregnant women in England and Wales.8 (d) Toxoplasmosis. Women contemplating pregnancy should be warned about the risks of handling soil, cat faeces or raw meat, and of eating undercooked meat and unwashed vegetables and fruits. Eighty per cent of British women are not immune at the time of pregnancy. There are currently no plans to offer widespread screening in the UK. (e) HIV. Most maternity units in the UK offer screening to all women booking for antenatal care, as precautions taken at delivery (antiretroviral medication and caesarean section) are highly effective at reducing vertical transmission. Several studies have suggested that screening only by risk factor will miss significant numbers of HIV positive women.9

Calcium * Consider giving calcium supplements to all patients at high risk, e.g. those economically deprived, Asian immigrants, repeated pregnancies, women who breast-fed for a prolonged period.

Diet * Advise a balanced diet high in fibre to avoid later constipation. * Recommend that all women take 400 |xg of folic acid a day from before conception until the end of the 12th week. This will prevent 95% of neural tube defects.10

Liver * Advise the woman not to eat liver or liver products, e.g. sausage or pate, because of the high vitamin A content11 or to reduce consumption to no more than 50 g of liver per week or lOOg of pate.12 Furthermore, see Listeria above.

ROUTINE ANTENATAL CARE Antenatal care varies from entirely hospital-based to entirely GP and community midwife-based, which gives equally good results in low-risk

ROUTINE ANTENATAL CARE 257

women.13 It is usually shared between the two. The right setting is that which best meets the needs of the woman concerned.14 Indications for referral for hospital-based antenatal care are: (a) (b) (c) (d) (e)

the patient's own choice; a poor obstetric history; a relevant medical problem; an obstetric complication in this pregnancy; multiple pregnancy.

Antenatal care should cover the following.

Booking (a) Age, ethnic origin of both parents, medical history, obstetric history, family history, alcohol, smoking, social circumstances, and diet. Recommend folic acid 400 jxg daily until week 12 if the patient is not already taking it. (b) Estimate expected date of delivery (EDD). (c) Check blood pressure, heart, weight, height, urine, oedema, teeth. (d) Blood tests: Hb, group and antibodies, rubella, syphilis serology and hepatitis B and HIV, after counselling. Tests for sickle-cell trait and thalassaemia if at risk. (e) MSU: Asymptomatic bacteriuria carries a high risk of overt infection during pregnancy (see p. 265). (f) Decide whether the patient is at normal or higher risk. (g) Discuss the place and mode of delivery, a plan for pain relief and involvement of partner. (h) Book an ultrasound scan for dating, placental site, multiple pregnancy, and gross anomalies. A patient with a history of a previous ectopic pregnancy or with other high risk factors (see below) for an ectopic should have an ultrasound scan as soon as pregnancy is suspected. (i) Counsel for Down syndrome screening by serum biochemistry, or by fetal nuchal scan (or in some centres by a combination test), (j) Arrange for a blood sugar 2 hours after a glucose load, at a later date, according to local policy. The WHO definition of gestational diabetes is a fasting sugar of 7mmol/L or above or a 2-hour sugar of 7.8mmol/L or above.15

Risk factors for ectopic pregnancy (a) (b) (c) (d) (e) (f)

A history of pelvic inflammatory disease; Previous tubal surgery; Previous ectopic pregnancy; Sterilization; Assisted reproduction; Progesterone IUD.

A patient in any of these categories should have an urgent USS as soon a pregnancy is suspected. An intrauterine pregnancy should be visible at 5-6 weeks gestation, or the serum (3HCG concentration should exceed lOOOmiu/L. Diagnosis of an ectopic pregnancy may not only be life-saving, but may also permit medical treatment with methotrexate provided the pregnancy is less than 3.5cm.16

Follow-up appointments What evidence there is suggests that reducing the number of visits is not associated with worse outcomes, except that women may be less satisfied.17 The Royal College of Obstetricians and Gynaecologists recommends a minimum of four appointments, after booking, at the following times: 20-22, 28-32,36 and 40 weeks. In addition, primagravida need a blood pressure check at 26,34 and 38 weeks. The case for fewer routine visits is, however, far from clear.18 Repeated weighing is no longer routinely offered.19

At each appointment check: (a) the patient's general health; (b) BP; (c) urine; (d) oedema; (e) symphysis-fundal height; (f) fetal movements or fetal heart sounds; (g) presentation (after 32 weeks). In addition: (a) at 16 weeks check that USS and alpha-fetoprotein (AFP) have been arranged, if wanted; (b) at 28 and 36 weeks take blood for Hb and ABO and rhesus antibodies; (c) screen for gestational diabetes according to local protocols.

258 OBSTETRIC PROBLEMS

PRENATAL DIAGNOSIS AND SCREENING A GP should be aware of the techniques of prenatal diagnosis in order to: (a) identify all women who might benefit from early assessment by the obstetrician; (b) counsel patients about the accuracy and risk of prenatal diagnosis; (c) make sure that the opportunity for prenatal diagnosis is not overlooked by the obstetrician. Prenatal diagnosis is generally performed to; (a) allow termination of pregnancy when a fetal abnormality is detected; (b) reassure parents when a fetus is unaffected; (c) prepare the parents and the neonatal unit for the birth of an abnormal baby. Women are routinely offered screening in the form of ultrasound, or by maternal serum testing using the 'triple test' (human chorionic gonadotrophin, estriol and alpha-fetoprotein). Several groups in the UK are now looking at combining ultrasound and serum screening either sequentially or concurrently, although the results of these studies are not yet available.

medication, need an informed discussion. For some abnormalities, early diagnosis is available by transvaginal scanning in tertiary centres (e.g. some cardiac anomalies, gastroschisis). For others USS at 18-19 weeks, or sometimes even later, is required (see below).

Down syndrome Triple test: a composite risk score, performed at 16-20 weeks, based on maternal age, serum alphafetoprotein, unconjugated estriol and human chorionic gonadotrophin. If the threshold for offering amniocentesis is set at a risk of 1 in 250, then about 5% of women will screen positive. About 1 in 40 of these will be shown to have an affected fetus.20 Detection rates of only 60% are claimed even if all women proceed to diagnostic testing. In reality, few centres achieve near 100% uptake for screening and advantages over screening on maternal age alone may be less than has previously been published.21 An integrated test, based on screening in the first and second trimesters, is more reliable but not widely available.22

Tests involved Chorionic villus biopsy (CVB)

All women who have a personal or family history of neural tube or other structural defect should be offered an ultrasound examination at 18-19 weeks. Ordinary ultrasound practised by a suitably trained operator should detect 90-95% of all cases of spina bifida, and all cases of anencephaly. Where ultrasonography of adequate quality is not available women should, ideally, be offered a scan in a region where it is, or a serum alpha-fetoprotein at 16-18 weeks (this detects 80% of open defects and about 90% of those with anencephaly). Confirmation by ultrasound is then needed.

This is usually performed after 11 weeks and allows termination of pregnancy in the first trimester, but has a miscarriage rate of 1.2%. There is also a further 1-2% chance of needing an amniocentesis to establish the diagnosis. CVB performed before 10 weeks may rarely cause limb abnormalities.23 It is indicated for, e.g. early diagnosis of chromosomal disorders, inborn errors of metabolism amenable to DNA analysis, haemophilia A and B, sickle-cell disease, alpha- and beta-thalassaemia, cystic fibrosis, muscular dystrophies, Huntington's chorea, alpha-1-antitrypsin deficiency, osteogenesis imperfecta, adult polycystic kidneys, tuberous sclerosis and von Recklinghausen's disease.

Other abnormalities

Amniocentesis

Women with a personal or family history of other abnormalities, or who have been put at risk by

This is usually performed at 15-16 weeks, which will allow termination by 20 weeks. It is associated

Spina bifida, anencephaly or other structural abnormality

PROBLEMS IN PREGNANCY

with a miscarriage rate of 1%. Many laboratories now use polymerase chain reaction technology to give results for Down and Edward syndromes in 48 hours, usually backed by culture results for other chromosomal anomalies available 2 weeks later.

259

72 hours of the bleeding starting, although, if a longer period has elapsed, it may still give some protection. In the second half of pregnancy the dose can be adjusted according to the results of the maternal Kleihauer test.

Ectopic pregnancy Ultrasonography

Routine ultrasonography can detect grosser forms of congenital abnormality, e.g. cranial and neural tube defects, severe skeletal dysplasia and abnormalities of the heart, chest and abdominal organs between 17 and 20 weeks. Hydrocephalus may be detected later. As technology and training improve the range of anomalies detectable prenatally continues to increase. Fetoscopy

Visualization of the fetus is necessary for assessment of some external malformations. It is also used for laser treatment of twin-to-twin transfusion syndrome.

PROBLEMS IN PREGNANCY VAGINAL BLEEDING AND MISCARRIAGE Guideline: Stillbirth and Neonatal Death Society. Pregnancy loss and the death of a baby: guidelines for professionals. London: SANDS, 1995.

* Rhesus status. If a rhesus-negative patient bleeds, anti-D immunization is recommended by the DoH at any stage of pregnancy, whether the bleeding leads to loss or continuation of the pregnancy. There is, however, no evidence that this is of benefit in threatened or naturally completed miscarriages, and it is reasonable to withhold anti-D immunoglobulin in miscarriages without evacuation of the retained products of conception (ERPC) under 12 weeks.24 * If anti-D immunoglobulin is needed, the dose depends upon the preparation used. Give it within

Ectopic pregnancy is usually associated with mild vaginal bleeding as well as pain. A negative pregnancy test (i.e. an HCG level below 50mU/ml) virtually excludes an ectopic. Intrauterine pregnancies should be visible on transvaginal ultrasound when serum HCG exceeds 1000 mU/L. * Admit any patient in whom the possibility of an ectopic is anything more than remote. It is the commonest cause of death in the first trimester and remains in the top five causes of death in pregnancy.25

Suspected miscarriage up to 14 weeks pregnancy Guidance: Ankum WM, Wieringa-de Waard M, Bindels PJE. Management of spontaneous miscarriage in the first trimester: an example of putting informed shared decision making into practice. SMJ2001; 322: 1343-6. Cahill DJ. Managing spontaneous first trimester miscarriage. 6M/2001; 322: 1315-16.

* Women referred to specialist care tend to undergo surgical evacuation, sometimes unnecessarily. * Expectant management at home can avoid referral in a large proportion of cases with possibly better outcomes. * Perform a vaginal examination to assess the cervix. * Do not use a pregnancy test to see whether the pregnancy is viable; too many false positives and false negatives occur. Threatened miscarriage

* If there is modest loss, minor pain and the os is closed, manage at home. Rest will reduce the

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OBSTETRIC PROBLEMS

amount of loss, although it will not affect the outcome. * Prepare the patient for what may happen; 50% will miscarry. * Arrange for an ultrasound detection of fetal heart movements. They are reliably detectable from the 7th week, and may be detectable from the 6th. If they are present, the pregnancy has a 95% chance of continuing.26 If bleeding and pain settle: * Advise the patient that, if the pregnancy continues, the risk of fetal abnormality is no greater than in patients who have not bled. (There is a higher risk of antepartum haemorrhage and early neonatal death, but it is difficult to justify telling the patient this unless asked.) * Arrange an ultrasound scan to confirm that the pregnancy is viable. * If a vaginal examination has not been performed, do so to look for local causes of bleeding, e.g. erosion, polyp or carcinoma. * The use of the triple test at 16 weeks is less reliable. Inevitable miscarriage

* If there is considerable loss, pain and the os is open, refer to hospital. * Remove any products of conception felt within the vagina. Send them to hospital with the patient. * If bleeding is heavy, give Syntometrine 1 ml im while awaiting the ambulance. The decision as to whether to perform a ERPC will be made by the gynaecologists. Not performing ERPC is as effective in four out of five women, but requires greater follow-up.27 Complete miscarriage

* If products have been lost, pain and bleeding have stopped and the os is closed: (a) inform the patient that the miscarriage is probably complete; (b) tell the patient to contact you if more bleeding occurs; (c) discuss with the parents whether to send the products of conception for histology. Some

may wish to have some tissue returned to them so that a burial service can be held. This can provide a focus for their grief. Even if no tissue is available, it is possible for a memorial service to be held. (d) Repeat the pregnancy test after 1 week. A negative test helps to exclude an ectopic. Follow-up after miscarriage

* Arrange to meet all patients who have miscarried about 4 weeks afterwards. Be aware that the patient has suffered a bereavement, and may need counselling; 50% are still likely to feel depressed.28 A quarter are likely to be suffering from post-traumatic stress disorder (PTSD).29 PTSD is also found in a fifth in the third trimester of the next pregnancy30 and is more likely if the next pregnancy follows within a year of the miscarriage. It may be more likely if the mother sees and holds the dead infant. * Attend to any physical issues and the possible need for contraception. The traditional advice to have two normal periods before trying to conceive again may be more important to allow time for the grieving process than for any physical reason. * Be aware that women are distressed by the fact that no reason for the miscarriage can usually be given. Explain that early miscarriage is due either to an abnormality of fetal development, or to a failure of implantation. * Explain that one miscarriage is followed by a slight or even no increase in risk of subsequent miscarriages.31 Investigations are unlikely to be fruitful unless three or more pregnancies have miscarried (see p. 254). Having three consecutive miscarriages gives a patient a subsequent risk of 40%. Referral for investigation is then traditional, but earlier referral would be justified as discussed above. Patient information and support: The Miscarriage Association, c/o Clayton Hospital, Northgate, Wakefield, West Yorkshire, WF1 3JS, helpline 01924 200799; administration 01924 200795 for leaflets about all aspects of miscarriage and ectopic pregnancy; www. miscarriageassociation .org. uk

PROBLEMS IN PREGNANCY

Suspected miscarriage after 14 weeks pregnancy The more advanced the pregnancy, the more advisable it is to admit the patient to hospital at the onset of bleeding, whether or not there has been pain. After 14 weeks, painless bleeding is due to placenta praevia until proved otherwise. Bleeding with severe pain is likely to be due to abruptio placentae. Do not do a vaginal examination in these patients. Patient information and support: For parents whose baby is stillborn or dies soon after birth: SANDS (the Stillbirth and Neonatal Death Society), 28 Portland Place, London W1N 4DE, helpline 020 7436 5881; administration 020 7436 7940; www.uk-sands.org

ABDOMINAL PAIN Abdominal pain may be due to a variety of causes, some of which are serious and which may present in an atypical way. Any cause which could occur outside pregnancy (e.g. renal calculus) must be considered. In the first 20 weeks, consider: (a) miscarriage; (b) ectopic pregnancy; (c) urinary infection; (d) appendicitis; (e) impaction of a retroverted uterus; (f) red degeneration of a fibroid (the maximum incidence is 12-18 weeks, but it may occur at any time); (g) torsion of an ovary or tube; (h) haematoma of the round ligament; (i) accident to an ovarian cyst. After 20 weeks, consider, in addition: (a) (b) (c) (d) (e) (f)

labour; abruptio placentae; haematoma of the rectus abdominis; red degeneration of a fibroid; uterine rupture; dehiscence of the pubic symphysis.

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INFECTION OR CONTACT WITH INFECTIOUS DISEASE Rubella contact • If a pregnant woman is infected with rubella, the risk to the fetus is greatest up to 11 weeks but 30% of fetuses between 11 and 16 weeks are affected. • Infection occurring before 16 weeks is usually considered to be grounds for termination, if requested. • If a pregnant woman is affected between 16 and 19 weeks, the risk of fetal damage is less than 2%. Deafness is the most likely problem. This is not usually considered to be sufficient grounds for termination. • After 19 weeks there appears to be no risk.32 • Accidental immunisation in pregnancy has not been associated with embryopathy. Rubella contact before 16 weeks

If the pregnancy is less than 16 weeks (regardless of whether the woman has had rubella or the vaccination or antibodies were previously detected:33 • Take blood for rubella antibodies. (a) If IgG is present and the blood was taken within 12 days of contact, inform the woman that she is immune and that there is little need to worry. (b) If IgG is present but the blood was taken more than 12 days after contact, request IgM levels. If IgM shows recent infection, discuss the risks of fetal abnormality and the question of termination of pregnancy. (c) If IgG is absent, repeat IgM 2 weeks later. If she has seroconverted, discuss the question of termination of pregnancy as above. The value of immunoglobulin in protecting the fetus is doubtful (see p. 27). (d) Any IgG-negative woman should be immunised in the puerperium. Note: If a woman contracts rubella and decides to continue the pregnancy, fetal blood sampling (from the umbilical vein) from 20 weeks onwards can indicate whether the baby has contracted it by assessing fetal IgM.

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OBSTETRIC PROBLEMS

Varicella (chickenpox) A congenital varicella syndrome, including limb deformities and scarring, may occur in 3% of pregnancies where the mother contracts chickenpox (although not shingles) in the first trimester.34 * A woman who is not known to be immune and who is in contact with chickenpox in the first trimester: check her varicella antibodies, and give VZIG if not immune (see p. 31). Give aciclovir if she develops varicella. * Offer an ultrasound anomaly scan to women contracting chickenpox before 20 weeks gestation. Note: A mother who contracts chickenpox in the week before giving birth, or just after, and delivers an unaffected baby, should be isolated from the baby until no longer contagious.35 Neonatal chickenpox in the first 10 days of life has a mortality of 30%. The baby needs VZIG.

Cytomegalovirus (CMV) CMV infection is now the commonest congenital infection in the UK. The birth incidence is between 0.2-2.5%, and 10% of these infections will produce an affected neonate. Two-thirds of congenital infection are thought to result from primary maternal infection and one-third from recurrent infection in the mother. Ganciclovir is effective in the neonate but has not been proven to be of benefit if given to the mother during pregnancy. Advice to a woman who seroconverts to CMV during pregnancy is difficult and such rare cases should be referred for tertiary centre advice.

Toxoplasmosis Toxoplasmosis causes fetal infection in about 15% of cases in the first trimester, rising to 70% in the third. The risk of serious fetal damage if the fetus is infected is however greater in early pregnancy. Overall, up to 90% of infected babies escape longterm damage. Most maternal cases are asymptomatic. The incidence of children born with definite or probable toxoplasmosis in the UK is very low. In 1989 only 14 cases were notified.36

* Where there is concern, check serology as soon as possible after conception and monthly thereafter. IgM may stay positive for months; timing infection in relation to pregnancy stage requires IgG avidity testing at the National Reference Laboratory. In the case of infection, the woman may be offered USS and amniocentesis to detect fetal abnormality, and fetal blood sampling between 20 and 24 weeks. Even if FBS demonstrates fetal infection, it cannot indicate whether the fetus has been damaged.36 Treatment with spiramycin in pregnancy is safe but of unproven benefit.

Listeria Maternal infection may be asymptomatic or associated with fever. The infection carries a risk of miscarriage, stillbirth or the birth of an infected baby. Symptoms in the mother are so non-specific that the diagnosis is rarely made during the acute illness, especially as the only useful diagnostic test is blood culture. Women with fever for 48 hours who are pregnant and who have no obvious other source of infection should have a blood culture specifying listeria. Treatment is with iv ampicillin.

Hepatitis A Reassure the mother that the infant will not be harmed, although it may be infected if occurring shortly before term.

Hepatitis B Hepatitis B is not influenced by pregnancy, but if the mother is infected there is a risk of acute infection of the fetus or neonate. For the management of babies born to HBsAg positive mothers, see p. 24.

Herpes genitalis Primary infection at delivery gives a 20-50% risk of neonatal herpes, which is frequently fatal. Recurrent herpes at delivery gives a risk of 1-2%. Lower segment caesarean section (LSCS) is usually only recommended if the patient is suffering

PROBLEMS IN PREGNANCY

her first attack during labour. Viral shedding is less in subsequent attacks and maternal IgG crosses the placenta to provide some fetal protection. There is probably little value in taking viral swabs in the last month of pregnancy in those with a history of infection but without active lesions.37 Aciclovir in the last month of pregnancy reduces viral shedding and vertical transmission.38

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more effective but toxicity has occurred in animal studies. * In late pregnancy exclude: UTI, pre-eclampsia and surgical causes of vomiting. * Hyperemesis gravidarum. Admit if the weight loss is more than 5% with ketosis. She needs iv fluids and possibly thiamine.

PRURITUS Genital warts See p. 44.

HIV • A mother who is HIV positive has a risk of fetal infection of 15-25%, but this can be substantially reduced by delivery by caesarean section and/or antiretroviral therapy. Most maternity units offer screening to all women. Some babies will be HIV-antibody-positive at birth due to maternal antibody, but will become negative as maternal antibody is cleared. • Breast-feeding doubles the chance of vertical transmission and is not recommended. If a woman must breast feed then exclusive breastfeeding rather than mixed feeding should be recommended as neonatal seroconversion may be greatest with the bowel inflammation secondary to artificial feeds. • Zidovudine can reduce the chance of vertical transmission if given to the mother antenatally and during labour, and to the baby postpartum.39 • Pregnancy does not appear to hasten the onset of AIDS in women who are HIV positive.

VOMITING AND REFLUX • Advise: (a) a biscuit before getting up; (b) small frequent meals; (c) a low-sodium, low-sugar antacid for heartburn; (d) stop iron if it is making symptoms worse. • Prescribe ranitidine 150mg b.d. to patients not controlled on the above measures. A lesser dose is no better than placebo.40 A PPI is likely to be

Pruritus, without jaundice, is the commonest manifestation of obstetric cholestasis, which, in turn, is associated with premature birth in 60%, fetal distress in up to 33% and intrauterine death in up to 2%.41 * Check LFTs. Transaminases are raised in 60% of cases, though only 25% have a raised bilirubin. * Refer all affected patients. Ursodeoxycholic acid will relieve pruritis. Most obstetricians will deliver the baby at 37 weeks.

EXCESSIVE WEIGHT GAIN * The average weight gain is 10-12.5 kg (0.65kg first quarter, 4 kg by midpregnancy, 8.5 kg by the third quarter, 12.5kg by term). Over a quarter of the total gain is due to fat deposition in the middle two quarters of pregnancy. * Excessive weight gain is associated with an increased incidence of pre-eclampsia. Check BP, urine and the presence of oedema.

FUNDAL HEIGHT * At 22 weeks the fundus should have reached the umbilicus, and at 32 weeks the lower rib border. * The symphysis-to-fundus height in centimetres should be within two of the number of weeks gestation between 20 and 36 weeks. * If the fundus is over 3 cm less than dates, refer for exclusion of fetal intrauterine growth retardation (IUGR) or oligohydramnios. * If the fundus is 3 cm more than dates, refer for assessment for possible multiple pregnancy or hydramnios.

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HYPERTENSION • Blood pressure falls early in the first trimester as the fall in peripheral resistance exceeds the rise in cardiac output. Blood pressure reaches its nadir by 16 weeks, plateaus until 22 weeks and then increases towards term. • Pregnancy-induced hypertension (PIH): Blood pressure which rises to exceed 90 mmHg diastolic on more than one occasion, in the second half of pregnancy, resolves after delivery and is not complicated by proteinuria. The International Society for the Study of Hypertension in Pregnancy has chosen this definition, rather than one based on a relative rise in diastolic pressure, because of the fact that any rise in pressure varies according to when the baseline was taken; and because a relative rise seems to correlate less well with outcomes than the use of an absolute cut-off point. • Pre-eclampsia: This is defined in the same way as above, but is complicated by proteinuria of >300mg/24h. The terms PIH and pre-eclampsia should not be used interchangeably as the former is not associated with poor maternal or fetal outcome, whilst pre-eclampsia is a leading cause of maternal and fetal morbidity. • Women with pre-existing hypertension are prone to develop pre-eclampsia as well, but, if this does not occur, moderate hypertension is compatible with a normal pregnancy. • Antihypertensives do not lessen the risk of developing PIH or alter its progression, but may be necessary for maternal health. • Aspirin prophylaxis (75 mg daily) may prevent the development of pre-eclampsia and moderate the condition once it has started. • Measuring the blood pressure. Use phase 4 diastolic pressure and not phase 5, which may be misleadingly low in pregnancy.42

Pre-existing hypertension • Ideally, all women should have their blood pressure measured preconceptually. If the first measurement is made in the first trimester, the vasodilation of normal pregnancy may mask a higher figure. A blood pressure of >140/90 before 20 weeks suggests pre-existing hypertension and needs specialist assessment.

Anti-hypertensives may reduce the chance that mild hypertension will become severe. Whether one drug is better than another is not clear.43

Pre-eclampsia * Arrange for the patient to be seen at the first available clinic, and: (a) consider aspirin 75 mg daily; (b) start a daily kick chart, with instructions to report if there has been a 50% drop in kicks by noon or less than 10 kicks by noon; (c) daily checks on urine for protein; (d) twice-weekly BP checks. * Admit if: (a) the BP is over 160/100. The patient needs antihypertensives; or (b) the urine has one plus or more of protein; or (c) there is evidence of intrauterine growth retardation; or (d) there are symptoms related to pre-eclampsia, e.g. headache, nausea, visual disturbance and epigastric pain. Admit these patients as an emergency.

Eclampsia • This is a medical emergency with a high maternal mortality. The patient must be resuscitated, and transferred to hospital as fast as possible. There is now good evidence that the anticonvulsant of choice to prevent further fits is magnesium sulphate. • Recurrence and follow up: many women will have PTSD when their normal physiological pregnancy has been taken from them. It is important to explain what happened and the relatively low chance of recurrence in future pregnancies (20%). Many units offer uterine artery Doppler as a screening test in subsequent pregnancies, with the possibility that aspirin, or vitamin C and E (antioxidants44), can reduce this rate further.

Proteinuria A trace of protein is acceptable. It may be due to contamination with vaginal secretions or to a delay since the urine was passed.

PROBLEMS IN PREGNANCY 265

One plus or more in the absence of raised BP * Arrange for an MSU for culture and microscopy. If negative, check the serum creatinine, the 24-hour urinary protein and refer. * Arrange to see the patient again within 2 days looking for clinical deterioration; 10% of patients who develop eclampsia have proteinuria without a raised BP.45

• Warn the patient of her 20-30% chance of developing diabetes in the next 5 years. This risk can be decreased, or postponed by maintenance of a 'diabetic diet' after pregnancy.

ANAEMIA

Glycosuria

• As the plasma volume increases in pregnancy, the haemoglobin falls. Only a haemoglobin <11 g/dl (WHO) or even <10g/dl in the second trimester is considered to be anaemia.47 • Iron stores are depleted in pregnancy; 30 mg/ day of elemental iron prophylactically will minimize this. One tablet of ferrous sulphate or one of the proprietary combined preparations is more than enough. 'Double iron' in the absence of anaemia achieves nothing except an increase in side-effects. • A low serum ferritin is not a reliable guide to iron deficiency in pregnancy. It may reflect a shift of iron stores to the increased red cell mass rather than a low total body iron.

This occurs in 70% of pregnant women. If glycosuria is detected:

Indications for prophylaxis

Bacteriuria Two to ten per cent of pregnant women have asymptomatic bacteriuria. If untreated, 30% will develop a urinary infection. Antibiotics are very effective in preventing pyelonephritis.46 * Treat with ampicillin, amoxicillin, cephalosporin or nitrofurantoin according to sensitivities. * Repeat the MSU 2 weeks after treatment.

* Repeat the urine test on a second morning specimen (i.e. the second urine sample passed in the morning while the patient is still fasting). * If still positive arrange for a modified glucose tolerance test (GTT). If the sugar level is raised (fasting sugar ^7mmol/L or the 2-hour sugar 3=7.8mmol/L; see p. 148), refer the patient urgently. Gestational diabetes Guideline: Recommendations for the management of pregnant women with diabetes (including gestational diabetes). London. Diabetes UK 2002. Online. Available: www.diabetes.org.uk

Iron The following women should definitely be given prophylaxis: (a) those who have previously suffered from iron deficiency anaemia; (b) those with a low booking Hb; (c) those on a poor diet; (d) those where chronic blood loss has occurred; (e) those where only a short interval has elapsed since the last pregnancy; (f) those where concomitant folate therapy, other than as prophylaxis, is given (except in haemoglobinopathies). Folate

* The patient should be managed intensively (see p. 267) if her 2 hour sugar is over 9mmol/L or her fasting glucose is over 6 mmol/L. For most patients, however, diet will be sufficient to control blood sugars. * Recheck the modified GTT 6 weeks postpartum to be sure that it has returned to normal.

Folate is usually given as combined therapy, except in cases of haemoglobinopathy. The following should receive folic acid 5mg daily: (a) those where previous folate deficiency has occurred;

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OBSTETRIC PROBLEMS

(b) those with malabsorption; (c) those with haemoglobinopathies; (d) those on anticonvulsant therapy, especially phenytoin; (e) the multiparous; (f) those with a multiple pregnancy; (g) adolescents. Anaemia developing in pregnancy

* Check full blood count, film and serum ferritin. Check vitamin B12 and red cell folate if there is macrocytosis. * Start iron in treatment doses if the haemoglobin is below lOg/dl or MCV below 82 fl, e.g. give ferrous sulphate 200 mg t.d.s. and folic acid 5 mg daily. * Repeat Hb in 2 weeks. It should rise by about 0.8 g/dl per week. * If there has been no response: (a) exclude occult infection, especially urinary; and either (b) consider arranging for parenteral iron; or (c) if the serum ferritin is normal, check the serum B12 and red cell folate (if not already checked) and start combined iron and folate if not already begun. * If the Hb remains low, seek expert advice. Consider an Hb electrophoresis regardless of the apparent ethnic origin.

RHESUS-NEGATIVE MOTHERS * Check antibodies at booking and in primagravida at 28 and 36 weeks; in multigravida, monthly from 24 weeks. If the maternal anti-D levels are 0.5-10.Oiu, antibody levels are needed every 2 weeks. A level above 10 means that fetal blood sampling is needed and referral to a fetal medicine unit is indicated. If gestation allows, delivery, rather than intrauterine transfusion, may be the preferred option. It is important to realize (and explain) that subsequent pregnancies may behave similarly. * Give anti-D antenatally, or check that the obstetrician has given it, in the following circumstances:

(a) abdominal trauma including external cephalic version; (b) chorionic villus biopsy and amniocentesis; (c) antepartum haemorrhage; (d) ectopic pregnancy; (e) termination of pregnancy or ERPC; (f) threatened or complete miscarriage after 12 weeks. Several studies have suggested that routine prophylaxis of all non-sensitised Rh-negative women is cost effective in preventing sensitization.48 NICE recommends routine anti-D prophylaxis in Rh-negative women at 28 and 34 weeks, except where a woman is sure she will have no more children, or where the father is Rh-negative.

ABNORMAL LIE * Check the lie from 30 weeks onwards. If a transverse lie is found at 32 weeks or later, then either: (a) refer; or (b) arrange a scan and reassess at 36 weeks. Refer if still transverse. Note: A breech presentation should be seen at the hospital by 37-38 weeks.

HIGH HEAD A 'high head' is one that lies completely out of the pelvis. Make sure that the bladder and rectum are empty before accepting that the head is high. * Primipam: refer by 36 weeks; * MuHipam: refer if still high at 38 weeks.

POST-MATURITY If the expected date of delivery was established by USS in early pregnancy, routine induction of labour at 41 weeks reduces perinatal mortality.49 Routine induction does not cause a rise in the rate of LSCS, nor in lower maternal satisfaction, and it is the cheaper option. The Drug and Therapeutics Bulletin recommends that the issue be discussed at 41 weeks with a decision by 42 weeks.50

PROBLEMS IN PREGNANCY

SPECIFIC MEDICAL CONDITIONS AND PREGNANCY Asthma Deterioration occurring during pregnancy is usually due to a reduction in therapy because of a fear that it will harm the fetus. Beta-sympathomimetics, inhaled steroids, theophyllines and ipratropium are safe. Oral steroids are safe and can be used for acute exacerbations and low dose maintenance.

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between 1 and 3 months and may present with features of hyperthyroidism, but more commonly with those of hypothyroidism, i.e. fatigue and lethargy, at a time when she is understandably tired anyway. Thyroxine is necessary for 6 months, followed by repeat TFTs. Antithyroid drugs are not usually needed for the hyperthyroid state, but beta-blockers may be given to control symptoms. * Follow the patient with yearly TFTs. Up to 20% develop hypothyoidism in the subsequent 4 years.52

Diabetes Optimum control of blood sugars should be achieved by the time of conception to reduce the risk of congenital malformation. The GP should emphasize the importance of good control, even though the majority of the management will be done by the hospital. * Patients should be seen 2-weekly before 28 weeks and then weekly. * The insulin dose may need to be doubled or tripled, and will need to be reduced immediately after delivery to the prepregnant dose. * Blood sugar profiles should show the majority of readings (pre-meals) below 6mmol/L. * Aim to keep the HbAlc at 6 or below. * Women should have serial ultrasound scans to exclude macrosomia and placental insufficiency.

Thyroid disorders Pre-existing thyroid disease

* Thyroid requirements change little during pregnancy, and the fetus is not affected by maternal thyroxine.51 Thyroid function should be checked at booking and 36 weeks. * Carbimazole can be continued throughout pregnancy, but may cause fetal goitre. Give the lowest dose that will control the condition and do not use the combination of high dose carbimazole and thyroxine. Postnatal thyroiditis

* Up to 10% of women develop transient autoimmune thyroiditis after delivery. This is usually

Epilepsy * The association between epilepsy and treatment with congenital malformations has been discussed (p. 255). Twenty per cent of patients have an increase of fits during pregnancy due either to poor compliance or to a fall in serum levels because of the physiological changes of pregnancy. Fits are more common when tired, which may explain the increased frequency of fits in the postnatal period. * Do not stop anticonvulsants or reduce their dose if a women presents already pregnant. Any teratogenic effect will have already occurred. Liaise with her neurologist to plan the rest of the pregnancy. In general it is not necessary to monitor serum levels of anticonvulsant in pregnancy, but to be guided by the clinical condition, and not to increase a drug unless the frequency of fits increases. A patient who drives or for whom a single fit would be a disaster may prefer drug level monitoring.53 * Ensure that an anomaly ultrasound is performed at 18-19 weeks. * Recommend folk acid 5 mg daily to all epileptic women until the end of the 12th week (see p. 255). This is particularly important in patients taking sodium valproate or carbamazepine or who have a history of a previous baby with a neural tube defect.10 * Prescribe vitamin K lOmg daily for the last 4 weeks of pregnancy to reduce the risks of maternal and fetal bleeding, in those taking enzymeinducing drugs, i.e. phenytoin, barbiturates and carbamazepine.

268 OBSTETRIC PROBLEMS

DRUG MISUSE * Amphetamines and cocaine: stop the drugs immediately. * Benzodiazepines: withdraw over 4 weeks (see p. 324). * Barbiturates: arrange admission. If there is any delay maintain the patient on phenobarbitone (see p. 330). * Opiates: arrange an urgent outpatient appointment. Maintain the patient on oral methadone meanwhile.

INTRAPARTUM CARE Policy statement: Royal College of GPs and the BMA (1997). General practitioners and intrapartum care: interim guidance. Available: BMA House, Tavistock Square, London WC1H 9JP. www.bma.org.uk

• The guidance above defines three levels of GP involvement in intrapartum care: (a) The GP provides general medical care only, with the responsibility to refer a patient to another professional to provide intrapartum care if the patient wishes to deliver at home; (b) The GP attends a patient in labour as non-specialist back-up for the midwife, with the midwife taking responsibility for the delivery; (c) The GP provides intrapartum care and has the competence, over and above that of the average GP, to do so. The GPC of the BMA advises GPs that, in order to assume this role, they must be skilled in the identification of abnormalities of labour, in perineal suturing, in the resuscitation of mother and baby and the insertion of an iv line.54 • It is the policy of the two relevant Royal Colleges that women who wish to have a home birth should be able to do so. • There is growing evidence that the risks of planned home delivery have been overemphasized in the UK, with a perinatal hazard associated with planned home births of <1%.55

* Home delivery is valued by those who choose it, even in those who are transferred to hospital during labour.56

When a woman requests a home delivery * Check that there are no medical or obstetric contraindications. Some contraindications, e.g. previous retained placenta, can be dealt with by 'domino', where most of labour takes place at home with the briefest possible admission for the delivery. * Refer to the community midwife, who has a statutory duty to provide a service at home, whether or not a GP is willing to provide maternity services.

POSTNATAL CARE * See the mother and baby within 24 hours of discharge, and then again as often as needed.

Mother * Examine fundal height, perineum, lochia and breasts. * Feeding. If necessary, reinforce the midwife's advice on breast feeding (see below). * Contraception. Hormonal methods should not be started before 4 weeks or before bleeding has stopped. * Rest. Stress the need for rest in the first week, but also that the mother should be ambulant from day 2 to prevent deep vein thrombosis. * Pelvic exercises. Urge the patient to perform pelvic exercises from the first day. * Warn the patient that she will inevitably receive conflicting advice from professionals, family and friends. Parents need to develop their own solutions and disregard dogma from all sides. * Depression. Identify vulnerable women (see p. 270) and arrange support to try to prevent depression.

POSTNATAL CARE

Baby * Examine the baby, unless this has already been done in hospital. * Vitamin K. If it has not already been given im, give it orally according to local guidelines. If the baby is breast-fed, it will need to be repeated at 1 and at 6 weeks. * Registration. Encourage early registration of the baby with the Registrar; the start of Child Allowance is dependent on this. Register the baby with the practice.

At 6 weeks Review the patient's experiences. * Ask about sleep, perineal pain and incontinence. Screen for depression (see p. 271). * Perform a vaginal examination only if symptoms dictate or if a smear is due.57 * Offer contraceptive advice. An explanation that libido is often low in the first year may prevent future problems. * Prevention of sudden infant death syndrome. Reinforce the advice already given by the health visitor by stressing: (a) the sleeping position (see p. 51); (b) the importance of not smoking; and of (c) not giving the baby a duvet.

Breast-feeding Guideline: Renfrew MJ, McGill HR. Enabling women to breastfeed. DoH 2000. London. The stationary office.

Advantages of breast feeding • Advantages for the baby: less infection, less chance of atopy or diabetes, better bonding. • Advantages for the mother: a lower risk of breast and ovarian cancer, weight loss, and less postpartum bleeding. It is also cheaper.

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jaw and tongue to drain the milk ducts under the areola. * Check that mother and baby are managing the following: (a) the baby's chest against the mother's chest, with the baby's chin to the breast; (b) the mouth wide open; (c) both lips curled back; (d) the lower lip at the junction of the areola and breast; (e) rhythmical movements of jaw muscles. Patient information: Breastfeeding: you and your baby. DoH 1996. Available: PO Box 410, Wetherby, WestYorksLS237LN.

Failing lactation * Suckling is the strongest stimulus. Ensure that the baby is sucking well (see above). * Advise the mother to go to bed for 2 days with the baby, to feed on demand (mother and baby). * In general drugs should not be used, but a short course of domperidone may restore prolactin secretion. Sore nipples These are usually due to the baby's tongue rubbing the nipple rather than the areola. * Check that the nipple is positioned in an upwards direction towards the roof of the baby's mouth. * Allow breast milk to dry on the nipple when not feeding, or, if the skin is broken, use white soft paraffin. Skin infection

Problems with breast feeding

This is usually due to Candida infection and may present as localized soreness, as pain around the areola and nipple or as pain in the breast after a feed.

The majority of problems centre around the infant's attempts to remove milk. The baby needs to be in a comfortable position in order to allow

* Treat the mother with miconazole cream and the baby with miconazole oral gel, whether the baby has signs of infection or not. It is

270 OBSTETRIC PROBLEMS

unnecessary for the mother to remove the cream before feeding. Blocked duct

* After stillbirth, or if there is another good reason to stop lactation, use bromocriptine 2.5 mg at night for 2 nights then 2.5 mg b.d. for 3 weeks. If used for a shorter period, a number of patients relapse.

This presents as a hard lump anywhere in the breast.

Vaginal bleeding after 24 hours

* Get the mother to massage the breast while feeding the baby from that breast. * The baby's position should be altered so that the lower lip is nearer the blocked duct.

* The traditional management of excessive bleeding more than 24 hours after delivery (secondary PPH) is surgical. However, ERPC yields placental tissue in less than 30% of these cases, and so the majority of patients do not benefit.

Breast engorgement

* Mild bleeding. Give: (a) ergometrine 0.5 mg im then 0.5 mg t.d.s. orally for 4 days; and (b) antibiotics, e.g. amoxycillin/erythromycin ± metronidazole for 5 days. * Severe bleeding, or if the os still admits one finger after 7 days: Admit. Admit more readily if there is no-one at home to look after the patient, if she is already anaemic, febrile or otherwise unwell.60

The pain of engorgement is one of the commonest reasons for stopping breast feeding in the first 2 weeks. Treatment is disappointing with cabbage leaves, ultrasound and cold packs being no better than placebo.58 * Give simple analgesia and support; * Attempt to prevent engorgement by removing any obstacles to easy breast-feeding. Mastitis

Fever

Early mastitis is inflammatory, not infectious, and may respond to effective emptying of the breast plus an NSAID.59

* Endometritis. Patients with fever, pain and foul-smelling lochia are likely to need admission, but early cases could be treated at home with amoxicillin/erythromycin and metronidazole (see above). * Be aware that DVT and UTI can cause fever without localized symptoms.

* Reassess the feeding position of the baby to ensure that milk is removed completely. * Prescribe an NSAID. * Treat with flucloxacillin for 10-14 days if the patient is unwell, febrile or there is any suspicion of abscess formation. Use erythromycin if the patient is allergic to penicillin. Warn mothers that the milk will change in taste and feeding may be harder initially, or that the baby may develop diarrhoea and need to feed more frequently. * Breast abscess. If an abscess forms which is large enough to need draining, admit the patient for incision and drainage. Suppression of lactation

This usually takes 4-5 days after stopping breast feeding. It may be very painful and require analgesia and a supportive bra.

Depression • This occurs in 10-15% in the first year after delivery, usually in the first 6 months. The symptoms are almost always present at 6 weeks. It is distinct from the transient 'blues' of the first 10 days, and from a puerperal psychosis, which is likely to need admission. • It is more likely in those: (a) with previous psychiatric illness; (b) with a family history of depression; (c) who were unhappy during pregnancy; (d) with marital difficulties; (e) with recent stressful events;

REFERENCES

(f) on low income; (g) in poor housing; (h) with physical illness or handicap in the family. * Patients often do not realize that they are depressed, and doctors recognize it even less frequently. Women often present with a feeling of not coping rather than with classical symptoms of depression. * Every woman should be screened at the postnatal examination. The Edinburgh Postnatal Depression Scale61 (see Appendix 15) may be administered by a trained professional. If the scale is not available, ask four simple questions: (a) How are you feeling? (b) How are you sleeping? (c) How are you eating? (d) Are you enjoying the baby? If depression is found * Check TFTs in those complaining mainly of tiredness (see p. 267). * Counsel. Simple non-directive counselling by health visitors, weekly for 8 weeks, doubles the recovery rate.62 * Antidepressants. Treat vigorously those scoring 5 or more on the test for major depression (see p. 309). Lofepramine is recommended in those who are breast-feeding because of its shorter halflife; the risk of drug accumulation in the infant is less.63 Fluoxetine is recommended in a woman who is not breast feeding.64 Consider referral if a multidisciplinary mental health team is able to

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offer more than the primary health care team. Ideally, if the woman requires admission, this should be to a mother and baby facility. * Refer urgently, to be seen within 24 hours, any women with ideas of suicide or of harming the baby. Patient information and support: The Association for Postnatal Illness, 145 Dawes Road, London SW6 EB, helpline 020 7386 0868; www.apni.org

Neonatal jaundice * Jaundice within 24 hours of birth: admit. * Deep jaundice developing after 24 hours: (a) admit if the baby is unwell or is at risk because of prematurity, small for dates or birth asphyxia; (b) otherwise, arrange for a serum bilirubin. If the level is 290mmol/L or above, discuss with the paediatric team. * Jaundice still present at 2 weeks: test the urine for bilirubin and the serum for conjugated bilirubin. If either is positive, admit. The baby may have biliary atresia. The earlier surgery is performed, the more successful it is likely to be.65 * Admit if jaundice is associated with any of the following: (a) pale fatty stools; (b) dark yellow urine; (c) failure to thrive; (d) poor feeding; (e) a tendency to bleed or bruise; (f) an enlarged liver or spleen, or ascites.

REFERENCES 1. Harris R et al. National confidential enquiry into counselling for genetic disorders by non-geneticists: general recommendations and specific standards for improving care. Br J Obst Gynaecol 1999; 106: 658-63. 2. Regan L et al. Influence of past reproductive performance on risk of spontaneous abortion. BMJ 1989; 299: 541-5. 3. Steel JM et al. Can pre-pregnancy care of diabetic women reduce the risk of abnormal babies? BMJ 1990; 301: 1070-1. 4. Holmes JB, Harvey EA, Coull BA et al. The teratogenicity of anticonvulsant drugs. New Eng J Med 2001; 344: 1132-8.

5. DTB. Epilepsy and pregnancy. Drug Ther Bull 1994; 32: 49-51. 6. Berkeley MI et al. Surveillance of antibody to rubella virus in Grampian: closing the immunity gap. BMJ 1991; 303: 1174-6. 7. Boxall EH. Antenatal screening for carriers of hepatitis B virus. BMJ 1995; 311: 1178-9. 8. Standing Medical Advisory Committee. The diagnosis and treatment of suspected listeriosis in pregnancy. London: Department of Health, 1992. 9. Lester P et al. The consequences of a positive prenatal HIV antibody test for women. / Accjuir Immiin Def Synd Hum Retrovir 1995; 10: 341-9.

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10. DTB. Folic acid to prevent neural tube defects. Drug Ther Bull 1994; 32: 31-2. 11. Department of Health. Vitamin A and pregnancy, DoH letter. 25 October 1990. 12. Nelson M. Vitamin A, liver consumption, and risk of birth defects. BMJ 1990; 301:1176. 13. Tucker JS et al. Should obstetricians see women with normal pregnancies? BMJ 1996; 312: 554-9. 14. Department of Health. Changing childbirth. Report of the Expert Maternity Group. London: HMSO, 1993. 15. Anon. Recommendations for the management of pregnant women with diabetes (including gestational diabetes). Diabetes UK. Online. Available: www.diabetes.org.uk 16. Mascarenhas L et al. The changing face of ectopic pregnancy. BMJ 1997; 315: 141. 17. Villar J, Carroli G, Khan-Neelofur D et al. Patterns of antenatal care for low-risk pregnancy (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 18. Sikorski J et al. A randomized controlled trial comparing two schedules of antenatal visits: the antenatal care project. BMJ 1996; 312: 546-53. 19. Dawes MD, Grudzinskas JD. Repeated measurement of maternal weight during pregnancy. Is this a useful practice? Br J Obst Gynaecol 1991; 98: 189-94. 20. Wald NJ et al. Antenatal maternal serum screening for Down's syndrome: results of a demonstration project. BMJ 1992; 305: 391-4. 21. Howe DJ et al. Six year survey of screening for Down's syndrome by maternal age and nuchal thickness ultrasound scans. BMJ 2000; 320: 606-10. 22. Gilbert RE, Augood C, Gupta R et al. Screening for Down's syndrome: effects, safety, and cost effectiveness of first and second trimester strategies. BMJ 2001; 323: 423-5. 23. Lilford RJ.The rise and fall of chorionic villus sampling. BMJ 1991; 303: 936-7. 24. Everett CB. Is anti-D immunoglobulin unnecessary in the domiciliary treatment of micarriages? BMJ 1988; 297: 32-3. 25. Department of Health. Report on confidential enquiries into maternal deaths in the UK 1988-90. London: HMSO, 1991. 26. Everett CB, Preece E. Women with bleeding in the first 20 weeks of pregnancy: value of general practice ultrasound in detecting fetal heart movement. Br J Gen Pract 1996; 46: 7-9. 27. Nielsen S, Hahlin M. Expectant management of first-trimester spontaneous abortion. Lancet 1995; 345: 84-6. 28. Friedman T. Women's experiences of general practitioners' management of miscarriage. / R Coll Gen Pract 1989; 39: 456-8. 29. Engelhard IM, van den Hout M, Arntz A. Posttraumatic stress disorder after pregnancy loss. Gen Hosp Psychiatry 2001; 23: 62-6. 30. Turton P, Hughes P, Evans CDH et al. Incidence, correlates and predictors of post-traumatic stress disorder in the pregnancy after stillbirth. Br J Psychiatry 2001; 178: 556-60. 31. Everett C. Incidence and outcome of bleeding before the 20th week of pregnancy: prospective study from general practice. BMJ 1997; 315: 32-4. 32. Jones G et al. Congenital rubella in Great Britain. Health Trends 1990; 22: 73-6.

33. Best JM et al. Fetal infection after maternal reinfection with rubella: criteria for defining reinfection. BMJ 1989; 299: 773-5. 34. Gilbert GL. Chickenpox during pregnancy. BMJ 1993; 306: 1079-80. 35. RCOG. Chickenpox in pregnancy. 2001. Clinical guideline, www.rcog.org.uk 36. Robinson R. Surveying rare diseases of childhood. BMJ 1991; 303: 1091. 37. RCOG. Management of genital herpes in pregnancy. Clinical Green Top Guideline 2002. www.rcog.org.uk 38. Braig S et al. Acyclovir prophylaxis in late pregnancy prevents recurrence of genital herpes and viral shedding. Eur J Obs Gyn Reprod Biol 2001; 96: 55-8. 39. Connor EM et al. Reduction of maternal-infant transmission of human immunodeficiency virus type 1 with zidovudine treatment. New Engl J Med 1994; 331:1173-80. 40. Larson JD et al. Double-blind, placebo-controlled study of ranitidine for gastroesophageal reflux symptoms during pregnancy. Obst Gyn 1997; 90: 83-7. 41. Milkiewicz P, Elias E, Williamson C et al. Obstetric cholestasis. BMJ 2002; 324: 123-4. 42. MacGillivray I, Thomas P. Recording diastolic blood pressure in pregnancy (letter). BMJ 1991; 302: 179. 43. Duley L. Pre-eclampsia and hypertension. Clinical Evidence, Issue 7. BMJ Publishing Group. 2002. 44. Chappell L et al. Effect of anti-oxidants on the occurrence of pre-eclampsia in women at increased risk: a randomised trial. Lancet 1999; 354: 810-16. 45. Douglas KA, Redman CWG. Eclampsia in the United Kingdom. BMJ 1994; 309: 1395-1400. 46. Smaill F. Antibiotics for asymptomatic bacteriuria in pregnancy (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 47. DTB. Routine iron supplements in pregnancy are unnecessary. Drug Ther Bull 1994; 32: 30-1. 48. Royal College of Obstetrics and Gynaecology. Use of anti-D immunoglobulin for Rh prophylaxis. RCOG guideline No 22. London: RCOG, 1999. www.rcog.org.uk 49. Crowley P. Interventions for preventing or improving the outcome of delivery at or beyond term (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2001. 50. DTB. Managing post-term pregnancy. Drug Ther Bull 1997; 35: 17-18. 51. Girling JC, de Swiet M. Thyroxine doses during pregnancy in women with primary hypoythyroidism. Br J Obst Gynaecol 1992; 99: 368-70. 52. DTB. The practical management of thyroid disease in pregnancy. Drug Ther Bull 1995; 33: 75-7. 53. O'Brien MD, Gilmour-White S. Epilepsy and pregnancy. BMJ 1993; 307: 492-5. 54. GPC. The role of the GP involved in intrapartem care. London: BMA, 1999. Online. Available: www.bma.org.uk (choose 'committees' then 'GPC Guidance' then 'General Practitioners and maternity medical services'). 55. Northern Region Perinatal Mortality Survey Co-ordinating Group. Collaborative survey of perinatal loss in planned and unplanned home births. BMJ 1996; 313: 1306-9. 56. Davies J et al. Prospective regional study of planned home births. BMJ 1996; 313: 1302-6.

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57. Noble T. The routine 6-week vaginal examination. BMJ 1993; 307: 698. 58. Snowden HM, Renfrew MJ, Woolridge MW. Treatments for breast engorgement during lactation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 59. Thomsen AC et al. Course and treatment of milk stasis, noninfectious inflammation of the breast, and infectious mastitis in nursing women. Am ] Obst Gyn 1984; 149: 492-5. 60. DTB. The management of postpartum haemorrhage. Drug Ther Bull 1992; 30: 89-92. 61. Cox JL, Holden JM, Sagorsky R. Detection of postnatal depression: development of the 10-item Edinburgh

62.

63. 64.

65.

Postnatal Depression Scale. Br J Psychiatry 1987; 150: 782-786. Holden JM et al. Counselling in a general practice setting: controlled study of health visitor intervention in treatment of postnatal depression. BMJ 1989; 298: 223-6. Nicholls KR, Cox JL. Lofepramine and breast feeding (letter). Psych Bull 1996; 20: 309. Hoffbrand S, Howard L, Crawley H. Antidepressant drug treatment of postnatal depression (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2001. Mackinlay GA. Jaundice persisting beyond 14 days after birth. BMJ 1993; 306: 1426-7.

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CHAPTER CONTENTS Urinary tract infection 275 Suspected uncomplicated lower urinary tract infection in women 275 'Frequency dysuria' or 'urethral' syndrome 277 Suspected lower urinary tract infection in men 277 Lower urinary tract infection in other situations 278 Suspected acute pyelonephritis 278

14 Urinary problems

Urinary incontinence 278 Difficulty voiding 281 Bladder outflow problems in men 281 Retention of urine in women 283 Carcinoma of the prostate 283 Urinary stones 283 Renal disease 284 Asymptomatic proteinuria 284 Asymptomatic haematuria 284 Renal insufficiency 285 References

285

URINARY TRACT INFECTION SUSPECTED UNCOMPLICATED LOWER URINARY TRACT INFECTION IN WOMEN • Half of the women presenting with frequency and dysuria do not have bacterial infection.1 • Half of those with bacterial infection resolve in 3 days without treatment.2 • Management of uncomplicated lower UTI in women can be as effective over the telephone as if the patients are seen in person.3 1. Decide on the need for immediate treatment. * Test the urine with a dipstick that includes tests for protein, blood, nitrites and leucocytes. * If positive for nitrites or leucocytes, assume that this is infection and treat with antibiotics without sending an MSU for culture unless the patient has recurrent or complicated infections, other complicating illnesses, or there is subsequently a poor response to treatment. * If negative for nitrites and leucocytes but positive for protein or blood, send an MSU for microscopy and culture and then treat with antibiotics. * If negative for all four tests, send an MSU for microscopy and culture without starting antibiotics. At least 90% will have a negative MSU.4 2. If treating, give antibiotics for 3 days. This is as effective as a 7-day course.2 Trimethoprim is still the drug of choice. Most infections respond even if reported as resistant in vitro, as the urine concentration of the drug exceeds that of the 275

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URINARY PROBLEMS

sensitivity disk.4 Nitrofurantoin is an alternative. Resistance to amoxicillin, in the UK, is about 40%. Co-amoxiclav has a higher incidence of cholestatic jaundice than amoxicillin (1 in 6000 adults), and should only be used when there are grounds to suspect resistant organisms.5 Quinolones should be reserved for complicated infections.6 3. Ask the patient to return or telephone to find out what the MSU showed. Patients without bacterial infection should not be given blind antibiotics on a subsequent occasion. Patients with resistant organisms may need a change of antibiotics. 4. Explain to the patient that symptoms should clear within 5 days. Advise the patient to contact the surgery or make an appointment if symptoms are still severe after 3 days.

Patients with a recurrence of symptoms * Repeat the MSU and assess whether the patient is suffering from: (a) relapse - the organism is the same; (b) reinfection - a different organism is present; or (c) the 'frequency dysuria syndrome', see below. Bacterial counts of >105/ml are significant, but lower counts may be found with organisms that are difficult to culture (e.g. Staphylococcus saprophyticus). Counts of 103 and 104/ml should be repeated if the symptoms suggest infection. Organisms without cells can be ignored, except in pregnant women and young children. Cells without organisms need further investigation, unless the MSU was taken after antibiotics had been started. Repeat the MSU and refer if cells are still present. Relapse

* Treat with an antibiotic to which the original organism was sensitive. Stress the importance of taking the whole course. * Look for a reason for the failure to clear the original infection. Consider stones and chronic retention.

Reinfection

* Use a different antibiotic; the new organisms are likely to be resistant to the one originally used, e.g. co-amoxiclav, a quinolone or a third generation cephalosporin. * Self help: Recommend that patients: (a) increase fluids; (b) increase the frequency of micturition (practise double voiding, i.e. attempting to pass urine a second time immediately after the first); (c) empty the bladder before sleep and after sexual intercourse; (d) wear loose-fitting cotton underwear and avoid tights; (e) avoid external sanitary towels; (f) make sure a diaphragm, if worn, fits comfortably; (g) use a lubricant, e.g. K-Y jelly, if vaginal dryness makes intercourse painful; (h) wipe from front to back and wash the vulva with soapy water; (i) consider drinking cranberry juice. A daily intake of 50 ml of concentrate (= 7.5 g of cranberry concentrate) can reduce the rate of reinfection in healthy young women by 56% (RRR) giving an NNT of 5 (95% CI 3 to 34)7 Frequent reinfection

* Give the patient an MSU bottle and a supply of antibiotics to keep at home so that there is no delay in starting treatment with each attack. * Prophylaxis. Consider low-dose prophylaxis in those with >4 attacks per year. Use nitrofurantoin lOOmg at night or trimethoprim 100 mg at night for 3-6 months in the first instance. They will not cause the development of resistant bowel organisms. However, if an infection occurs while on prophylaxis, use a different drug as treatment. * Consider investigating, particularly in the older patient, to rule out underlying abnormalities such as a large residual urine. In young women the usual recommendation is for a plain film of the abdomen and an USS after more than three infections. If normal, there is no need for specialist referral.

URINARY TRACT INFECTION

Infections occurring after intercourse

* Advise emptying the bladder after intercourse. * Give a single dose of antibiotics to be taken within 2 hours of intercourse, e.g. trimethoprim 200 mg.

'FREQUENCY DYSURIA' OR 'URETHRAL SYNDROME Review: Hamilton-Miller JM. The urethral syndrome and its management. J Antimicrobial Chemother 1994; 33 (Suppl. A): 63-73.

* Examine to exclude: (a) vaginal infection, especially Candida, chlamydia, trichomonas, gardnerella or gonorrhoea; (b) urethral herpes or warts; (c) significant anterior prolapse; (d) atrophic vaginitis. * Arrange for 3-monthly MSUs to exclude intermittent infection. Ask for counts as low as 102 to be reported. * Ask the patient to keep a diary of input, output and symptoms for 1 week, and manage as for detrusor instability. * Self help. Patients should: (a) alkalinize the urine, e.g with potassium citrate mixture; (b) avoid coloured toilet paper, scented soaps, bubble baths, douches, antiseptics, talcum powder, vaginal deodorants and deodorized tampons; (c) ensure that sexual intercourse is not traumatic because, for instance, of lack of lubrication. * Do not treat with antibiotics, as overgrowth with lactobaccilli may be encouraged.8 * If symptoms are disabling, refer to a urologist.

SUSPECTED LOWER URINARY TRACT INFECTION IN MEN * Examine: the abdomen (for a palpable bladder); the testes and epididymis to assess the extent of infection; the prostate, and the urethral meatus for discharge. * Exclude diabetes.

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* Arrange for an MSU. * Treat with antibiotics for 5 days. Note: Most episodes of dysuria in young men will be due to urethritis, and need referral to a GUM clinic.

Proven UTI * Repeat the MSU after treatment and refer to a urologist. If infection recurs, consider low-dose prophylaxis until seen.

Acute bacterial prostatitis * Take urine for an MSU. * Start antibiotics, e.g. trimethoprim, a quinolone, erythromycin or azithromycin, all of which penetrate prostatic fluid well. Continue for 2 weeks in those that resolve promptly but 6 weeks in those that respond more slowly. * Give analgesics or an NSAID for symptomatic relief. * Admit patients who are severely ill or in whom rectal examination suggests a prostatic abscess.

Chronic prostatitis Only 5-10% of patients with this symptom complex have bacterial infection. * Establish the diagnosis: (a) order microscopy and culture on the first part of the stream of the first urine passed in the morning. Threads of white cells suggest prostatitis; (b) massage the prostate and smear the urethral secretions onto a slide. Air dry it. More than ten leucocytes per high power field suggests prostatitis. * If culture positive: treat with minocycline 500 mg b.d. or doxycycline 200 mg daily or ciprofloxacin 500 mg b.d. for 4-12 weeks or until symptoms have completely settled, whichever is the longer. * If culture negative: no treatment has been clearly shown to aid resolution.9 Offer a trial of NSAIDs for symptomatic relief or referral to a urologist. The value of referral is to confirm the diagnosis and assist in explaining it to the patient rather than to provide specific treatment.

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URINARY PROBLEMS

LOWER URINARY TRACT INFECTION IN OTHER SITUATIONS

Children * Refer all girls under the age of 13 and all boys (see p. 80).

Pregnancy * Give a cephalosporin or nitrofurantoin for 7 days. * Repeat an MSU 1 to 2 weeks after completion of treatment then monthly until delivery. * Pyelonephritis - admit.

Women after the menopause Review: Cardozo L. Post-menopausal cystitis. BMJ1996;313: 129.

Recurrent UTIs are common after the menopause, affecting more than 10% of women. This is in addition to symptoms due to urogenital atrophy. * Oestrogen therapy. Prescribe low-dose estrogen therapy in those with no contraindications. It has been shown to change the colonization of the vagina and decrease infections. Topical treatment may be sufficient. * Culture-negative 'cystitis'. Organize a further culture, if symptoms persist, asking specifically for Ureaplasma urealyticum and Mycoplasma hominis. Treat with tetracycline or erythomycin for 3 months. * Refer for urodynamic investigation women not responding to the above measures or unsuitable for oestrogen. * Interstitial cystitis. Treatment is palliative and should be decided by the urologist. The options are: (a) oral therapy with, e.g. a tricyclic antidepressant; or (b) intravesicular with, e.g. dimethyl sulfoxide. Patient support: The Interstitial Cystitis Support Group (UK), 76 High Street, Stony Stratford, Bucks MK11 1AH, tel. 01908 569169; www.interstitialcystitis.co.uk

Unusual organisms * Women who are infected with unusual organisms (e.g. Proteus or Pseudomonas, but not Escherichia coli or Staphylococcus albus) need a repeat MSU and further investigation if still present.

Indwelling catheter Seep. 281.

Neurogenic bladder * Patients with a neurogenic bladder need a follow-up MSU to ensure that it is sterile.

SUSPECTED ACUTE PYELONEPHRITIS * Admit, if too ill to take oral fluids. Otherwise: * Arrange an MSU. * Prescribe a 7-day course of antibiotics, choosing one to give the widest antibacterial cover, e.g. co-amoxiclav or a quinolone. Give it for 2 weeks if there is underlying structural abnormality. * Consider the need for analgesics. * Review the MSU result and confirm that the antibiotic was appropriate. * Repeat the MSU at least 1 week after the antibiotics are finished. * Follow-up: Order either a plain film of the abdomen and an ultrasound scan, or an intravenous urogram (IVU) (according to local policy) in all patients except for women where the attack was clearly precipitated by the start of sexual intercourse or its resumption after a break.

URINARY INCONTINENCE Guidelines: Royal College of Physicians. Incontinence causes, management and provision of services. A report of the Royal College of Physicians. London: RCP, May 1995. MeReC. Urinary incontinence in adults. MeReC Bulletin 1997; 8: part 1: 33-6; part 2: 41-4. Online. Available: www.npc.co.uk/merecjndex.htm

URINARY INCONTINENCE 279

* Up to 20% of women and 10% of men living at home aged >65 suffer from incontinence, as do up to 7% of women aged <45.10 * Only a quarter of women with incontinence consult a doctor about it spontaneously.11 * It is possible to help 70% of patients with incontinence. A practice must decide either to offer a full assessment and management programme itself, or to refer patients to an incontinence adviser.12 The use of a nurse with only 3 weeks training can lead to improvement or cure in 68% of patients against 5% in controls.13

(e) symptoms suggesting bladder outflow obstruction; (f) symptoms suggesting a large residual urine. * Refer to a neurologist if there appears to be a neurological cause.

* Be ready to ask anyone about incontinence if they have a condition which puts them at risk. A question like 'do you ever have trouble holding your water?' is less threatening than the word 'incontinence'. * Check, from the history for other urinary symptoms. Incontinence is usually part of a larger problem. * Identify the type of incontinence from the history: stress, urge, overflow or continuous, or a mixture. Most sufferers have at least 2 types. * Examine the abdomen, the genitals and prostate in men, with a pelvic examination in women and a rectal examination in both sexes for constipation and for the integrity of the anal reflex. * Test the urine for sugar and send an MSU to test for infection. * Frequency/volume diary. Ask the patient to complete a diary in order to get a clearer picture of the problem. * Consider an USS to assess residual volume, especially if there is a possibility that overflow incontinence is present or if the patient does not respond promptly to the measures below. A residual volume of 200 ml or more is abnormal. * Consider referral for urodynamic studies if the type of incontinence is not clear or a trial of treatment fails. * Refer to a urologist or gynaecologist at this stage if there is: (a) severe vaginal or uterine prolapse; (b) a pelvic mass; (c) a vesical fistula; (d) persistent or recurrent infection;

* Reduce intm-abdominal pressure by weight loss, avoidance of constipation and reducing cough by stopping smoking. * Increase external sphincter tone (87% cured or improved):14 (a) by pelvic floor exercises. Teach the patient to practice stopping the flow of urine momentarily midstream and then continue tensing those same muscles for 4 seconds at a time with 4 seconds rest, for a total of 1 hour a day. Short, repeated bouts of exercises are the most useful. Continue for 3 months before deciding that it has not helped. (b) use of intravaginal weighted cones (available through urologists or from Colgate Medical Ltd, Shirley Avenue, Windsor, Berks SL4 5LH). The patient inserts the lowest weight cone, with the pointed end downwards, and learns to retain the cone. The weight of the cones is steadily increased to 100 g. (c) by referral to physiotherapists for Faradism or interferential treatment. * Postmenopausal women with an atrophic vagina. Consider a trial of local or oral oestrogen replacement and assess the symptoms after 3 months. * Referral. Refer those with troublesome symptoms that do not respond to the measures above. Surgery cures or improves 85% of those operated on.

Management of patients in general practice Where the problem is primarily stress incontinence

Where the problem is primarily urge incontinence * Reduce excessive fluid intake and try avoiding caffeine.

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* Bladder training (87% cured or improved).14 Instruct the patient to: (a) keep a frequency/volume chart for a week; (b) return for discussion of the pattern the diary reveals. In addition, check that the total volume of urine passed does not suggest that the patient is drinking too much; (c) practise holding the urine when the urge to pass it is there; and (d) slowly increase the interval between voiding up to 2-3 hours. * Anticholinergic drugs are helpful in up to 83%,15 but are limited by side-effects: (a) oxybutynin or tolterodine; the latter is more expensive but may have fewer adverse effects;16 (b) flavoxate has fewer side-effects than oxybutynin, but is less effective; (c) imipramine 25-75 mg nocte may help nocturia. * Desmopressin nasal spray in a single evening dose may help night-time symptoms. * HRT may help a woman who has evidence of vaginal atrophy. * Referral. Refer for consideration of augmentation cystoplasty those still sufficiently troubled by symptoms despite treatment. Where the problem is primarily overflow incontinence * Refer for assessment by a urologist, neurologist or other specialist, according to the cause of the overflow. Incontinence in the frail elderly or the cognitively impaired * Assess: (a) whether it is due to infection. Older people often do not experience the typical symptoms of urinary infection; (b) whether it is a symptom of another physical illness; (c) whether drugs may be making the situation worse, e.g. diuretics, sedatives; (d) the practicalities of passing urine - how easy is it to get to the toilet? Can mobility be improved? Can clothing be made easier to unfasten?

* Encourage the patient to pass urine regularly, e.g. 2-hourly, to try to pre-empt the incontinence.

Coping with incontinence * Where the situation does not respond to any of the above measures, the continence adviser will be able to advise the patient on appliances, some of which are prescribable. Others may be available through the social services (including bath and laundry allowance) (see also p. 5). Patient information: The Continence Foundation, 307 Hatton Square, 16 Baldwin Gardens, London EC1N 7RJ, tel. 020 7404 6875; helpline 020 7831 9831; www.continence-foundation.org.uk The Incontinence Advisory Service, The Disabled Living Foundation, 380-4 Harrow Road London W9 2HU, tel. 020 7289 6111; helpline 0845 130 9177; www.dlf.org.uk ERIC (Enuresis Resource and Information Centre for children and young adults). 34 Old School House, Britannia Road, Kingswood, Bristol BS15 8DB, tel. 0117 960 3060; www.eric.org.uk

Odour control * Advise patients or carers to: (a) soak immediately and then wash clothing and linen that has become soiled; (b) wash soiled carpets, upholstery and slippers; (c) seal absorbent padding in disposable bags. Bedding protection * Plastic mattress covers and one-way sheets can be bought if not available from the Community Nursing Service. They not only increase comfort but also reduce bed sores. Pads and appliances * Inco pads may be available from the Community Nursing Service. * Cellulose wadding can be prescribed. * Body-worn pads and waterproof pants usually have to be bought, e.g. Urocare, Kanga and Kanga pouch.

DIFFICULTY VOIDING

* Collecting devices such as commodes, pans and urinals can be bought from pharmacists or hired from the Red Cross. * Penile sheaths are available on prescription. Conveen provide a free sizing kit (catalogue no. 6902) and sheaths from 17mm to 35mm. Downs Medical offer a free advice service and small, medium and large sheaths. Urinary drainage bags * Assess the patient's requirements: (a) the length of tubing; short for wearing on the thigh or long on the calf; (b) the type of tap; selection should depend on the patient's dexterity; (c) the capacity needed (350, 500, 750 ml). Night bags hold 2 litres and should only be used for bed-bound patients or over night.

Urinary catheters Review: Nicolle LE. Prevention and treatment of urinary catheter-related infections in older patients. Drugs and Ageing 1994; 4: 379-91.

* Infection makes catheterization an unattractive solution but one that is likely to be necessary in those: (a) with retention or neurogenic bladder dysfunction; (b) with severe pressure ulcers; (c) who are terminally ill; or (d) who cannot cope with less invasive appliances. * Catheters for long-term use should always be silicone with a 5 ml balloon. If a catheter is unlikely to be needed for more than 3 weeks, use a cheaper latex catheter. * Catheter insertion should be under sterile conditions. * Catheter changes should only be done when there is malfunction or contamination. * Debris may be reduced by acidification of the urine with ascorbic acid. Infection * Ninety per cent of patients with an indwelling catheter have bacteriuria after 17 days.16

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* Infection cannot be prevented by topical antimicrobials applied to the meatus, nor by irrigation with antimicrobials or antiseptics. * Give antibiotics only if the infection is systemic (i.e. the patient is febrile) or due to Proteus. Proteus may give rise to triple-phosphate stones and is worth eradicating with antibiotics, even though this is likely to substitute a Pseudomonas in its place. * If giving antibiotics, remove the catheter if the patient can manage without it for a few days. Insert a new one once the urine is sterile.16 Bypass

This is caused by obstruction of the catheter or by detrusor instability, not by the catheter being too small. * Check that the bag is lower than the bladder. * Change the catheter and see if the old one was blocked. * Use a small bulb size, e.g. 5 ml. * Exclude and, if necessary, treat bladder stones and infection. * If there is no improvement use anticholinergic drugs, e.g. oxybutynin or flavoxate.17 Catheterizing a patient in chronic retention * Haematuria is inevitable if the bladder has been distended for some time. Clamping to release the urine in stages does not prevent this. * Diuresis may occur after the obstruction is relieved. Diuretic doses may need to be reduced. Admit patients whose diuresis is severe.

DIFFICULTY VOIDING BLADDER OUTFLOW PROBLEMS IN MEN Guideline: Royal College of Surgeons and British Association of Urological Surgeons. Guidelines on management of men with lower urinary tract symptoms suggesting bladder outflow obstruction. London: Royal College of Surgeons, 1997. Systematic review: NHS Centre for Reviews and Dissemination. Benign prostatic hyperplasia. Effective Health Care 1995; 2 (2).

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* Identify whether the patient has: (a) voiding symptoms (poor flow, hesitancy, terminal dribbling and incomplete emptying); and/or (b) filling symptoms (frequency, urgency, urge incontinence and nocturia). These terms have replaced the older ones of bladder outflow obstruction and detrusor instability because of the poor correlation between the symptoms and objective findings of obstruction and instability.18 * For patients with the above symptoms: (a) examine the prostate; (b) examine for a palpable bladder; (c) check the blood pressure. It tends to rise in obstructive uropathy; (d) check the urine for infection, microscopic haematuria and sugar (a dipstick is adequate); (e) check serum creatinine and prostatespecific antigen (PSA). The use of the PSA to assess the symptomatic patient is appropriate even when population screening with PSA is not recommended; but some sophistication is needed to interpret it (see box below) (f) arrange an ultrasound examination to assess the residual urine and exclude bladder calculi.

PSA interpretation: 1. The upper limit of normal rises with age: Age <50: 2.5 ng/ml Age 50-59: 3.5 Age 60-69: 4.5 Age 70-79: 6.5 2. Repeat a borderline level; the result can alter by up to 30%. 3. Double the result if the patient is taking finasteride. 4. Rectal examination does not raise the PSA but more invasive manoeuvres (even catheterization) can, as can DTI, prostatitis and benign hypertrophy.

* Strong grounds for referral exist for men with: (a) evidence of a malignant prostate on digital examination or a raised PSA; (b) obstructive uropathy or overflow incontinence; (c) microscopic or frank haematuria; (d) urinary infection or stones;

(e) very troublesome symptoms or a large residual urine, i.e. over 300ml; (f) impaired renal function. * Assist other men to make a decision about referral for surgery. This should be based on the extent to which symptoms are interfering with their life at the moment. Symptoms do not necessarily worsen, and may improve.19 The size of the prostate is irrelevant. A symptom score can be useful (see Appendix 16) especially for monitoring changes over time, but there is a poor correlation between symptom scores and the degree of obstruction. The single most important question is the general one: Tf you had to spend the rest of your life with these symptoms, how would you feel about it?' Fully informed men tend to be less interested than their physicians in surgery. * Outline the treatment options: (a) Watchful waiting. Advise the patient to reduce fluid intake to 2L/day and avoid caffeine. Recommend bladder training to those with filling symptoms. Briefly, this means gradually lengthening the time between passing urine. Review the patient yearly. In one study only 7% required surgery over the next 3 years.20 (b) Drug treatment: - Alpha-blockers are the drugs of first choice.21 They produce a modest improvement in symptoms within 2 weeks. A trial is therefore feasible, using a symptom score. All alpha-blockers share this effect; a considerable saving can be made by choosing the least expensive. If there is benefit, it is felt within 6 weeks and lasts for at least 3 years. -A 5-alpha-reductase inhibitor, e.g. finasteride, produces a benefit that is even more modest. In one trial there was virtually no benefit.22 The benefit may be greater in men with large prostates (>40ml). No benefit may be seen for up to 6 months. If there is benefit it is sustained while the drug is continued. Sexual dysfunction occurs in 9% more patients than in controls. (c) Surgery (usually TURP or TUIP) is much more effective than drugs in improving symptoms, but only in two-thirds of those who undergo it. It does, however, carry not only the

URINARY STONES

short-term risks of any operation but also, in the case of TURP,23 the risks of: incontinence in one-third, although only 6% were bothered by it; erectile problems in one-third; retrograde ejaculation in two-thirds; in 9%, the need for reoperation within 5 years.

283

(b) screening can miss a quarter of prostate cancers; (c) if screening is positive, a biopsy will be needed. Only about 1 in 3 who have a positive screen turn out to have cancer; (d) It is not clear that early treatment saves lives; and the treatments that may be offered have potentially severe adverse effects; (e) the stress of knowing that you have cancer is considerable and will affect insurance applications.

RETENTION OF URINE IN WOMEN Retention may be due to pelvic masses, introital pain or psychogenic causes. * Examine the pelvis and consider ordering an ultrasound scan. * Check serum creatinine. * Refer to a urosurgeon or gynaecologist, according to findings. Acute retention will need immediate admission.

Patient information: The PSA test for prostate cancer. Online. Available: www.nelc.org.uk (choose 'prostate cancer' then 'PSA test' then 'patient information leaflets').

The finding of a raised age-specific PSA in a man with a 10-year life expectancy, or of a high PSA (>20ng/ml) in a man with a clinically malignant prostate or bone pain, are grounds for urgent referral under the NHS 2-week rule.

CARCINOMA OF THE PROSTATE Carcinoma of the prostate is the third leading cause of cancer deaths in men in the UK.24 It raises difficult issues relating both to screening and to the question of the value of treatment in the asymptomatic man in whom carcinoma is discovered incidentally. There is no doubt that the symptomatic patient will benefit from referral. The following statements may assist a decision in these difficult areas: • There is no good evidence that early detection and treatment can increase the chance of cure, although trials are underway. • The PSA test is not specific and not very sensitive for carcinoma of the prostate.25 • Population screening for cancer of the prostate is not justified,26 but should be available for a man who requests it. • Early treatment in the form of androgen deprivation prolongs survival in advanced local and in metastatic disease.27

Counselling for the patient who requests PSA screening28,29 Explain that: (a) a rectal examination is needed as well as the blood test;

URINARY STONES Review: Dawson C, Whitfield H. Urinary stone disease. 6M71996;312: 1219-21.

* Analgesia. Use an NSAID parenterally, e.g. diclofenac 75 mg im. Opioids may be necessary if that fails. * Check for microscopic haematuria with a dipstick. If negative, reconsider the diagnosis.30 Check MSU and serum creatinine. * Urgent IVU or USS according to local policy:

(a) to establish the diagnosis; (b) to assess the size, position and number of stones; (c) to look for obstruction. * Admit for: (a) uncontrolled pain; (b) unable to drink adequate liquids; (c) infection; (d) complete uni- or bilateral obstruction on IVU; (e) known renal insufficiency; (f) known to have a single kidney.

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URINARY PROBLEMS

* Refer to outpatients all those not requiring admission. Arrange: (a) a plain abdominal X-ray (kidneys, ureter and bladder; KUB) and IVU or USS. The latter may miss small stones; (b) give oral, sublingual or rectal NSAIDs or analgesics. * Instruct the patient to save any stone passed by passing urine through a filter, e.g. a woman's stocking. Send it for analysis. Once the patient is discharged from follow-up: * If conservative management has been chosen, support the patient with the information that 90% of small stones (<5mm) pass spontaneously and that 50% of stones that are 5-10 mm in diameter also pass.31 * Check that an attempt has been made to find a cause, i.e. serum calcium and uric acid and a 24-hour urinary calcium. If there is a family history or the patient has recurrent stones, the following should also be checked: (a) 24-hour urine for pH, oxalate, phosphate and uric acid; (b) random urine for cystine. * If an abnormality is found: (a) Treat hypercalciuria with a low calcium diet, potassium citrate and thiazides; (b) Treat hyperuricaemia with allopurinol; (c) Explain the importance of a longterm high fluid intake.

* Check that the sample was not taken after exercise, during menstruation or in the presence of vaginal or urethral discharge. * Protein + or more is likely to be significant, but false positives and negatives occur. Confirm with a second sample taken on waking, to exclude orthostatic proteinuria, at least 1 week later. If only the first sample shows proteinuria, repeat in 6 months. * Refer promptly (without waiting for the results of other investigations) if the dipstick shows protein + + + or + + + + . The protein loss is likely to be heavy enough to lead to nephrotic syndrome. * Refer less urgently all other patients: (a) with a protein excretion of more than 500mg/L, or a protein/creatinine ratio of >30mg/mmol even if they have a primary condition which is likely to be the cause (e.g. diabetes); (b) with a protein excretion of>250mg/L or a protein/creatinine ratio of >20mg/mmol if they also have a raised creatinine or hypertension; (c) with haematuria and any degree of proteinuria. * If proteinuria is present but does not meet the criteria for referral, repeat the tests 6 monthly.

ASYMPTOMATIC HAEMATURIA Guideline: SIGN. Investigation of asymptomatic microscopic haematuria in adults. Scottish Intercollegiate Guidelines Network, 1997. Online. Available: www.sign.ac.uk

RENAL DISEASE ASYMPTOMATIC PROTEINURIA Guideline: SIGN. Investigation of asymptomatic proteinuria in adults. Scottish Intercollegiate Guidelines Network, 1997. Online. Available: www.sign.ac.uk

Work-up if the dipstick shows proteinuria (a) Blood pressure; (b) MSU and a dipstick for haematuria; (c) 24-hour urinary protein or protein/creatinine ratio; (d) Serum creatinine and electrolytes; (e) Fasting blood sugar.

Macroscopic haematuria This is rarely due to glomerular disease. Refer to a urologist.

Microscopic haematuria • A finding of one + or more is significant, as is the repeated finding of a trace. About half of these patients will have glomerular disease.32

REFERENCES 285

* Check that the test was not carried out shortly after strenuous exercise, during menstruation or during a UTI. * Check the MSU. More than five red cells per high power field is abnormal. If normal, repeat the test for blood with a dipstick. If still positive, ignore the negative MSU result. Red cells lyse easily in urine in the time it takes for an MSU to reach the laboratory. * Check serum creatinine, abdominal X-ray and ultrasound of the urinary tract. * Refer: (a) children and young adults, and patients with red cell casts or a raised creatinine, without evidence of urinary obstruction, to a nephrologist; (b) older adults (e.g. over 40 years old) to a urologist. Ten per cent of asymptomatic haematuria in adults is due to malignancy, mainly in those over 40. They need a cystoscopy.

RENAL INSUFFICIENCY

* Check that there is no remediable cause for the decline in renal function: (a) drugs, especially NSAIDs, diuretics or ACE inhibitors; (b) dehydration; (c) gastrointestinal bleeding. * Refer to a renal physician for diagnosis and, if appropriate, for the management of the uraemia. * Patients not regularly followed up by a renal physician should:

(a) be regularly reviewed to try to reduce the rate of progression of the renal disease and to reduce the risk of cardiovascular disease. Screen for hypertension, anaemia, dyslipidaemia, hyperparathyroidism and hyperphosphataemia; (b) be referred back to the renal physician if the creatinine reaches 250 mmol/L unless a decision has been made to offer no further treatment. Even if the patient does not wish to have dialysis or transplantation, the quality of life might be improved with dietary advice, treatment of renal bone disease, and erythropoietin.

Review: Walker R. General management of end-stage renal disease. BMJ 1997; 315: 1429-32.

REFERENCES 1. Hamilton-Miller JM. The urethral syndrome and its management. / Antimicrobial Chemother 1994; 33 (Suppl. A): 63-73. 2. Brumfitt W, Hamilton-Miller JMT. Consensus viewpoint on management of urinary infections. / Antimicrobial Chemother 1994; 33 (Suppl. A): 147-53. 3. Barry HC, Hickner J, Ebell MH et al. A randomized controlled trial of telephone management of suspected urinary tract infections in women. J Fam Pract 2001; 50: 589-94. 4. Urinary tract infection. MeReC Bulletin 1995; 6 (8): 29-32. 5. Committee for Safety of Medicines. Revised indications for co-amoxiclav. Curr Prob Pharmacovigil 1997; 23: 8. Available on www.mca.gov.uk/ 6. Baerheim A. Empirical treatment of uncomplicated cystitis. BMJ 2001; 323: 1197-8. 7. Kontiokari T, Sundqvist K, Nuutinen M et al. Randomised trial of cranberry-lingonberry juice and lactobacillus GC drink for the prevention of urinary tract infections in women. BMJ 2001; 322: 1571-3. 8. Anon. The mysterious 'urethral syndrome' (letter). BMJ 1991; 303: 361-2. 9. McNaughton Collins M, MacDonald R, Wilt T. Interventions for chronic abacterial prostatitis (Cochrane

10. 11. 12. 13. 14. 15.

16. 17. 18.

Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. MeReC. Urinary incontinence in adults. MeReC Bulletin 1997; 8: part 1: 33-6; part 2: 41-4. Burgio KL et al. Prevalence, incidence and correlates of urinary incontinence in healthy middle-aged women. / Lira/ 1991; 146: 1255-9. Duckett JRA. Women with urinary incontinence should be referred to a specialist. BMJ 1996; 313: 754. O'Brian J, Long H. Urinary incontinence: long-term effectiveness of nursing intervention in primary care. BMJ 1995; 311: 1208. US Department of Health and Human Services. Urinary incontinence in adults. Rockville MD: Agency for Health Care and Policy Research (AHCPR), 1992. Malone-Lee JG, Walsh IB, Maugourd M-F et al. Tolterodine: a safe and effective treatment for older patients with overactive bladder. J Am Geriatr Soc 2001; 49: 700-5. DTB. Managing urinary tract infection in women. Drug Ther Bull 1998; 36: 30-2. Foster MC et al. Urological myths. BMJ 1990; 301: 1421-3. Abrams P. Managing lower urinary tract symptoms in older men. BMJ 1995; 310: 1113-17.

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19. Ball AJ et al. The natural history of untreated 'prostatism'. Br J Urol 1981; 53: 613-16. 20. Wasson JH, Reda DJ, Bruskevitz RC et al. A comparison of transurethral surgery with watchful waiting for moderate symptoms of benign prostatic hyperplasia. New Engl J Med 1995; 332: 75-9. 21. Farmer A, Noble J. Drug treatment for benign prostatic hyperplasia. BMJ 1997; 314: 1215-16. 22. Lepor H et al. Veterans Affairs Cooperative Studies Benign Prostatic Hyperplasia Study Group. The efficacy of terazocin, finasteride, or both in benign prostatic hyperplasia. New Engl J Med 1996; 335: 533-9. 23. Emberton M et al. The effect of prostatectomy on symptom severity and quality of life. Br J Urol 1996; 77: 233-47. 24. Woolf SH. Should we screen for prostate cancer? BMJ 1997; 314: 989-90. 25. NHS Centre for Reviews and Dissemination. Screening for prostate cancer. Effectiveness Matters 1997: 2(2). 26. Schroder FH. Detection of prostate cancer. BMJ 1995; 310: 140-1.

27. MRC Prostate Cancer Working Party Investigators Group. Immediate versus deferred treatment for advanced prostatic cancer: initial results of the Medical Research Council trial. Br J Urol 1997; 79: 235-46. 28. NHS Centre for Reviews and Dissemination. Screening for prostate cancer: the evidence. Information for men considering or asking for PSA tests. Effectiveness Matters 1997: 2 (2). Available on www.york.ac.uk/inst/crd/ welcome.htm 29. Chan ECY, Sulmasy DP. What should men know about prostate-specific antigen screening before giving informed consent? Am J Med 1998; 105: 266-74. 30. Dawson C, Whitfield H. Urological emergencies in general practice. BMJ 1996; 312: 838-40. 31. Prodigy. Guidance on the national electronic library for health guidelines database. Online. Available: www.prodigy.nhs.uk (choose 'urology' then 'renal colic-acute'). 32. Topham PS et al. Glomerular disease as a cause of isolated microscopic haematuria. Quarterly J Med 1994; 87: 329-35.

15

CHAPTER CONTENTS Head injury 287 Head injury in young children 288 Treatment after any head injury 288 Rehabilitation after a head injury 289

Surgical problems

Orthopaedic trauma in primary care 289 Whiplash injury 289 Acute knee injury 289 Acute ankle injury 290 Breast problems 290 Peripheral vascular disease 292 Buerger's disease 294 Raynaud's phenomenon 294 Abdominal aortic aneurysm 294 Gallstones 295 Cholecystitis 295

HEAD INJURY

Superficial wounds 296 Burns 296 Dog bites 296 Lacerations 297 Common postoperative complications Returning to work after operations References

299

299

297

Indications for referral for skull X-ray or CT scan1,2,3 (a) history of unconsciousness; or post-traumatic amnesia (PTA) of more than 10 minutes; (b) history of severe trauma including, in a child under 5, a fall of over 60 cm on to the head; (c) suspected foreign body or penetrating injury; (d) convulsion; (e) severe persistent headache; (f) persistent vomiting; (g) clinical signs of skull fracture e.g. crepitus, skull depression, 'bogginess', serious scalp laceration or haematoma, blood or cerebrospinal fluid (CSF) in the nose or ear, or CSF in the wound; (h) altered conscious state at the time of the examination (Glasgow Coma Scale (GCS; Table 15.1) <15); (i) focal neurological signs at the time of examination; (j) complicating medical factors (e.g. anticoagulant use, alcohol misuse); (k) difficulty in assessing the patient, due e.g. to extremes of age or intoxication. Note: If a patient with a minor head injury and a normal skull X-ray is not improving, do not hesitate to refer back for a CT scan.4 One in seven will have an abnormal scan.

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288 SURGICAL PROBLEMS

Table 15.1

Glasgow Coma Scale5

Parameter

Response

Numerical value

Eye opening

Spontaneous To speech To pain None Orientated Confused Inappropriate Incomprehensible sounds None Obeys commands Localize to pain Flexion to pain - withdrawal Flexion - abnormal Extension to pain None Total

4 3 2 1

Best verbal response

Best motor response to painful stimulus

5 4 3 2 1 6 5 4 3 2 1 3-15

Non-referral Patients with normal imaging who may be sent home6 are those with no impairment of consciousness; and (a) PTA <5 minutes; and (b) no severe headache, vomiting, irritability, abnormal behaviour; and (c) no seizure at any stage; and (d) no neurological signs; and (e) no complicating medical problems; and (f) adequate supervision at home. If the patient is not admitted, warn the patient's attendant, verbally and in writing, to report: (a) (b) (c) (d) (e) (f)

unusual drowsiness; severe headache; persistent vomiting beyond 4 hours; visual disturbance; strange behaviour; anything else worrying.

* Record the warning in the patient's notes. * Give paracetamol or codeine for headache, but nothing stronger.

HEAD INJURY IN YOUNG CHILDREN * Small children can be difficult to assess. Refer if in doubt.

* The inability to rouse a child is a worrying sign. Refer. Sleepiness alone is not worrying. * Persistent crying may indicate that the child has a headache; small children will not usually complain of headache. * If the child does not cry immediately after head trauma, suspect moderate to severe head injury and refer.

Skull fractures in children7 • Radiological evidence of a skull fracture is found in almost one-third of children with a history of head injury. These children are normally neurologically intact at the time of examination and remain so. • Nearly 50% of children who die of an acute brain injury have no evidence of a skull fracture. • Subdural haematoma is twice as likely in the absence of a skull fracture after head injury in children compared with adult head injury without skull fracture. • Epidural haematoma occurs in 50% children with skull fracture. • Location and type of skull fracture are rarely correlated with symptoms and physical findings in children who have suffered head trauma. • Depressed skull fractures require surgical decompression if the depression is more than 3-5 mm.

TREATMENT AFTER ANY HEAD INJURY * Explain that headache, dizziness, tinnitus, poor concentration and balance and fatigue are common. They will subside over about 6 months after head injury.8 * Depression or anxiety. Treat with antidepressants (NNT 5,95% CI = 4 to 7).9 Note: Prophylactic antiepileptic drugs reduce early seizures after moderate to severe head injury when given within hours and continued for several weeks (RRR 66%, 95% CI = 46 to 79%; NNT 16, 95% CI = 11 to 35) but do not reduce late seizures, neurological disability, or death (RRR 15%, 95% CI= -11 to 51).10

ORTHOPAEDIC TRAUMA IN PRIMARY CARE

REHABILITATION AFTER A HEAD INJURY • Residual problems, which are common after severe head injury, can be reduced by rehabilitation. • Neuropsychiatric problems are common after minor head injury. • Attempt to keep the patient in the rehabilitation programme and consider re-referring patients still troubled by cognitive, behavioural and emotional disturbances. Patient organization: HEADWAY: National Head Injuries Association, 7 King Edward Court, King Edward Street, Nottingham NG1 1EW, tel. 0115 9121000; www.headway.org.uk Website for patients and professionals: www.neuroland.com

289

* Consider referring to a physiotherapist for training in active mobilization of the neck. This is more effective than rest in a soft collar. Furthermore, early referral (before 2 weeks) is better than later referral.15 * Resist overtreatment; instead, try to reward patients for getting better. Discourage symptom diaries.12

Chronic whiplash injury * Refer to a specialist any patient who has chronic pain after whiplash injury and who has not improved after 3-6 months. Consider referring to a specialist skilled in diagnostic cervical zygapophyseal joint blocks and radiofrequency neurotomy.16 Of these patients, 46% (95% CI22 to 79%) will improve with treatment.13 ACUTE KNEE INJURY

ORTHOPAEDIC TRAUMA IN PRIMARY CARE WHIPLASH INJURY Acute whiplash injury • Whiplash injury is self-limiting in the absence of bone or nerve injury.11 • Soft collars are of no value.11 • Impending compensation delays recovery.11 • Careful examination is important as imaging studies are rarely helpful. The act of examination helps to relieve anxiety and encourages selfmanagement.12 * Check for weakness with or without pain in one or both arms. It would suggest nerve root involvement. This is an unusual complication of whiplash but requires urgent specialist evaluation. * Reassure that 90% recover within 6 months, although up to 40% of these patients have some neck pain after 15 years!13 * Give paracetamol with or without codeine and/or an NSAID and recommend that they be taken regularly. * Encourage normal activities rather than rest. It speeds resolution.14

• The 'Ottowa Knee Rules' reduce the need for an X-ray after acute knee injury by 26%.17 Patients younger than 18 or those with major trauma are not suitable candidates for the use of the decision rule. • The economic impact of adopting this approach could be a cost saving to the NHS of nearly £2 million.18 • The rules state that a knee X-ray series is only required for knee injury patients with any of these findings: (a) age 55 or older; (b) isolated tenderness of the patella (that is, no bone tenderness of the knee other than the patella); (c) tenderness at the head of the fibula; (d) inability to flex to 90 degrees; (e) inability to bear weight both immediately and when examined (they should be able to take four steps, i.e. take their weight on each leg twice even if they limp). If there is no fracture:

* Mobilize as soon as pain permits. * Encourage quadriceps exercises (see p. 170). * Provide suitable analgesia.

290

SURGICAL PROBLEMS

Table 15.2 Reliability of the Ottowa ankle rules for identifying ankle and foot fractures or avulsion fractures Patient groups

Sensitivity (95% Cl)

Specificity (95% Cl)

+ LR

-LR

All patients

99%

39%

1.62

0.03

(91 to 100%)

(34 to 45%)

Ankle injuries

98%

45%

1.78

0.04

Midfoot injuries

(88 to 100%) 100% (85 to 100%)

29%

1.41

0.00

(39 to 50%) (27 to 32%)

ACUTE ANKLE INJURY • The Ottowa Ankle Rules (Table 15.2) reduce the need for X-rays following ankle injury by almost 20%.19 • An ankle X-ray is required if there is any pain in the malleolar zone and: (a) there is bone tenderness at the posterior edge or tip of the lateral malleolus; or (b) there is bone tenderness at the posterior edge of the medial malleolus; or (c) the patient is unable to weight-bear both at injury and when seen. • A foot X-ray is required if there is pain in the midfoot zone and: (a) there is bone tenderness at the navicular; or (b) there is bone tenderness at the base of the 5th metatarsal; or (c) the patient is unable to weight-bear both at injury and when seen. * The immediate treatment (as for any injured joint or limb) is RICE (rest, ice, compression, elevation). * Analgesia, figure-of-eight strapping, and partial weight bearing all have a role in subsequent care of the damaged ankle.

Rehabilitation after ankle injuries * Recommend active mobilization to restore proprioception. This can be achieved by regular exercises: (a) Imagine writing the alphabet with the foot, first capitals then small letters. (b) Balance on the injured leg while moving the free leg forward and backward and

side-to-side; initially with eyes open then with eyes shut. (c) Use a wobble board.

BREAST PROBLEMS Referral criteria for breast problems20 Refer urgently if there is: • a new discrete lump in a woman age >45; or • other signs of cancer: ulceration, a skin nodule or skin distortion. Lump

* Refer, but not necessarily urgently, if there is: (a) a new discrete lump in a younger woman; or (b) a new lump in pre-existing nodularity; or (c) asymmetrical nodularity in a woman with a family history of breast cancer or who is age 35 or over. In a younger woman only refer if the asymmetry persists after the next period; or (d) an abscess; or (e) recurrence of a previously drained cyst. • Young women with tender, lumpy breasts and older women with symmetrical nodularity, provided there is no localized abnormality, do not need referral to specialist. • For women with recurrent multiple cysts, aspiration is acceptable by GP with the necessary skills. Discharge

* Refer if there is discharge from the nipple without a lump and the patient is: (a) 50 years old or over; or (b) under 50 with discharge that is: -bilateral and heavy enough to stain clothing; or -bloodstained; or -persistent from a single duct. • Women aged under 50 who have nipple discharge that is from more than one duct or is intermittent and is neither bloodstained nor

ORTHOPAEDIC TRAUMA IN PRIMARY CARE

troublesome do not require referral to a breast specialist. Other problems Refer if there is: (a) nipple abnormality: eczema or a retracted or distorted nipple; or (b) a change in breast contour (best seen with arms raised while patient sitting upright); or (c) a dimple or change in skin texture of the breast; or (d) localized, non-cyclical breast pain; or (e) intractable cyclical breast pain which fails to respond to medical treatment; or (f) a strong family history of breast cancer (see P- 292). 21

Breast pain without a lump

• Cyclical mastalgia is usually bilateral; noncyclical breast pain may be due to true chest wall discomfort. • Nearly 70% women have breast pain at some time in their life, most commonly aged 30 to 50 years. • Cyclical pain resolves within 3 months in 20-30% of women. Non-cyclical pain resolves eventually in nearly 50%. • Women with minor and moderate degrees of breast pain who do not have a discrete palpable lesion can be managed in general practice. • There is no evidence of effectiveness for many potential remedies: evening primrose oil, vitamin E, LHRH agonists, progestogen cream, pyridoxine, antibiotics, or diuretics. • Recommend analgesics, a supportive bra and a low-fat, high-carbohydrate diet, which can reduce breast pain and tenderness.22 • If pain is more severe, use danazol for 3-6 months (200 mg daily reducing to l00mg daily once pain is controlled). Pain relief continues for at least 6 months after stopping the drug (NNT = 3, 95% CI = 2-37).23 Side-effects are common. • Consider bromocriptine, starting at 1 mg nocte and increasing to a maximum of 2.5 mg b.d. However, its side-effects tend to outweigh any

291

benefit.24 If the response is good, treat for 6 months. * HRT. Consider changing a conventional oestrogen/progestogen combination to tibolone if the former is causing mastalgia.25 * Oral contraception. Consider a change to an alternative method. * Refer patients not controlled by the above measures for specialist consideration for tamoxifen23 or gestrinone.25 Breast cancer screening and breast cancer26 General practitioners have a role in supporting the breast screening programme but should know its limitations. • A GP with 2000 patients will see nearly two new cases of breast cancer annually (incidence = 118 per 100,000) and she/he will be looking after five women with breast cancer (prevalence = 0.5%). • 10% of screening mammograms are abnormal and need further assessment. Only 5% of these turn out to show cancer. • At an initial screen up to 20% of the breast cancers found will be in situ, 20-25% will be invasive lesions under 1 cm in diameter, and 25% will be invasive lesions between 1-2 cm in diameter.27 • Mammography misses 10% of cancers. • Over 90% of breast cancers are found by women themselves. Information the GP needs to support the breast cancer screening programme

(a) Women under 50. There is insufficient evidence to demonstrate that screening women under 50 is effective in reducing mortality from breast cancer. The harms may be greater than any potential benefit.28 (b) Women aged 50-64. There is a large body of evidence for regular screening mammography.29'30 Five-year survival is almost 75% and 10-year mortality is reduced by 25-30%.31 (c) Women with a family history of breast cancer: see Table 15.3.31 Women with a family history that does not meet these criteria can be reassured, with

292

SURGICAL PROBLEMS

Table 15.3

Genetic risk for women with a family history of breast cancer33

Risk category

Criteria determining level of risk

Recommended management

High risk (more than four times the average age-related risk)

Four or more relatives affected at any age by breast or ovarian cancer Three relatives affected with breast or ovarian cancer, with an average age at diagnosis of the breast cancer of less than 60 years Two relatives affected by breast cancer, with an average age at diagnosis of less than 40 years One relative with both breast and ovarian cancer at any age

Referral for specialist genetic counselling at a regional genetic centre Genetic testing may be appropriate for some of these patients

Moderate risk (at least three times the average age-related risk)

One first-degree relative* with breast cancer diagnosed under the age of 40 years Two first- or second-degree** relatives with breast cancer diagnosed under age 60, or ovarian cancer at any age Three first- or second-degree relatives with breast or ovarian cancer diagnosed at any age One first-degree relative with bilateral breast cancer under age 60 One first-degree male relative with breast cancer at any age

Current sparsity of evidence to support intervention. Referral for management within clinical studies can be considered Genetic testing is not appropriate for these patients

*A first-degree female relative is a mother/sister/daughter; **a second-degree female relative is a granddaughter/ grandmother/aunt/niece

an explanation of the difference between familial and non-familial cancer. (d) Women with symptoms between routine mammography. Women should continue to be aware of minimal symptoms and to report these without delay to their GP even if they have been recently screened. A woman with symptoms should be referred for investigation to a surgeon with a special interest in breast disease working in a hospital that can provide a multidisciplinary approach to breast disease. (e) Women with breast cancer. Women who have undergone surgery for breast cancer should have mammography yearly for the first 5 years and then 2-yearly. (f) Women receiving hormone replacement therapy. Women aged 50-64 on HRT do not need more frequent screening. Women do not need a baseline mammogram before receiving HRT. HRT for 5 years is associated with two extra cases of breast cancer by age 70 per 1000 women; cases of breast cancer per 1000 women for 10 and 15 years of exposure are six and 12, respectively. This small increased risk for HRT users disappears 5 years after cessation of therapy.32

Information for patients: The Breast Care and Mastectomy Association, 15-19 Britten Street, London SW3 3TZ, tel. 020 7867 1103; www.obgyn.net CancerBACUP, 3 Bath Place, Rivington Street, London EC2A 3JR, www.cancerbacup.org.uk CancerHelp, www.cancerhelp.org.uk Macpherson & Waller (Eds). Women's health, 4th edition. Oxford: Oxford University Press, 1997. (Chapter 5 on breast problems is excellent for patients and health professionals alike.)

PERIPHERAL VASCULAR DISEASE (PVD) Intermittent claudication • The prevalence in the population is 1.7% to 2.2% with a male to female ratio of 11.34 • Once the finding of peripheral vascular disease (PVD) has been confirmed, the patient needs all appropriate measures to reduce the risk of coronary heart disease and stroke as well as reducing the risk of progression of the peripheral lesion (see p. 109).

PERIPHERAL VASCULAR DISEASE 293

Prognosis for PVD35 * Symptomatic patients have a 30% risk of death within 5 years and a 50% risk within 10 years (of which 60% is from myocardial infarction and 12% from stroke). The risks are more than doubled in patients with severe disease (requiring surgery). * Asymptomatic patients (whose ankle-brachial pressure index is <0.9) have a 2- to 5-fold increased risk of fatal or non-fatal cardiovascular events.

Evaluation when PVD is suspected * Check the history for CHD risk factors. Ask about erectile dysfunction. * Examine for femoral, popliteal and foot pulses; bruits, including iliac bruits; limb temperature; and venous filling time to determine whether arterial disease is present and, if so, where the obstruction is.36 * Use Buerger's test37 if the diagnosis is in doubt. Raise both legs to 60 degrees for 2 minutes. Check the time taken for each limb to become pallid. The veins in the elevated ischaemic limb may become depressed beneath the skin surface, and if the legs are then rapidly swung into a dependent position an ischaemic limb shows a delayed but pronounced hyperaemia response. * Check the ankle-brachial index (ABI).38 * Check blood tests: Hb, U&Es, random blood sugar, lipid profile. * Perform other investigations: urinalysis, ECG, and USS of the abdomen.

To measure ABI: * Measure systolic pressure in both arms. Use the higher. * Measure systolic pressure in the dorsalis pedis and the posterior tibial arteries of each leg with Table 15.4 The ankle-brachial index ABI

Interpretation

>1.3

Non-compressible, i.e. the artery is calcified at the ankle - test invalid Normal Mild to moderate peripheral arterial disease Severe peripheral arterial disease

0.91-1.3 0.41-0.90 0.00-0.40

the cuff placed just above the malleoli. Use the higher on each side. * Calculate the ABI for each leg as the ankle systolic over the brachial systolic pressure.

Management and secondary prevention39 Non-surgical treatment is sufficient in many patients. Only a quarter continue to deteriorate. * Stop smoking. Doing so reduces stroke risk, preventing one stroke for every 67 hypertensive men, smoking at least 20 cigarettes/day, who stopped for 5 years.40 It also slows the progression of the limb ischaemia.38 * Exercise despite pain. This can treble the distance that can be walked and improve rest pain. Walking should be for at least 30 minutes at least three times a week for at least 6 months before benefit is likely.41,34 * Antiplatelet therapy is indicated in all cases. Lowdose aspirin given to 100 patients with peripheral vascular disease will protect nine from acute arterial occlusion over 19 months.42 Antiplatelet drugs reduce all cardiovascular events in patients with claudication from 11.8% to 9.7% over 27 months (NNT = 48). * Treat hyperlipidaemia. Aim for a total cholesterol <5.0mmol/L and an LDL-cholesterol <3.0mmol/L (see p. 109).

Referral * Refer if walking distance is 50% less than normal or < 100 yards. * Refer if there is rest pain. * Patients under 50. Refer all young patients presenting with intermittent claudication. They are more likely to have a nonatheromatous cause for their claudication. * Refer urgently if the limb is at risk. * Refer immediately if there is acute limb ischaemia, manifested by a pale, pulseless, painful and cold limb.

Revascularization * Angioplasty is the treatment of choice if the lesion is amenable.

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* Mortality after aortoiliac (AI) reconstruction is 3% and after femoropopliteal (FP) reconstruction is 1%. * Grafts may remain patent on average for 8 years for AI surgery, but 4 years for FP surgery.

Drug treatment * Pentoxifylline has been found to increase walking distance in some studies but not in others. * Naftidrofuryl has been shown to increase painfree walking distance on a treadmill, although not maximal walking distance. In neither is the possible benefit sufficient to justify the expense when more effective treatment options are available.43

BUERGER'S DISEASE * Smoking cessation is imperative. * Refer to exclude embolic disease, especially if a cardiac murmur or arrhythmia is present; the differential diagnosis includes multiple emboli. * Refer if digital gangrene develops.

RAYNAUD'S PHENOMENON44 Evaluation when Raynaud's phenomenon is suspected: * History: ask about duration, precipitating factors, CHD risk factors. * Examination: peripheral skin integrity, pulses, heart rhythm. * Blood tests: Hb, ESR, autoantibodies (especially cold agglutinins), U&Es, LFTs, TFTs. * Other investigations: CXR (thoracic inlet; cervical rib). Hand X-rays may reveal evidence of bone ischaemia or anatomical variants that impinge on the digital circulation.

Treatment Mild symptoms: * Encourage the patient to keep the hands warm and not to smoke. * Give vitamin C l000mg and vitamin E 500 mg daily as a trial.

Moderate symptoms: * Calcium channel blockade (e.g. nifedipine 5-20 mg daily). * An ACE inhibitor (for bradykinin effect). Severe symptoms * Refer, especially if there is ulceration or gangrene. They may benefit from iv prostacyclin.45 Patient organization: The Raynaud's and Scleroderma Association, 112 Crewe Road, Alsager, Cheshire ST7 2JA, tel. 01270 872776; www.members.aol.com/Raynauds/medic.htm

ABDOMINAL AORTIC ANEURYSM (AAA) * All-cause mortality for patients with small aneurysms 6 years after diagnosis is 30% regardless of whether 'watchful waiting' or operation is chosen.46 * The 30-day mortality after repair of asymptomatic aneurysms is 1.4%. For ruptured aneurysms it is over 40%, ignoring those who die before they reach surgery. * Elective surgical repair is recommended to patients when:47 (a) the aneurysm diameter is >5.5cm; or (b) the growth rate is >1 cm/year; or (c) the aneurysm becomes tender; or (d) iliac or thoracic repair is needed. * Order an abdominal USS for people under 60 with a family history of a first-degree relative with AAA. The frequency of screening is not certain, but in high-risk individuals scanning is usually performed every 3-5 years or as agreed by the local vascular team and radiology department. * Refer all clinically detectable aneurysms unless the quality of life precludes surgery. * Order an USS if the aorta appears prominent but it is not clear clinically whether it is aneurysmal. * Admit any aneurysm that is tender. Rupture may be imminent. * Organize a CHD secondary prevention programme for all patients after detection of AAA (see p. 109).

GALLSTONES

GALLSTONES (CHOLELITHIASIS) * Do not refer patients with asymptomatic gallstones unless they fall into one of the categories below. They are often discovered during evaluation of other problems and are best left alone unless symptoms appear. Only 20% will develop pain over the next 20 years. On a population basis, people with stones are no more likely to have pain than those without. If, however, the gall bladder is removed, the prevalence of pain is greater than if it is left in situ. Even doctors think that up to 30% of cholecystectomies are inappropriate.48 * Refer patients with symptomatic gallstones. Half will develop severe symptoms or complications over the next 20 years. Cholecystectomy is indicated. * Also refer: (a) patients who are jaundiced; (b) children with pigment stones; (c) where the risk from complications is high, e.g. where a patient has diabetes (diabetics have a 20% chance of developing emphysematous cholecystitis), or is on long-term parenteral nutrition or is immunosuppressed; (d) Where there is a risk of cancer:49 -the gall bladder is non-functioning, or there is scarring, calcification or thickening; or - the gall bladder epithelium shows adenomyosis or papillomatosis; or -the history is chronic.

Outcomes of Cholecystectomy • 90% patients will be symptom free after cholecystectomy. • 5-10% patients will continue to be troubled by pain. Some of these people will have retained bile duct stones; others will have biliary dyskinesia (see below).

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cholestasis, or elevation of serum amylase and lipase levels. • The biliary tree, and less often the pancreatic duct, may be dilated on direct cholangiography and pancreatography. • Sphincter pressures may be elevated when measured during endoscopic ductal manometry. Small residual calculi are discovered in some patients. • Endoscopic sphincterotomy may be curative in patients with objective findings but is controversial in patients with pain alone. • Episodic pain caused by papillary disorders may have been responsible for the symptoms that prompted Cholecystectomy and may be the cause of continued pain after surgery.

Patients unsuitable for surgery50 • Oral bile acids will dissolve some stones, especially small radiolucent stones <5 mm in diameter. • Ursodeoxycholic acid 750 mg daily will reduce stone size by 80% after 6 months and by 90% after 12 months providing the gallstones are no more than 1cm in diameter and the gallbladder is functioning. * Consider referral for treatment with ursodeoxycholic acid. * Monitor progress by USS 3-monthly until the stones have dissolved. * If successful, consider maintenance at low dose. This appears to halve the risk of recurrence.

CHOLECYSTITIS Patients with acute cholecystitis are frequently referred to hospital but can be managed successfully at home if the diagnosis is clear and the condition relatively mild.

Post-cholecystectomy syndrome

Treatment of acute cholecystitis in primary care

• This is caused by biliary dyskinesia, which is a structural or functional disorder of the ampulla of Vater. Patients experience periodic colicky pain with variable associated findings of transient elevation of bilirubin or liver enzymes, suggesting

* Encourage fluids and a bland fat-free diet. * Give adequate analgesia. * Give oral antibiotics covering aerobic and anaerobic bacteria (co-amoxiclav; or a cephalosporin plus metronidazole).

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* Request FBC, LFTs and U&Es - many hospitals can provide results within 24 hours if notified. * Request an abdominal USS as an outpatient to confirm the diagnosis - refer to the surgeons for cholecystectomy if gallstones are confirmed. * Refer immediately if jaundiced (cholangitis is a medical emergency) or there is no improvement within 48-72 hours or if the patient is too ill to take oral fluids and antibiotics.

SUPERFICIAL WOUNDS

if the wound is likely to ooze. Change the dressing every 2-3 days. (b) Tullegras, e.g. Bactigras, covered with cotton gauze and a crepe bandage. Leave on for 1 week. (c) Calcium alginate dressings, e.g. Sorbsan or Kaltostat. (d) Occlusive dressings, e.g. Opsite, Tegaderm, Spyroflex, Granuflex or Tegasorb. These are less bulky than the above and permit the patient to wash without the wound getting wet. They should only be changed weekly. They should not be used if infection is present, or there has been a delay in presentation or the wound is dirty.

The expertise of GPs in relation to wounds varies hugely. The general principle should be: only deal with wounds that you are competent to manage; refer if in doubt.

Burn wounds are easily contaminated. Provide a booster if necessary (see p. 30).

BURNS51

Special areas

Who can be managed without referral?

* Hands can be treated with Flamazine (silver sulphadiazine) inside polythene gloves. * Leave burns on the face and perineum exposed.

Patients with: (a) a burn affecting <5% of the body area (the patient's hand is 1%); and (b) a partial-thickness burn only. Full-thickness burns need early surgery; and (c) the ability to care for themselves. Note: Partial-thickness burns are pink, blister, blanche on pressure, and sensation to pinprick is intact. Full thickness burns are white or charred, and pin-prick is not felt as being sharp.

First aid (a) Cool the burn with cold water from the tap. (b) Cover the burn with cling-film or a clean plastic bag. Leave it on until the patient is either seen in hospital or a dressing can be applied in general practice.

Dressings in general practice (a) Flamazine cream on gauze held in place with a crepe bandage. Put cotton wool under the bandage

Tetanus prophylaxis

Refer the above patients (a) If healing is incomplete after 2 weeks. (b) If there is invasive sepsis needing iv antibiotics. DOG BITES About 200,000 people are bitten by dogs each year in the UK. Many victims regard their injury as too trivial to seek medical help. Because a small number go on to develop overwhelming infection, the injury can only be regarded as trivial in retrospect. * Wound toilet. Clean all dog bites. Where there is dead or devitalized tissue, this should be done in hospital. * Wound closure. Only suture a dog bite if: (a) it is in an area with good blood supply, e.g. the head; and (b) the wound is recent (<6-8 hours); and (c) there is minimal tissue damage.

COMMON POSTOPERATIVE COMPLICATIONS

* Antibiotics. Give antibiotics to those with wounds that are: (a) closed primarily; or (b) more than 8 hours old; or (c) crush or puncture wounds; or (d) wounds to the hands or feet; or (e) in children under the age of 5, the elderly or the immunocompromised. Prescribe co-amoxiclav for 5 days or (in those who are allergic) doxycycline 200 mg daily. In children, pregnant women or breast-feeding mothers, use erythromycin. * Tetanus. Check the patient's tetanus status and give a booster if necessary. * Rabies. Consider the risk of rabies if the bite occurred abroad (see p. 35).

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is no improvement, or pus develops, commence oral antibiotics (flucloxacillin or erythromycin). * People with diabetes, vascular disease or suppressed immunity. Give prophylactic antibiotics for all but the smallest of wounds. * Facial lacerations rarely become infected because of .the excellent blood supply. However, always check for intraoral penetration - 'through and through' wounds require referral. Further reading: Sambandan S. Surgery in the surgery. Oxford: Radcliffe Medical Press, 1999. Brown JS. Minor surgery: a text and atlas, 4th edition. London: Arnold, 2000. Calne R, Pollard SG. Operative surgery. London: Gower Medical Publishing, 1992. Jamieson GG. The anatomy of general surgical operations. Edinburgh: Churchill Livingstone, 1992.

LACERATIONS * Control bleeding: 10 minutes of pressure usually suffices, unless a vein or artery is damaged. * Clean the wound with sterile normal saline solution. * Decide if you are competent to close the laceration - if not, refer to the local Emergency Department. * Check for damage to peripheral nerves (altered sensation beyond the wound or weakness of muscles supplied by nerves in the vicinity of the wound) and tendons. Check the integrity of the blood supply to structures beyond the wound. This advice is particularly important for lacerations of face and limbs. If a problem is detected, refer to the local Emergency Department or plastic surgery team. * Check tetanus status: provide a booster if necessary. * Removal of sutures: healing times vary with the site of the laceration and the age of the patient. When in doubt it is better to remove sutures earlier than later; the cosmetic result is better. Slight gaping of wound can be treated with Steristrips. Remove sutures as follows: (a) face: after 3-5 days; (b) upper limb, scalp, trunk: after 5-7 days; (c) lower limb, back: after 7-10 days. Always remove sutures if the suture line is moist and red - this signals local wound infection. If there

COMMON POSTOPERATIVE COMPLICATIONS • With reduced hospital stays for surgical patients and an upsurge in day-case surgery, family doctors are facing a rising incidence of postoperative problems. In general, early referral back to the patient's surgeon is encouraged for all but the most trivial of complications. • Major surgery triggers a catabolic response that is followed by anabolic activity. Patients can experience dramatic loss of weight and suffer fatigue during the weeks and months after major surgery. Nutritional deficiency may occur during recovery if the diet does not meet the needs of the healing process. • Low mood after operations is common. This can be a bereavement reaction, especially if patients have had a major amputation or suffered the removal of vital structures. Disfigurement and deformity after surgery can cause a great deal of distress. Standard care for depressive illness can be deployed with success for such cases. • Pain: postoperative pain syndromes are protean. Often there is no clear explanation for the pain. The symptoms are very real for the patient

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and require a sympathetic approach to achieve control (see p. 422). Referral to the local Pain Clinic is advised if there is no identifiable reason for the pain and simple measures for controlling the pain have not succeeded.

Abdominal wounds * Cellulitis: use an antibiotic which covers streptococci and staphylococci, e.g. erythromycin or co-amoxiclav. * Abscess: incise or refer immediately. * Serous ooze: refer for an early outpatient appointment. * Dehiscence: cover with clean tissues or a towel and admit immediately. Treat shock with iv morphine.

Gastrointestinal surgery * Be aware of the possibility of: (a) B12 deficiency after terminal ileum resection or gastric surgery; (b) malabsorption after small bowel resection; (c) blind loop syndrome after colonic surgery.

* Suspect graft infection if a patient with a graft develops a fever without obvious cause.

Eye surgery * Refer immediately if any of the following occur: (a) hyphaemia; (b) intraocular infection; (c) iris prolapse; (d) acute glaucoma; (e) retinal detachment (1-2% of all cataract extractions); (f) iritis which was not present on discharge; (g) postoperative conjunctivitis not settling after 10 days of antibiotic/steroid topically.

Gynaecological surgery * Termination: readmit if bleeding is more than a normal period. There may be retained products of conception. * Fever is frequently due to urinary infection after catheterization. Otherwise, after hysterectomy, consider vault haematoma, which will need rereferral. * Laparoscopy may cause diaphragmatic irritation with shoulder-tip pain. It will settle.

Anal surgery * Salt baths thrice daily and especially after stool. * Dab the anus dry with a paper towel. * Keep stool soft but not bulky. Use a laxative (but not fibre) and glycerol suppositories. * Prevent impaction with lignocaine gel administered into the anal canal via a nozzle. * If impaction occurs, use a stool-softening enema, e.g. Micolette or Fletcher's Enemette. * Treat diarrhoea promptly; it will excoriate the perianal wound.

Arterial surgery * Refer immediately if: (a) a graft blocks that was previously patent; or (b) emboli occur distal to a graft. * Refer urgently to outpatients if a pulsatile swelling appears at the site of a graft. It is probably an anastomotic aneurysm.

Tonsillectomy * Throat pain and otalgia: give antibiotics. * Tonsillar bed infection: give antibiotics. * Haemorrhage: admit if the loss is more than one cupful or continues for more than 24 hours. Even milder haemorrhage may require admission if there is not adequate supervision at home.52

Urosurgery * Cystoscopy: haematuria more than 24 hours after cystoscopy may signify infection. Patients need an MSU, antibiotics and rest. If severe, take blood for Hb, give tranexamic acid 1 g t.d.s. and consider admission. * TURP: The haematuria, urgency, dysuria, frequency and incontinence may take 2-3 weeks to resolve. Worsening symptoms from the second week suggest infection.

REFERENCES

Endocrine surgery * Be aware of the possibility of: (a) hypocalcaemia after thyroid surgery; (b) Addison's disease after pituitary/adrenal gland surgery; (c) diabetes mellitus after pancreatic surgery; (d) diabetes insipidus after pituitary/brain surgery; (e) premature ovarian failure after gynaecological procedures.

RETURNING TO WORK AFTER OPERATIONS (a) Time off is not usually needed after: vasectomy; varicose vein injections; anal dilatation; pilonidal sinus, provided the patient can be released for regular dressings. (b) 2 weeks for sedentary workers, 4 weeks for heavy workers: unilateral inguinal or femoral hernia; appendicectomy. (c) 4 weeks for sedentary workers, 6 weeks for heavy workers: bilateral inguinal hernias; small umbilical or incisional hernias. (d) 4 weeks for sedentary workers, 12 weeks for heavy workers: gastric and duodenal surgery; femoropopliteal grafts.

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(e) 6 weeks for sedentary workers, 12 weeks for heavy workers: cholecystectomy; abdominal or vaginal hysterectomy; large incisional or umbilical hernias; repair of a previous inguinal hernia repair; colectomy; aortoiliac and aortic grafts; coronary artery bypass and heart valve surgery; nephrectomy; an abdominal wound weakened by postoperative infection or haematoma.

Special cases (a) Simple mastectomy: as soon as the wound has healed. (b) Varicose vein ligation, anal fistula: 2 weeks. (c) Haemorrhoidectomy, deep vein thrombosis, TURP: 4 weeks. (d) Retinal detachment: If surgery is successful, the heaviest manual work should be avoided permanently. Individual differences in personality, wound healing and the presence of other illnesses will lead to variation in the advice given. For further details, see Fitness for Work: The Medical Aspects (The Joint Report of the Royal College of Physicians and the Faculty of Occupational Medicine).

REFERENCES 1. Masters SJ et al. Skull X-ray examinations after head trauma. Recommendations by a multidisciplinary panel and validation study. New Engl J Med 1987; 316: 84-91. 2. RCR Working party. Making the best use of a department of clinical radiology: guidelines for doctors. London: The Royal College of Radiologists, 1998. 3. Expert Panel on Neurologic Imaging: American College of Radiologists. Head trauma. In: ACR Appropriateness Criteria. American College of Radiology, 2000. Online. Available: http://www.acr.org 4. Voss M, Knottenbelt JD, Peden MM. Patients who re-attend after head injury: a high risk group. BMJ 1995; 311: 1395-8. 5. Jennett B, Teasdale G. Assessment of coma and impaired consciousness. A practical scale. Lancet 1974; 2 (7872): 81-4. 6. SIGN. Early management of head injury. Scottish Intercollegiate Guidelines Network 2000. Online. Available: www.sign.ac.uk

7. Haslam RHA. Head injuries. In Nelson textbook of pediatrics, 15th edition. Philadelphia, W.B. Saunders Company, 1996. 8. Greenwood RJ et al. Effects of case management after severe head injury. BMJ 1994; 308: 1199-1204. 9. Gill D, Hatcher S. A systematic review of the treatment of depression with antidepressant drugs in patients who also have a physical illness (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 10. Schierhout G, Roberts I. Prophylactic antiepileptic agents after head injury: a systematic review. J Neural Neurosurg Psychiatry 1998; 64: 108-12. 11. Cassidy JD et al. Effect of eliminating compensation for pain and suffering on the outcome of insurance claims for whiplash injury. New Engl J Med 2000; 342: 1179-86. 12. Livingstone M. Whiplash injury: why are we achieving so little? J R Soc Med 2000; 93: 526-9. 13. Binder A. Neck pain. In Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org

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14. Borchgrevink GE, Kaasa A, McDonagh D et al. Acute treatment of whiplash neck injuries. Spine 1998; 23: 25-31. 15. Rosenfield M, Gunnarsson R, Borenstein P. Early intervention in whiplash-associated disorders. Spine 2000; 25: 1782-7. 16. Lord SM, Barnsley L, Wallis BJ et al. Percutaneous radiofrequency neurotomy for chronic cervical zygapophysealjoint pain. New Engl J Med 1996; 335: 1721-6. 17. Stiell ID, Wells GA, Hoag RH et al. Implementation of the Ottowa knee rule for the use of radiography in acute knee injuries. JAMA 1997; 278: 2075-9. 18. Nichols G, Stiell IG, Wells GA et al. An economic analysis of the Ottowa knee rule. Ann Emerg Med 1999; 34: 438-47. 19. Auleley GR et al. Validation of the Ottowa ankle rules in France: a study in the surgical emergency department of a teaching hospital. Ann Emerg Med 1998; 32: 14-18. 20. Austoker J et al. Guidelines for referral of patients with breast problems. NHS Breast Screening Programme & The Cancer Research Campaign, 2nd edition; London: HMSO, 1999. 21. Bundred N. Breast pain. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org 22. Boyd NF et al. Effect of a low-fat high carbohydrate diet on symptoms of cyclical mastopathy. Lancet 1988; 2: 128-32. 23. Kontostolis E et al. Comparison of tamoxifen with danazol for treatment of cyclical mastalgia. Gynecol Endocrinol 1997; 11: 393-7. 24. Mansell RE et al. European multicentre trial of bromocriptine in cyclical mastalgia. Lancet 1990; 335: 190-3. 25. Colacurci N et al. Effects of tibolone on the breast. Eur J Obs Gynaecol and Rep Bio 1998; 80: 235-8. 26. Mittra I et al. Is clinical breast examination an acceptable alternative to mammographic screening? BMJ 2000; 321: 1071-3. 27. Miller AB et al. Canadian National Breast Screening Study 2: 13 year results of a randomised trial in women aged 50-59 years. / Nat Cancer Inst 2000; 92: 1490-8. 28. Canadian Task Force on Preventive Health Care. Preventive health care, 2001 update: screening mammography among women 40-49 years at average risk of breast cancer. CMAJ 2001; 164: 469-76. 29. Kerlikowske K, Grady D, Rubin SM et al. Efficacy of screening mammography. A meta-analysis. JAMA 1995; 273: 149-54. 30. Tabar L, Vitak B, Chen H-HT et al. Beyond randomized controlled trials: organized mammographic screening substantially reduces breast carcinoma mortality. Cancer 2001; 91: 1724-31. 31. Blanks RG et al. Effect of NHS breast screening programme on mortality from breast cancer in England and Wales, 1990-8: comparison of observed with predicted mortality. BMJ 2000; 321: 665-9. 32. Collaborative Group on Hormonal Factors in Breast Cancer. Breast cancer and hormone replacement therapy: collaborative reanalysis of data from 51 epidemiological studies of 52,705 women with breast cancer and 108,411 women without breast cancer. Lancet 1997; 350: 1047-59.

33. British Association of Surgical Oncology Guidelines. Eur J Surg Oncol 1998; 24: 464-76. In: Interim advice to GPs on familial breast cancer. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/ publications 34. McDermott MM, McCarthy W. Intermittent claudication: the natural history. Surg Clin N Am 1995; 75: 581-606. 35. Smith GD, Shipley MJ, Rose G. Intermittent claudication, heart disease risk factors and mortality: the Whitehall study. Circulation 1990; 82: 1925-31. 36. McGee SR, Boyko EJ. Physical examination and chronic lower-extremity ischemia. A critical review. Arch Intern Med 1998; 158: 1357-64. 37. Insall RL, Davies RJ, Prout WG. Significance of Buerger's test in the assessment of lower limb ischaemia. J R Soc Med 1989; 82: 729-31. 38. Hiatt WR. Medical treatment of peripheral arterial disease and claudication. New Engl J Med 2001; 344: 1608-21. 39. Tierney S, Fennessy F, Bouchier Hayes D. ABC of arterial and vascular disease: secondary prevention of peripheral vascular disease. BMJ 2000; 320: 1262-5. 40. Wannamethee SG, Shaper AG, Whincup PH et al. Smoking cessation and the risk of stroke in middle-aged men. JAMA 1995; 274: 155-60. 41. Gardner AW, Poehlman ET. Exercise rehabilitation programs for the treatment of claudication pain. A metaanalysis. JAMA 1995; 274: 975-80. 42. Antiplatelet Trialists' Collaboration. Collaborative overview of randomised trials of antiplatelet therapy II: Maintenance of vascular graft or arterial patency by antiplatelet therapy. BMJ 1994; 308: 159-68. 43. MeReC. Drug treatment of intermittent claudication. MeReC Bulletin 1994; 5: 17-20. 44. Isenberg DA, Black C. ABC of rheumatology: Raynaud's phenomenon, scleroderma, and overlap syndromes. BMJ 1995; 310: 795-8. 45. Wigley FM, Wise RA, Seibold JR et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis: a multicenter, placebocontrolled, double-blind study. Ann Intern Med 1994; 120: 199-206. 46. The UK Small Aneurysm Trial Participants. Mortality results for randomised controlled trial of early elective surgery or ultrasonographic surveillance for small abdominal aortic aneurysms. Lancet 1998; 352: 1649-55. 47. Thompson MM, Bell PRF. ABC of arterial and venous disease: arterial aneurysm. BMJ 2000; 320: 1193-6. 48. Johnson AG. Gall stones: the real issues. BMJ 1993; 306: 1114-15. 49. Tait N, Little JM. The treatment of gallstones. BMJ 1995; 311: 99-102. 50. DTB. Managing patients with gallstones. Drug Ther Bull 1994; 32: 33-5. 51. Muir IFK, Barclay TL, Settle JAD. Burns and their treatment, 3rd edition. London: Butterworths, 1987. 52. Kuo M et al. Early post-tonsillectomy morbidity following hospital discharge: do patients and GPs know what to expect? Health Trends 1995; 27: 98-100.

16

CHAPTER CONTENTS The National Service Framework 301

Psychiatric problems

Acute psychotic disorders 302 Compulsory admission 303 Chronic schizophrenia Drug treatment 305

304

Bipolar affective disorder 307 General management 307 Depression 309 Postnatal depression 313 Stress reactions 314 Post-traumatic stress disorder 315 Generalized anxiety 315 Panic disorder Phobias

316 Guideline: Department of Health. National Service Framework for Mental Health. London: The Stationery Office, 1999. Online. Available: www.doh.gov.uk/nsf/nsfhome.htm

317

Dealing with violence 317 Unexplained somatic complaints References 318

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THE NATIONAL SERVICE FRAMEWORK The National Service Framework for Mental Health for England and Wales1 provides clear goals for local service developments and indicates the roles and responsibilities of GPs, primary care groups, NHS Trusts, local authorities, service users and carers. Standard 2 relates most directly to primary care, requiring that: Any service user who contacts their primary health care team with a common mental health problem should: • have their mental health needs identified and assessed • be offered effective treatments, including referral for specialist services for further assessment, treatment and care if they require it.

Standards 4 and 5 indicate criteria for the provision of effective services for people with severe mental illness. Working groups need to be formed to develop the policies, protocols and guidelines that are feasible and acceptable within the available resources. Every practice should have a register of patients with severe and enduring mental illness that includes all patients with schizophrenia and bipolar affective disorder. The register, or repeat prescribing data, should be used to 301

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ensure that the patients are being reviewed at least 6-monthly. Practice issues: Members of the primary health-care team involved in mental health should meet regularly to agree: (a) policies, procedures and guidelines; (b) roles and responsibilities; (c) criteria for referral procedures and urgency; (d) to use the register appropriately; (e) to use computer templates to record management; (f) to ensure appropriate educational material is available and used; (g) to agree audit procedures and outcomes.

ACUTE PSYCHOTIC DISORDERS The possibility of acute psychosis is raised by the following: (a) hallucinations; (b) delusions; (c) disorganized or strange speech; (d) agitation or bizarre behaviour; (e) extreme and labile emotional states; (f) family concern about recent changes in personality, behaviour or function.

* If necessary, organize support and do not go alone. * Try to ensure that a relative or friend is present. * Tell someone in the practice whom you are visiting and when. * Arrange for the practice staff to phone you after a specified time. * Have an action plan if you do not respond to the phone call. Forcible tranquillization is rarely necessary. Tranquillization makes subsequent assessment difficult and sedated patients need observation which may not be possible. However, if it is needed, tranquillize the patient forcibly if there is no alternative, but only when sufficient police have arrived. For a strong patient, four male officers are likely to be needed. Give: (a) lorazepam 2-4 mg orally or, preferably, sublingually; or (b) haloperidol 5-10 mg orally; or, if refused, (c) lorazepam 1-2 mg im; or (d) haloperidol 5-10 mg im. If using haloperidol, have procyclidine 10 mg injection ready in case of dystonia.

Medication Initial assessment

If the patient needs medication before referral:

* Obtain a careful history from family or friends of recent events and changes in the patient's behaviour. * Ask about drug or alcohol misuse. * Assess the mental state. * Make a differential diagnosis if this is a first episode. Consider the possibilities of acute confusional state, drug-induced psychosis and epilepsy. * If this is a relapse, review past management and outcomes. * Decide if admission or referral is necessary. * Explain to the family what you think is wrong and what action is needed.

* Discuss the options with the duty psychiatrist; or * If the patient has relapsed because treatment was stopped, restart the previous medication if it was tolerated and acceptable. * Include details of any emergency medication in the referral letter and the time it was given.

Home visit When doing a home visit to assess a disturbed patient: * Ensure there is no past history of violence or delusions focused on the GP.

Referral and admission Refer all patients suffering from acute psychosis to the psychiatrist or community mental health team. The specialist services will usually decide whether the patient needs admission or can be managed at home. Admission is usually required in acute psychosis especially if: (a) it is the first episode; or (b) there is a significant risk of suicide, violence or neglect; or

ACUTE PSYCHOTIC DISORDERS

(c) the patient is non-compliant or has serious side-effects; or (d) there is coexisting alcohol or drug misuse. Consider managing at home a patient who is one suffering from a relapse of a known mental illness with a previous good response to treatment, where the patient agrees to restart, is at low risk, where home care is safe, and community support available.

COMPULSORY ADMISSION Mental Health Acts www.whoguidemhpcuk.org for a brief guide. England and Wales: www.doh.gov.uk/mhact1983.htm for both the explanatory memorandum to the Act and the Code of Practice.

Indications for use of the Mental Health Act (England and Wales) 1983 The Mental Health Act can only be used if the following conditions are met: (a) the patient is suffering from a mental disorder of a nature or degree which warrants detention in a hospital for assessment (or assessment followed by medical treatment) for at least a limited period; and (b) he or she ought to be detained in the interests of his or her own health or safety, or with a view to the protection of others; and (c) there is no alternative management other than compulsory admission to hospital. • Mental disorders are mental illness, psychopathic disorder, mental impairment and severe mental impairment. The latter two disorders refer to patients with learning difficulties associated with abnormally aggressive or seriously irresponsible conduct. • Although often called by relatives or neighbours, the doctor's role is to safeguard the patient's welfare and not that of the relatives or neighbours, unless they are in danger. • Copies of the medical recommendation forms can be obtained from: DoH Store, Health

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Publication Unit, No. 2 Site, Manchester Road, Heywood, Lancashire OL10 2PZ. • Approved social workers (ASWs) will carry the forms, as well as forms on which the GP can claim a fee.

Types of section Section 2 Allows admission for 28 days. The Mental Health Code of Practice2 stresses that this is the preferred section procedure. The assessment is by ASW, GP and approved psychiatrist. Section 4 Only permits admission for 72 hours. It should be reserved for emergencies when the doctor cannot leave the patient for fear of injury and waiting for a second doctor would cause 'undesirable delay'. The patient must be admitted within 24 hours. The assessment is by ASW and GP. Note: If the patient is about to injure him- or herself, or others, then a doctor may restrain the patient or give emergency treatment knowing that, under common law, such action is defensible if done in good faith. Such action would normally be followed by admission under Section 2 or 4. Section 3 Allows for admission for treatment for 6 months and is usually applied for when Section 4 or 2 have already been used and are about to expire. Application is made by the ASW supported by recommendations from GP and approved psychiatrist but efforts must be made to contact the nearest relative and obtain consent. Section 136 Allows police to remove a person from a public place to a hospital or police station, for a maximum period of 72 hours, if they are: (a) suffering from a mental disorder; and (b) in immediate need of care and control.

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Section 135 Allows a magistrate to authorize a police officer (with a doctor and approved social worker) to enter any premises to which access has been denied, and remove the patient to a place of safety if there is reasonable cause to suspect that the person is: (a) suffering from a mental disorder; and (b) is being ill-treated or neglected or not kept under proper control; or (c) is unable to care for themselves and lives alone. The GP can usually gain entry legally without using Section 135.

of two doctors, one of whom may be the patient's GP, and a social worker or another approved mental health professional. After 28 days, a mental health tribunal will review the compulsory treatment powers. Advice for patients and professionals: MIND provides a booklet on the Mental Health Act 1983: An Outline Guide. Send s.a.e. to MIND, 22 Harley Street, London W1N 2ED, tel. 020 7637 0741. The WHO Guide contains information on compulsory admission for professionals and patients. It is available on www.whoguidemhpcuk.org

Regulations for Scotland and Northern Ireland

Voluntary and compulsory admission procedures

These follow the same principles as those for England and Wales but the brief details of the Acts are as follows:

* Use the locally-agreed care pathway that indicates the procedure to be followed when a patient needs admission. Examples are given in Appendix 26. GPs need to adapt them to meet the local situation.

Mental Health (Scotland) Act 1984 Situation

Section

Emergency

24

Less urgent

18

Power of entry

117

Professionals

Duration

GP and MHO or relative GP and MHO and approved psychiatrist MHO obtains from JP GP and police enter

72 hours 6 months

Driving Patients who have an acute psychotic illness or a relapse of bipolar affective disorder should not drive and the DVLA must be informed4 (see p. 9). Further information is available from the DVLA Senior Medical Advisor.

Mental Health (Northern Ireland) Order 1986 Situation

Section

Professionals

Duration

Follow-up

Emergency Power of entry

4 129

GP and MHO or relative MHO obtains from JP. GP and police enter

7 days

* Agree clear guidelines between the primary care team and the mental health team on the roles and responsibilities of different people in providing immediate follow-up, long-term care, support and education of patient and family (see Schizophrenia, below).

ASW, approved social worker; GP, general practitioner; JP, justice of the peace; MHO, mental health officer. For more details, see www.psychiatry.ox.ac.uk/cebmh/ whoguidemhpcuk/mha.html

Compulsory community treatment The UK government White Paper Reforming the Mental Health Act published in 20003 envisages changes to the Mental Health Act including the possibility of compulsory care in the community under a treatment plan to be drawn up by a team

CHRONIC SCHIZOPHRENIA Review: McGrath J, Emmerson WB. Treatment of schizophrenia. BMJ 1999; 319: 1045-8.

CHRONIC SCHIZOPHRENIA

Department of Health. National Service Framework for Mental Health. London: The Stationery Office, 1999. Online. Available: www.doh.gov.uk/nsf/nsfhome.htm

• Between 0.5 and 1% of a practice population will suffer from schizophrenia at some time in their life and between 25 and 40% will lose contact with secondary services while still chronically ill.5 • Structured care offers a chance of improving the management of such patients. Structured care This entails: (a) Establishing a register of patients with severe and enduring mental illness. This can be done from existing clinical codes, from repeat prescriptions for psychotropic drugs, from addresses of hostels and homes catering for the mentally ill, and opportunistically.6 (b) Using the register to ensure that patients are seen at least 6-monthly. (c) Working with local mental health teams to identify agreed policies and guidelines for treatment, referral, and the management of relapse. (d) Adapting existing computer templates to record long-term follow-up and undertaking practice audit of the above goals.

Referral * Arrange for urgent assessment of the following: (a) those presenting for the first time; (b) those relapsing, or showing the prodrome of a relapse; (c) those at risk of relapse because of: -a deterioration in their home circumstances; or -poor compliance; or - abuse of drugs or alcohol; (d) those suffering from side-effects of medication. Such an assessment is better done at home than in the outpatient department if possible. Patients will also need to be referred, although not necessarily urgently, if (a) they are becoming increasingly disabled by their illness; or (b) a care plan needs to be drawn up; or (c) the patient or family request referral or the therapeutic relationship has broken down.

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General management * Education. The patient and the family need to know: (a) That 85-90% recover from the first episode in the following 2 years.7 In the 5 years following the first episode, half do not relapse, or relapse but recover completely between episodes. (b) How to identify early signs of relapse. Looking for early warning signs can prevent relapse or reduce its severity.8 The Early Warning Signs Form (see Appendix 27) can be used to help with this. (c) How to obtain early treatment. (d) That avoiding extremes of expressed emotion by family members, whether hostility or overprotectiveness, can reduce the risk of relapse. They need to know who can help them deal with expressed emotion in this way.9 * Advance directives. Discuss the use of advance directives with patient and family. These indicate what the patient wants to happen if a relapse occurs. * Refer the patient for training if needed in daily living or social skills to the psychiatric rehabilitation team at a day hospital or centre. * If fit for sheltered work, liaise with the mental health team to review options. * Assess the patient's housing needs. * Help the patient to claim the disability living allowance if eligible (see p. 5).

DRUG TREATMENT • Early drug treatment seems to lead to better medium- and long-term outcomes.10 • Without drugs, 60% of patients relapse in the 9 months after an acute attack. Maintenance with antipsychotics reduces this by over a half. • Newer (atypical) antipsychotics are now alternative first-line drugs in the UK.11

Initial choice of neuroleptics The choice of long-term medication should be made by the psychiatrist.

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PSYCHIATRIC PROBLEMS

Relapse Ascertain whether the relapse is due to the patient discontinuing medication. If it is: * assess the reason for stopping treatment, e.g. the presence of side-effects, failure to obtain a prescription, etc.; * decide if restarting previous medication is appropriate and acceptable; * review the need for urgent psychiatric assessment; * if assessment is needed but will be delayed, and there is concern about medication, phone for advice from the psychiatrist.

Medication review These patients need a medication review at least once every 6 months.12 The repeat prescription slip should indicate when a medication review is due. Someone in the practice needs to be responsible for ensuring such patients have attended for this review, and to know what action to take if the patient defaults. The medication review will include: (a) assessment of mental state and compliance; (b) presence of side effects or drug interactions; (c) existence of drug or alcohol problems; (d) whether the patient has been admitted in the past 6 months.

Side-effects Local guidelines are needed for GPs, community psychiatric nurses (CPNs), patients and families to identify side-effects and take appropriate action. The most frequently seen include: (a) Parkinsonian symptoms, e.g. tremor and akinesia, which occur in 10-15%. Ask the psychiatrist for advice on whether to reduce the neuroleptic dose. Start orphenadrine 50 mg t.d.s. rather than procyclidine, which is a stimulant and can be abused. Withdrawal of antiparkinsonian drugs should be attempted after 3 months without symptoms. (b) Acute dystonias or spasms, which can be managed as above.

(c) Akathisia (motor restlessness), which occurs in 20% initially and 40% long term. It may be improved by dosage reduction or beta-blockers. Switching to an atypical antipsychotic, e.g. olanzapine may help. Refer for advice. (d) Tardive dyskinesia occurs in 20% overall, and is much more likely in the elderly and in very long-term treatment. It does not usually improve on stopping treatment. An increase of dose helps temporarily but the symptoms return, together with other dose-related side-effects. Dose reduction may also help and is safer. Tetrabenazine may help to reduce the abnormal movements. Tardive dyskinesia may be less likely with atypical antipsychotics.13

Annual review The annual review is easier to do if there is a checklist or computer template that helps to record an agreed set of observations. These include: (a) agreed clinical codes; (b) current mental state including presence of depression, delusional thoughts, anxiety, hallucinations, signs of self-neglect; (c) problems, crises or admissions since last seen; (d) daily activities, employment, training, income and disability benefits; (e) substance abuse; (f) accommodation needs; (g) carers, relationships, dependants (children), home support; (h) assessment of physical health including review of blood pressure, cervical screening, smoking status, family planning needs; (i) medication review and blood tests if necessary; (j) whether care is GP only or shared with psychiatrist, CPN, or social worker; (k) assessment of carer's needs. Many practices use computer templates to record long-term care of diabetics and asthmatics. Similar practice templates have been developed to record and audit long-term care of patients with severe and enduring mental illness.

BIPOLAR AFFECTIVE DISORDER 307

Patient-held shared-care records There is evidence that patient-held shared-care records increase the effectiveness of long-term care for some patient with psychoses and schizophrenia.14 However, doubt has been raised about their value unless a dedicated coordinator is available to facilitate such an innovation.15 Such records are acceptable to patients with severe mental illness and: (a) increase patient autonomy; (b) improve communication between all involved in care; and (c) improve the effectiveness and safety of shared-care.

Patient organizations: The National Schizophrenia Fellowship, 30 Tabernacle Street, London EC2A 4DD, tel. 020 7330 9100/01; national advice line 020 8974 6814; www.nsf.org.uk MIND, Granta House, 15-19 Broadway, Stratford, London, E15 4BQ, tel. 020 8519 2122; Mind/nfoline 020 8522 1728; helpline 020 8522 1728; www.mind.org.uk SANE, First Floor, Cityside House, 40 Alder Street, London E1 1EE, tel. 0207 375 1002; helpline 0345 678 000; www.sane.org.uk/ Carer information: Wilkinson G, Kendrick T, Moore B. A carer's guide to schizophrenia. London: RSM Press Ltd, 1999. The WHO Guide to Mental Health in Primary Care provides excellent information for professionals and patients in book form and on disk. It is published by the Royal Society of Medicine Press Ltd, 2000 and is available online: www.whoguidemhpcuk.org

Carers' needs (see p. 408) People who care for someone with severe and enduring mental illness should have:

BIPOLAR AFFECTIVE DISORDER

(a) a needs assessment at regular intervals; (b) a care plan which is reviewed annually; (c) links with local carer support groups.

GENERAL MANAGEMENT

Carers need to be identified and this information should be put on their summary card or computer problem list. The practice should have a carers' register and a practice policy on whom to assess, when and by whom. Social services should record each carer's needs, draw up an agreed care plan which includes:

* Manage as for depression but with an awareness of the possibility that the patient will swing into mania. * Assess the risk of suicide or harm to others. Close supervision by family and friends may be needed.

(a) information about the mental health needs of the patient; (b) how to identify a relapse and what action to take; (c) advice on benefits, housing and employment; (d) arrangements for short-term breaks; (e) social support including access to carers' support groups; (f) information about appeals or complaints procedures. The plan should be confirmed in writing and communicated to the primary care team. The GP is the professional most likely to identify signs of stress or deteriorating health of a carer. The plan will indicate who should review the carer's needs at such times and the GP can ensure that this is done.

During episodes of depression

During episodes of mania * Encourage the patient and family to identify early warning signs of mood swings and to seek help as soon as these develop (see Appendix 27). * Avoid unnecessary confrontation. * Identify impulsive behaviours, e.g. act to prevent large amounts of money being spent irresponsibly. * Explain to the patient and family the importance of a regular routine to the patient's life. Mania can be triggered by disruption of routine, even if the reason for the change is not in itself stressful.16 Tell the family who to contact if the patient becomes very agitated or severely disruptive. * Enter the patient on a practice register of patients with severe mental illness.

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PSYCHIATRIC PROBLEMS

* Ensure recall for annual reviews (as in schizophrenia, see p. 306); * Identify the carer's needs to provide support and consider respite care.

Medication * Antipsychotic medication may be needed initially if behaviour is very disturbed (e.g. haloperidol 1.5-4mg t.d.s.). Use the smallest dose that relieves symptoms and aim to stop once the manic episode is over. Continued use can precipitate depression.17 * Benzodiazepines may also be needed to control acute agitation. Use diazepam 5-10 mg t.d.s. or lorazepam 2-4mg sublingually or 1-2 mg im (although it must be refrigerated and diluted with an equal quantity of water or saline before being injected.) * Lithium helps to relieve mania and depression and can prevent episodes from recurring. It should only be started by a specialist. * Anticonvulsants such as carbamazepine and valproate are now being used as alternative and adjunctive treatments to lithium. Cochrane Reviews on their efficacy are under way.18 Lithium The GP needs to: * Ensure clear agreement on who is responsible for monitoring lithium treatment. * Have an agreed protocol for monitoring blood levels, identifying side-effects and taking appropriate action. * Provide patients with a written description of drug side-effects and what to do if these develop e.g. lithium toxicity in The WHO Guide to mental health in primary care.19 * Check the patient knows to increase fluid intake and avoid dehydration and NSAIDs. * Put the patient on the recall register for monitoring blood levels. * Discuss pregnancy plans with women on lithium. * Check that the patient understands that the lithium should not be stopped abruptly unless toxicity occurs, as relapse may occur.

* Lithium levels. Check lithium levels every 3 months. Take blood 12 hours after the last dose. Levels of 0.4-0.8 mmol/L are adequate for maintenance but in acute mania a level of 0.8-1 mmol/L is necessary. * Change in dose. If the dose is changed, check levels weekly for 4 weeks, then monthly for 3 months and then 3-monthly. * Check thyroid and renal function annually.

Management of suspected lithium toxicity Symptoms of toxicity include muscle weakness, shaking, trembling, fasciculation, nausea, vomiting or diarrhoea, ataxia, confusion, slurred speech, toxic psychosis, convulsions, coma and cardiac arrhythmias. * Check the lithium level and U&Es. * Stop lithium. Levels usually fall to safety within 24 hours. If the lithium level is more than 1.5 mmol/L, or there is diarrhoea and vomiting, stop lithium immediately and seek urgent psychiatric advice. * Identify cause of toxicity before restarting lithium. Discuss the patient with their specialist. * If no cause is found, restart at a lower dose. * Monitor levels monthly for 3 months after toxicity for no obvious reason. Advice for patients and families: Manic Depression Fellowship, Castle Works, 21 Saint George's Road, London SE1 6ES, tel. 020 7793 2600; www.mdf.org.uk The Mental Health Foundation UK Office, 7th Floor, 83 Victoria Street, London SW1H 0HW, tel. 020 7802 0300. Scotland Office, 5th Floor, Merchants House, 30 George Square, Glasgow G2 1EG, tel. 0141 572 0125; www.mentalhealth.org.uk WHO Guide to Mental Health in Primary Care. See resource leaflets in Section 5-1: Living with bipolar disorder and 5-2: Lithium toxicity. Inside out: a guide to self-management of manic depression. Available from the Manic Depression Fellowship, 8-10 High Street, Kingston on Thames KT1 1EY. Copeland ME. Living without depression and manic depression: a workbook for maintaining mood stability. USA: New Harbinger Press.

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DEPRESSION Guidelines: Anderson IM, Nutt DJ, Deakin JFW. Evidence-based guidelines for treating depressive disorders with antidepressants: a revision of the 1993 British Association for Psychopharmacology guidelines. J Psychopharmacol 2000; 14: 3-20. Show V, Lascher S, Mottir-Pilson C. Pharmacologic treatment of acute major depression and dysthymia: clinical guideline part 1. Ann Int Med 2000; 132: 738-42. Williams JW Jr, Mulrow CD, Chiquette E et al. A systematic review of new pharmacotherapies for depression in adults: clinical guideline part 2. Ann Int Med 2000; 132: 743-56.

• GPs fail to diagnose up to half of their patients with a major depressive illness, and often fail to treat adequately those whom they do recognize.20,21 • Depression in people from the AfricanCaribbean, Asian, refugees and asylum seekers communities is often overlooked, although the prevalence is 60% higher than in the white population.1 People from black and minority ethnic communities are much less likely to be referred to psychological therapies.1 • Those patients whose depression is most likely to be missed are those with somatic complaints or with physical illness, those with long-standing depression, those who do not look depressed and those who do not realize they are depressed.20,22 • Depression is often accompanied and masked by anxiety,23 yet treatment of the anxiety alone is insufficient and may appear to worsen the depression.

Presenting complaints * Screen for depression with two specific questions: During the past month have you felt: (a) low, depressed or hopeless? (b) little interest or pleasure in doing things?24 * Confirm the diagnosis of depression using the DSM IV criteria as follows. Used by GPs this is highly specific.25 A major depressive illness exists if patient has low mood or diminished interest or pleasure for at

least 2 weeks, occurring most of the day or nearly every day, and a positive score on at least five of the following: (a) depressed mood; (b) loss of interest or pleasure; (c) change in weight or altered appetite almost every day; (d) disturbed sleep; (e) agitation or slowing of movement or speech; (f) fatigue or loss of energy; (g) guilt or low esteem; (h) poor concentration; (i) recurrent suicidal thoughts or acts. Note: The scoring system cannot be used in patients reacting appropriately to a life crisis or who are schizophrenic and the symptoms should indicate significant distress or impairment in functioning. Minor depression is characterized by fewer than five of the above. The evidence that it responds to antidepressant drugs is poor, unless it lasts for at least 2 years, when it may be called dysthymia, which may respond to drugs.21,26

Differential diagnosis * Schizophrenia. Look for evidence of schizophrenia, which may present with depression. If delusions or hallucinations are also present, depression should not be the initial diagnosis. * Bipolar affective disorder. Ask about a past history of a manic episode. The management of bipolar affective disorder is different. * latrogenic cause. Check for drugs that can cause depression, e.g. antihypertensives, H2-blockers, steroids, and beta-blockers. * Life events. Ask about recent life events such as recent childbirth, bereavement, termination of pregnancy, loss of job, work stress, family illness or divorce. They may have triggered the depression.

Risk of suicide * 40-50% of suicides are patients with undiagnosed or inadequately treated depression. * The diagnosis and adequate treatment of depression are effective ways to prevent suicide.

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* In all patients with depression ask a series of questions, only stopping when the answer is no: (1) have you thought how nice it would be if you did not wake up one morning? (2) have you thought of killing yourself? (3) have you decided how to do it? (4) have you decided when to do it? * Judge the risk according to the following risk factors: (a) a history of previous attempts, especially if determined or violent methods were used, or if a suicide note was left; (b) intense feelings of hopelessness and worthlessness; (c) major mental illness including depression; (d) suicidal ideation or evidence of planning, e.g. when, and if a sudden and infallible method is identified;27 (e) chronic physical illness or in much pain; (f) recent bereavement or other significant loss including job; (g) males over 45; (h) alcohol or other substance misuse; (i) previous inpatient psychiatric treatment; (j) family history of mental illness, suicide, or alcoholism; (k) AIDS or HIV-positive patients; (1) unmarried, separated or divorced, especially if living alone.

Management of suicidal patients Low risk

* Manage at home patients who have thoughts of suicide but who have: (a) no clear suicidal plans or past history of a serious suicidal attempt; (b) good rapport with their GP or local psychiatric services; (c) 24-hour home support; (d) a stable personality (e) no psychotic illness, chronic physical illness, or drug or alcohol misuse * Drugs. If prescribing, give small quantities of medication, initially.

High risk

* Refer urgently to the mental health team. Check that the patient meets the previously agreed local criteria for same-day referral. A domiciliary visit by a psychiatrist may be indicated (even if a patient refuses all offers of help). * If the GP and psychiatrist cannot visit together, make sure that the GP is notified immediately of the outcome of the visit. * Ensure that the carers can provide observation for 24 hours a day, until the risk diminishes. The family may need help to provide this level of care. If drug treatment is started, a relative or carer ought to be asked to supervise the medication.

Follow-up after attempted suicide Often the first time a GP hears about a suicidal patient is when a hospital discharge report is received. When this occurs the doctor should: (a) review the records to see if the patient has recently attended; (b) discuss the management with the mental health care team, if the patient has a severe longstanding mental illness; (b) identify anything that might indicate the patient was a suicide risk; (c) enter suicide attempt on summary card and on computer; (d) ask the patient to make an appointment to see the GP for review; (e) follow-up to: -identify depression or other precipitating life events; -assess need for treatment including counselling; -make a care plan to review outcome and prevent recurrence; (f) start treatment as below if indicated.

General management of depression • The GP will manage 90% of depressed patients without consultant referral. * Decide if this is minor or major depression, or dysihymia. Antidepressants will be needed in the

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last two of these, but counselling or psychotherapy may be more appropriate in the first (see p. 312). * Life events. Identify relevant life events which may have precipitated the illness and focus on small steps that might be taken to reduce their impact. * Explore effects on behaviour and relationships at home and work. * Explain the nature of the illness, its treatment and good prognosis. * Discuss the link between physical symptoms and mood. * Encourage the patient not to make major or irreversible decisions about work or family until their mental state improves. * Exercise. Recommend exercise every day, even (or especially) on days when the patient feels like staying in bed.28 * Drug therapy. Decide whether medication is appropriate and acceptable to the patient. Explain the side-effects and time taken to change mood (see below). * Counselling. Assess the need for counselling and discuss this option with the patient (see below). * If the patient decides not to take medication, explore other options and review weekly. Complementary therapies are popular but the evidence of benefit is poor.29 * Follow up. Explain the need for and frequency of GP follow-up. * Patient information. Provide one of the leaflets listed in the box on p. 313.

Referral Most patients are managed completely in primary care. However, psychiatric referral of a non-suicidal patient may be needed if: (a) the differential diagnosis may include psychosis or schizophrenia; (b) admission is being considered, e.g. severe depression, risk of self-neglect, social isolation, little family support, poor compliance; (c) selection of antidepressant is difficult because of associated physical illness or drug interactions; (d) there have been previous episodes of major depression and prophylactic drug treatment may be needed;

(e) other forms of treatment are needed, e.g. cognitive behavioural therapy; (f) the patient is an adolescent with severe depression; (g) there has been a poor response to an appropriate antidepressant in maximum dosage, with good compliance, taken for an adequate period of time.

Medication Evidence and policies • Patients will respond to antidepressants if their depression is sufficiently severe or prolonged, whether it is judged to be reactive or endogenous. • A score of 5 or more on the DSMIV criteria (see above) or a duration of over 2 years indicates that the patient is likely to respond to antidepressants. • There is no evidence that SSRIs are more effective than tricyclic antidepressants (TCAs)30 and all antidepressants have a similar delay in onset. • The dropout rate with SSRIs in clinical trials is 10% better than with TCAs.31 Patients need to be warned that side-effects will occur in the first 2 weeks before any benefit has occurred. These will lessen if treatment is continued. • As side-effect profiles differ, some drugs may be more acceptable to particular patients than others. • Locally agreed guidelines are needed to identify drugs of first choice. A switch from TCAs to SSRIs as first line in 1994 would have cost the NHS in England and Wales an extra £100 million pounds. However, economic analyses in which doctor time as well as drug costs have been included have shown that SSRIs can be more costeffective.32 The appropriate drug must be given at the right dose for the right length of time. A UK study has shown that few patients given TCAs by GPs receive an effective dose for long enough.33 Drug dosage is not a problem with SSRIs but they tend to be given for inadequate duration in the same way as TCAs. • Patients with an inadequate response to firstline drugs need referral to consider using augmentation with drugs such as lithium, which have been shown to be effective in such cases.1 • Patients need reassurance that they will not become drug dependent.

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Choice

* SSRIs can be started at full therapeutic dose.

* Use locally agreed guidelines but in doing so, tailor the choice to the patient as follows: (a) the elderly: avoid highly anticholinergic drugs (e.g. amitriptyline, clomipramine, doxepin, imipramine and maprotiline); (b) prostatism or glaucoma: avoid all anticholinergic drugs; (c) cardiac disease: avoid tricyclics; (d) suicide risk: avoid tricyclics; (e) lethargy: avoid sedative drugs, e.g. amitriptyline, clomipramine, dothiepin, doxepin, maprotilene, mianserin, trazodone and trimipramine; (f) anxiety or insomnia: use a sedative drug; (g) alcohol misuse: choose one of the SSRIs which do not interact with alcohol (see BNF Section 4.3.3.); (h) obesity: use an SSRI; (i) epilepsy: avoid SSRIs. * Recurrence. Use a previously successful drug if there is a recurrence. * St John's Wort. In some patients who may not want to take conventional medication, consider recommending St John's Wort. At a dose of hypericum extract of 350 mg t.d.s. it is superior to placebo and as good as imipramine 100 mg daily in mild-moderate depression.34,35 It has mild monoamine oxidase inhibitor (MAOI) properties so should not be combined with other antidepressants and caution may be needed with diet.36 It is an inducer of drug-metabolizing enzymes and so can reduce the effectiveness of the following: oral contraceptives, warfarin, digoxin, theophylline, cyclosporin, indinavir. It may also reduce the effectiveness of other HIV protease inhibitors, non-nucleoside reverse transcriptase inhibitors, and anticonvulsants (phenytoin, carbamazepine and phenobarbitone).37 More information is given onwww.open.gov.uk/mca/mcahome.htm

Patients should be advised to consult a doctor before stopping medication.

Dose * TCAs. Amitriptyline should be started with 75 mg at night for 3 weeks, then increased to 150 mg unless there is already an adequate response. The elderly will need lower starting doses.

Counselling/psychotherapy * There is evidence that psychological therapy can lead to faster resolution of depression than usual GP care in the short term, without increased costs, but with no difference in outcome after 1 year.38,39 * In the mild-moderate end of the spectrum of major depression, cognitive and behavioural therapies can be more effective than antidepressants and may prevent relapse.40 * In major depression, generic counselling can be as effective as antidepressants, if the counsellors are experienced and the counselling is available without delay. However, patients receiving antidepressants may recover more quickly. More patients would choose counselling than drugs if given the choice.41 In major depression, a combination of antidepressants and cognitive behavioural therapy can be more effective than either alone.42 Other psychotherapies have not shown a benefit from being combined with drugs.43 * Most of the evidence for the benefit of psychological therapies in depression lies with certain techniques only, mainly cognitive behavioural and problem-solving ones,43 and cannot be assumed to exist for all forms of counselling.44 * Minor depression: offer counselling or psychotherapy. * Major depression: offer patients a choice of counselling, psychotherapy, or drug treatment, provided a therapist is able to see the patient promptly. Even then, counselling or psychotherapy without drugs is not appropriate for patients at the severe end of the spectrum of major depression.

Follow-up * Look for changes in mood, affect, behaviour and function at home and work. * Review patients weekly initially, then monthly. * Continue antidepressants for at least 4-6 months after recovery. This reduces the relapse rate from 50% to 20%. A reduction in dose should only be made if side-effects are a problem.

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• Stopping treatment. Tail the drug off over 4-6 weeks to avoid a discontinuation reaction.45 Review patients 4 weeks later to ensure they remain well. Advise them to return at the earliest sign of a recurrence.

Prevention of recurrence • Seventy-five per cent of patients with a first episode of major depression have a recurrence within the next 10 years.46 A second episode during the next 4 years requires full initial treatment, followed by psychiatric referral to consider prophylaxis with long-term antidepressant medication. In the first 2 years this will reduce the rate of recurrence from 75% to 30%. • Long-term prophylaxis after a single episode may be appropriate in the following because their risk of recurrence is even higher: (a) the elderly; (b) those with a first-degree relative with bipolar disorder or recurrent major depression.

(b) with a family history of depression; (c) who were unhappy during pregnancy; (d) with marital difficulties; (e) with recent stressful events; (f) on low income; (g) in poor housing; (h) who are single mothers; (i) with physical illness or handicap in the family. * Every practice should be aware of the need to identify 'at risk' patients during the antenatal period and should ensure that they receive more intensive help after birth. Risk factors should be identified during pregnancy and the primary care team need to decide: (a) what extra care this group should receive; and (b) who is responsible for identifying this condition as early as possible? * Patients often do not realize that they are depressed, and doctors recognize it even less frequently. Women often present with a feeling of not coping rather than with classic symptoms of depression.

Information for patients: The Samaritans 0345 909090. The Mental Health Foundation (see p. 308) www.mentalhealth.org.uk Leaflets: patient guide on depression from The WHO Guide to Mental Health in Primary Care available on www.whoguidemhpcuk.org Royal College of Psychiatry leaflet Help is at Hand: Depression, available on www.rcpsych.ac.uk/info/index.htm Books: Butler G, Hope A. Manage your mind: the mental fitness guide. Oxford: OUP, 1995. Rowe D. Depression: the way out of your prison. London: Routledge and Kegan Paul, 1995.

POSTNATAL DEPRESSION • This occurs in 10-15% in the first year after delivery, usually in the first 6 months. The symptoms are almost always present at 6 weeks. It is distinct from the transient 'blues' of the first 10 days, and from a puerperal psychosis which is likely to need admission. • It is more likely in those: (a) with previous psychiatric illness;

Every woman should be screened at the postnatal examination. The Edinburgh Postnatal Depression Scale47 may be used (see Appendix 15). If the scale is not available, ask four simple questions: (a) (b) (c) (d)

How are you feeling? How are you sleeping? How are you eating? Are you enjoying the baby?

If depression is found * Check TFTs in those complaining mainly of tiredness (see p. 267). * Counsel. Simple, non-directive counselling by health visitors, weekly for 8 weeks, doubles the recovery rate.48 * Literature. Recommend Marshall F. Coping with postnatal depression. Shelden Press 1993. * Antidepressants. Treat vigorously those scoring 12 or more on the EPDS scale (see Appendix 15). Lofepramine is recommended because of its shorter half-life, as the risk of drug accumulation in the infant is less.49

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* Consider referral if a multidisciplinary mental health team is able to offer more than the primary health-care team. * Refer immediately for urgent psychiatric assessment any women with ideas of suicide or of harming the baby. Patient organizations: The Association for Post Natal Illness, 145 Dawes Road, Fulham, London SW6 7EB, tel. 020 7386 0868; www.apni.org The Mental Health Foundation (see p. 308) www.mentalhealth.org.uk

STRESS REACTIONS • Stress reactions may be: (a) acute and brief; (b) an adjustment reaction which may last a few months; or (c) post-traumatic stress disorder (PTSD), with a delay between the stress and the onset of symptoms. • Stress may be a reaction to loss or trauma. Symptoms may be those of anxiety, depression, abnormal behaviour or inability to cope with normal events. Symptoms may have been present for a long time before help is sought. • Stressful events include: (a) crime or accidents involving psychological and/or physical trauma; (b) bereavement, including siblings in a family where a child has died; (c) termination of pregnancy, miscarriage; (d) redundancy, loss of job, occupational stress; (e) divorce, separation, relationship difficulties; (f) housing or financial problems; (g) surviving a disaster; (h) having to perform in front of an audience, or have work appraisal; (i) seeking asylum; (j) drug or alcohol withdrawal.

Management * Identify underlying precipitating events and the steps taken by the patient to modify or cope with the situation.

* Exclude a physical or drug cause for the symptoms (drug or alcohol withdrawal, sudden stopping of a beta-blocker, hyperthyroidism). * Identify others who might help, e.g. Relate, Citizens' Advice Bureau, Victim Support, union representative, local police domestic violence unit. * Review what support can be obtained from family, friends, work colleagues and sources of community support. * Discuss coping strategies. * Assess the need for counselling or posttraumatic stress management. * Decide if short-term time off work might be helpful. * Consider prescribing a beta-blocker if somatic symptoms (shaking and tachycardia) are prominent. Avoid anxiolytic drugs but if the symptoms are very severe give them for 3 days only.

Insomnia * Check that the insomnia is not due to an underlying disorder, either mental or physical, which needs treatment in its own right. * Explain that failing to sleep is a natural part of a reaction to stress and not harmful; and that worrying about not sleeping makes sleep harder to achieve. * Check that the patient is not using alcohol to sleep. It causes early morning rebound awakening. * Explain that simple rules will help and offer an leaflet, e.g. www.psychiatry.ox.ac.uk/cebmh/ whoguidemhpcuk/index.html (choose 'self-help leaflets' then 'overcoming sleep problems'). Behavioural techniques can be as effective as drug treatment, with longer-lasting benefit, but they take longer to administer.50,51 * Offer medication only if the patient's functioning is affected by the lack of sleep and the reason for the insomnia is temporary (e.g. jetlag or examinations). Obtain the patient's agreement that the course will not extend beyond 14 days because of the danger of dependence. Use a short-acting benzodiazepine or zopiclone, which may have less rebound insomnia but is still only licensed for short-term use.

GENERALIZED ANXIETY 315

POST-TRAUMATIC STRESS DISORDER (PTSD) • 23% of assault victims and 80% of rape victims develop PTSD. Presentation may be delayed for several months after the trauma. • The WHO guide to mental health in primary care19 describes this as a specific disturbance occurring more than 1 month after the event with: (a) history of a very threatening or catastrophic event producing feelings of intense horror, fear or helplessness; (b) intrusive symptoms; memories, flashbacks and nightmares; (c) avoidance of thoughts, activities, situations associated with the event; (d) possible suicide risk if severe depression or guilt develops; (d) symptoms of autonomic arousal, e.g. hypervigilance, insomnia, irritability, excessive anger, and impaired concentration and/or memory; (e) possible drug or alcohol abuse; (f) functional impairment at home and work.

may be needed. Startle and hyperarousal symptoms may be helped by beta-blockers (BNF Section 2.4) The evidence for benzodiazepines is poor. * Refer to secondary services if severe disability persists in spite of the above measures. Posttraumatic stress counselling is a specialized skill, which may be available locally. Behaviour therapy or cognitive methods may help.54 * Inform the patient about available community resources listed below. Advice: Victim Support: emotional and practical support for victims of crime. Tel. 020 7735 9166; Supportline 0845 30 30 900 (9 a.m.-9 p.m., Mon-Fri; 9 a.m.-7 p.m. Sat, Sun.) Refugee Support Centre: counselling for refugees, asylum seekers, tel. 020 7820 3606. Trauma Aftercare Trust: information about counselling and treatment, tel. 01242 890498. Medical Foundation for Care of Victims of Torture, tel. 020 7482 0219. CombatStress supports ex-service people with PTSD. tel. 020 8543 6333.

Management * Debriefing. Do not routinely offer debriefing after a traumatic event. It appears to be useless and may be harmful.52 * Provide information for patient and family (see WHO resource leaflets19). * Encourage discussion of the precipitatory event once PTSD has developed. * Encourage the patient to discuss his or her feelings and fears. * Explain how avoidance of trauma cues maintains fears and distress. * Explore a gradual plan to face avoided activities and situations. * Alcohol. Warn about the need to avoid excess use of alcohol. * Antidepressants. Consider antidepressant medication including TCAs and SSRIs for treating intrusion and avoidance symptoms. Sertraline has been shown to increase the response rate from one-third (with placebo) to a half, although the reduction in symptoms was small.53 It may take 8 weeks before effects are seen and high doses

GENERALIZED ANXIETY Management * Look for other conditions: (a) thyrotoxicosis; (b) depression; (c) psychotic illness; (d) alcohol or drug misuse or withdrawal; (e) post-traumatic stress disorder; (f) reactions to recent life event or trauma; (g) phobic states or panic disorder; (h) a side-effect of new medication. If the anxiety has no obvious underlying cause: * Explain how anxiety causes physical symptoms and they in turn increase anxiety. * Assess the patient's disability. How does it affect family relationships, sex, work, and physical and mental state? Is a job at risk? Is alcohol or caffeine intake excessive? * Help the patient to identify lifestyle changes that might reduce stress.

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* Give the patient some guidance on how to reduce anxiety. Two easily understood techniques for the patient are: (a) to challenge the importance of the thing causing anxiety: 'will this matter in 5 years time?' (b) to challenge the idea that what they fear is likely to happen. * Recommend Manage your mind: the mental fitness guide by Gillian Butler and Tony Hope (OUP, 1995) for details of strategies that patients can use for themselves. * Assess the need for cognitive behaviour therapy, or anxiety management. These methods are more effective than drug therapy55 and the best evidence of benefit is for cognitive behaviour therapy.56 All patients whose symptoms are chronic and disabling should be referred. * Help the patient to identify relevant self-help groups, or relaxation or yoga classes. * Give the patient a copy of one of the leaflets described below. * Help the patient to make a management plan and set a date for review. Drug treatment is not first-line therapy but: (a) beta-blockers may reduce severe somatic symptoms although there is a dearth of evidence;57 (b) benzodiazepines are effective in the short term but have serious side-effects of sedation, with the risk of accompanying road and industrial accidents, and of impaired memory and dependence. They should be avoided if at all possible. If prescribed, they should only be used for less than 2 weeks to tide a patient over a crisis. They are the subject of a special warning from the CSM to limit their use (BNF section 4.1). Avoid the daytime use of short-acting benzodiazepines (e.g. temazepam or lorazepam); (c) antidepressant drugs, whether SSRIs, TCAs or related antidepressants, appear to be more effective than benzodiazepines and may be used longterm. Typical NNTs for moderate or pronounced improvement over 8 weeks are 3-4.58

Information for patients: Leaflets: patient guide on anxiety from the WHO Guide to Mental Health in Primary Care available on www.whoguidemhpcuk.org

Royal College of Psychiatrists' leaflet Help is at Hand: Anxiety and Phobias available on www.rcpsych.ac.uk/info/index.htm Book: Butler G, Hope A. Manage your mind: the mental fitness guide. Oxford: OUR 1995.

PANIC DISORDER * Follow the management outlined under Generalized Anxiety. It is even more important to explain the link between the feeling of panic and the physical symptoms it produces, since patients often see it as a physical illness. * Rehearse the patient in saying: 'This is only a panic attack. It will pass in a few minutes. Nothing awful is going to happen.' * Explain how hyperventilation can worsen some of the physical symptoms associated with panic. * Explain how breathing slowly can help.

Drug therapy * Benzodiazepines should not be given. They are not very effective, and they are inappropriate in a disorder that usually lasts over 4 weeks and where dependence commonly occurs. * Beta-blockers, e.g. propranolol 40 mg t.d.s., will abolish the somatic symptoms of panic and may help the subjective terror as well.59 This should be the drug of first choice if patient is not asthmatic. * Antidepressants. Both TCAs and SSRIs, given regularly, may stop panic attacks completely. Use them only in resistant cases. Warn the patient that symptoms may worsen in the first 1-2 weeks. * Refer patients whose attacks are severe or frequent or continue for more than 1-2 months. There is evidence of benefit from cognitive behaviour therapy and applied relaxation but not from other therapies nor from counselling.44

Information for patients: 'No Panic' helpline 01952 590545; information line 0800 783 1531. Leaflets: patient guide on anxiety from the WHO guide to mental health in primary care, available on www.whoguidemhpcuk.org

DEALING WITH VIOLENCE 317

Royal College of Psychiatrists' leaflet Help is at hand: anxiety and phobias, available on http://www.rcpsych.ac.uk/info/index.htm Book: Butler G, Hope A. Manage your mind: the mental fitness guide. Oxford: OUR 1995.

PHOBIAS * Phobias require energetic management if they present early in order to prevent them from becoming fixed. Even those presenting late are amenable to treatment if the patient is willing to undergo therapy. All patients with phobias need treatment. The first decision is whether or not this can be provided by the GP or needs referral for behavioural therapy. * Needle phobia is a problem that is important to treat. Patients need to decide whether to have treatment now, or be forced to confront their greatest fear when least able to deal with it. If the GP has the skills, this can be effectively dealt with in practice over five or ten short consultations. * Try to identify any factors that precipitated the phobia in the first place. Anxiety about this may need counselling. * Urge the patient not to avoid the phobic situation. Avoidance increases the strength of the phobia. * Check that the patient is not using alcohol to try to cope. * Plan a programme of gently increasing exposure to the phobia. Do this with imaginary exposure if real exposure is not possible. Exposure must be daily, and the patient must stay in the phobic situation until the panic has subsided. Ask the patient to keep a diary to discuss at each appointment. * Find out what would be 'the worst possible scenario'. Often it turns out to be unpleasant but acceptable. Usually it is that other people will see that the patient is being phobic. Being prepared to 'own up' to a phobia is often a great step forward. * Recommend Manage your mind: the mental fitness guide by Gillian Butler and Tony Hope (OUP, 1995) for details of strategies that patients can use for themselves.

* Drug treatment: avoid giving benzodiazepines for a phobia. The patient is likely to become even less able to face the situation without drugs than before treatment began. Consider a beta-blocker, e.g. propranolol 40 mg 1-2 hours before exposure to the phobic situation in those who cannot tolerate the physical symptoms of panic. * Cognitive behaviour therapy. Refer any patient with a phobia that is not responding to treatment. There is insufficient evidence of benefit from other forms of psychological therapy or counselling.44 * Antidepressants. Consider prescribing a TCA or SSRI in resistant cases, in consultation with the patient's therapist or psychiatrist. Information for patients: Self-help group: Triumph over Phobia, PO Box 1831, Bath BA1 3YX, tel. 01225 330353. Leaflets: patient guide on phobias from the WHO guide to mental health in primary care, available on www.whoguidemhpcuk.org Royal College of Psychiatrists' leaflet Help is at hand: anxiety and phobias, available on www.rcpsych.ac.uk/info/index.htm Books: Butler G, Hope A. Manage your mind: the mental fitness guide. Oxford: OUP, 1995. Marks IM. Living with Fear. New York: McGraw-Hill, 1980.

DEALING WITH VIOLENCE There should be a practice policy for dealing with violent patients. Many of these patients have a past history of violent or threatening behaviour. There should be: (a) a notice in the waiting room stating that physical or verbal abuse, racism, threats or violence to staff will result in removal from the practice; (b) panic buttons in consulting rooms and a clear procedure to follow if one is set off; (c) clear guidelines on who should deal with an aggressive, abusive or violent patient in the waiting room;

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(d) risks can be assessed and there needs to be agreement on how this should be done and what to do about it;60 (e) a protocol for management of a patient whose delusions are focused on a specific doctor or nurse; (f) written indications on when to call the police and who should do so; (g) guidance on how to deal with home visit requests to a potentially violent patient; (h) debriefing counselling for staff exposed to violent situations; (i) a policy of informing local GPs that a violent patient who has been removed from the practice list may want to register with them; (j) a record kept of all such incidents; (k) a way to report these incidents as 'significant events' so that lessons can be learnt by others; (1) a PCO plan to deal with violent patients GPs refuse to treat. Guideline: Braithwaite R. Violence, understanding, intervention and prevention. Oxford: Radcliffe Professional Press. This book can be used to develop a practice response to potentially dangerous situations.

UNEXPLAINED SOMATIC COMPLAINTS Review: Goldberg R, Dennis H, Novack M, Gask L. The recognition and management of somatization: what is needed in primary care training. Psychosomatics 1992; 33: 55-61.

There is a group of patients characterized by: (a) voluminous records;

(b) frequent attendances for physical symptoms intensively investigated by many hospital referrals with no cause found; (c) repeated reassurance does not alter the belief that physical illness is the underlying cause; (d) repeated demands for further investigations and referrals are made; (e) symptoms of depression and anxiety are common but rejected by the patient as the primary problem.

Management * Acknowledge the fact that the symptoms are real and explain the relationship between somatic and psychological symptoms. * Treat any associated depression or anxiety. * Try to reduce polypharmacy. * Offer a second opinion from another partner. * Document discussions with partners or hospital colleagues to share responsibility for management decisions. * Try to see the patient in time-limited scheduled appointments with the same doctor to ensure a consistent approach and reduce frequent requests for home visits or requests for emergency appointments. * Try a low-dose TCA. * Avoid multiple referrals to hospital specialists. * Consider referral to a liaison psychiatrist if the service is available. * Consider cognitive behavioural therapy if the patient will accept it.61 * Give the patient the leaflet on unexplained physical complaints from the WHO guide to mental health in primary care,19 as well as those on learning to relax, and problem solving if appropriate.

REFERENCES 1. Department of Health. National Service Framework for Mental Health. London: The Stationery Office, 1999. Online. Available: www.doh.gov.uk/nsf/nsfhome.htm 2. Mental Health Act 1983. Memorandum on parts 1-6, 8 and 10. London: The Stationary Office, 1998. 3. Reforming the Mental Health Act. Government White Paper. London: The Stationary Office, 2000.

4. Driver and Vehicle Licensing Agency. At-a-glance guide to medical aspects of fitness to drive. Swansea: DVLA, 1998. 5. DTB. Long-term management of people with psychotic disorders in the community. Drug Ther Bull 1994; 32: 73-7. 6. Kai J, Crosland A, Drinkwater C. Prevalence of enduring and disabling mental illness in the inner city. Br J Gen Pract 2000; 50: 988-94.

REFERENCES

7. World Health Organization. Schizophrenia: an international follow-up study. Chichester: John Wiley & Sons, 1979. 8. Falloon IRH, Laporta M, Fadden G, Graham-Hole V. Managing stress in families: cognitive and behavioural strategies for enhancing coping skills. London: Routledge, 1993. 9. Falloon I, Coverdale J, Laidlaw T et al. Family management in the prevention of morbidity of schizophrenia: social outcome of a two-year longitudinal study. Psychol Med 1998; 17: 59-66. 10. Loebel, AD et al. Duration of psychosis and outcome in first-episode schizophrenia. Am J Psychiatry 1992; 149: 1183-8. 11. NICE. Guidance on the use of newer (atypical) antipsychotic drugs for the treatment of schizophrenia. 2002. Available: www.nice.org.uk 12. Primary Care Schizophrenia Consensus Group: Guidelines for the care of schizophrenia in general practice. Online. Available: www.eguidelines.co.uk 13. Beasley CM, Dellva MA, Tamura RN et al. Randomized double-blind comparison of the incidence of tardive dyskinesia in patients with schizophrenia during long-term treatment with olanzapine or haloperidol. Br J Psychiatry 1999; 174: 23-30. 14. Essex B, Doig R, Renshaw J. Pilot study of records of shared care for people with mental illness. BMJ 1990; 300: 1442-6. 15. Warner JP, King M, Blizard R et al. Patient-held shared care records for individuals with mental illness: randomized controlled evaluation. Br J Psychiatry 2000; 177: 319-24. 16. Malkoff-Schwartz S, Frank E, Anderson B et al. Stressful life events and social rhythm disruption in the onset of manic and depressive bipolar episodes. Arch Gen Psychiatry 1998; 55: 702-7. 17. Licht RW. Drug treatment of mania: a critical review. Acta Psych Scand 1998; 97: 387-97. 18. Young AH, Calabrese JR. Treatment of bipolar affective disorder. BMJ 2000; 321: 1302-3. 19. World Health Organization Collaborating Centre for Research and Training for Mental Health. WHO Guide to Mental Health in Primary Care. Adapted for the UK. London: Royal Society of Medicine Press Ltd, 2000. Online. Available: www.whoguidemhpcuk.org 20. Freeling P, Rao BM, Paykel ES et al. Unrecognised depression in general practice. BMJ 1985; 290: 1880-3. 21. Anderson IM, Nutt DJ, Deakin JFW. Evidence-based guidelines for treating depressive disorders with antildepressants: a revision of the 1993 British Association for Psychopharmacology guidelines. / Psychopharmacol 2000; 14: 3-20. 22. Gill D, Hatcher S. Antidepressants for depression in medical illness (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. 23. Goldberg D, Bridges K. Screening for psychiatric illness in general practice: the general practitioner versus the screening questionnaire. J R Coll Gen Pract 1987; 37: 15-18. 24. Whooley MA, Avins AL, Miranda J et al. Case finding instruments for depression. Two questions are as good as many. / Gen Intern Med 1997; 12: 439-45. 25. Van Weel-Baumgarten EM, Van Den Bosch WJ, Van Den Hoogen HJ et al. The validity of the diagnosis of depression in general practice: is using criteria for

319

diagnosis as a routine the answer? Br J Gen Pract 2000; 50: 284-7. 26. Barrett JE, Williams JW, Oxman TE et al. Treatment of dysthymia and minor depression in primary care. J Fam Practice 2001; 50: 405-12. 27. Vining RLM. Assessing risk of suicide. BMJ 1995; 310: 126-7. 28. Geddes J, Butler R. Depressive disorders: what are the effects of treatments? Exercise. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org 29. Ernst E, Rand JI, Stevinson C. Complementary therapies for depression: an overview. Arch Gen Psychiatry 1998; 55: 1026-32. 30. Schulberg H, Katon W, Simon G et al. Best clinical practice: guidelines for managing major depression in primary care. J Clin Psychiatry 1999; 60 (Suppl. 7). 31. Anderson IM, Tomenson BM. Treatment discontinuation with selective serotonin re-uptake inhibitors compared with tricyclic antidepressants: a meta-analysis. BMJ 1995; 310: 1433-8. 32. Stewart A. Choosing an anti-depressant: effectiveness based pharmacoeconomics. / Affect Disorders 1998; 48: 125-33. 33. MacDonald TM, McMahon AD, Reid 1C et al. Antidepressant drug use in primary care: a record linkage study in Tayside, Scotland. BMJ 1996; 313: 860-1. 34. Philipp M, Kohnen R, Hiller KO. Hypericum extract versus imipramine or placebo in patients with moderate depression: randomised multicentre study of treatment for eight weeks. BMJ 1999; 319: 1534-9. 35. Woelk H for the Remotiv/Imipramine Study Group. Comparison of St John's wort and imipramine for treating depression: randomised controlled trial. BMJ 2000; 321: 536-9. 36. Breckenbridge A. Important interactions between St John's wort (Hypericum perforata) preparations and prescribed medicines. Committee for Safety of Medicines, 29 Feb 2000. 37. CMO's Urgent Communication CEM/CMO/2000/04. 38. Ward E, King M, Lloyd M et al. Randomised controlled trial of non-directive counselling, cognitive-behaviour therapy, and usual general practitioner care for patients with depression. 1: Clinical effectiveness. BMJ 2000; 321: 1383-8. 39. Bower P, Byford S, Sibbald B et al. Randomised controlled trial of non-directive counselling, cognitive-behaviour therapy, and usual general practitioner care for patients with depression. II Cost effectiveness. BMJ 2000; 321: 1389-92. 40. Gloaguen V, Cottraux J, Cucherat M et al. A meta-analysis of the effects of cognitive therapy in depressed patients. / Affect Disorders 1998; 49: 59-72. 41. Chilvers C, Dewey M, Fielding K et al. Antidepressant drugs and generic counselling for treatment of major depression in primary care: randomised trial with patient preference arms. BMJ 2001; 322: 772-5. 42. Keller MB, McCullough JP, Klein DN et al. A comparison of nefazodone, the cognitive behavioural-analysis system of psychotherapy, and their combination for the treatment of chronic depression. New Engl J Med 2000; 342: 1462-70. 43. Mynors-Wallis LM, Gath DH, Day A et al. Randomised controlled trial of problem solving treatment,

320

44.

45. 46. 47. 48.

49. 50.

51.

52.

53.

PSYCHIATRIC PROBLEMS

antidepressant medication, and combined treatment for major depression in primary care. BMJ 2000; 320: 26-30. Department of Health. Treatment choice in psychological therapies and counselling: evidence based clinical practice guideline. London: DoH, February 2001. Online. Available: www.doh.gov.uk/mentalhealth/treatmentguideline Haddad P, Lejoyeux M, Young A. Antidepressant discontinuation reactions. BMJ 1998; 316: 1105-6. Angst J. A regular review of the long-term follow-up of depression. BMJ 1997; 315: 1143-6. Cox J, Holden J (Eds). Use and misuse of the Edinburgh Depression Scale. London: The Royal College of Psychiatrists, 1994. Holden JM, Sagovsky R, Cox JL. Counselling in a general practice setting: controlled study of health visitor intervention in treatment of postnatal depression. BMJ 1989; 298: 223-60. Nicholls KR, Cox JL. Lofepramine and breast feeding (letter). Psychol Bull 1996; 20: 309. Review: individual applied relaxation and cognitive behavioural therapy are effective psychological treatments for generalised anxiety disorder. Evidence-Based Mental Health 2000; 3 (3). Fisher PL, Durham RC. Recovery rates in generalized anxiety disorder following psychological therapy: an analysis of clinically significant change in the STAI-T across outcome studies since 1990. Psychol Med 1999; 29: 1425-34. Bisson J. Post-traumatic stress disorder: What are the effects of preventive psychological interventions? Clinical Evidence 2001; Issue 5. London: BMJ Publishing Group, 2001. Brady K, Pearlstein T, Asnis GM et al. Efficacy and safety of sertraline treatment of post-traumatic stress

54. 55.

56.

57.

58.

59.

60. 61.

disorder: a randomised controlled trial. JAMA 2000; 283: 1837-44. Bisson J. Post-traumatic stress disorder. What are the effects of psychological treatments? Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Gould RA, Otto MW, Pollack MH, Yap L. Cognitive behavioural and pharmacological treatment of generalised anxiety disorder: a preliminary meta-analysis. Behaviour Ther 1997, 28 (2): 285-305. Gale C, Oakley-Browne M. Generalised anxiety disorder: What are the effects of cognitive therapy? Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org Gale C, Oakley-Browne M. Generalised anxiety disorder: what are the effects of drug treatments? Beta-blockers. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org Gale C, Oakley-Browne M. Generalised anxiety disorder: what are the effects of drug treatments? Anti-depressants. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org Meibach RC, Dunner D, Wilson LG et al. Comparative efficacy of propranolol, chlordiazepoxide, and placebo in the treatment of anxiety: a double-blind trial. / Clin Psychiatry 1987; 48: 355-8. Shaw J. Assessing the risk of violence in patients. BMJ 2000; 320: 1088-9. Speckens A, Van Hemert A, Spinhoven P et al. Cognitive behavioural therapy for medically unexplained physical symptoms: a randomized controlled trial. BM/ 1995; 311: 1328-32.

17

CHAPTER CONTENTS Alcohol problems

321

Benzodiazepine withdrawal Opiate misuse

324

Substance abuse

325

Other substances 329 Cannabis 329 Stimulants: amphetamines, cocaine and ecstasy 330 Barbiturate withdrawal 330 Chlormethiazole 330 Volatile substance abuse 330 Smoking cessation 331 Drug treatment to aid smoking cessation 332 Behavioural support 332 Pregnant smokers 332 References 333

ALCOHOL PROBLEMS Guideline: National Health Committee (New Zealand). Guidelines for recognising, assessing and treating alcohol and cannabis abuse in primary care. July 1999. Online. Available: www.nzgg.org.nz/library.cfm

• Twenty-eight per cent of men and 11% of women in Britain drink more than the limits associated with minimal risk1 (Table 17.1). • In 1995 the Department of Health (England and Wales) revised this advice, advocating: (a) limits of up to 4 units per day for men and 3 for women; (b) a daily tally rather than a weekly one; Table 17.1 per week2

Men Women

Health risk from alcohol in units of alcohol

Minimal risk (no. units)

Moderate risk (no. units)

High risk (no. units)

<22 <15

22-50 15-35

>50 >35

1 unit = 10g ethanol =1/2pint of beer, a single measure of spirits or a glass of sherry. 1 bottle of sherry = 15 units, 1 bottle of spirits (75 cl) = 30 units. Note: the number of units in a drink may be calculated by the formula:

Thus, a 440 ml can of extra strong beer is 4 units, a glass of 12% wine is 1.5 units and a bottle is 9 units.3 321

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(c) that these maximum sensible limits should not be drunk every day. • The UK Royal Colleges have supported the second and third points, but continue to recommend daily limits of up to 3 units for men and 2 for women.4 • The New Zealand guidelines recognize the danger of binge drinking and define that as >6 units for men, >4 for women, on one occasion. Drinking more than 6 units a day more than doubles the mortality over non-drinkers.5

* Ask the patient to keep a drink diary for the next week to confirm the amount drunk. * Examine for hepatosplenomegaly and signs of cirrhosis. * Take blood for: (a) a full blood count (for macrocytosis); (b) liver function tests, including gammaglutamyl transferase (GGT). Note that the GGT is raised in only one-third of those with an alcohol problem, and that a raised level is not specific for alcohol abuse.7 However, a raised MCV and a raised GGT detects 75% of problem drinkers.8

Detection Problem drinking is so common that the regular screening of all teenagers and adults is worthwhile. The New Zealand guidelines recommend a 3-yearly screening for everyone over age 14. * Ask:

1. How often do you have a drink containing alcohol? Score 1 for once a month or less and 4 for 4 or more times a week; 2. How many drinks containing alcohol do you have on a typical day when you are drinking? Score 1 for 3 or 4 drinks and 4 for 10 or more a day;

3. How often do you have 6 or more drinks on one occasion? Score 1 for less than monthly and 4 for daily or almost daily. This is the Audit-C questionnaire. A score of 5 for a man is 78% sensitive and 75% specific for problem drinking.6 For a woman it is only 50% sensitive (although 93% specific) and a score of 4 or more should prompt further questioning. Note: The CAGE questionnaire appears to be the least useful of all the screening instruments in current use.6 If the screening test is positive, take a full history of the patient's alcohol use and its consequences. * Consider alcohol as a cause of 'black-outs', accidents, obesity, pancreatitis, dyspepsia, impotence, anxiety, depression, insomnia, a poor employment record, or a criminal record. * Be aware of risk factors, such as employment in the licensing trade, a family history of alcohol problems, a lack of social support.

Management of patients drinking excessively but not dependent ('problem drinkers') * This group manifests the greatest number of alcohol-related problems because of the large numbers at risk and is therefore the most costeffective group to treat.1 * Brief intervention by a GP can lead to a 24% reduction in drinking at follow-up 6-12 months later.9 * Examine and investigate as above. * Explain the dangers of excess drinking: liver, cardiovascular, cerebral damage, peripheral neuropathy and psychological disorders (depression, anxiety and dependence). * Check that the drinking is not secondary to some other disorder, e.g. anxiety, phobia, depression, or insomnia. * Driving. Assess, in a patient who drives, whether the drinking is putting other people at risk (see p. 9). * Occupation. Assess whether the patient's job means that other people are being put at risk. * Agree on a goal; either abstinence or low risk drinking. * Give a booklet or the print-out from the WHO guide to mental health in primary care (www.whoguidemhpcuk.org). * Follow up after 1 month, then 3-monthly for as long as the patient seems to be at risk. * Ask the patient to keep a drink diary for 1 week before each appointment.

ALCOHOL PROBLEMS

Management of alcohol-dependent drinkers * Examine and investigate as above. Severely disordered liver function may require referral to a hepatologist. * Refer to the community alcohol team patients with the following characteristics: (a) no support at home. AA may, however, be able to provide a rota of sitters; (b) multiple drug problems, including benzodiazepine dependence; (c) other psychiatric problems, especially depression with suicidal ideas; (d) physical illness, e.g. jaundice or pancreatitis; or Wernicke's or Korsakoff's syndromes; (e) a previous history of fits or delerium tremens; (f) severe dependence; or (g) previous failed attempts at abstinence. * Consider referring to the community alcohol team patients who do not come into the above categories but who would benefit from the specialist care or individual or group counselling that they can provide. * Driving. Warn the patient that persistent alcohol misuse or dependency must be reported to the DVLA and that revocation of the licence for a minimum of 6 months is likely10 (see p. 9). * Occupation. Assess whether the patient's job means that other people are being put at risk. * Begin with the steps outlined above, but with abstinence as the goal unless the patient refuses. * Involve family, friends and organizations to provide support. Family members who enrol in Al-Anon facilitation therapy can succeed in getting previously unmotivated drinkers into treatment.11 Evidence from randomized controlled trials (RCTs) supports the use of the 12-Step Facilitation Programme of A A.12 * Identify others who will attempt to sabotage the patient's attempts at abstinence because of their own drinking habits.

Home management of potential withdrawal symptoms * The dangers of fits are real. Untreated, delerium tremens has a mortality of 20%.13

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* The risk of delerium tremens is significant in a patient who has been drinking 12 units a day or more. 'DTs' may occur after consumption as low as 8 units a day and as late as 72 hours after stopping drinking.14 Long-acting benzodiazepines are the drugs of choice;15 chlormethiazole has greater addiction potential. * With the exception of those who have found from experience that their withdrawal symptoms are mild, sedatives should be used in adequate doses. * Ensure that you, as physician, have experience in the management of alcohol withdrawal or discuss the plan with a colleague who has. * Advise the patient to take 1 week off work and stay at home. * Obtain the patient's agreement not to take alcohol while on sedative drugs. * Enlist another person to give the patient the drugs; or arrange for the pharmacist to dispense daily. * See daily at first to assess the physical condition and ensure compliance. Tailor the benzodiazepine dose to the patient. A starring point would be, for diazepam:16 1. 20 mg q.d.s. for 2 days; then 2. 15 mg q.d.s. for 2 days; then 3. 10 mg q.d.s. for 2 days; then 4. 10 mg t.d.s. for 2 days; then 5. 5 mg q.d.s. for 2 days; then 6. 5 mg b.d. for 2 days then stop. * Increase the dose if tremor is still present, especially in the first 3 days. Reduce it if the patient is over sedated. * Use half-doses of the above if the patient is small (e.g. <11 stone) or if the alcohol intake was <100 units per week. * Refuse requests for further supplies of diazepam, which would merely substitute one addiction for another. * Give thiamine 50 mg b.d. orally for 1 month. Parenteral administration of high-potency vitamins B and C may be given where there are facilities for resuscitation but not at home because of the danger of anaphylaxis. * Admit under the physicians any patient who convulses or becomes confused, agitated, paranoid or hallucinates or who develops other new neurological signs.

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SUBSTANCE ABUSE

Long-term management * Follow up regularly. * Agree on a contract with a clear goal; this should be abstinence for patients who have needed help with withdrawal. * Look again for any underlying psychiatric disorder, especially depression. Do not attempt this assessment until at least 2 weeks after the patient has stopped drinking. * Attend again to the patient's social situation. Homeless patients do better in hostels catering specially for those with alcohol problems and, indeed, can be successfully detoxified there.17 * Consider (especially in the period immediately after detoxification): (a) acamprosate: to reduce craving. Abstinence after 1 year can be increased from 7% (with placebo) to 18% (NNT = 9).18 (b) disulfimm: It can reduce the number of days on which drinking takes place but the evidence that it promotes abstinence is minimal.19 It is most effective if there is a partner or friend who can give it to the patient every day.20 It may also be useful to help patients over stressful periods later. They must be clear that it is dangerous to drink from 12 hours before the first dose until 1 week after the last dose. (c) Naltrexone: can reduce craving and reduces the enjoyment of alcohol. It reduces heavy drinking but does not necessarily lead to abstinence.21 Self-help groups: Alcoholics Anonymous; see telephone directory or www.alcoholics-anonymous.org.uk AI-Anon Family Groups UK and Eire 24-hour Helpline 020 7403 0888, Drinkline (National Alcohol 24-hour Helpline) 0700 0780977.

BENZODIAZEPINE WITHDRAWAL Guidelines: UK Department of Health. Drug misuse and dependence - guidelines on clinical management. London: The Stationary Office, 1999. Online. Available: www.doh.gov.uk/drugdep.htm

Lader M, Russell J. Guidelines for the prevention and treatment of benzodiazepine dependence: summary of a report from the Mental Health Foundation. Addiction 1993;88:1707-8.

Every practice needs a policy to avoid initiating dependence in patients given benzodiazepines. For example: (a) Benzodiazepines will only be used for the short-term relief of severe anxiety or insomnia, or for specific medical emergencies such as alcohol detoxification or the management of convulsions. (b) Short-acting compounds (such as lorazepam) will not be given for daytime use. (c) No repeat prescriptions will be given except to those already established as long-term users. (d) No patient not already on benzodiazepines will be prescribed a course of more than 4 weeks. (e) Current benzodiazepine users will be identified and invited to attend to discuss withdrawal. The invitation will be repeated annually until the drug is withdrawn. An exception to this might be the older patient maintained long-term symptomfree on the same dose. (f) An annual audit of the number of benzodiazepine users will be conducted to monitor success and to learn from failures.

Management of withdrawal • Withdrawal symptoms can be distinguished from a return of anxiety and include: (a) disordered perceptions: feelings of unreality, increased sensory perception, or a sensation of movement; (b) serious psychiatric and neurological adverse effects: convulsions or acute psychosis. • Symptoms typically emerge in the first week after stopping or reducing the dose. Occasionally they occur in patients on a stable dose, and are temporarily abolished by an increase in the dose. They usually last for up to 3 months but may last for over a year. • Withdrawal symptoms may be seen in patients who have been on benzodiazepines for as little as 3 weeks and they occur in up to almost half of patients who have taken them for over 3 months.22

OPIATE MISUSE 325

* Two-thirds will be able to stop with some disturbance of sleep but without the true withdrawal syndrome. They may, however, need help in finding a different solution to the problem for which they were taking the benzodiazepine. * Offer withdrawal with explanation to all patients on long-term benzodiazepines who have become dependent inadvertently following therapeutic use. Record the discussion in the notes. * Refer patients who are misusing other drugs or alcohol, as well as benzodiazepines, to the specialist services. Concurrent withdrawal of more than one drug is not recommended. * Refer patients requiring very high doses or who are injecting the drugs. A number of withdrawal regimens have been used. There is a spectrum of possibilities with the two proposed below at opposite ends of the spectrum. The two principles behind all regimens are to use a long-acting benzodiazepine and to reduce slowly, or very slowly. Rapid withdrawal

For patients who give no indication that a withdrawal syndrome is likely. * Reduce the benzodiazepine dose by a quarter of the original dose every 2 weeks and see the patient each time. If true withdrawal symptoms develop then change to a slow withdrawal.

* Be prepared to renegotiate the plan if the patient develops severe withdrawal effects. Keep patients on each dose long enough for them to settle before the next reduction. * If the patient requests a slower rate of reduction because of psychological rather than physical dependence, it is better to keep to the agreed plan and intensify support. * Be prepared for the emergence of anxiety, depression or insomnia, and manage accordingly. Patients on long-term benzodiazepines often have inappropriate reactions to difficulties in their lives. Withdrawal of the drug must be accompanied by re-education by the GP or referral, for instance, to a benzodiazepine-withdrawal support group. * Symptom control. For patients not willing to tolerate the symptoms of withdrawal, consider using other drugs temporarily: (a) promethazine 25 mg at night for insomnia; (b) propranolol 10 to 40 mg t.d.s. for somatic symptoms; (c) tricyclic antidepressants, e.g. amitriptyline 25 to 75 mg daily, for depression and anxiety. This should be started 4 weeks before reducing the benzodiazepine in those in whom its need can be predicted. * Avoid using buspirone or major tranquillizers. They may make withdrawal worse. Do not use zopiclone or chlormethiazole; they have crossdependency with benzodiazepines.

Slow withdrawal

For patients in whom a withdrawal syndrome is likely (because of evidence of tolerance or previous symptoms of withdrawal). * Change patients on short-acting to long-acting benzodiazepines, e.g. diazepam. For equivalent doses see BNF (chapter 4.1), e.g. lorazepam 2 mg is equivalent to diazepam 20 mg. If the changeover proves difficult, do it in stages, e.g. changing 1 mg lorazepam to l0 mg diazepam at a time. * Start with a plan agreed with the patient, e.g. reducing the daily dose by1/8per fortnight, with smaller reductions in the final stages. Expect to take several months or even a year. If the patient has been obtaining the drug illegally, start with a dose considerably below the amount claimed.

Self-help groups: Tranx (Tranquillizer Recovery And New Existence); address available from the local Drug Information Service. CITA (Council for Involuntary Tranquilliser Addiction) tel. 0151 9490102.

OPIATE MISUSE Guideline: The UK Department of Health. Drug misuse and dependence: guidelines on clinical management. London: The Stationary Office, 1999. Online. Available: www.doh.gov.uk/drugdep.htm

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SUBSTANCE ABUSE

• The UK Department of Health guidelines stress that drug misusers have the same right as other patients to medical services. The UK General Medical Council has ruled that it is unethical for a doctor to withhold treatment on the basis of a moral judgement that the patient's activities or lifestyle might have contributed to the condition for which treatment was being sought. • The guidelines stress the importance of: (a) shared care, primarily shared between primary and secondary care; (b) a multidisciplinary approach. • Notification. Doctors are expected to report, on the database reporting forms, treatment demands of drug misusers to the Regional Drug Misuse Database (DMD). This applies not only to opioids and cocaine but also to any other drug that is being misused.

The patient requesting opiates urgently, or the known opiate user with a poor prognosis * Do not prescribe opiates unless you are threatened with violence, and then contact the police immediately it is safe to do so. * Harm minimization. Ensure that the user knows about the local needle exchange scheme and the risk of HIV and hepatitis B and C. Explain that infection can be acquired from sharing filters or the same water supply as well as from sharing needles and syringes. Give a copy of Harm minimisation: advice about safer drug use available on www.whoguidemhpcuk.org (choose 'information and self-help leaflets'). * Cleaning equipment. Be sure that the user knows to: (a) draw up thin, undiluted household bleach and squirt it down the sink, twice; (b) draw up clean cold water and squirt it down the sink, three times. Explain that cleaning equipment is not as good as using sterile needles and syringes. Cleaning reduces HIV risk but may not protect against hepatitis C. * Discuss safe injecting techniques: (a) never inject alone;

(b) inject with the blood flow; (c) rotate sites and avoid infected areas and swollen limbs; (d) avoid the neck, groin, breast, feet and hands; (e) mix powders with sterile water and filter the solution before injecting; (f) dispose of equipment safely, e.g. place the needle inside the syringe and put the syringe in a drinks can. * Safe sex. Check that the user is using condoms. Drug users are a high-risk group for HIV, and may acquire it sexually. * Offer immunization against hepatitis B (see p. 23). * Offer HIV antibody testing with counselling (see p. 46). * Specialist help. Urge the patient to attend the Drug Dependency Clinic. * Admission. Arrange urgent admission for the few patients in whom it is important, i.e. the woman in late pregnancy and those physically ill (e.g. those with multiple abscesses or septicaemia). If it proves impossible to admit a pregnant addict it is better to give her 3 days methadone and arrange an urgent appointment than to risk her losing her baby from withdrawal.

Symptom control * If only mild withdrawal symptoms are predicted, use promethazine 25 mg up to three times a day if needed, for 3 days. * If more severe symptoms are likely, offer a 4-day course of: (a) co-phenotrope (Lomotil) 2 tablets q.d.s. to prevent abdominal cramps; and (b) a neuroleptic, e.g. chlorpromazine 50 mg t.d.s. to prevent agitation; and (c) metoclopramide for nausea and vomiting; and (d) ibuprofen for muscle pains.

Seeing a patient where violence is a possibility * Try not to see the patient alone. * Ask staff to check on you at regular intervals during the consultation.

OPIATE MISUSE

* If visiting, consider having a mobile phone 'open' to the surgery so that the consultation can be monitored. * Report criminal behaviour to the police. It makes similar behaviour by others less likely.

Patients seeking help, rather than an immediate prescription * Care should be shared between primary and secondary care. How much takes place in primary care will depend on the experience available in the primary health-care team and the degree of access to a specialist unit. * Refer to the specialist services patients: (a) with other serious mental or physical health problems; or (b) misusing more than one drug; or (c) with a serious forensic history; or (d) needing specialist psychological interventions or help with housing or employment; or (e) needing a residential rehabilitation programme; or (f) not responding to an oral substitute regimen. The following guidelines are appropriate where a GP delivers the bulk of the medical care. First appointment

* Discover what has prompted the patient to present at this time and what he or she expects from the consultation. Assess their motivation to change. * Assess the patient's psychological and physical state; look especially for needle marks. If female, establish whether she is pregnant. Refer patients with severe mental health problems to the mental health team. * Assess the social situation, including contacts with other drug users. Establish whether there is a child-care issue, whether a court case is pending and whether the patient is working and housed. * Assess the past and current drug situation. Check which other drugs are being used, including

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alcohol. Refer anyone likely to need more than 80 mg of methadone (i.e. on more than 1 g daily of injected street heroin or 1.5g if smoked, or equivalents of diconal, dihydrocodeine and other opiates). The UK guideline contains a list of equivalents. * Assess the need for HIV and hepatitis B and C screening. If the patient chooses to be tested, postpone the test until he or she is stabilized on methadone lest a positive result sabotage the detoxification. * Ask about previous attempts at treatment. If the patient is new to the practice, contact the previous GP. * Send a urine sample to the laboratory for a full drug screen. It should be passed with a witness present. Give no drugs until the urine result is available. Almost all drugs of misuse are detectable 24 hours after ingestion. Heroin is detectable 48 hours after and methadone 7-9 days after. * Take blood, if the patient gives informed consent, for creatinine, LFTs, hepatitis B and C serology and HIV antibodies. * Notify the Regional Drug Misuse Database on the appropriate form (address in the BNF). The data is anonymised by the Regional Centre. Notification is required at first presentation, even if previously notified by another doctor, and at representation after a gap of at least 6 months. * Avoid all other psychoactive drugs. * Advise the patient that it is his or her responsibility to notify the DVLA, if holding a driving licence. * Give the patient information about the detoxification (e.g. The detox handbook, available from Drugscope, 32-36 Loman Street, London SE1 OEE, tel. 020 7928 1211; www.drugscope.org.uk (choose 'drugscope publications' then 'drug users and families'). * Keep full records of the discussion.

If the urine sample confirms opiate use, at the next appointment

* Discuss any planned pharmacological treatment with a specialist or a specialized generalist16 before the appointment.

328 SUBSTANCE ABUSE

* Agree a treatment plan with the patient: either detoxification, or maintenance on methadone. A written contract between doctor and patient can be helpful.

Information and help for relatives: ADFAM National, which offers counselling for family and friends of drug users, tel. 020 7928 8900. Families Anonymous, which runs self-help groups for parents of drug users, helpline 020 7498 4680.

Detoxification The options are:

Methadone reduction programme

(a) symptom control with co-phenotrope, chlorpromazine, metoclopramide, ibuprofen or promethazine, as above if only mild to moderate symptoms are predicted; or (b) detoxification on lofexidine if more severe symptoms are predicted but the patient is stable on a low or moderate dose of illicit opiate (see below); or (c) a methadone reduction programme23 (see below).

* Prescribe a single dose initially of oral methadone mixture 1 mg per ml to assess the starting maintenance dose. Do not prescribe tablets; they are likely to be crushed and injected. For a table of equivalents, see the UK guideline (in the box on p. 325). For example, expect to need to give 20 mg methadone a day as a single dose to someone injecting 10 mg pharmaceutical diamorphine per day. Street heroin is too variable to give equivalents; the dose will need to be titrated against symptoms. Start with between l0 mg and 40 mg per day. Death from overdose is a real possibility at higher doses. * Write the prescription in ink on an FP10 (MDA) in England and Wales, GP10 in Scotland. State the form and strength of the preparation, with the dose and the total dose in words and figures with the amount in each instalment and the frequency of instalments. Up to 14 days treatment may be prescribed, to be dispensed daily with two doses on Saturday if the chemist is shut on Sunday. Order three doses for bank holiday weekends. * Specify the pharmacy to be used, and speak personally to the pharmacist about every prescription. Ask the pharmacist to supervise the patient's taking of the drug. After 3 months without difficulties, dispensing may be increased to weekly. * Give no other drugs with abuse potential. * See after 4 hours. Reduce the dose if the patient is oversedated. If seriously oversedated, reverse the effect with naloxone 0.8-2 mg iv repeated every 2 minutes to a maximum of 10 mg and admit. A naloxone infusion is likely to be needed. If moderately undertreated, add 5-10 mg of methadone. If seriously undertreated, add 20-30 mg of methadone.

Note: There is no place for dihydrocodeine, which has to be taken 4 times a day and so cannot be supervised. * Whichever option is chosen, draw up a contract specifying what you will prescribe and that the patient agrees to keep appointments, not to consult other doctors and not to take other drugs. * Discuss the necessary changes in lifestyle: the patient will need to give up all friends who are themselves users. It may be possible to arrange residential rehabilitation for when the patient is drug free. * Recommend Narcotics Anonymous or Release and any other source of support. * Agree who will provide support from the statutory services. This might be the community drug team, community psychiatric nurse, probation officer, social worker or street agency. Patient information: UK National Drugs helpline, tel. 0800 776600. Narcotics Anonymous, tel. 020 7730 0009 for details of local groups. Release helpline tel. 020 7603 8654 for advice and support to drug users and their friends and families. Drugs in School helpline tel. 0808 8000 800.

OTHER SUBSTANCES

* See daily, to check that the dose is right. If further increases are needed, increase the daily dose by up to 10 mg, but no faster than weekly; it takes 5 days from the change in dose for the new plasma level to be reached. Do not go above 80 mg per day unless experienced. * Once the patient is stable (on average after 6 weeks) tailor the planned reduction to the patient's needs. A rapid withdrawal might involve reducing the methadone by 5mg every 3 days; a slow withdrawal would be 5 mg every 2 weeks. Be prepared to hold the dosage level if the patient runs into a crisis. * Be prepared to change the reduction programme to a maintenance programme if that seems necessary to keep the patient in treatment. * If the patient omits more than 5 days methadone, restart the programme but at a lower dose, in case tolerance to methadone has been lost. * If the patient claims that a prescription has been lost or stolen, cover the time to the next prescription with lofexidine, not a duplicate prescription for methadone. * Organize random supervised urine tests. The laboratory will be able to tell if drugs other than methadone are being used. The community drug team may be willing to undertake this for the GP. If illicit drugs are found, renegotiate the contract. Note: Most detoxifications do not run smoothly. Be prepared to be flexible unless you sense that the patient has no intention of cooperating.

Detoxification on lofexidine • Lofexidine will reduce the symptoms of opiate withdrawal but will not abolish the craving for the drug. It suppresses noradrenergic neuronal hyperactivity during opiate withdrawal.24 It can be as effective as methadone in achieving successful withdrawal.16 * Give 200 ug twice a day, and increase by 200 ug twice a day until symptoms are controlled, up to a maximum of 2.4 mg per day. Continue for 7-10 days, then tail off over a further 2-4 days.

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* Check the blood pressure at baseline and again daily for the first 3 days. Stop lofexidine if there is significant hypotension.

Methadone maintenance * Methadone maintenance has been shown to be more effective than withdrawal in keeping patients in treatment25 and to result in lower heroin use rates.26 * Higher doses (60-120 mg/day) are associated with better outcomes than lower doses.27 * Methadone maintenance has the potential to reduce crime and reduce HIV transmission, as well as to reduce illicit opiate use; but it should be given as part of a structured programme.28 * Start as for methadone reduction. * See 2-4 weekly once stable. * Perform random urine tests for methadone and other drugs. * Look again at psychological and social issues once the patient is stable. * Review the decision not to reduce the dose, roughly 3-monthly.

OTHER SUBSTANCES CANNABIS Guideline: National Health Committee (New Zealand). Guidelines for recognising, assessing and treating alcohol and cannabis abuse in primary care. July 1999. Online. Available: www.nzgg.org.nz/library.cfm

• 4% of the US population is dependent on cannabis at some point in their lives. This is higher than any other drug of misuse.29 • Of those who use cannabis once, 9% become dependent. • Only 17% of long-term users who try to quit are abstinent 1 year after treatment.30 • Ask about cannabis use in younger patients who are also using alcohol and tobacco.

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SUBSTANCE ABUSE

* Explain the dangers: subtle specific cognitive defects (memory loss, poor concentration), chronic bronchitis and carcinoma of the lung, precipitation of latent psychosis and possible infertility, as well as deterioration in relationships and performance at work. * Explain that withdrawal symptoms are likely to be mild and short-lived: insomnia, sweating, anxiety, tremor and paranoia. * Agree a goal along the line of the approach taken with problem drinkers and arrange to see the patient again. Brief interventions of this sort can be as effective as extensive ones.31 STIMULANTS: AMPHETAMINES, COCAINE AND ECSTASY * Craving for stimulants is real. Withdrawal symptoms mainly take the form of psychiatric reactions, including depression, anxiety, agitation and acute psychosis. * Advise users to discontinue the drugs abruptly. Do not give substitute drugs. * Warn patients that they will go through a period of prolonged sleep, with subsequent lethargy and depression. * Arrange for counselling and social support.

Amphetamine

BARBITURATE WITHDRAWAL This is more dangerous than heroin, and should be done as an inpatient. If that is impossible, a reducing course of phenobarbitone over 8 days gives some protection from convulsions. Roughly, l00 mg of short-acting barbiturate is equivalent to 30 mg of phenobarbitone. Do not give more than 300 mg of phenobarbitone a day. CHLORMETHIAZOLE Withdrawal from high doses (i.e. more than 4g a day) should be done in hospital. There is a real risk of acute psychosis, and phenobarbitone and diazepam give no protection against this. VOLATILE SUBSTANCE ABUSE • One in ten secondary school children have sniffed solvents; 2% for a period of weeks or months and a further 1% long-term. • Most sniffing is out of curiosity, and stops spontaneously. The sniffing that is most likely to continue is when it is used as an escape from severe family or personal problems. • Sniffing accounts for 1 in 60 of all teenage deaths. This is an improvement on the rates in the 1980s.32 • Substances can be stopped abruptly without danger.

* Refer long-term heavy users of amphetamine (>1 g daily for >3 days a week for >3 months) to the appropriate psychiatrist for an opinion on maintenance with dexamphetamine. Their depression following withdrawal can be severe and unremitting. Dexamphetamine is not licensed for this indication and its use is beyond the expertise of a GP.

Explain the dangers: (a) cerebral damage; (b) lung damage; (c) liver damage; (d) cardiac arrhythmias and sudden death; (e) accidental injury while intoxicated.

Cocaine

Sniffing due to underlying problems

* Craving for cocaine can be reduced with a TCA. Start it several weeks before the patient stops cocaine. * Refer cocaine users to Cocaine Anonymous (020 7284 1123 or www.cauk.org.uk).

* Advise about 'safer sniffing', e.g. don't sniff alone, don't put a bag over the head, don't use solvents other than glue as they can be fatal, don't sniff in dangerous places, e.g. rooftops. Stress that sniffing is never safe.

Experimentation sniffing

SMOKING CESSATION

* Mobilize all possible help for the underlying problems. Patient information and groups: A Parent's Guide to Drugs and Alcohol available from the National Drugs Helpline, tel. 0800 776600. Re-Solv, The Society for the Prevention of Solvent and Volatile Substance Abuse, 30a High Street, Stone, Staffordshire ST15 8AW, tel. 01785 817885; www.re-solv.org

SMOKING CESSATION Guideline: Smoking cessation guidelines for health professionals: an update. Thorax 2000; 55: 987-99. Online. Available: http://thorax.bmjjournals.com/ cgi/content/full/55/12/987

• Smoking remains the largest preventable cause of ill health in the UK, causing over 120,000 deaths per year, around one in five of all deaths.33 • More than one in four deaths between the ages of 35 and 65 are caused by smoking (1:3 for men andl: 5 for women).33 • Smoking is estimated to be responsible for 90% of lung cancer deaths, the commonest cancer in the UK (21% of all cancer deaths).34,35 • Smoking in pregnancy causes adverse fetal outcomes, such as an increased risk of miscarriage, low birth weight and perinatal death. The risk of sudden infant death syndrome or 'cot-death' is significantly increased by exposure to tobacco smoke.36 • Smoking cessation greatly reduces the health risks associated with smoking. The risks from smoking decline immediately on cessation until after 10-15 years of abstinence, depending on the number of years as a smoker, the risk of death returns to almost that of a life-long non-smoker.37 • In 1998, in England, 27% of adults smoked cigarettes, of whom 69% wanted to give up.38 • There is no evidence that acupuncture39 or hypnosis40 are effective in assisting patients to stop smoking. • Brief advice by GPs, drug treatment such as nicotine replacement therapy (NRT), bupropion

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(Zyban) and behavioural support have all been shown to increase the number of people successfully stopping smoking.41 * Brief advice (up to 5 minutes) from a GP leads to an increase in the number who have stopped smoking (in addition to those who would have stopped anyway) at 6 months of between l-3%.42 Approximately 40% of smokers make some form of attempt to quit in response to GP advice.43,44 The following features are essential components of a smoking cessation programme in primary care - ask, advise, assist, arrange:41 * ASK patients about smoking on a regular basis (at least once a year) when they visit the surgery and assess their interest in stopping. Record smoking status and their interest in stopping in the notes. 1. 'Do you smoke?' 2. 'How do you feel about your smoking?' 3. 'Have you ever tried to stop?' 4. 'Are you interested in stopping now?' * ADVISE all smokers of the value of stopping (at any age) and the risks to health of continuing to smoke. The advice should be clear, firm and personalized, whilst remaining non-judgemental. (a) Do not limit this advice to patients with smoking-related diseases. (b) Try and link the advice to the reasons for the patient consulting and/or relevant to the individual. (c) Reassure patients who have failed on a previous attempt that most do so before finally succeeding.45 * ASSIST the smoker if they would like to stop and are well motivated by providing further help, advice and referral. 1. Advise them to set a date to stop and stop completely on that date. 2. Offer prescription for NRT or bupropion for smokers of more than 10-15 cigarettes per day (see drug treatment, below). 3. Refer for behavioural support at specialist clinic; or 4. Refer to intermediate service (at primary care level) provided by nurses or other health professionals specifically trained and employed to provide smoking cessation support.

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SUBSTANCE ABUSE

* ARRANGE follow up - although smokers will be followed-up with the above support services you may also wish to review the patient yourself 1-2 weeks after their quit date. DRUG TREATMENT TO AID SMOKING CESSATION

Nicotine replacement therapy (NRT) • NRT increases quit rates by 1.5- to 2-fold for those smoking more than ten cigarettes per day.46 • No form of NRT seems to be more effective than others but smokers given the choice prefer nicotine patches.47-49 • There is evidence of long-term use and dependence with nicotine gum and nasal spray (13-38% using it for 1 year), but there is no evidence of long-term use of patches.50-52 • NRT appears to be safe when given to smokers with cardiovascular disease.53

Bupropion • Bupropion may work by counteracting the effects of nicotine withdrawal by increasing levels of dopamine and noradrenaline in the brain. • Bupropion has been shown to be at least as effective as NRT when used in smokers of more than 15 cigarettes per day who are well motivated to stop and who also receive regular counselling.54,55 • Common side-effects of bupropion include insomnia and dry mouth. Less common sideeffects include seizures which has been reported in 1 in 1000 people taking bupropion 300 mg (modified release). An urgent message from the Chairman of the Committee on Safety of Medicines, 30 May 2001 (CEM/CMO/2001/7) included the following information: Bupropion is contraindicated in:

(a) history of seizure; (b) diagnosis of bulimia or anorexia nervosa;

(c) known CNS tumour; (d) alcohol or benzodiazepine withdrawal. Bupropion should not be prescribed in the following unless there is compelling clinical justification (where benefit outweighs increased risk of seizure):

(a) high alcohol intake; (b) recent head injury; (c) treatment with: antidepressant, antipsychotics, theophylline, systemic steroids, antimalarials, tramadol, quinolones, sedating antihistamines, hypoglycaemics, insulin, stimulants and anorectic products. A provisional list of those drugs that might interact with bupropion has been published56 and is available on http:// www.pharmj.com/pdf/zyban.pdf BEHAVIOURAL SUPPORT • Behavioural support further increases the chance of quitting, enabling an additional 1 in 20 smokers to quit for 6 months or more.57 • There is no evidence that individual counselling is more effective than group counselling or vice versa.57 • There is currently no evidence that more intensive counselling, in terms of frequency and duration of contact, is more effective.57 • Behavioural support for smokers provided by nurses specifically employed to provide for this purpose has been shown to be effective58 whereas when given as part of their general duties it has not been shown to be effective in aiding smoking cessation.58,59 • Smokers' helplines and access to telephone counselling, on their own or in addition to written materials, have been shown to be effective.60 PREGNANT SMOKERS • For pregnant smokers, referral for support by someone specifically trained and employed for that purpose encourages 1 in 15 to stop smoking for the remainder of the pregnancy (when they would not otherwise have done so).61,62 • Support by midwives as part of their normal duties has not been shown to be effective.63,64 Support for pregnant women should be on

REFERENCES 333

a 1:1 basis, as very few are willing to attend group sessions.65 • Small studies on the effects of NRT on the fetus have not revealed significant problems66,67 and it is likely to be considerably safer than smoking.

Telephone support and leaflets: Quitline tel. 020 7487 3000; NHS Smoking Helpline 0800 169 0 169; or 0800 169 9 169 (pregnant smokers). Leaflets can be ordered free on www.givingupsmoking.co.uk (choose 'publications').

REFERENCES 1. NHS Centre for Reviews and Dissemination, University of York. Brief interventions and alcohol use. Effective Health Care 1993; 7. 2. Department of Health, GMSC, RCGP. Better living, better life. London: DoH, 1993. 3. Webster-Harrison PJ, Barton AG, Barton SM et al. General practitioners' and practice nurses' knowledge of how much patients should and do drink. Br J Gen Pract 2001; 51: 218-20. 4. Gaziano JM, Hennekens C. Royal Colleges' advice on alcohol consumption. BMJ 1995; 311: 3-4. 5. Rehm J, Greenfield TK, Rogers JD. Average volume of alcohol consumption, patterns of drinking and all-cause mortality: results from the US National Alcohol Survey. Am J Epidemiol 2001; 153: 64-71. 6. Aertgeerts B, Buntinx F, Ansoms S et al. Screening properties of questionnaires and laboratory tests for the detection of alcohol abuse or dependence in a general practice population. Br J Gen Pract 2001; 51: 206-17. 7. Bernadt MW et al. Comparison of questionnaire and laboratory tests in the detection of excessive drinking and alcoholism. Lancet 1982; i: 325-8. 8. Ashworth M, Gerada C. Addiction and dependence II: alcohol. BMJ 1997; 315: 358-60. 9. Anderson P. Effectiveness of general practice interventions for patients with harmful alcohol consumption. Br J Gen Pract 1993; 43: 386-9. 10. DVLA. At-a-glance guide to the current medical standards of fitness to drive. Swansea: DVLA, 2001. Online. Available: www.dvla.gov.uk/at_a_glance/content.htm 11. Miller WR, Meyers RJ, Tonigan JS. Engaging the unmotivated in treatment for alcohol problems: a comparison of three strategies for intervention through family members. / Consult Clin Psychol 1999; 67: 688-97. 12. Longabaugh R, Wirtz PW, Zweben A et al. Network support for drinking, Alcoholics Anonymous and long-term matching effects. Addiction 1998; 93: 1313-33. 13. National Health Committee (New Zealand). Guidelines for recognising, assessing and treating alcohol and cannabis abuse in primary care. July 1999. Online. Available: www.nzgg.org.nz/library.cfm 14. Chick J. Delirium tremens. BMJ 1989; 298: 3-4. 15. Mayo-Smith MF et al. Pharmacological management of alcohol withdrawal: a meta-analysis and evidence-based practice guideline. JAMA 1997; 278 (2): 144-51. 16. UK Department of Health. Drug misuse and dependence guidelines on clinical management. London: The Stationary Office, 1999. Online. Available: www.doh.gov.uk/drugdep.htm

17. Haigh R, Hibbert G. Where and when to detoxify single homeless drinkers. BMJ 1990; 301: 848-9. 18. Whitworth AB, Fischer F, Lesch OM et al. Comparison of acamprosate and placebo in long-term treatment of alcohol dependence. Lancet 1996; 347: 1438-42. 19. West SL, Garbutt JC, Carey TS et al. Pharmacotherapy for alcohol dependence. Evidence report number 3. AHCPR publication No. 99-E004. Rockville MD: Agency for Health Care Policy and Research, 1999. 20. Heather N. Disulfiram treatment for alcoholism. BMJ 1989; 299: 471-2. 21. Garbutt JC, West SL, Carey TS. Pharmacological treatment of alcohol dependence. JAMA 1999; 281: 1318-25. 22. Kan CC, Breteler MH, Zitman FG. High prevalence of benzodiazepine dependence in outpatient users, based on the DSM-III-R and ICD-10 criteria. Ada Psychiatr Scand 1997; 96: 85-93. 23. Farrell M et al. Methadone maintenance treatment in opiate dependence: a review. BMJ 1994; 309: 997-1001. 24. Washton AM et al. Opiate withdrawal using lofexidine, a clonidine analogue with fewer side-effects. J Clin Psych 1983; 44: 335-7. 25. Ball JC, Ross A. The effectiveness ofmethadone maintenance treatment: patients, programs, services and outcome. New York: Springer-Verlag, 1991. 26. Sees KL, Delucchi KL, Masson C et al. Methadone maintenance vs 180-day psychosocially enriched detoxification for treatment of opioid dependence. JAMA 2000; 283: 1303-10. 27. Ball JC, Ross A, Jaffe JH. Cocaine and heroin use by methadone maintenance patients. NIDA Res Monogr 1989; 95: 328. 28. Farrell M, Ward J, Mattick R et al. Methadone maintenance treatment in opiate dependence: a review. BMJ 1994; 309: 997-1001. 29. Stephens RS, Roffman RA, Curtin L. Comparison of extended vs brief treatments for marijuana use. / Consult Clin Psychol 2000; 68: 898-908. 30. Stephens RS, Roffman RA, Simpson EE. Treating adult marijuana dependence: a test of the relapse prevention model. / Consult Clin Psychol 1994; 62: 92-9. 31. Stephens RS, Roffman RA, Simpson EE. Treating adult marijuana dependence: a test of the relapse prevention model. / Consult Clin Psychol 1994; 62: 92-9. 32. Field-Smith ME, Taylor JC, Norman CL et al. Trends in deaths associated with abuse of volatile substances 1971-99. London: St George's Hospital Medical School, 2001. Online. Available: www.vsareport.org

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33. Callum C. The smoking epidemic. London: Health Education Authority, 1998. 34. Doll R, Peto R, Wheatley K et al. Mortality in relation to smoking: 40 years' observation on male British doctors. BMJ 1994; 309: 901-11. 35. Department of Health. Our healthier nation: a contract for health, Cm 3852. London: The Stationery Office, 1998. 36. Poswillo, D. Report on the Scientific Committee on Tobacco and Health. London: Health Education Authority, 1998. 37. The Surgeon-General. Reducing the health consequences of smoking: 25 years of progress. Bethesda, MD: Department of Health and Human Services, Public Health Service, Centers for Disease Control Office on Smoking and Health, 1989. 38. Department of Health. Statistics on smoking: England, 1978 onwards. Statistical Bulletin 2000; 17: 1-36. 39. White AR, Rampes H, Ernst E. Acupuncture for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 40. Abbot NC, Stead LF, White AR, Barnes J, Ernst E. Hypnotherapy for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 41. West R, McNeill A, Raw M. Smoking cessation guidelines for health professionals: an update. Thorax 2000; 55: 987-99. 42. Silagy C. Physician advice for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 43. Kreuter MW. How does physician advice influence patient behaviour? Arch Fam Med 2000; 9: 426-33. 44. Russell MAH. Effect of general practitioners' advice against smoking. BMJ 1979; 2: 231-5. 45. Gourlay SG. Double-blind trial of repeated treatment with transdermal nicotine for relapsed smokers. BMJ 1995; 311: 363-6. 46. Silagy C, Mant D, Fowler G, Lancaster T. Nicotine replacement therapy for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 47. Cummings KM, Hyland A, Ockene JK. Use of the nicotine skin patch by smokers in 20 communities in the US. Tobacco Control 1997; 6 (Suppl. 2): S63-70. 48. Shaw JP, Ferry DG, Pethica D et al. Usage patterns of transdermal nicotine when purchased as a non-presciption medicine from pharmacies. Tobacco Control 1998; 7:161-7. 49. West R, Hajek P, Nilsson F et al. Individual differences in preference for and responses to four nicotine replacement products. Psychopharmacology (Berl) 2001; 153: 225-30. 50. Benowitz NL (ed.). Nicotine safety and toxicity. New York: Oxford University Press, 1998. 51. Hughes JR. Dependence potential and abuse liability of nicotine replacement therapies. Biomed Pharmacother 1989; 43:11-17.

52. Blondal T, Gudmundsson L, Olafsdottir I et al. Nicotine nasal spray with nicotine patch for smoking cessation: randomised trial with six year follow up. BMJ 1999; 318: 285-9. 53. Benowitz NL, Gourlay SG. Cardiovascular toxicity of nicotine: implications for nicotine replacement therapy. JAm Coll Cardiol 1997; 29: 1422-31. 54. Hurt RD, Sachs DPL, Glover ED et al. A comparison of sustained-release bupropion & placebo for smoking cessation. New Engl J Med 2001; 337: 1195-202. 55. Jorenby DE, Leischow SJ, Nides MA et al. A controlled trial of sustained-release bupropion, a nicotine patch, or both for smoking cessation. New Engl J Med 1999; 340: 685-91. 56. Cox A, Anton C, Ferner R. Take care with Zyban. The Pharmaceutical Journal 2001; 266: 718-22. 57. Lancaster T, Stead LF. Individual behavioural counselling for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 58. Rice VH, Stead LF. Nursing interventions for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 59. Steptoe A, Doherty S, Rink E et al. Behavioural counselling in general practice for the promotion of healthy behaviour among adults at increased risk of coronary heart disease: randomised trial. BMJ 1999; 319: 943; discussion 947-8. 60. Lancaster T, Stead LF. Self-help interventions for smoking cessation (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 61. Lumley J, Olover S, Waters E. Interventions for promoting smoking cessation during pregnancy (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 62. Melvin CL, Dolan-Mullen P, Windsor RA et al. Recommended cessation counselling for pregnant women who smoke: a review of the evidence. Tobacco Control 2000; 9 (Suppl. 3): III80-4. 63. Hajek P, West R, Lee A et al. Randomised control trial of a midwife-delivered brief smoking cessation intervention in pregnancy. Addiction 2001; 96: 485-94. 64. Wisborg K, Henriksen TB, Secher NJ. A prospective intervention study of stopping smoking in pregnancy in a routine antenatal care setting. Br J Obst Gynaecol 1998; 105: 1171-6. 65. West R. Smoking cessations interventions in pregnancy: guidance to purchasers & providers. London: Health Education Authority, 1994. 66. Lambers DS, Clarke KE. The maternal and foetal phsyiologic effects of nicotine. Semin Perinatal 1996; 20:115-26. 67. Linblad A, Marsal K, Andersson KE. Effect of nicotine on human fetal blood flow. Obstet Gynecol 1988; 72: 371-82.

CHAPTER CONTENTS

The ear 335 Pain in the ear 335 Discharge from the ear 337 Deafness 338 Giddiness 340 Meniere's disease 341 Benign positional vertigo 342 Tinnitus 342 Facial palsy 343 Bell's palsy 343

18 Ear, nose and throat problems

The nose 344 Sinusitis 344 The common cold 345 Rhinitis 345 Nasal polyps 346 Snoring and obstructive sleep apnoea 346 The throat and mouth 347 Sore throat 347 Vincent's angina 349 Infectious mononucleosis 349 Oral thrush 349 Sore throat due to postnasal drip 349 Tonsillectomy 349 Dysphagia 349 Hoarseness 349 Post-laryngectomy 350 The feeling of a lump in the throat 350 Sore mouth 350 Dental problems 351 Halitosis 352 References

352

THE EAR

PAIN IN THE EAR * Examine the external canal and drum. If these are normal then the pain is unlikely to be due to ear disease. * Consider: (a) mumps; (b) cervical lymphadenopathy; (c) dental causes; (d) sinus pain; (e) temporomandibular (TM) joint pain; (f) cervical spine pain; (g) pharyngeal tumours.

Acute otitis media (AOM) • 85% of children with acute otitis media (AOM) and otalgia are free of pain within 24 hours, without antibiotics.1 • Acute otitis media should be distinguished from myringitis and otitis media with effusion (OME). * Control pain and fever with: (a) paracetamol; by suppository if the patient is vomiting; or (b) ibuprofen suspension in children over 1 year old. * Do not give decongestants or antihistamines. They are probably worse than useless;2 steroid/ antibiotic ear drops are similarly unhelpful. 335

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Pink suffused drum (myringitis)

* Do not give antibiotics. * Explain that it is part of a viral infection. Red bulging drum

This is the only specific sign for AOM.3 • Antibiotics reduce pain at 2-7 days and reduce the risk of developing contralateral AOM, but have no effect on pain in the first day, and no effect on the incidence of deafness at 1 month. • There is no evidence that antibiotics improve outcomes in children with uncomplicated AOM under 2.4 • Penicillin reduces the number of children with symptoms and signs of AOM at 1-2 weeks (NNT 7) with no significant advantage from broadspectrum antibiotics.5 The use of broad-spectrum antibiotics is, however, hallowed by consensus although not by research evidence. • Writing a prescription for antibiotics, but suggesting to the parents that they only use it if the otitis media has not resolved in 3 days, can reduce the number taking antibiotics to a quarter with only minor delay in the resolution of symptoms. Parents given a delayed prescription are less likely to believe that they need to consult a doctor in future episodes.6 • Discuss with the parents the question of whether to prescribe an antibiotic. Use penicillin V. Consider suggesting that they only use it if symptoms persist after 3 days. * If symptoms persist in the presence of a red bulging drum prescribe a second antibiotic.7 Use amoxycillin, erythromycin or a cephalosporin. * Review in 4-6 weeks for deafness and /or effusion. Half of all children will have an effusion at 1 month and 10% will have an effusion at 3 months. Persistent fluid is not an indication for further antibiotic treatment.3

(b) the patient has five or more attacks of AOM in one winter, or 6 or more in one year; 8 (c) the ear drum is retracted;8 (d) a perforation is still present at 1 month. Refer urgently8 if there is: (a) a sudden severe hearing loss; or (b) a sudden onset of dizziness with nystagmus. AOM prevention

This remains largely unsubstantiated. * Encourage breast feeding. * Feed upright where possible. * Avoid cigarette smoke exposure. AOM prophylaxis

There is some evidence to support antibiotic prophylaxis in children with recurrent otitis media (NNT of 9). The type, duration and criteria for antibiotic use are not clear.9

Mastoiditis Admit any patient with: (a) tenderness, redness or oedema over the mastoid; or (b) vertigo, vomiting, increasing deafness and nystagmus; or (c) acute otitis media with continuing fever despite antibiotics.

Acute furunculosis of the external canal * Refer if the tympanic membrane is obscured. * Early cases respond to antibiotic/corticosteroid drops. * Surrounding cellulitis needs oral antibiotics as well, e.g. flucloxacillin or erythromycin. * Exclude diabetes.

Referral Refer to ENT if:

Bloody discharge (bullous myringitis)

(a) deafness or effusion (which may lead to hearing loss) is still present at 3 months;

The patient usually suffers severe pain followed by bleeding. A number of serosanguinous bullae

THE EAR 337

appear on the tympanic membrane or surrounding skin. * Prescribe adequate analgesia and advise the patient to keep the canal dry. * Reserve broad-spectrum antibiotics for patients with middle ear effusions.10 * Refer if the patient suffers persisting pain or hearing loss.11

Herpes zoster (Ramsay Hunt syndrome) As well as a vesicular eruption of the auricle or external auditory meatus, the patient may develop facial palsy or hearing loss and vertigo. * Treat as for shingles anywhere, with oral acyclovir and adequate analgesia (see p. 374). * Arrange ENT assessment of VIIth and VIIIth nerve function.

DISCHARGE FROM THE EAR Otitis externa * This may be due to bacterial, viral or fungal infection, and a predisposing factor is any scaling skin disorder (e.g. eczema, psoriasis). * Clean the canal with a Jobson Home wool carrier, or by gently syringing and mopping with cotton wool. Where there is profuse discharge: * See daily to clean the canal; teach the patient to do it at home with cotton wool wound on to an orange stick. Commercial cotton buds are too thick and hard; or refer for microsuction. * Prescribe ear drops containing antibacterial agents and steroid. * Tell the patient that reinfection is likely if the ear canal is scratched, cotton buds are used or water is allowed to enter the canal. When contact with water is unavoidable, e.g. showering, the meatus should be plugged with cotton wool impregnated with petroleum jelly. * Make sure that patients prone to recurrent attacks have ear drops at home to use at the onset of symptoms.

If the canal is severely swollen: * Refer for the insertion of an antibiotic/corticosteroid wick or insert half-inch ribbon gauze soaked in steroid cream. Change it daily. Refractory otitis externa

This is usually due to Candida or aspergillus, and may follow prolonged use of topical antibiotics. The canal is filled with blackish spots or brownishcream deposits. * Swab for bacteria and fungi. * Mop or syringe out the debris gently and dry with cotton wool or a Jobson Home wool carrier. * Treat with clotrimazole solution 1% instilled t.d.s. and continued for 14 days after the disappearance of obvious infection.

Chronic suppurative otitis media (CSOM) * Refer all patients for initial assessment. * Take an ear swab if antibiotic drops have been used recently. It may show resistant bacteria or fungi. Central perforations - 'safe disease'

* Manage an acute exacerbation as in otitis externa, but with even more frequent mopping: 1. mop the ear; 2. administer antibiotic drops;12 3. instruct the patient to pump the drops through the perforation by repeated pressure on the tragus. Attic or marginal perforations - 'unsafe disease'

This carries the highest likelihood of ototoxicity on topical drug treatment: 1. ensure that the patient is under ENT supervision; 2. mop and instill drops as above; 3. refer urgently if vertigo develops; there may be a fistula into a semicircular canal causing vestibulotoxicity.

338

EAR, NOSE AND THROAT PROBLEMS

Antibiotic ear drops and perforation • There is agreement that antibiotic drops, except possibly those below, are indicated in the presence of middle ear infection and a perforation; yet many patients are still being denied adequate treatment. Only 34% of GPs would give topical antibiotics in the presence of a perforation, and only 14% in the presence of grommets.13 • There is less agreement about the use of aminoglycosides and polymyxins in the presence of a perforation. The British Association of Otolaryngology states that such antibiotic drops should not be discontinued as long as gentamicin is never used in an uninfected ear and its use not prolonged longer than ten days.14 The Drug and Therapeutics Bulletin15 is more cautious. However, the CSM warns doctors against prescribing topical aminoglycosides in the presence of a perforation.16 It seems wise, therefore, not to do so in primary care.

DEAFNESS • At least one-third of patients over 70 and half of patients over 80 have deafness severe enough to be helped by a hearing aid (i.e. roughly a 35 dB hearing loss at speech frequencies). • All deaf people, particularly the elderly, may become isolated, depressed and difficult to live with. When complicated by tinnitus, speech discrimination may deteriorate and auditory hallucinations can occur.17 Assess whether the hearing loss is: (a) perceptive (lesion of the inner ear or of the eighth cranial nerve); or (b) conductive (middle or outer ear). See p. 340 for a description of the tests.

Perceptive deafness • Examine the drum and remove any wax which may be contributing. • Refer patients with: (a) Unilateral deafness of acute onset. If the patient is seen within 3 weeks of onset, arrange for outpatient assessment within 24 hours. This is most

important in patients with barotrauma, especially if they also experienced vertigo. The round window membrane may have ruptured, (b) Unilateral deafness with a longer history. Referral is less urgent, but the patient may have a treatable cause (e.g. acoustic neuroma). * Be active in detecting deafness and encouraging patients to consider a hearing aid. * Refer patients for a hearing aid early rather than late, and encourage them to persevere with it once supplied. Deaf patients lose the ability to discriminate between sounds, and have to relearn this skill. They may take up to 1 year to get the full benefit from an aid. Questions about how patients are functioning are more reliable than a hearing test in the surgery. Useful questions to screen for hearing loss18 Do other people mumble a lot? Do you find yourself frequently saying 'pardon'? Does the family say the TV is too loud? Do you miss hearing the doorbell or phone? Do you occasionally get the wrong end of the stick in conversation?

* Refer direct to the audiology department for a hearing aid if: (a) the deafness is chronic, perceptive and bilateral; and (b) there is no other ear pathology which needs ENT assessment first. * Lip reading will help many patients. If not available through the local ENT department, contact The Association of Teachers of Lip Reading to Adults, 6 Ellerby Street, London SW6 6EZ. * Cochlear implant devices are most suitable for children over 2 and adults with profound or severe deafness. Most benefit is seen in those people for whom deafness occurred after language and speech is acquired.19 Refer to a local ENT specialist for onward referral.20 * Aids: A number of aids are available for deaf people, e.g. special telephones, flashing-light alarm clocks, loop amplifiers for radio and TV sets which allow the patient to hear the sound through their hearing aid, and typetalk - a system by which deaf people can communicate via texrphones and others can communicate with them.

THE EAR 339

* Communication: Explain to the family how to communicate with a deaf person: (a) face the person when speaking; (b) speak clearly without shouting. Shouting may distort the sound and make it harder to hear. Patient advice For access to a range of information on aids, rights and other help, contact: The Royal National Institute for the Deaf (RNID), 19-23 Featherstone st, London ECIY 8SL; or Floor 3 Crowngate Business Centre, Brook st, Glasgow G40 SAP, www.rnid.org.uk The British Deaf Association, 1-3 Worship st, London EC2A 2AB, tel. 0870 770 3300, www.bda.org.uk The British Association of the Hard of Hearing, 7-11 Armstrong Road, London W3 7JL, tel. 0208743 1110/1353.

Conductive deafness * Assume that all conductive deafness is treatable. This is true whether the drum looks normal, as in otosclerosis, or abnormal, as in middle ear effusion or CSOM. * Examine the canal and drum. If wax is present: (a) syringe if the wax is soft; (b) disimpact hard wax with a wax hook, then syringe; or (c) suggest that the patient instils ear drops into the canal for 2-7 nights and then reattends for syringing. Instillation of either an oil-based or a water-based solvent for 1 week will clear wax in 20% of patients21 and facilitate syringing in the others. * Refer all patients with conductive deafness not due to wax, with the exception of a recent perforation not due to CSOM. Even then, refer if the perforation has not healed in 6 weeks.

Otitis media with effusion (OME) Fluid in the middle ear without signs or symptoms of inflammation can result in hearing loss but a causal link has not been established between glue ear and significant disability.22

Children • This is also known as secretory otitis media, or glue ear. • Half of affected children resolve spontaneously within 3 months.23 However, 2-3% of children under 7 have a more prolonged hearing loss due to OME and need specialist assessment. • Even those having specialist assessment are unlikely to be helped by surgery, the benefits of which are only temporary. Watchful waiting and immediate presurgery testing may reduce inappropriate surgery.24 For instance, grommets improve hearing 6 months after insertion but very little after 12 months. Adenoidectomy improves hearing, but for only up to 2 years. Tonsillectomy and/or myringotomy do not help hearing.25 • A 10-day course of antibiotics (at normal dose) has been shown to increase the rate of resolution by 14%.25 Meta-analysis has not confirmed the benefit of oral steroids.25 • OME is more common in children whose parents smoke in the same room. • Antihistamines and decongestants, orally or topically, are of no value. • Refer to the community audiology service, once deafness due to OME is discovered, or even merely suspected by the parents, to determine: (a) severity; (b) that the deafness is conductive; (c) the overall disability. • Refer direct to ENT outpatients only if: (a) deafness is persistent, i.e. more than 3 months; or (b) deafness is severe (i.e. a hearing loss of more than 25 dB or the child is having difficulties with hearing, speech, learning or social functioning because of it); and (c) medical treatment has failed (e.g. a 4-week trial of antibiotics, with or without oral steroids); or (d) deafness is associated with persistent pain. • If a child has other disabilities, refer if there is any degree of deafness because normal hearing is crucial. • Following acute otitis media: arrange to see the child at 6 weeks before deciding to refer.

340 EAR, NOSE AND THROAT PROBLEMS

Adults OME may follow a URTI or barotrauma. * Advise regular autoinflation with a Valsalva manoeuvre. * Prescribe ephedrine nose drops or a steroid nasal spray if there is generalized rhinitis. * Refer if the effusion persists for over 6 weeks. A nasopharyngeal tumour must be excluded, especially in Chinese patients. Grommets • The majority of grommets are extruded within 9 months, and only need to be reinserted if deafness recurs. • Discharge should be treated by aural toilet and antibiotic/corticosteroid drops (see p. 337). • Swimming and bathing pose no danger, but diving should be banned.

Hearing tests Rinne's test A 512 cps tuning fork is struck and held close to the meatus until the patient signals that it can no longer be heard. The base is then applied to the mastoid process. If the patient cannot hear it, this is a normal or positive Rinne's test, i.e. if deafness is present, it is perceptive. If the patient hears the fork applied to the mastoid process, this is a negative Rinne's test, i.e. if deafness is present, it is conductive. False negatives can occur in severe unilateral perceptive deafness. Bone conduction appears better than air conduction, but the sound is in fact being heard by the good ear. Weber's test Once the side of the deafness has been discovered, apply the tuning fork to the top of the head. (a) If the sound is heard on the deaf side, then the deafness is conductive. (b) If the sound is heard on the good side, the deafness is perceptive.

Whereas Rinne's test can only detect a hearing loss of 30 dB, Weber's can detect a 5 dB loss. The 'whisper test' This is a good screening test and is easily performed.26 1. Cover one of the child's ears with a finger in the meatus. Move it gently to and fro to mask the whisper in that ear. 2. Stand with your head 30 cm behind the child's other ear. 3. Whisper, and ask the child to repeat what he or she has heard: for children under 6 years old e.g. 'bread and butter' or 'Father Christmas'; for children over 6 years old multisyllabic numbers, e.g. '362436'.

GIDDINESS The duration and type of symptom will often indicate the cause. It is important to distinguish between vertigo, unsteadiness and the sensation of light-headedness.

Unsteadiness Episodic (a) Unsteadiness lasting a few seconds indicates a physiological overload of the vestibular or central systems. It occurs most frequently on rapid movement, associated with a minor inadequacy of the proprioceptive or labyrinthine systems. In young people it occurs in the later stages of recovery after head injury, and in the elderly after standing or turning rapidly. (b) Unsteadiness lasting hours or days is due to temporary impairment of the central connections or decompensation of the vestibular system. It commonly occurs with alcohol or drug overdose, normal doses of tranquillizers or sedatives, and hyperventilation. Prolonged Unsteadiness lasting weeks or months is usually due to central or vestibular inadequacy, and is common in the elderly.

THE EAR 341

Vertigo

Treatment

Episodic

Acute vertigo

(a) Vertigo lasting only a few seconds or minutes indicates a short-lived depression or stimulation of the labyrinth or central connections. It is present on sudden changes in posture, and is commonly due to benign positional vertigo (see below). (b) Vertigo lasting a few minutes to a few hours indicates a physiological or metabolic disturbance of the labyrinth. It occurs in Meniere's disease.

* Give prochlorperazine 12.5 mg im followed by 25 mg suppositories 8-hourly until the vomiting stops. Continue with prochlorperazine 5-10 mg t.d.s orally until the vertigo ceases.

Prolonged Vertigo lasting more than 24 hours. The clinical picture is of severe incapacitating vertigo associated with nausea and vomiting, and is due to: (a) a peripheral lesion, usually vestibular neuronitis, in which there is no hearing loss.27 Less common causes are an extension of acute or chronic suppurative otitis media, trauma or, in the elderly, vascular lesions of the inner ear; or (b) a central lesion which is usually associated with other signs, as in multiple sclerosis, stroke, a posterior fossa tumour or secondary deposits in the brain stem.

Examination This should include: (a) taking the patient's temperature and testing for neck stiffness in the acute attack; (b) inspection of the ear drums for acute or chronic otitis media; (c) testing the hearing for the deafness of Meniere's disease or acoustic neuroma; (d) looking for nystagmus. In peripheral lesions the nystagmus will be horizontal with the fast phase towards the healthy side. Looking towards that side will increase the amplitude of the nystagmus. In central lesions the nystagmus may be horizontal, vertical or rotatory; (e) looking for other neurological signs, especially cerebellar and cranial nerve signs and papilloedema, as in acoustic neuroma or central lesions.

Recurrent vertigo Treatment should be directed at encouraging central compensation. Labyrinthine sedatives should be tailed off as soon as possible because they may delay this process. * Labyrinthine sedatives. Give labyrinthine sedatives only if the vertigo is due to labyrinthine dysfunction, e.g. cinnarizine 15-30 mg t.d.s., prochlorperazine 5-10 mg t.d.s., or cyclizine 50 mg t.d.s. Diazepam has potent labyrinthine sedative effects but leads to increased unsteadiness if the dizziness is due to vestibular inadequacy. * Compensation may occur naturally, especially in young people, but can be accelerated by vestibular rehabilitation exercises. One study in primary care found that two 30-minute sessions tripled the number of patients who improved.28 Vestibular rehabilitation exercises (a) In bed, performed slowly initially then more rapidly: -eye movements - up and down, side to side, focusing on a finger as it moves from 1 m away to 30 cm away; -head movements - moving head forwards then backwards and turning from side to side. (b) Sitting: rotating the head; bending down and standing up with eyes open and closed. (c) Standing: throwing a small ball from hand to hand, turning through 360°. (d) Moving about: walking across the room, up and down a slope, up and down stairs with eyes open and then closed.

MENIERE'S DISEASE • This is characterized by the triad of vertigo, hearing loss and tinnitus. Hearing loss and tinnitus may be unilateral. Symptoms vary in severity.

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In the long term, deafness may remain but the vertigo and tinnitus to a lesser degree. Some people develop a severe bilateral disorder and may need surgery. • The incidence is variably reported but a practice of 6000 patients may see one new case a year.29

Refer to an ENT consultant (a) To confirm diagnosis before starting treatment. (b) To exclude an acoustic neuroma in patients with persistent unilateral symptoms. The diagnosis may be made by electrocochleography in an ENT department.

Medical treatment: acute No RCTs exist for acute management with betahistine, benzodiazepines or anticholinergics. The most recent analysis of betahistine trial data concludes that there is no evidence that it is effective or ineffective in patients with Meniere's disease or syndrome.30 Earlier work favoured the use of betahistine in doses of 8-16 mg t.d.s, stating that it may decrease the number and duration of attacks of vertigo as well as reduce tinnitus and the feeling of fullness in the ear.29 Any benefit may take several months to develop. • Give a labyrinthine sedative (see above) if vertigo remains troublesome. • Consider referral to ENT for those severely affected. Surgical options are: (a) sac decompression; or (b) selective division of the 8th nerve; or (c) labyrinthectomy.

Prophylactic • There is no evidence for prophylactic management of Meniere's disease or syndrome.31 One trial shows some control of vertigo with diuretics.32

Hallpike manoeuvre. Neurological referral is not necessary. The Hallpike manoeuvre The Hallpike manoeuvre involves moving the patient quickly from the sitting to the lying position with the head extended and turned to one side, and then repeating the manoeuvre with the head turned to the other side. In benign positional vertigo, nystagmus will be seen within 30 seconds of lying the patient down in one of the head positions only. The patient will experience vertigo at the same time.

* Explain that the vertigo may last for a few weeks only or may continue with remissions and relapses for many years. * Do not give labyrinthine sedatives. * Teach the patient to minimize the vertigo by sitting up or lying down in stages. * Discuss with the ENT department whether they offer adaptive physiotherapy or Epley's manoeuvre. The latter involves repeated rapid positioning of the head, the theory being that this moves otoliths out of the labyrinth. Epley has shown that two sessions 1 week apart give relief in 97% of patients33 with 70% cured after a single session.34

TINNITUS Guideline: Clinical Guidelines Working Group of the British Tinnitus Association. New developments in the management of tinnitus: guidelines for general practitioners, 1997. Online. Available: send an e-mail to [email protected]

1-2% of the population has tinnitus that severely affects the quality of life. * Reassure the patient that 15% of people experience tinnitus. For most, it improves with time. Worry about it can lead to a vicious circle of increasing distress and more intrusive tinnitus. * Exclude a drug cause, e.g. aspirin, NSAIDs, quinine, loop diuretics, tricyclics and aminoglycosides. Refer patients with:

BENIGN POSITIONAL VERTIGO • It is usually possible to diagnose this condition from the history and by performing the

(a) sudden onset; (b) associated vertigo or deafness. They may have Meniere's disease or middle ear disease;

THE EAR 343

(c) unilateral tinnitus, for exclusion of cerebellopontine angle tumours, especially if there is perceptive deafness on the same side;35 (d) tinnitus due to occupational disease. If confirmed, patients may be entitled to financial benefits. (e) neck and skull bruits (carotid artery stenosis or a-v fistula).

Treatment The Centre for Reviews and Dissemination36 and Bandolier37 record their difficulty in drawing conclusions from available studies. However, some patients may benefit with: (a) alprazolam, although some benzodiazepines make symptoms worse; (b) tricyclic antidepressants (nortriptyline and amitriptyline); and (c) electrical stimulation over the mastoid. * Non-specific support and counselling are probably helpful. * Consider sedation for those unable to sleep. * Look for depression associated with tinnitus and treat it in its own right. Suicide is known as a consequence of tinnitus. There is no evidence that psychological treatments help tinnitus in the absence of depression. * Masking: there is no research evidence of a significant effect but considerable clinical experience of benefit. Encourage the patient to mask the noise with: (a) a background radio; (b) a hearing aid to amplify ordinary background noise; (c) a masking device available through ENT departments. * Tinnitus retraining therapy (TRT): This combines a low level noise generator with psychological retraining to reduce the attention that the patient pays to the tinnitus. There is insufficient evidence to suggest benefit. Patient organization: British Tinnitus Association, 4th Floor, White Building, Fitzalan Square, Sheffield S1 2AZ, tel. 0114 279 6600; Helpline 0800 018 0527; www.tinnitus.org.uk

FACIAL PALSY (LOWER MOTOR NEURONE) Refer urgently if there is evidence of: (a) middle ear infection or cholesteatoma; (b) parotid tumour; (c) cerebellopontine angle tumour, i.e. deafness, loss of facial sensation, diplopia and cerebellar signs; (d) trauma; or (e) Ramsay Hunt syndrome (look for herpetic vesicles on the pinna or in the external auditory canal).

BELL'S PALSY Overview: Williamson IG, Whelan TR. The clinical problem of Bell's palsy: is treatment with steroids effective? Br J Gen Pract 1996; 46: 743-7.

• Characterized by a unilateral facial weakness with the inability to close the affected eye properly. The weakness may be associated with mild pain behind the ear and some hearing loss. The onset is typically hours or days. It is more common in pregnant women, in those with diabetes, and following a recent upper respiratory infection. • Up to 85% recover in 3 months without treatment.

Referral (a) If loss of power is complete - refer to an ophthalmic surgeon be seen that day. Tarsorraphy is likely to be needed. (b) If the eyelid, when closed, does not cover the cornea. Refer to an ophthalmic surgeon. (c) If recovery has not started at 6 weeks. Refer to an ENT surgeon to look for an underlying cause. (d) If recovery at 9 months is incomplete. The patient may benefit from cosmetic surgery.

Management * Protect the eye with a patch and/or artificial tears. Warn the patient that the eye is not protected by reflex blinking and must be safeguarded. This

344 EAR, NOSE AND THROAT PROBLEMS

is especially true in windy conditions and during sleep. • Consider oral prednisolone, despite the lack of evidence favouring the use of steroids over placebo in terms of good recovery of function or in reducing the proportion of patients with sequelae.38 If choosing to give it, give 1 mg/kg per day up to a maximum of 80 mg for 10 days. • Combining antiviral medication such as aciclovir with prednisolone is of unknown effectiveness.39 • Physiotherapy has no benefit. • Surgical decompression of the nerve is not indicated. There is no evidence that it is beneficial.

THE NOSE SINUSITIS Guidelines and reviews: American Academy of Allergy, Asthma and Immunology et al. Parameters for the diagnosis and management of sinusitis, 1998. Online. Available: www.guideline.gov Institute for Clinical Systems Improvement. Acute sinusitis in adults, 1999. Online. Available: www.guideline.gov de Bock GH, Dekker FW, Stolk J et al. Antimicrobial treatment in acute maxillary sinusitis: a meta-analysis. J Clin Epidemiol 1997; 50: 881-90. Williams JW Jr, Aguilar C, Makela M et al. Antibiotics for acute maxillary sinusitis (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford, Update Software, 2000.

• Diagnosis is usually clinical. • X-rays are unlikely to affect management in general practice because of the poor correlation between clinical symptoms and radiological findings.40

Management of acute sinusitis • Decongestants (topical or oral) are used widely but there are no studies available which support their use. • Most trials of antibiotics report findings for maxillary sinusitis and the relatively small studies from which this data comes tend to use objective

diagnostic criteria rather than clinical diagnosis. A recent well-conducted trial in which diagnosis was clinical showed no benefit from antibiotics. * A few studies suggest that intranasal steroids may have some benefit as an adjunct to antibiotics in treatment of acute sinusitis but also for the treatment of underlying rhinitis.41 * Advise the patient to: (a) take simple analgesics; (b) use steam inhalations. * Decide whether to give an antibiotic, e.g. penicillin or amoxycillin for 10 days. Trimethoprim can be used in patients who are penicillin sensitive. If not responding after 10 days change to a second-line drug, e.g. a macrolide, and add metronidazole 400 mg t.d.s.42 Candidates for an antibiotic are those with: (a) high fever with prior history suggestive of sinusitis; or (b) tooth pain; or (c) severe symptoms; or (d) a known anatomical blockage.

Management of chronic sinusitis * Give a 10-day course of a beta-lactamaseresistant antibiotic and metronidazole. If the response is partial, this may need to be continued for up to 6 weeks. * Use a decongestant spray, e.g. xylometazoline (less prone to rebound congestion than ephedrine) for a maximum of 5 days, and intranasal steroids (see p. 346). For greatest effect, the patient should adopt the following position to insert nose drops: kneel with the head on the floor, drip the drops into the nostril and maintain the position for 2 minutes. Insert two drops 2-3 times a day for no more than 1 month. More prolonged use can lead to systemic side-effects. Do not give more than six courses a year.43 * Where there is no evidence of preceding upper respiratory tract infection, consider an upper molar tooth abscess or possible malignancy. Refer urgently: (a) for surgical drainage if there is no response to the above regimen;

THE NOSE 345

(b) patients with orbital or facial cellulitis or who are severely ill. They need admission.

THE COMMON COLD Guideline: Mossad SB. Treatment of the common cold. BMJ 1998; 317: 33-6.

A consultation about the common cold is an opportunity for the education of the patient in self-treatment as well as discussion of any underlying issues. However, in the occasional patient where treatment is needed (singers, examinees) the options are as follows: • Oral first-generation antihistamines will reduce rhinorrhoea and sneezing but with anticholinergic adverse effects especially sedation. • Ipratropium bromide nasal spray will reduce rhinorrhoea with a 26% reduction in symptoms at day 4 (NNT 6) but with a risk of nasal dryness and headache.44 • Oral or nasal decongestants reduce symptoms by 13% after a single dose but the benefit disappears with repeated doses.45 Courses longer than 5 days carry the risk of rebound congestion. • Steam inhalations give subjective benefit but evidence of objective benefit is conflicting. • Oral vitamin C may reduce duration and severity but the dose required is not clear. It is likely to be in excess of 1 g/day. • Oral zinc has shown benefit in some studies and not in others. If it does work, the dose required is above that found in most vitamin preparations. • Co-amoxiclav has been shown in one study to have benefited the 20% of patients in whom H. influenzae, M. catarrhalis or S. pneumoniae were subsequently cultured.46 However, if all patients with the common cold are treated, no benefit can be detected.

RHINITIS Guidance: GPIAG opinion sheet no. 6. Online. Available: www.gpiag-asthma.org

Seasonal and perennial rhinitis • Affects 15-20% of the population; 3% of GP consultations are for allergic rhinitis. • Treatment is best tailored to the individual's symptoms; where possible identify the underlying cause. • Allergens should be avoided where possible, e.g. by reducing the dust in the house or driving with windows and air vents closed.

Intermittent mild symptoms or acute response to allergen • Antihistamines reduce the nasal itching, watery hypersecretion and sneezing, but do little for nasal blockage.47 Intermittent use may be enough. * Use an oral, minimally-sedating H1-selective antihistamine with a rapid onset, e.g. loratadine. * Add an antihistamine nasal spray in someone in whom oral antihistamines are not enough, but not in patients who need regular protection, for whom steroid sprays are more effective.48

Regular symptoms • Antihistamines may be taken regularly, and for some people are all that is necessary. • Intranasal steroid sprays are the most effective prophylactic treatment. There is often a delayed onset of action possibly up to a week. Combining them with an antihistamine adds no further benefit.49 * Instruct the patient on their proper use: (a) use once or twice a day. The patient should point the spray upwards for one puff and backwards for the other, occluding the other nostril; (b) in seasonal rhinitis, start 2 weeks before the beginning of the season; (c) they should be continued until the rhinitis is likely to have subsided. * Use an aqueous preparation (e.g. Beconase Aqueous, Syntaris or Flixonase) if irritation or epistaxis occurs with a pressurized preparation.

Nasal blockage * Use a topical decongestant, e.g. xylometazoline spray for 5 days, to open the airway enough for

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the steroid spray to penetrate. Longer use is likely to lead to rebound congestion on stopping. * Use betamethasone drops 0.1%. For greatest effect the patient should use these in the 'head down' position. Insert two drops 2-3 times a day for no more than 1 month. More prolonged use can lead to systemic side-effects. Do not give more than 6 courses a year.50 Try to maintain improvement after each course with a steroid spray. * Use sodium cromoglycate nasal spray in a child already using inhaled steroids for asthma and in whom you do not want to increase the total steroid dose. It is less effective than steroids, and is limited by being needed four times a day. * Use sodium cromoglycate eye drops regularly in patients with allergic conjunctivitis. * Consider ipratropium bromide nasal spray in those troubled mainly by watery rhinorrhoea. It does not, however, relieve sneezing, itching or nasal obstruction, and therefore is most helpful in perennial rather than seasonal rhinitis.

Severe symptoms * Oral steroids can be given if symptoms warrant them, e.g. at the time of an examination, but courses should not be repeated frequently. Give oral prednisolone 5-10 mg daily or 30-40 mg daily for 1 week, in dire situations. Intramuscular depot injections of steroids (e.g. Kenalog) last for 3 months, with the inevitable pituitary adrenal suppression, and are not recommended for use in rhinitis. * Referral is needed for continuing severe symptoms. Nasal endoscopy may reveal pathology, e.g. a polyp, which was not otherwise visible. In addition, patients with nasal deformity may benefit from surgery.

NASAL POLYPS Review: Lund JL. Diagnosis and treatment of nasal polyps. BMJ 1995; 311: 1411-14.

• Nasal polyps may be associated with asthma and aspirin sensitivity, but if they occur in the presence of rhinitis it is probably coincidental.

Cystic fibrosis should be considered in any child under 16 with nasal polyps. * The initial treatment should be medical. Shortterm systemic steroids are as effective as nasal polypectomy in controlling polyps over the subsequent year. * Stop aspirin and consider stopping other NSAIDs; there is some cross-reaction. * Give antibiotics if nasal discharge is purulent. Thick, green-brown secretions may indicate fungal infection. This is more likely to be detected on microscopy than by culture.

Treatment * Give betamethasone drops 0.1% using the 'head down' position (see above), or oral prednisolone (40 mg a day for 3 days, then 20 mg a day for 3 days, then l0 mg a day for 3 days). Significant improvement may occur within 48 hours. * If using oral steroids, start the spray simultaneously. Continue for at least 3 months. Some patients need indefinite prophylaxis. * Refer those not responding to the above. Malignancy A polyp that does not have the typical smooth, pale or slightly reddened appearance may be malignant, especially if unilateral. Refer urgently.

SNORING AND OBSTRUCTIVE SLEEP APNOEA (OSA) Guideline: Millman RR Do you ever take a sleep history? Ann Intern Med 1999; 131: 535-6. Royal College of Physicians. Sleep apnoea and related conditions. Summary of a report of the working party of the Royal College of Physicians. J R Coll Physicians Lond 1993; 27: 363-4.

• 25% of middle-aged men are snorers and 10% of middle-aged women. 4% of middle-aged men and 2% of middle-aged women suffer from OSA, with at least five episodes of apnoea/hypopnoea an hour at night plus daytime sleepiness.51 • Men under 65 who admit to both snoring and daytime sleepiness are twice as likely to die as men with just one of these symptoms and men

THE THROAT AND MOUTH 347

without either symptom. The excess deaths are mainly due to cardiovascular disease.52 Symptoms

Signs

Loud snoring

Obesity

Restless sleep with recurrent wakening; nightmares

Sinusitis Nasal or nasopharyngeal obstruction

Daytime fatigue or poor concentration; sleepiness

Large tonsils Large tongue

Family history of OSA

Small chin

Contacts: The British Snoring and Sleep Apnoea Association (BSSAA). www.britishsnoring.demon.co.uk or UK Freephone 0800 0851097. The British Sleep Society, PO Box 247, Huntingdon PE28 3UZ, www.british-sleep-society.org.uk

THE THROAT AND MOUTH SORETHROAT

Refer to a sleep study centre (or possibly directly to ENT) patients: (a) with excessive daytime sleepiness; (b) where a partner reports apnoeic episodes or restless sleep; (c) where loud snoring is causing relationship problems; (d) where curable pathology in the nose could be contributing, e.g. polyps or a deviated septum. They may be appropriate for septoplasty, turbinectomy, tonsillectomy, adenoidectomy or uvulopalatoplasty. Thermal ablation techniques may be considered as may night-time continuous positive airways pressure (CPAP).

Snoring without apnoea * Advise patients to make the following lifestyle changes: (a) avoid alcohol before bed-time; (b) stop smoking; (c) discontinue night-time sedation; (d) lose weight if appropriate; (e) sleep on the side; (f) lift the head of the bed up; (g) dilate the nostrils using a Nozovent or pinch nostrils completely with a diver's nose clip. * Examine for nasal obstruction and consider a trial of decongestants and intranasal steroids. * Consider whether the patient could be hypothyroid. * Protriptyline 5-10 mg t.d.s. may help some patients by lightening the depth of sleep. * Recommend ear plugs for the partner.

Guideline: SIGN 34: Management of sore throat and indications for tonsillectomy. Edinburgh: RCPE, 1999. Online. Available: www.sign.ac.uk/guidelines Systematic review: Del Mar CB, Glasziou PP, Spinks AB. Antibiotics for sore throat (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000.

The following evidence demonstrates the limited value of throat swabs and of antibiotics in the management of sore throat: • Approximately 20% of sore throats are bacterial, namely beta-haemolytic streptococci group A (GABHS) and groups C and G. • The incidence is dependent on age. Only 15% are bacterial under the age of 3, while 50% are bacterial between the ages of 4 and 1353 and 10% in adults.54 • The sensitivity and specificity of the throat swab are low, at most 30% and 80% respectively. Up to 40% of adults are carriers of GABHS. A positive swab is therefore not proof of infection. • Streptococcal tonsillitis cannot reliably be diagnosed clinically, although it is more likely if the patient is <11 years old with: (a) myalgia; (b) tender or swollen cervical lymph glands; (c) history of fever; (d) tonsillar exudates; and is less likely if there is cough or earache.55 • Penicillin speeds up recovery only slightly in patients with Streptococcal tonsillitis (7 days after the start of treatment penicillin shows no advantage over placebo in relief of symptoms). It does

348 EAR, NOSE AND THROAT PROBLEMS

not get patients back to school or work more quickly.56 • Antibiotics do not protect against the rare non-suppurative complications in patients with streptococcal tonsillitis, namely rheumatic fever57 and acute glomerulonephritis58,59 or, if they do, the incidence of those complications in developed countries is so rare that it should not influence management.60 • Antibiotics do protect against the suppurative complications of sore throat, reducing the incidence of acute otitis media by three quarters and of acute sinusitis by a half. However, these complications are uncommon. It would be necessary to treat 30 children and 145 adults to prevent one case of acute otitis media.60 • Antibiotics have no effect on the incidence of URTI, whether bacterial or viral, in the subsequent 6 months. The early use of antibiotics may even increase the chance of recurrence.61 • Patients given penicillin for immediate use are more likely to re-attend than those not given antibiotics or given a presciption only to be used three days later if symptoms have not resolved.62,63 If penicillin is used, a 10-day course is more successful than a 5-day course in eradicating streptococci from the throat. The former eradicates 94% of streptococci while the latter only eradicates 76%. However, it is not clear whether eradication is of clinical importance given the high carriage rate in the population. • Patients who are more satisfied with the consultation get better more quickly.62

Management The aim is to:

(a) avoid unnecessarily widespread use of penicillin, with its disadvantages: the dependence on the GP that it encourages; the possibility of antibiotic resistance and the adverse effects of the drugs; (b) give the ill patient the possible benefit of early penicillin. • Recommend lozenges. • Recommend analgesics and antipyretics.64 Paracetamol is the drug of choice.65 It can decrease

throat pain after 3 hours by up to 50%. Ibuprofen 400 mg t.d.s may be even more effective.66 * Check what it is that concerns the patients. Explain the probable viral nature of the condition, and how they can manage subsequent attacks themselves at home.65 * Instruct patients to return in 1 week, if still unwell, for a throat swab, FBC and glandular fever antibodies. * Be alert to the possibility that the sore throat is really an excuse to consult about some more difficult issue.

Special cases * Ill patients, e.g. with high fever, dysphagia, marked cervical lymphadenopathy and severe pharyngitis: (a) Take a throat swab or a rapid antigen test. (b) Give penicillin V 250 mg q.d.s. or 500 mg b.d. Give erythromycin if the patient is allergic to penicillin. (d) Instruct the patient to telephone for the result. (e) If pathogenic streptococci are grown, urge the patient to complete the 10-day course; * Sore throat associated with stridor or breathing difficulty: admit immediately.65 * Patients with sore throat who are members of a closed community where there is an outbreak of streptococcal pharyngitis. Treat as 'ill patients'. * Patients with prolonged tonsillitis: (a) With negative investigations: - repeat glandular fever antibodies weekly for 2 more weeks; -take blood for an ASO titre. The throat swab may be a false negative. (b) With persistent streptococcal infection: -give a cephalosporin or co-amoxiclav. Beta-lactamase-producing organisms may be destroying the penicillin. * Patients with a past history of rheumatic fever should already be taking prophylactic penicillin V 250 mg b.d. If they develop a sore throat they should be given a cephalosporin or co-amoxiclav, in case they have beta-lactamase-producing organisms in the pharynx that are destroying the penicillin.

THE THROAT AND MOUTH

* Patients on chemotherapy, immunosuppressive drugs or carbimazole. Check the WBC. * Patients with a past history of quinsy. Treat as 'ill patients'.

VINCENT'S ANGINA This is an ulcerative gingivostomatitis, which can spread to the tonsils. It is due to an infection with fusiform bacilli and spirochaetes. * Give metronidazole 400 mg b.d. for 3 days.

INFECTIOUS MONONUCLEOSIS * Consider prednisolone 40 mg daily orally for 5 days in patients with severe tonsillitis, to enable them to swallow and avoid airway obstruction. * Consider a high-dose NSAID to improve the general wellbeing of patients at an important time of their lives, e.g. when taking examinations. * Avoid ampicillin-based antibiotics, including co-amoxiclav.

ORALTHRUSH * Confirm with a mouth swab. * Treat with an oral antifungal without waiting for the swab result. * Consider a predisposing cause, e.g. diabetes, the use of broad spectrum antibiotics or inhaled steroids, HIV infection or other causes of immunosuppression.

SORE THROAT DUE TO POSTNASAL DRIP Treat with intranasal steroids (see p. 345).

TONSILLECTOMY * Not all sore throats are due to tonsillitis. * In children with recurrent severe tonsillitis, surgery offers the chance of avoiding two moderate or severe throat infections in the next 2 years. Children with a history that is less severe benefit less.67 * There is no convincing evidence for tonsillectomy in adults.

349

Indications for referral * Recurrent acute tonsillitis, five or more episodes a year. Attacks should be severe enough to interfere with the child's normal functioning. * Airway obstruction, especially if there is sleep apnoea. * Chronic tonsillitis, for over 3 months, especially if associated with halitosis. * Symptoms are disabling and prevent normal functioning. * Quinsy or peritonsillar cellulitis, after recovery, in patients under age 25 with a history of recurrent tonsillitis. * Bacterial carriers who have not responded to antibiotics, e.g. the diphtheria carrier and the carrier of haemolytic streptococcus group A who has had rheumatic fever. * Unilateral tonsillar enlargement or ulceration. It may be malignant.

DYSPHAGIA * Check Hb and refer urgently to an ENT or a general surgeon to exclude a local cause. * If the appointment is not imminent, order a barium swallow. This is the one situation where a barium study should precede endoscopy.

HOARSENESS * Refer urgently anyone suffering from hoarseness for more than 3 weeks. Early tumours confined to the vocal cord treated by radiotherapy have an 80-90% chance of 5-year survival. * Consider re-referring a patient with persistent constant hoarseness, even after a normal laryngoscopy performed in the early stages. A previously undetected nodule or carcinoma may now be visible. * Do not diagnose chronic laryngitis in smokers without laryngoscopy. It is they who are also at risk of laryngeal carcinoma. * If laryngoscopy is normal, refer to a speech therapist or voice teacher. Voice abuse is common in teachers, mothers of young children and anyone who shouts. * Distinguish hoarseness from functional dysphonia, in which the patient adopts a whisper for

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EAR, NOSE AND THROAT PROBLEMS

psychological reasons. These patients need laryngoscopy to exclude a pathological cause before urgent referral to a speech therapist, psychiatrist or psychologist. Voice teachers Two organizations run workshops and keep a register of voice teachers: 1. The Voice Care Network UK, 29 Southbank Road, Kenilworth, Warwickshire CVS 1LA, tel. 01926 864000; e-mail [email protected]; www.voicecare.org.uk 2. The Voice and Speech Centre, 40A High Street, Downham Market, Norfolk, PE38 9HH, tel. 01366 386014; e-mail [email protected]

SORE MOUTH

Red or white patches in the mouth * Refer all as erythroplakia or leukoplakia unless the typical skin lesions show that it is lichen planus. The risk of malignancy over 10 years is 3-6% with leukoplakia, and far higher with erythroplakia.

Ulcerated sore mouth * Be aware that ulcers may be associated with skin, genital or eye lesions as part of a larger syndrome. Primary herpes stomatitis or labialis

POST-LARYNGECTOMY Review: Gleeson M, Jani P. Long-term care of patients who have had a laryngectomy. BMJ1994; 308: 1452-3.

Systematic review: Worrall G. Herpes labialis. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org

* Treat all respiratory infections vigorously, and admit patients early if they are failing to expectorate their secretions. * Check that the patient: (a) is attending speech therapy; (b) is eating enough. The sense of smell diminishes because the nose is not being used to sniff. Patients can be taught to sniff by speech therapists; (c) has been referred to the National Association of Laryngectomy Clubs, 6 Rickett Street, London SW6 1RU, tel. 020 7381 9993.

* First attack: if seen within the first 48 hours give an oral antiviral agent, e.g. aciclovir, unless mild. It can halve the time to healing, at least in children. * Recurrent attacks: use 5% aciclovir cream five times a day for 5 days. Any benefit is, however, marginal. If the patient really needs treatment, use an oral course. * Prevention of recurrent attacks: (a) sunblock will dramatically reduce the rate of recurrence in those in whom sun is a factor; (b) prophylactic oral antivirals probably do reduce the number of attacks and the size and duration of lesions. They need to be given continuously or at least 7 days before a high risk activity, such as skiing.

THE FEELING OF A LUMP IN THE THROAT

Aphthous ulcers

When diagnostic clues are absent: (a) Treat as oesophagitis for 2 weeks with raft antacids. (b) Refer non-responders to the ENT department in order to exclude a physical cause. (c) Counsel those with negative findings to try to uncover the cause for their probable globus hystericus.

These may be associated with stress, menstruation, poor overall health or, occasionally, coeliac disease. Thirty per cent of patients with recurrent ulcers have low iron, B12 or folate levels. Large single ulcers can reach 1-2 cm in diameter and take 6 weeks to heal, but at 3 weeks some improvement should already be seen. * Give Adcortyl in Orabase, thinly applied 2-4 times a day; or hydrocortisone pellets - dissolve

THE THROAT AND MOUTH

one pellet slowly in contact with the ulcer, 3-4 times a day. Traumatic ulcers

(a) Treat the cause. (b) Try a local anti-inflammatory agent, such as salicylate cream, applied 3-4 times a day. Refer for biopsy any ulcer which has not healed within 3 weeks after the removal of any local cause. It may be a squamous cell carcinoma.

Gum disease In its most severe form, gum disease is acute necrotizing ulcerative gingivitis. * Give a chlohexidine mouthwash and metronidazole 400 mg b.d. for 5 days. * Stop smoking. * Improve oral hygiene.

Sore mouth without ulceration Dry mouth may be due to anxiety, drugs, dehydration or Sjogren's syndrome. * Prescribe an artificial saliva product. Do not encourage the sucking of sweets unless they are sugar-free. Caries is already a hazard for patients with a dry mouth. * Oral thrush in a patient wearing dentures. Sterilize the dentures by soaking them overnight in dilute hypochlorite solution, then apply miconazole oral gel q.d.s. to the part of the denture in contact with the gum. Work-up where diagnosis is not clear (a) Swab for Candida. (b) FBC, B12, folate, serum iron.

DENTAL PROBLEMS Toothache * Sensitive to hot, cold and sweet stimuli: this is probably due to exposed dentine. Advise the patient to see a dentist.

351

* Increasing pain, initially temperature sensitive: This is probably pulpitis. Advise the patient to see a dentist. * Severe pain, touch sensitivity with or without local lymphadenopathy: This is periapical periodontitis. Refer for drainage. If there is likely to be delay, prescribe: (a) penicillin V 500 mg q.d.s. or erythromycin 500 mg b.d.; and metronidazole 400 mg b.d. (b) adequate analgesia. Try an NSAID first.

Loss of a tooth through trauma * Re-implantation of a secondary tooth within 12 hours is likely to succeed, and is worth attempting after an even longer time. 1. Pick the tooth up by its crown, not by the root. 2. If dirty, wash it in cold water. 3. Push it back into its socket or under the patient's tongue, or store it in a cup of milk or in the patient's saliva. 4. Send the patient immediately to a dentist or Accident and Emergency department for re-implantation.

Complications after extraction Haemorrhage

1. Allow the patient to rinse the mouth out. 2. Place a damp gauze swab over the socket and ask the patient to bite hard for 10-15 minutes. 3. If this fails, either suture across the socket with 3.0 silk or refer to a dentist or oral surgeon. Painful socket, bad taste, halitosis

This usually occurs 4-5 days after an extraction and is due to the clot not forming or being removed and the socket filling with debris that gets infected. * Refer to a dentist. * Give strong analgesics. * Advise the patient to wash the mouth out with warm saline. * Start antibiotics: penicillin V 500 mg q.d.s. or erythromycin 500 mg b.d. and metronidazole 400 mg b.d.

352 EAR, NOSE AND THROAT PROBLEMS

HALITOSIS Overview: Scully C, Porter S, Greenman J. What to do about halitosis. BMJ1994; 308: 217-18.

Ninety per cent of halitosis is due to bacterial putrefaction in the mouth.68 * Encourage mouth hygiene. Trapped food is a common cause of halitosis, especially in denture wearers. * Treat any local infection. This may be tonsillitis, gingivitis, sinusitis or even bronchiectasis. A dental check is worthwhile even if there is no obvious pathology. * Look for a furry tongue, although treatment is difficult. Stopping smoking and scrubbing the tongue with a toothbrush may help. * Check whether the patient suffers from a dry mouth. This may be due to drugs, DXR or Sjogren's syndrome, or to mouth breathing. Sugar-free mints or gum will aid salivation. * Ask whether the halitosis only occurs when the patient is hungry. Hunger halitosis is wellrecognized, although the cause is not clear. * Consider certain foods as the cause. Patients usually recognize for themselves the connection with garlic or onions, but may not notice the

connection with a diet high in dairy products, especially yoghurt. * If there is no specific pointer to the cause of the trouble, consider an empirical trial of toothbrushing and flossing after every meal with an antiseptic mouthwash night and morning for 2 weeks at a time. Longer courses put the patient at risk of fungal overgrowth. * If the above fails, then consider investigating for rare causes such as: (a) metabolic disorders; uraemia, ketosis and liver failure; (b) small bowel pathology where bacterial overgrowth may be the cause of the halitosis (e.g. blind loop syndrome). Evidence for this may be haematological, (anaemia; iron deficiency; low serum B12 and a raised red cell folate) or radiological (barium meal and follow-through); (c) nasopharygeal malignancy. * If small bowel pathology is not suspected, try a 2-week course of antibiotics (amoxicillin or tetracycline). Chronic bacterial overgrowth of the gut may still be the cause. If the condition improves, consider long-term intermittent use of antibiotics. * Delusional halitosis may be the only complaint of a patient with a hypochondriacal depression. Refer to a psychiatrist.

REFERENCES 1. van Buchem FL et al. Therapy of acute otitis media: myringotomy, antibiotics, or neither? A double-blind study in children. Lancet 1981; ii: 883-7. 2. DTB. The management of acute otitis media. Drug Ther Bull 1984; 22: 53-5. 3. Alberta Medical Association. Guideline for the diagnosis and treatment of acute otitis media in children. Online. Available: www.amda.ab.ca 4. Damoiseaux RA, van Balen FA, Hoes AW et al. Primary care based randomised, double blind trial of amoxycillin versus placebo for acute otitis media in children aged under two years. BMJ 2000; 320: 350-4. 5. Rosenfeld RM, Vertrees JE, Carr J et al. Clinical efficacy of antimicrobial drugs for acute otitis media: metaanalysis of 5400 children from thirty-three randomised controlled trials. / Pediatrica 1994; 124: 355-67. Quoted in: O'Neill P. Acute otitis media: antibiotics. Clinical Evidence, Issue 5, 2001. London: BMJ Publishing Group. Available on www.clinicalevidence.org 6. Little P, Gould C, Williamson I et al. Pragmatic randomised controlled trial of two prescribing strategies for childhood acute otitis media. BMJ 2001; 322: 336-42.

7. Bloomington MN. Institute for Clinical Systems Improvement; 2001. Diagnosis and treatment of otitis media in children. Online. Available: www.ngc.gov 8. DTB. Management of acute otitis media and glue ear. Drug Ther Bull 1995; 33: 12-15. 9. O'Neill P. Long term antibiotic treatment. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org 10. Marais J, Dale BAB. Bullous myringitis: a review. Clin Otolaryngol 1997; 22: 497-9. 11. Biedlingmaier JF. Two ear problems you may not need to refer. Otitis externa and bullous myringitis. Postgrad Med 1994; 96 (5): 141-5. 12. Acuin J, Smith A, Mackenzie I. Interventions for chronic suppurative otitis media (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2000. 13. Bickerton RC et al. Survey of general practitioners' treatment of discharging ear. BMJ 1988; 296: 1649-50. 14. Lancaster JL, Mortimore S, McCormick M, Hart CA. Systemic absorption of gentamicin and the managememt of active mucosal chronic otitis media. Clin Otolaryngol Allied Sci 2001; 24 (5): 435-9.

REFERENCES 353

15. DTB. Management of acute otitis media and glue ear. Drug Ther Bull 1995; 33: 12-15. 16. Committee for Safety of Medicines. Reminder: ototoxicity with aminoglycoside eardrops. Curr Prob Pharmacovigil 1997; 23. Available on www.mca.gov.uk 17. Hickish G. Hearing problems of elderly people. BMJ 1989; 299: 1415-16. 18. Brooks D. How best to help adult patients who have hearing difficulties. Pulse 1995; 29: 77-80. 19. National Guideline Clearing House. Cochlear implants in adults and children. Online. Available: www.ngc.gov 20. Douek E. Sensorineural deafness. BMJ 1990; 301: 74-5. 21. Keane EM et al. Use of solvents to disperse ear wax. Br J Clin Pract 1995; 49 (2): 71-2. 22. Bandolier. Glue ear - a sticky problem. Feb 1994. Online. Available: www.jr2.ox.ac.uk/Bandolier 23. Managing otitis media with effusion in young children. American Academy of Pediatrics. 1997. Online. Available: www.ngc.gov 24. NHS Centre for Reviews and Dissemination. The treatment of persistent glue ear in children. Effective Health Care 1994; 1 (4) with update 1996. The University of York. 25. Agency for Health Care Policy and Research. Otitis media with effusion: clinical practice guideline No. 12. Rockville MD: Agency for Health Care and Public Research (AHCPR), 1994. 26. Eekhof JAH et al. The whispered voice: the best test for screening for hearing impairment in general practice. Br J Gen Pract 1996; 46: 473-4. 27. Cooper CW. Vestibular neuronitis: a review of a common cause of vertigo in general practice. Br J Gen Pract 1993; 43: 164-7. 28. Yardley L, Beech S, Zander L et al. A randomised controlled trial of exercise therapy for dizziness and vertigo in primary care. Br J Gen Pract 1998; 48: 1136-40. 29. Bandolier. Menieres disease. Bandolier, 1995; 13. Online. Available: www.jr2.ox.ac.uk/Bandolier 30. James AL, Burton MJ. Betahistine for Meniere's disease or syndrome (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2001. 31. James A, Thorp M. Meniere's disease. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org 32. Bandolier. Tinnitus and Meniere's update. Bandolier, April 2000. Online. Available: www.jr2.ox.ac.uk/ Bandolier 33. Lempert T, Gresty MA, Bronstein AM. Benign positional vertigo: recognition and treatment. BMJ 1995; 311: 489-91. 34. Ruckenstein MJ. Therapeutic efficacy of the Epley canalith repositioning maneover. Laryngoscope 2001; 111: 940-5. 35. Luxon LM. Tinnitus: its causes, diagnosis, and treatment. BMJ 1993; 306: 1490-1. 36. Dobie RA. A review of randomized clinical trials in tinnitus. Laryngoscope 1999; 109: 1202-11. Reviewed in the Database of Abstracts of Reviews of Effectiveness. The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 37. Bandolier. Tinnitus and Meniere's update. Bandolier, 2000. Online. Available: www.jr2.ox.ac.uk/Bandolier 38. Salinas R. Bell's palsy: corticosteroids. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. Available: www.clinicalevidence.org

39. Salinas R. Bell's palsy: antiviral treatment. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. Available: www.clinicalevidence.org 40. Evans KL. Diagnosis and management of sinusitis. BMJ 1994; 309: 1415-22. 41. American Academy of Allergy, Asthma and Immunology et al. Parameters for the diagnosis and management of sinusitis, 1998. Online. Available: www.guideline.gov 42. Lund VJ. Sinusitis. Prescribers Journal 1990; 33: 9-12. 43. DTB. Chronic sinusitis. Drug Ther Bull 1989; 27: 69-70. 44. Hayden FG, Diamond L, Wood PB et al. Effectiveness and safety of intranasal ipratropium bromide in common colds. A randomised, double-blind, placebocontrolled trial. Ann Intern Med 1996; 125: 89-97. 45. Taverner D, Bickford L, Draper M. Nasal decongestants for the common cold (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2000. 46. Kaiser L, Lew L, Hirschel B et al. Effects of antibiotic treatment in the subset of common-cold patients who have bacteria in nasopharyngeal secretions. Lancet 1996; 347: 1507-10. 47. Pearlman DS, Lumry WR, Winder JA et al. Once daily cetirizine effective in the treatment of seasonal allergic rhinitis in children aged 6-11 years: a randomized double-blind placebo-controlled study. Clin Ped 1995; 36: 209-15. 48. Ratner PH, van Bavel JH, Martin BG et al. A comparison of the efficacy of fluticasone proprionate aqueous nasal spray and loratadine, alone and in combination, for the treatment of seasonal allergic rhinitis. J Fam Pract 1998; 47: 118-25. 49. Weiner JM, Abramson MJ, Puy RM. Intranasal corticosteroids versus oral H1 receptor antagonists in allergic rhinitis: systematic review of randomised controlled trials. BMJ 1998; 317: 1624-9. 50. DTB. Chronic sinusitis. Drug Ther Bull 1989; 27: 69-70. 51. Young T, Palta M, Dempsey J et al. The occurrence of sleep-disordered breathing among middle-aged adults. New Engl J Med 1993; 328: 1230-5. 52. Lindberg E, Janson C, Svardsudd K et al. Increased mortality among sleepy snorers: a prospective population based study. Thorax 1998; 53: 631-7. 53. DTB. Diagnosis and treatment of streptococcal sore throat. Drug Ther Bull 1995; 33: 9-12. 54. Cooper RJ, Hoffman JR, Bartlett JG et al. Principles of appropriate antibiotic use for acute pharyngitis in adults: background. Ann Intern Med 2001; 134: 509-17. 55. Dobbs E A scoring system for predicting Group A streptococcal throat infection. Br J Gen Pract 1996; 46: 461-4. 56. Dagnelie CF et al. Do patients with sore throats benefit from penicillin? Br J Gen Pract 1996; 46: 589-93. 57. Howie J, Foggo B. Antibiotics, sore throat and rheumatic fever. J R Coll Gen Pract 1985; 35: 223-4. 58. Taylor JL, Howie J. Antibiotics, sore throat and acute nephritis. J R Coll Gen Pract 1983; 33: 783-6. 59. Glasziou P, Del Mar C. Upper respiratory tract infection: antibiotics. Clinical Evidence, Issue 4. London: BMJ Publishing Group, 2000. Available: www.clinicalevidence.org 60. Del Mar CB, Glasziou PP, Spinks AB. Antibiotics for sore throat (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2000.

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61. El-Daher NT, Hijazi SS et al. Immediate versus delayed treatment of group A haemolytic streptococcal pharyngitis with penicillin V. Pediatr Infect Dis J 1991; 10: 126-30. 62. Little P et al. Open randomized trial of prescribing strategies in managing sore throat. BMJ 1997; 314: 722-7. 63. Little P, Gould C, Williamson I et al. Re-attendance and complications in a randomised trial of prescribing strategies for sore throat: the medicalising effect of prescribing antibiotics. BMJ 1997; 315: 350-2. 64. Snow V, Mottur-Pilson C, Cooper RJ et al. Principles of appropriate antibiotic use for acute pharyngitis in adults. Ann Intern Med 2001; 134: 506-8.

65. SIGN 34: Management of sore throat and indications for tonsillectomy. Edinburgh: RCPE, 1999. Online. Available: www.sign.ac.uk/guidelines/ 66. Thomas M, Del Mar C, Glasziou P. How effective are treatments other than antibiotics for acute sore throat? Br J Gen Pract 2000; 50: 817-20. 67. Marshall T. A review of tonsillectomy for recurrent throat infection. Br J Gen Pract 1998; 48: 1331-5. 68. Tessier JF, Kulkarni GV. Bad breath: etiology, diagnosis and treatment. Oral Health 1991; 81: 9-24.

19

CHAPTER CONTENTS The gritty irritable red eye 355 The painful red eye that is not 'gritty'

358

Eye problems

Lash, lids and lacrimal problems 358 Visual acuity testing

359

A'cute disturbance of vision 359 Acute painless loss of vision 359 Decreased vision with pain 360 Acute distortion of vision 360 Gradual loss of vision 360 Cataracts 361 Age-related <maeular..degeneration Chronic simple glaucoma 361 Visual handicap References

362

361

THE GRITTY IRRITABLE RED EYE

363

Acute infective conjunctivitis In an adult or child * The history and examination may give grounds for distinguishing between bacterial, viral and chlamydial infection, but not reliably. * Even in proven bacterial conjunctivitis, antibiotics only modestly improve the rate of resolution. Over half will resolve without treatment in 2-5 days.1 If the visual acuity is normal and the cornea is clear, the patient should: * Wipe away discharge. * Instil chloramphenicol eye drops four times a day with ointment at night for 1 week. Fears of bone marrow aplasia2 from topical chloramphenicol seem to be unfounded.3 Fusidic acid and gentamicin are alternatives. * Wash hands after touching the eyes. * Use separate towels. It seems logical to recommend that children should stay away from school even though this is not the recommendation of the Public Health Laboratory Service (www.phls. * If the discharge is purulent, use the drops hourly and return for assessment on alternate days. * If symptoms are still present at 1 week but there are no complications, stop all treatment and review weekly. Adenoviral infection typically takes 2-3 weeks to resolve. 355

356

EYE PROBLEMS

* refer to eye casualty at any stage if the visual acuity is reduced or the cornea is involved. * Orbital cellulitis: admit immediately. In a neonate The likelihood and the dangers of sexually transmitted disease are greater. * If the eye is mildly sticky: take a swab and review. * If the discharge is purulent: take swabs for viral, bacterial and chlamydial infection and a smear for gonococci. Then start chloramphenicol eye drops hourly and refer for an ophthalmic opinion the same day. * Admit for intensive antibiotic therapy if: (a) herpes simplex, chlamydia or gonococcus is grown; or (b) the clinical situation worsens, with a red eye, cloudy cornea, blepharitis, marked preauricular lymphadenopathy or an unwell child. * Notify as ophthalmia neonatorum. Instilling into the eye Instilling drops: pull the lower lid down with the patient looking up. Squeeze one drop onto the lower fornix. Instilling ointments or gels: as above but squeeze 1 cm onto the inner surface of the lid. Warn the patient it will blur the vision for a short while.

Allergic conjunctivitis Acute * Reassure the patient that the eye will not be harmed and that the problem will settle soon. * Give antihistamine drops, e.g. antazoline with xylometazoline. Chronic or recurrent allergic eye disease * Establish, if possible, what the allergen is and reduce or avoid contact. * Use an antihistamine plus a vasoconstrictor topically, e.g. antazoline with xylometazoline but only for short periods. Prolonged use of xylometazoline can lead to rebound hyperaemia when it is

stopped. Use a pure antihistamine instead, e.g. levocabastine or emedastine. * Prophylaxis. Give sodium cromoglycate, nedocromil sodium or lodoxamide drops q.d.s. Benefit may not be seen for 2 weeks. Where symptoms are seasonal, start treatment before the season starts. * Give an oral non-sedating antihistamine if symptoms are still not controlled. * Severe cases not responding to the above: Consider using topical steroids for up to 5 days, but only if: (a) the cornea is monitored for dendritic ulceration; (b) the patient is referred after 5 days if treatment is not successful. More prolonged use carries the risk of raised intraocular pressure. * If a patient worsens dramatically after starting to use drops consider contact sensitivity. The usual cause is the preservative in the drops.

Blepharitis * Reassure the patient that the condition will not threaten sight. Explain that it is caused by an abnormality of secretion from the meibomian glands, and that treatment has to be long-term. * Lid hygiene. Encourage the patient to clean the eyelid margins of debris by gently scrubbing the eyelid margins with a cotton bud dipped in a solution of mild baby shampoo diluted 1:3 with warm water. This should be repeated twice a day until symptoms improve. * Treat patients as if they have dry eyes, as the tears evaporate more rapidly. Use hypromellose by day and a paraffin-containing preparation at night. Do not continue if there is no benefit. * If there is no improvement, give chloramphenical ointment to be applied to the lid margins b.d. for a month for presumed staphylococcal infection. Encourage the patient to continue lid hygiene. * Treat those not still responding and those with acne rosacea, with long courses of oral antibiotics, e.g. oxytetracycline 250 mg b.d. or doxycycline 50 mg o.d. * Consider referral to an ophthalmologist of those: (a) remaining symptomatic despite treatment for a few months;

THE GRITTY IRRITABLE RED EYE 357

(b) who have associated skin disease; (c) where there is suspicion of corneal involvement.

Dry eye * Encourage the patient to use artificial tears as often as necessary. Start with hypromellose 0.3%. Drops may be needed every 30 minutes. Higher strengths may give relief for longer. * Add or substitute products that last longer either: (a) by clinging to the surface, e.g. carbomers; or (b) by increasing the persistence of the tear film, e.g. polyvinyl alcohol. * Prescribe paraffin-containing ointments, e.g. Lacri-Lube, for use at night. * Treat with drops containing acetylcysteine if excess mucus is a problem. * Refer: (a) urgently if there is a sudden increase in discomfort, redness or deterioration in vision. These patients are at increased risk of microbial keratitis; (b) routinely patients who are having to instill drops very frequently. Surgery may benefit those who have not responded to treatment.

Trauma Foreign body

* Suspect a foreign body in any unilateral red eye with or without a history of 'something going into the eye'. * Always fully evert the upper eyelid when looking for a foreign body. If a foreign body is seen, it can usually be removed from the conjunctiva without difficulty. * If there is a history of pain while hammering metal or of exposure to glass splinters, refer to exclude an intraocular foreign body. Corneal foreign body

1. Insert local anaesthetic. Start with benoxinate and when the stinging has stopped, deepen the

anaesthesia with amethocaine 1% drops. Continue until the drops feel cold but do not sting; 2. Remove the foreign body with a cotton wool bud or the tip of a sterile needle. Do not dig into the cornea; at its centre it is only 1 mm thick; 3. After removal, prescribe antibiotic eye ointment b.d. for 2 days as prophylaxis; 4. Give a cycloplegic, e.g. cyclopentolate 1% drops q.d.s. if pain continues; 5. Do not apply an eye pad;4 6. Examine after 24 hours: (a) with fluorescein to be sure the epithelium has healed; (b) to exclude a rust ring if the foreign body was metallic. If present, refer to an ophthalmologist urgently for it to be removed. Chemical bums

These are acid or alkali burns of which alkali (usually in the form of cement, lime, caustic soda or ammonia) are the more serious. * Irrigate with tap water or normal saline for 20 minutes. * Examine the cornea with fluorescein and send to eye casualty if it is not clear. * Cement. Look for cement adhering to the eye, under the lids or in the conjunctival fornices. If found, give local anaesthetic drops (e.g. benoxinate) and remove it, however distressing this is to the patient. * Detergent. If the conjunctiva shows punctate epithelial loss, treat with chloramphenicol and review in 3 days. Ultraviolet light burns

Arc eye is usually due to exposure to UVC through welding or using a sunbed without eye protection. * Give local anaesthetic drops, e.g. benoxinate once, but do not repeat them; they retard corneal healing. * Give cyclopentolate 1% drops q.d.s. for as long as the pain lasts. Paralysing the ciliary muscle will relieve some of the pain.

358

EYE PROBLEMS

* Treat with chloramphenicol drops 2-hourly for 24 hours and 4-hourly for the next 24 hours. * Pad the worst of the eyes. Few patients would tolerate bilateral padding. * Give sufficient sedatives and analgesics to ensure a night's sleep. Opioids are likely to be needed. Cessation of blinking during sleep enables the corneal epithelium to heal. * Advise patients not to drive until they have finished the drops (i.e. at least 48 hours).

for 2 weeks. Refer if not improved within a month. (b) Recurrent uveitis. Treatment with topical steroids can be started, provided: - their use has been previously sanctioned by a consultant; - the visual acuity is normal. (c) a dendritic ulcer is excluded with fluorescein; but arrange for specialist assessment. Ophthalmic supervision is always necessary because intensive topical steroids are usually needed.

Corneal abrasion * Confirm the abrasion with fluorescein dye. * Check that there is no deeper damage to the eye, e.g. hyphaema or retinal detatchment. * Treat with chloramphenicol ointment 2-hourly for 24 hours and then q.d.s. for a further week. * Give a cycloplegic, e.g. cyclopentolate 1% drops q.d.s. if pain continues. * Do not apply an eye pad.4 * Give oral analgesia if the pain is severe. * Refer if the epithelium has not healed after 24 hours.

Problems with contact lenses (a) Corneal abrasion: see above. (b) Bacterial keratitis: see below. (c) Other problems need slit-lamp examination to distinguish them. Advise the patient to leave the lenses out and contact the lens prescriber, or refer the patient to an ophthalmologist. NHS facilities are available to lens wearers if pathological problems have arisen.

THE PAINFUL RED EYE THAT IS NOT 'GRITTY' Most need referral to eye casualty: (a) keratitis, ocular herpes simplex, corneal ulcer, scleritis and uveitis the same day; (b) acute glaucoma immediately.

LASH, LIDS AND LACRIMAL PROBLEMS Acute dacrocystitis * Treat aggressively for presumed staphylococcal or streptococcal infection, e.g. with flucloxacillin and amoxycillin, for 1 week. * Review after 24 hours. If the symptoms are worse, refer for an ophthamological opinion that day. * Refer once recovered, for an ophthalmological opinion and possible surgery.

Chalazion * Advise the patient to use a warm compress, e.g. a face flannel, over the lump and massage towards the lid margin. Antibiotic ointments or drops are traditionally given (on to the eye, in the hope that they will penetrate the chalazion through the conjunctival surface) despite the lack of evidence of benefit. * Treat secondary infection with oral antibiotics (see dacrocystitis). * Review the patient, once recovered, and treat any blepharitis present (see above). * Refer if a hard lump has failed to resolve after 6 months or is particularly troublesome.

The GP may treat:

Styes

(a) Episderitis with a topical NSAID if there is discomfort, e.g. diclofenac or flurbiprofen

* Treat the infected eyelash follicle with topical antibiotics.

ACUTE DISTURBANCE OF VISION 359

Twitching

ACUTE PAINLESS LOSS OF VISION

* Mykomia. Reassure the patient that twitching of a small area of the orbicularis muscle is common and is not sinister. It is often exacerbated by tiredness. * Facial twitching. Refer to a neurologist. * Blepharospasm. Forcible contractions of the entire orbicularis muscle should be referred to an ophthalmologist. Botulinum toxin injections can give short-term relief.

Central retinal artery occlusion

Watery eye

If seen within 24 hours * Arrange for the patient to be seen immediately in eye casualty. If any delay is anticipated: (a) Temporal arteritis. If the history and examination suggest temporal arteritis give 200-300 mg of hydrocortisone or 500 mg of methylprednisolone by slow intravenous injection. (b) Give aspirin 300 mg (unless contraindicated).

This results from the involution of the nasolacrimal duct. * Treat any infection with topical antibiotics. * Refer patients who are bothered by it to an ophthalmologist. They may benefit from creation of a new passage between the lacrimal sac and the nasal mucosa. Massage of the lacrimal sac does not help.

VISUAL ACUITY TESTING Tests of visual acuity have a reliability of >98%, but only if the following steps are taken: (a) The Snellen chart should be the recommended distance from the patient for the size of chart used. The distance should be marked and the patient should stand behind the mark. (b) The chart should be illuminated with 480 lux (e.g. a spotlight). One study found that few practices fulfilled these criteria.5

If seen after 24 hours There is no treatment for the eye and the object is to try to prevent retinal artery occlusion of the other eye. * Refer to an ophthalmologist but less urgently. * Give aspirin 75 mg daily. * Take blood for an ESR. If the clinical picture suggests temporal arteritis, give steroids while awaiting the result. * Look for evidence of more widespread cardiovascular disease. Check blood pressure, fasting blood sugar and lipids. * Consider the possibility that the occlusion was embolic. If so, refer to a vascular surgeon.

Central or branch retinal vein occlusion Work-up: (a) BP; (b) FBC for polycythaemia and leukaemia; (c) Serum lipids; (d) Blood sugar.

ACUTE DISTURBANCE OF VISION * Establish that the loss is acute, and not just sudden awareness of a pre-existing field loss, or cataract. * Distinguish transient from continuing visual losses and treat as appropriate. For migraine, see p. 189; for TIA, see p. 200.

* Refer to eye casualty. The patient may need photocoagulation for chronic macular oedema and ischaemia following a branch vein occlusion. Even if no treatment is available, as in central vein occlusion, complications can develop early. 50% develop neovascular glaucoma within 3 months.

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EYE PROBLEMS

Vitreous haemorrhage * Refer for an ophthalmological opinion within 24 hours to establish the cause of the bleeding. Retinal detachment will require surgical removal of the vitreous. Posterior vitreous detachment is self limiting. * Check BP, INR (if on warfarin) and fasting blood sugar.

Posterior vitreous detachment and retinal detachment * Refer to be seen that day if a patient reports the sudden appearance of a number of floaters. Assess the visual acuity before referral. A reduced visual acuity may indicate a retinal detachment and increases the urgency.

DECREASED VISION WITH PAIN Acute closed angle glaucoma * Refer urgently to eye casualty or admit. * Treat nausea and vomiting with prochlorperazine 12.5 mg im. * Do not attempt to dilate the pupil of anyone with suspected acute angle glaucoma.

Retrobulbar neuritis * Progressive reduction in vision over a few days with pain on eye movements. Colours appear washed out. Symptoms may be worse after a hot shower or bath. * Improvement occurs within 2 weeks and full recovery is usual within 3 months. * Between 50 and 70% aged 20 to 40 with retrobulbar neuritis in the UK will develop multiple sclerosis, almost all of them within 5 years. A second episode increases that risk fourfold.6 * Refer urgently patients having their first attack. Arrange for the patient to be seen again if symptoms are not improving within 2 weeks. * Refer patients with a second attack to a neurologist because of the chance that this is multiple sclerosis.

* Treat subsequent attacks with steroids if they seemed helpful previously; only refer if the attack fails to resolve.

ACUTE DISTORTION OF VISION * Refer urgently. Older patients may have acute wet age-related macular degeneration, especially if that diagnosis has already been made in the other eye. Typically they report that straight lines appear crooked. If vision is still better than 6/18 they may benefit from laser treatment, provided they are seen within 10 days of the onset of symptoms.

Floaters and flashes * Refer patients who report: (a) multiple floaters, especially if associated with flashing lights. They may have a posterior vitreous detachment, which carries a 5% chance of causing a retinal tear; (b) the appearance of a single large blob, which then fragments. They may have a vitreous haemorrhage. * Be more ready to refer if: (a) there is a family history or a personal history of retinal tear or detachment; (b) the patient has high myopia; (c) there is a history of cataract surgery; (d) there is a field loss or decrease in acuity. These imply that retinal detachment has occurred. It may be visible ophthalmoscopically. Note: Retinal tears are unlikely to be detected ophthalmoscopically. A normal fundus is therefore no grounds for inaction.

GRADUAL LOSS OF VISION * See if vision improves when looking through a pinhole. If it does, then a refractive error is at least part of the problem. * Suggest the following to all patients with visual difficulty: (a) use adequate lighting (e.g. an angle-poise lamp), especially for reading and close work;

GRADUAL LOSS OF VISION

(b) rearrange the room to sit with the main window behind the patient and the television away from the window. * Recommend the See for yourself leaflets available from the RNIB. Website: www.rnib.org.uk and search for 'leaflets'.

CATARACTS Systematic review: Management of cataract. Effective Health Care 1996; 2(3).

* In a child, refer urgently. * In a younger adult, refer. * In the elderly: (a) Exclude retinal pathology. If in doubt, refer to an ophthalmologist so that the presence of coexisting eye disease can be detected before the cataract obscures the fundus. (b) Assess visual acuity. If this improves with a pinhole the patient may benefit from a change in refraction. (c) Exclude diabetes. (d) Refer to an ophthalmologist when loss of vision is interfering with the quality of life. For most people this occurs when the visual acuity (VA) in the better eye is less than 6/18. Glare will result in a visual acuity worse than that tested in the surgery. Those wishing to continue driving, or who are engaged in work or hobbies which involve fine work, will need surgery earlier. (d) Refer patients for surgery on the other eye if it is affected. The benefit is likely to be nearly as great as from surgery on the first eye. (e) Surgery. If VA falls after surgery, refer the patient back. Twenty per cent develop opacification of the posterior capsule within 2 years of operation. They need laser treatment.

AGE-RELATED MACULAR DEGENERATION This occurs in elderly patients who present with progressive loss of central vision and, frequently, distortion effects. The vision is best in dim light. The eye appears normal except for the macular changes.

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* Refer all patients for specialist assessment: (a) to confirm the diagnosis and to assess suitability for laser photocoagulation. Refer urgently those with sudden deterioration (see p. 360). (b) to provide low vision aids; (c) to consider registration as visually handicapped. * Reassure patients that although they have lost some of their central reading vision they will never be completely blind. Patient information: Macular Degeneration Network. Online. Available: www.macular-degeneration.org

CHRONIC SIMPLE GLAUCOMA • Loss of visual acuity is a late sign of chronic glaucoma. By this stage, disc cupping and field loss are likely to be advanced. • Fundoscopy for glaucomatous cupping is the most effective single screening manoeuvre, and is easily performed by GPs. Pathological cupping is: (a) a pale disc with a sharp rim; (b) oval vertically; (c) asymmetrical; (d) characterized by a cup: disc ratio of 1:3 or more, i.e. the wider the cup the more likely it is to be pathological; (e) associated with angulation and nasalization of blood vessels. • The incidence rises with age. In the UK it is 1 in 5000 at age 40-49 years, and only really rises over age 60. Over 85, it is 1 in 10. • The risk increases: (a) with a family history (X10); (b) in high myopes (X3); (c) in diabetics (X3); (d) in African Caribbeans; (e) in users of topical steroid drops for over 5 days. • Family history. Screen those with a first-degree relative with glaucoma as follows, or ensure an optician screens them. They are entitled to free NHS eye tests once they are 40 years old: (a) age 45-65: 5-yearly; (b) age 66-75: 2-3-yearly; (c) age 76 onwards: 1-2-yearly.

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EYE PROBLEMS

* Screen others over 45 every 5 years, especially those in the other high-risk groups. * Refer patients with: (a) intraocular pressure (IOP) >26mmHg. Patients without cupping or field loss do not necessarily have glaucoma. The majority have ocular hypertension and only develop glaucoma at the rate of 1% per year; (b) IOP 22—25mmHg with pathological cupping or loss of visual fields; (c) pathological cupping, even if the pressures are normal. Cupping occurs with normal pressures in one-fifth to one-third of patients with glaucoma; (d) visual field loss that persists on repeated testing, even with no other signs. * Ensure follow-up of patients with an IOP of 22-25 mmHg but no evidence of glaucoma. They are seven times more likely to develop glaucoma than patients with lower pressures.7 * Driving. Advise the patient to notify the DVLA, if there is significant bilateral visual field loss.8 Patient information: International Glaucoma Association, 108c Warner Road, kondran, SE5 9HQ, tel 020 7737 3265; www.iga.org.uk

• Treatment is managed by the hospital eye department It is helpful to know that: (a) the aim is to keep the pressure below 21 mmHg; (b) timolol eye drops can exacerbate asthma and heart failure; (c) pilocarpine eye drops frequently cause headaches, but they tend to disappear after the first few weeks of use; (d) pilocarpine also causes miosis, and so poor night vision.

• Blind registration is for those with a VA below 3/60, or with a VA of up to and including 6/60 but with gross visual field restriction. • Registration as partially sighted is not defined by statute. It is appropriate for patients with VA of 6/60 or worse with full visual fields, or those with a VA better than that but with restriction of visual fields. A patiertt with a homonymous hemianopia, for instance, is likely to be eligible.

Benefits Registration means that a social worker will contact the patient and provide: (a) access to a mobility officer, a teacher of braille, and daily living courses; (b) free radio and cassette aids; (c) details of the RNIB talking-book service. In addition, the registered blind (but not necessarily the partially sighted) are eligible for: (a) a slightly higher rate of income support; (b) extra Housing Benefit, Income Support, and Council Tax Benefit; (c) increased income tax allowance; (d) reduced fares.and a Blue parking badge; (e) free sight tests; and (f) reduction in the TV licence fee. The Royal National Institute for the Blind (RNIB) provides information, support and advice for anyone with a serious sight problem, tel. 020 7388 1266; website www.rnib.org.uk; helpline 0845-766 99 99 (Interpreters are available);,e-mail [email protected] Textphone users can call via Typetalk 0800 515152. RNIB Customer Services provide publications, equipment, etc. PO Box 173, Peterborough PE2 6WS, tel. 0845 702 3153.

Prescribing for patients with contact lens9 VISUAL HANDICAP

Registration • Registration is by a consultant ophthalmologist on form BD8, which is submitted to the Director of Social Services.

Topical applications Avoid: (a) eye ointments; (b) eye drops containing preservatives (in soft lenses); (c) topical steroids;

REFERENCES 363

(d) coloured drops: fluorescein and rose bengal; (e) sympathomimetics, Systemic drugs Avoid: (a) drugs that reduce or alter tear secretion: oral contraceptives, HRT, drugs with anticholinergic

activity (e.g. tricyclic antidepressants), betablockers, diuretics, isotretmein; (b) drugs that reduce blinking: benzodiazepines; (c) drugs that stain the lens: nitrofurantoin, rifampicin, sulfasalazine; (d) drugs that are concentrated, in the lens and can irritate the eye: aspirin.

REFERENCES 1. Sheikh A, Hurwitz B. Topical antibiotics for acute bacterial conjunctivitis: a systematic review. Br J Gen Pract 2001; 51: 473-7. 2. Doona M, Walsh JB. Use of chloramphenicol as topical eye medication: time to cry halt? BMJ 1995; 310: 1217-8. 3. Lancaster T, Swart AM, Jick H. Risk of serious haematological toxicity with use of chloramphenical eye drops in a British general practice database. BMJ 1998; 316: 667. 4. Easty DL. Is an eye pad needed in cases of corneal abrasion? BMJ 1993; 307: 1022.

5. Pandit JC. Testing acuity of vision in general practice: reaching recommended standard. BMJ 1994; 309: 1408. 6. Kanski JJ. Clinical ophthalmology, 2nd edition. London: Butter-worths, 1989. 7. Crick RP, Tuck MW. How can we improve the detection of glaucoma? BMJ 1995; 310: 546-7. 8. Potamis T et al. Driving, glaucoma and the law. BMJ 1994; 309: 1057-8. 9. Mitchell R, Edwards R. Prescribing for patients who wear contact lenses. Prescriber 2001; July: 40-4.

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20

CHAPTER CONTENTS Eczema and dermatitis

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Seborrhoeic eczema and dandruff Acne 368 Acne rosacea Psoriasis

Skin problems

368

370

370

Skin infections 373 Viral infections 373 Herpes simplex labialis Herpes zoster 374 Bacterial infections

373 374

Fungal infections 375 Fungal infections of nails Lichen planus 376 Alopecia 376

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Skin diseases can be the easiest and the most difficult conditions to manage in general practice. Because the pathological process is so easy to inspect it can be easy. Once a diagnosis is reached, treatment is usually obvious. However, many skin eruptions are the common final pathways of many disparate causes.

Skin infestations 377 Scabies 377 Lice 377 Papular urticaria 378 Malignancy 378 Basal cell carcinomas 378 Squamous cell carcinomas 379 Melanoma 379 Solar keratoses 379 Hyperhidrosis 380 Generalized hyperhidrosis 380 Hyperhidrosis of the palms, soles and axillae Gravitational ulcers Urticaria

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References

381

380

ECZEMA AND DERMATITIS 380 Systematic review: Charman C. Atopic eczema. In: Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org Guidelines: Primary Care Dermatology Society. Guidelines for the management of atopic eczema 2000. Online. Available: www.eguidelines.co.uk McHenry PM, Williams HC, Bingham EA for the British Association of Dermatology and the Royal College of Physicians Working Party. Treatment of atopic eczema. BMJ 1995; 310: 843-7.

• The rash of acute eczema is weepy and excoriated, with indistinct margins. In atopic eczema the distribution is symmetrical and can be widespread. It has a predilection for the flexures of the elbows and knees, plus the face in infants. • Acute dermatitis from contact with irritants is initially confined to the area in contact with the offending agents, but can spread to adjacent areas. • Chronic dermatitis presents as thickened, erythematous scaling skin. Both the acute and chronic forms are itchy. Scratch marks can be a prominent 365

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SKIN PROBLEMS

feature of the clinical appearance, as can lichen planus (see below). • Either type of rash can occur in sun exposed skin when certain drugs have been taken (e.g. thiazide diuretics, allopurinol). • Other body systems may be involved, particularly the respiratory system (hay fever and asthma) and the gut in infants (cows milk allergy). • Intrinsic predisposing factors include varicose veins (where stasis in the lower limb may cause accumulation of waste products). • Persistent environmental provocation - particularly to chemicals at work and home - can cause dermatitis. Organic chemicals such as petroleum products, soaps and detergents are common culprits, as are metals (especially nickel, the metal used in much jewellery).

Management * Controlled studies support the efficacy of topical steroids as the primary treatment of atopic eczema. * Evidence for the use of emollients is less strong; they have rarely been subjected to rigorous trials.1 * There is insufficient evidence to recommend evening primrose oil.1 Evidence for many other interventions is poor. * Find out how the eczema affects the patient's life. Over 50% of eczema sufferers feel that the eczema interferes with their life. * Explain the nature of the condition and establish the patient's expectations. A complete cure is not realistic, but treatment should give good control and an acceptable quality of life. 60% of children with atopic eczema have grown out of it by adolescence.2 * Stress the importance of the correct use of emollients or topical steroids. Explain that the dangers of topical steroids are associated with the potent and very potent preparations and, especially, with their prolonged use. * Advise the patient to return if control deteriorates. * Find out if there are trigger factors that can be avoided: wool, heat, cold, exercise, preparations containing lanolin and emotion.

* Hand dermatitis. Ask about occupational exposure to solvents, petroleum products, detergents, non-ferrous metals and latex. Ask about washing up, peeling fruit and vegetables, handling polishes and solvents and other irritants, and even about shampooing the hair. Avoidance of irritants involves keeping the hands dry and warm and wearing cotton gloves for handling solids, and cotton-lined rubber gloves for handling liquids.

Emollients * Explain that some emollients (e.g. white soft paraffin) work by providing a lipid film on the skin, which slows the water loss from it. The most effective time to apply them is after a bath, when the water content of the skin is greatest. Others (e.g. aqueous cream) work by adding water to the skin and may be applied at any time. Urea helps water bind to the skin. * Encourage the patient to apply emollients as freely as possible and as frequently as reasonable. A child with widespread eczema will need at least 250 g per week and an adult 500 g per week. The quantity of emollient used should be >10 times that of topical steroid. * Use the oiliest preparation that the patient can tolerate. The patient may need one preparation for the body and a less oily one for the hands and face. * The patient should use a bath oil and not soap.

Topical steroids * Safety. Hydrocortisone 1% (cream or, preferably, ointment) is safe for all ages and at all sites bar one: the sole exception is the eyelid, where prolonged use may cause glaucoma. Potent preparations should not be prescribed for children, nor for flexures or the face. Their use should be restricted in sites prone to striae; the breasts, abdomen, upper arms and thighs. * Rates of absorption vary according to site: scrotal skin absorbs 40 times more than the palm, with the forehead, axilla, and back absorbing 6 times, 4 times and twice as much, respectively. * Steroid strength. More potent steroids should be used as a course of treatment to cope with an

ECZEMA AND DERMATITIS

exacerbation, rather than as maintenance (see also Infection, below). * Prescribe enough: to cover the hands twice daily for a month, an adult needs at least 60 g. To cover the trunk or both legs for the same time, 400 g.3 * Prescribe within the following maximums: moderately potent, 100 g per week; potent, 50 g per week; very potent, 25 g per week. * Base. Use ointments and not creams, except where the patient cannot tolerate the greasiness of ointments. In that case, use an ambiphylic cream such as Locoid Lipocream. * Pompholyx is relatively resistant to steroids. Use a potent or very potent steroid. Consider occlusion with plastic gloves or bags. * The face. Restrict steroids to 1% hydrocortisone. Stronger preparations may cause telangiectasia. * Amounts. Patients need to be told how much cream or ointment to apply. Explain the concept of the fingertip unit (FTU); the amount of cream or ointment that covers the finger from tip to distal crease. 1 FTU (= 0.5 g) should cover an area equivalent to the palms of two adult hands.

Acute exacerbations * Infection is often the cause of an exacerbation, whether or not there is clinical evidence of it. S. aureus is the most frequent pathogen, colonizing 80% of patients with eczema. Use oral antibiotics, e.g. flucloxacillin or erythromycin. Continue them until the exacerbation is over. * Herpes simplex. Discuss with a dermatologist or arrange for the patient to be seen urgently. * Excoriations: (a) Paste bandages are helpful in excoriated or lichenified eczema. Tar bandages are most effective and messiest; ichthammol, or zinc and calamine, may be more acceptable. Bandages can be put on at night on top of steroid ointment. In children they prevent scratching. (b) Gloves may need to be worn in bed as well. (c) 'Wet wrapping' may be helpful. Soak a tubigrip bandage or tubular gauze dressing in emollient, apply it over the eczema, and cover it with a dry bandage.

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* Steroids: Use a short course of increased strength, as above. If the skin is moist, use a cream rather than an ointment, to permit evaporation.

Second-line treatments Antihistamines Sedative antihistamines will help children sleep, but they have no specific effect on itch.1 If using an antihistamine for sedation, use the highest recommended doses; lower doses often stimulate them. If going above the recommended doses, increase to that level slowly. Regular use leads to tolerance. Use on alternate nights, or use for 2 weeks and then omit for 2 weeks. Exclusion diets These may help a small number of children, although what studies there are have failed to show benefit.4 Foods commonly implicated are milk, eggs, citrus fruits, colourings, preservatives, nuts, fish, wheat, tomatoes, lamb, chicken and soya! If undertaken at all, an exclusion diet must be conducted under the supervision of a dietician to avoid malnutrition. House dust mite Controlling the house dust mite may improve eczema but the reduction in dust levels must be extreme, with synthetic mattress covers, spraying and high filtration vacuuming.4 Oral steroids Oral corticosteroids can be used as rescue therapy while waiting for an urgent consultant opinion. Patients should not be maintained on steroids or courses of steroids for prolonged periods.

Referral Refer patients when: (a) the diagnosis is uncertain; (b) there is failure to respond to mildly potent steroids in children;

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(c) there is failure to respond to potent steroids in adults, or where regular courses of these steroids are needed; (d) when specialist assistance would be valuable in counselling patients and the family; (e) for confirmation of contact allergy where job or lifestyle is at stake; (f) there is a severe acute exacerbation; (g) there is bacterial infection that has not responded to a course of oral antibiotics; (g) there is eczema herpeticum.

Patient organizations: The National Eczema Society, Hill House, Highgate Hill, London N19 SNA, tel. 020 7281 3553; helpline 0870 241 3604 (Mon to Fri 1-4.00 p.m.), www.eczema.org Eczema Association of Australia Inc. PO Box 1784 DC, Cleveland, Queensland, 4163, tel. 07 3821 3297; www.eczema.org.au

SEBORRHOEIC ECZEMA AND DANDRUFF Infants Reassure the parents that the majority of children are better by the age of 1 year. * Cradle cap. Advise the parent to soften skin scales with arachis or olive oil and gently scrape them off. * Use emollients freely, and mild steroids if erythema is marked (but not on the face).

Adults * Use emollients. * Treat with topical hydrocortisone 1% for 4-6 weeks. If there is no improvement, treat with ketoconazole cream twice daily for 4 weeks. * Scalp. Treat with selenium sulphide shampoo or ketoconazole shampoo twice weekly for 2-4, weeks.

ACNE Guideline: MeReC. The treatment of acne vulgaris: an update. MeReC Bulletin 1999; 10(8): 29-32.

* Explain to the patient what is known about the aetiology of the condition: (a) an increase in sebum production; (b) keratinization of the follicle, resulting in lesions that are not inflamed (blackheads, whiteheads); (c) colonization of the pilosebaceous unit with Propionobacterium acnes; (d) development of inflammation. * Explain to the patient that the aims of treatment are to: (a) prevent scarring, by reducing noninflammatory and inflammatory lesions; (b) reduce the unsightly appearance of the disease; and so (c) reduce the psychological stress and social morbidity. * Explain that the treatment may take several months to work, and that the patient should re-attend until the condition is satisfactory. Encouragement is important. In one study, 80 out of 120 patients admitted they were noncompliant at 3 months.5 * Assess the type of acne and treat accordingly: (a) Comedone-dominated acne is best managed by keratolytic activities such as frequent washing (but not scrubbing, which can increase the inflammation6), or use of topical benzoyl peroxide, azelaic acid or topical retinoids. Sunlight is effective, but of course risks increased skin cancer in later life. (b) pus-dominated acne responds to topical or oral antibiotics. Sometimes patients must take them for many months to maintain a remission.

Details of treatment * Benzoyl peroxide: (a) Start at a low strength, i.e. 2.5%, and apply at night;

ACNE

(b) Warn the patient that mild erythema and transient skin irritation are common, and that it may bleach clothes; (c) Increase the strength gradually to 10% at 2-weekly intervals, but stop at any stage if irritation occurs. * Topical antibiotics work by reducing P. acnes. They are no more effective than benzoyl peroxide or tretinoin, but cause less skin irritation.7 Topical tetracycline is the cheapest, but patients who frequent clubs should know that it will fluoresce under ultraviolet light. * Azelaic acid cream is keratolytic, alters the fatty composition of skin lipids and reduces bacterial concentrations. It avoids the problem of bacterial resistance, but can cause local irritation and photosensitization. Use it daily for 1 week then twice a day for at least 8 weeks, but for no more than 6 months.8 * Topical retinoids. (a) Start at a low strength (0.025%) every other night. Warn the patient to expect redness and scaling. When it is clear that it is tolerated, increase to nightly, then b.d., then slowly increase the strength of the preparation until a response is seen. (b) Use the cream on dry and fair skin, the gel on dark and oily skin, especially in severe acne, and the lotion for large areas such as the back; (c) Avoid direct sunlight and UV lamps. Do not use in pregnancy. * Oral antibiotics may be added to benzoyl peroxide or retinoids, but to reduce the risk of resistance, should not be combined with a different topical antibiotic. (a) Use oxytetracycline 500 mg b.d. or Ig daily, for 3 months in the first instance. (b) Warn the patient that: - improvement is unlikely before 6 weeks, and it continues for 6 months; - strict compliance is essential; -it must be taken at least half an hour before food and, especially, not taken with milk. (c) If effective, decrease to 500 mg daily then 250 mg daily, at 3-monthly intervals.

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(d) Reassess 6-monthly whether to stop for 2 months to see if the problem has resolved. If the condition recurs, restart the original antibiotic. (e) If a patient responds initially and then worsens, bacteria may have developed resistance. Change to another antibiotic or to azelaic acid cream. Alternative antibiotics are erythromycin 250-500 mg b.d., trimethoprim 100-200 mg b.d or doxycyline l00 mg daily. Because of fears of hepatitis and pneumonitis, minocyline should be reserved when other antibiotics have failed. Even then there is no evidence that it is better than other antibiotics.9

Referral * Refer patients with: (a) severe acne and acne still failing to respond; (b) acne cysts, for consideration of intralesional steroid injections; (c) psychological distress despite full primary care management. Treatment options include isotretinoin, UV therapy, and high-dose antibiotics. Isotretinoin is very expensive, is teratogenic and often causes cracking of the lips and facial dermatitis. * Consider starting antibiotics whilst awaiting the appointment. Hormonal treatment in women * Consider changing women who are on combined oral contraceptives with progestogens that may aggravate acne (i.e. those containing norethisterone or levonorgestrel) to one containing a less androgenic preparation, e.g. desogestrel, gestodene or norgestimate. They should be counselled about the risks of thrombosis (see pp. 228, 230). * Consider using cyproterone acetate and ethinylestradiol (Dianette) in women, whether or not they need oral contraception. Note: (a) it is not licensed as a contraceptive but is effective as such; (b) if applicable, write 'for contraceptive use' on the prescription to avoid a prescription charge;

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(c) treatment is necessary for several months before benefit is seen.

Self-help group: Acne Support Group, PO Box 230, Hayes, Middlesex. UB4 OUT, tel. 020 8561 6868; www.m2w3.com/acne

PSORIASIS Review: Naldi L, Rzany B. Chronic plaque psoriasis. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org Guideline: Recommendations for the initial management of psoriasis. London: British Association of Dermatologists, 1997. Online. Available: www.bad.org.uk

ACNE ROSACEA • This eruption occurs on the face in the middle aged. Some patients report persistence of a flushing tendency that they had in their youth. • The redness is often inflamed and is accompanied by dilated blood vessels. • There is often hypertrophy of the skin adnexae. The sebaceous glands may form rhinophyma or 'strawberry' nose.

Management * Treatment, unexpectedly, is best with tetracyclines (administered in the same way as for acne, see above). * Steroids usually make things worse (after an apparent initial response from rebound erythema), and should be avoided. * Explain that it should be possible to get rid of the papules and pustules, but often the erythema and telangectasia remain. * Encourage the patient to avoid triggers if possible, e.g. caffeine, alcohol, temperature changes, sunlight. * Burning. Prescribe emollients to reduce the burning sensation. * Oral antibiotics. Long-term oxytetracycline is extremely effective. Start with 500 mg b.d. or Ig daily for 2 months. Half or a quarter of this dose is often sufficient for maintenance. * Topical antibiotics. Topical metronidazole is as effective as oral tetracycline, but much more expensive. Topical clindamycin has been shown to be effective, but is not currently licensed for use in this condition.

• The characteristic lesion of psoriasis is a thickened plaque of skin that may include prominent scale. If scale is not present, the lesions are thickened and shiny, and have a well-demarcated edge. • The lesions present in a wide variety of locations. Most common is the presence of large plaques over the extensor aspects of the elbows and knees, and in large areas of the scalp. Large plaques can occur virtually anywhere in the body, except the face. Smaller lesions can occur over virtually any body surface except the face. • If lesions occur as multiple lesions, it is called guttate (drop-like) psoriasis. Plaques of psoriasis can recur in areas of previous skin trauma (the Koebner phenomenon). Pustular psoriasis is a form with small sterile pustules within the plaques.

Assessment • Look for systemic disease, notably inflammatory arthritis. The small joints of the hands are most commonly involved. However, other larger joints (singly or symmetrical), and the spine can be affected. Psoriasis is commonly associated with nail pitting. • Consider the presence of trigger factors, notably trauma, beta-haemolytic streptococcal sore throat, some drugs (beta-blockers, antimalarials and lithium), and emotional upset.

Management • Emollients and coal tar preparations reduce psoriatic activity. • Topical steroids improve psoriasis in the short term. These need to be fluorinated steroids,

PSORIASIS 371

e.g. 0.05% betamethasone valerate used twice daily. In the longer term, while there is a trade-off between benefit and harms, steroids can reduce or nearly eliminate psoriasis in 60% compared to 20% with placebo. • Other agents which can be used include calcipotriol (44% improvement), topical retinoids (tazarotene), dithranol, methotrexate, cyclosporin, phototherapy and psoralens with UVA (PUVA). Each has either short-term difficulties or long-term harms that may limit their usefulness (Table 20.1). Patients with mild to moderate psoriasis can usually be managed in primary care, although the patient may appreciate a single specialist appointment to discuss the condition. Do not delay treatment while waiting for a dermatological opinion. * Explain that psoriasis is common, that it is likely to be chronic with remissions and relapses, that it is not contagious and that the intensity of the treatment will depend on how hard the patient wants to work at it. Table 20.1 Potential harms in selected treatments for psoriasis known to confer benefits Treatment

Potential harm

Steroids

Skin atrophy, striae, telangectasia, adrenocortical suppression

Emollients

Local irritation and contact dermatitis Local irritation and contact dermatitis Smell, staining and local burning Perilesional irritation from calcipotriol

Coal tar preparations Dithranol Topical calcipotriol (± local steroids) Oral retinoids

Phototherapy PUVA Methotrexate

Cyclosporin (oral) (topical)

Mucocutaneous affects: skin dryness, cheilosis, conjunctivitis. Low grade hepatotoxicity (1%), spinal ligament thickening, teratogenicity Photo-ageing, increased skin cancer risk Increased skin cancer risk Dose-related myelosuppression, liver fibrosis and pulmonary toxicity. Renal dysfunction, hypertension Possibly neither renal dysfunction or hypertension

* Look out for the psychological impact the psoriasis is having and refer for counselling those not coping well. * Prescribe the following general measures for all patients who want treatment, regardless of what other treatment they are using: (a) a soap substitute, e.g. aqueous cream; and (b) a tar-based bath additive, e.g. coal tar solution BP; and (c) an emollient to be used after bathing. Use the oiliest preparation that can be tolerated. This usually means using an oily preparation, e.g. emulsifying ointment, for the body and a cream, e.g. aqueous cream for the hands and face. Prescribe enough. A patient with widespread psoriasis may need 500 g of emollient per week.

Localized plaque psoriasis * Warn the patient that no benefit will be seen for at least 3 weeks. * Choose between: (a) A tar-based cream. Small plaques may respond to a 1-5% proprietary coal tar preparation once or twice a day but large thick plaques need a strong coal tar dressing, e.g. 7.5% coal tar paste BP, covered with a dressing. Few patients are prepared to tolerate this when there are other more effective and convenient treatments. (b) A tar and steroid mixture. This is a halfway house, which attempts to reap the benefits of tar and steroids while using low strengths of both in order to reduce adverse effects. It may suit some patients with mild psoriasis. (c) Short-contact dithranol. This is more effective than coal tar, but harder work. Start at 0.1% for creams and 0.5% for wax sticks. Apply daily for 30 minutes, then wash off and apply an emollient to stop the skin drying out. Longer contact is no more effective. Increase the strength by doubling it weekly, up to 2%, provided 'burning' does not occur. If there is 'burning' omit the dithranol for a few days, use emollients until the erythema subsides, then restart dithranol. Warnings: -do not apply to sensitive skin, e.g. the flexures or the face, and avoid putting it on normal skin around the plaques;

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-it stains! Rinse it off the skin, and the bath, afterwards and wear an old dressing gown during the 30 minutes that the dithranol is applied. Wash hands at the end of each day's treatment. (d) A vitamin D analogue, e.g. calcipotriol or tacalcitol. They are easier to use than dithranol but more expensive. They do not stain but, like dithranol, cannot be used on the face or flexures. The maximum total dose of calcipotriol is 100 g per week. Tacalcitol should be restricted to two courses of 12 weeks each per year. (e) A topical steroid. This is the first-line treatment for the face or flexures. Use 1% hydrocortisone on the face but use a moderately potent steroid elsewhere, e.g. 0.025% betamethasone valerate. Other than the face and flexures, steroids are not first-line because of the problems of rebound exacerbation and skin thinning (see box below). Restrict use to l00 g per month and impose regular breaks from use.

the advantage of combining a steroid with salicylic acid, which helps get rid of scale. * Severe crusting. Use a coconut oil compound (Cocois) every night, washed out with a tar shampoo in the morning. It is messy and smelly, will need to be covered with a shower cap to avoid staining the pillow, and should be reduced to twice weekly applications as soon as a response is achieved.

The hairline * Use a moderately potent, or even a potent steroid cream, with a warning that it must not be used on the face.

Soles and palms * Use a potent steroid ointment possibly combined with salicylic acid, e.g. Diprosalic ointment. Response is likely to be poor.

Guttate psoriasis Guidelines for the use of topical steroids in psoriasis From: Greaves MW. Current management of psoriasis. J Dermatol Treat. 1997; 8: 35-6. * Do not use steroids for more than 4 weeks without review. Do not use potent steroids regularly for >7 days. Do not give repeat prescriptions without review. Do not give >100g per month of a moderately potent teroid. Try to rotate steroids with alternative treatments. Only use potent or very potent steroids under specialist supervision.

Guttate psoriasis should resolve in a few months without treatment. If treatment is needed: * Use an emollient for dryness. * Use a mild tar and steroid cream or calcipotriol to suppress the lesions if the patient prefers a more active approach. * Refer early for phototherapy if the psoriasis is widespread and troubles the patient.

Referral Refer when:

Widespread plaque psoriasis * Refer. Systemic treatment will probably be needed.

Scalp psoriasis * Shampoo daily with a tar shampoo, e.g. Polylar. * Exacerbations. Continue coal tar shampoo and also apply a daily steroid scalp application or calcipotriol scalp solution twice a day (but not prior to shampooing). Diprosalic scalp application has

(a) the diagnosis is uncertain; (b) the psoriasis is severe; (c) the psoriasis fails to respond to the above treatments; (d) the patient needs more intensive education or counselling than is possible in primary care; (e) there is psoriatic arthropathy; (f) there is widespread guttate psoriasis; (g) there is generalized pustular or erythrodermic psoriasis. Referral is urgent: it may be lifethreatening. It is especially likely to occur if potent steroids are used and then withdrawn.

SKIN INFECTIONS

Patient groups: Australia: The Psoriasis Association Inc. 16 Musgrave Street, Kirra, Queensland 4225, tel. 190 2272 100 or e-mail [email protected] New Zealand: The Psoriasis Association of New Zealand Inc. Online. Available: www.everybody.co.nz/support/psoriasis.html l/AOThe Psoriasis Association. 7 Milton Street, Northampton NN2 7JG, tel. 01604 711129.

SKIN INFECTIONS VIRAL INFECTIONS Warts (other than genital warts) Systematic reviews: Gibbs S, Harvey I, Sterling JC et al. Local treatments for cutaneous warts (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2001. DTB. Tackling warts on the hands and feet. Drug Ther Bull 1998; 36: 22-4.

• Human papilloma virus (HPV) causes a wide range of different lesions, from cauliflower-like lesions to flat mosaic lesions. Many wart infections are completely asymptomatic, yet shed virus to transmit them. • Genital warts are of greatest concern because they are associated with malignancy, particularly some subtypes with cervical cancer. • Warts will probably always remit spontaneously but the time to remission may be very variable. • First-line topical treatment remains salicylic acid. Between 67% and 84% of warts are cleared by 3 months of daily treatment.10 • Surgical treatment includes of a wide range of destructive methods, including cryotherapy cautery, laser treatment, and excision. They may be no more effective than chemical methods. • There is not enough evidence to establish whether any one is superior to another (either in establishing cure or reducing the chance of recurrence, which ranged from 5-35%), or that treatment reduces infectivity (among genital warts).

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* If the patient is not troubled by the wart, suggest that it is left alone. The majority will disappear spontaneously as immunity is gained. However, if the patient elects for treatment, explain that the wart must be rubbed down with an emery board before application of a topical agent. Pare down thick warts with a scalpel in the surgery. * Choose agents from the following groups. Explain that 3 months treatment may be needed and that the agent should not touch normal skin. (a) salicylic acid for a wart anywhere except on the face; (b) podophyllin, glutaraldehyde or formalin for plantar warts. * Plantar warts. Prescribe a salicylic acid gel for children who wish to use swimming pools, and give them a note to confirm that they do not need a verruca sock. * Filiform warts. Curette them under local anaesthetic. * Plane warts should be left to resolve spontaneously. * Surgery. Warts not responding to treatment after 3 months, or those that are a particular nuisance, are candidates for freezing or curettage and cautery. Children under 10 tolerate these procedures badly.

Referral Refer: (a) if the diagnosis is uncertain, especially if sites other than hands, feet or face are affected; (b) if the patient is immunosuppressed; (c) when there is a sudden widespread eruption of warts. Malignancy may be the cause; (d) warts that have failed to clear after 3 months intensive topical treatment; (e) facial warts if cryotherapy is not available.

HERPES SIMPLEX LABIALIS * Topical aciclovir appears to speed resolution, with agents in a lipophilic base more effective than those of a hydrophilic base. * Oral aciclovir (200 mg five times daily for 10 days), valaciclovir (500 mg three times daily for

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7 days) and famciclovir (250 mg three times daily for 7 days) similarly reduce the duration of the disease. • Topical local anaesthetic cream (e.g. Xylocaine gel) may ease the pain. • There is some evidence that a course of oral aciclovir or famciclovir before exposure to intense UV light reduces the incidence and duration of attacks. Sunscreen prophylaxis similarly reduces the risk of attacks.

(b) the immunocompromised; or (c) patients in moderate to severe acute pain; or (d) older patients, e.g. over age 50. Starting the drug after 72 hours may be of value if new vesicles are still appearing. * Corticosteroids/idoxuridine. The evidence is too weak to recommend their use.

HERPES ZOSTER

BACTERIAL INFECTIONS

Systematic reviews: Lancaster T, Silagy C, Gray S. Primary care management of acute herpes zoster: systematic review of evidence from randomized controlled trials. Br J Gen Pract 1995; 45:39-45. Yaphe J, Lancaster T. Postherpetic neuralgia. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org

* The prevention of post-herpetic neuralgia (PHN) and the prompt treatment of ophthalmic zoster are the most important issues. PHN follows shingles in 50% of patients aged over 60 and 75% of patients over 70, of whom half will still be suffering 1 year later.11 * Oral aciclovir (800 mg five times daily for 5 days), famciclovir (250 mg t.d.s. for 7 days), and valaciclovir (1 g t.d.s for 7 days) taken within 72 hours of the appearance of the rash can shorten the duration and severity of the rash, and reduce the risk of persistent post-herpetic pain. The latter two have better bioavailability than aciclovir and may be more effective in reducing the risk of PHN.12,13

Acute herpes zoster * The patient with zoster on exposed skin should remain isolated until the last lesion scabs over. They can transmit the virus to non-immune individuals, who can contract chicken pox. * Give adequate analgesia. Opioids may be required. * Oral antiviral agents. Prescribe at high dose for 7 days, starting within 72 hours of the start of the rash, for: (a) ophthalmic zoster; or

For treatment of PHN, see p. 424.

* Check for diabetes in recurrent or unusually severe skin infections. * Topical antibiotics inevitably lead to colonization of the skin with resistant organisms. Do not therefore use antibiotics topically which may be needed systemically. * Topical neomycin, if used for less than 10 days, rarely leads to allergy.

Cellulitis and erysipelas * Use oral penicillin or erythromycin. Antibiotics effective against streptococcus (penicillin, ceftriaxone, roxithromycin and flucloxacillin) are equally useful, so penicillin is usually the drug of choice. Oral versus intravenous antibiotics have not been tested in a controlled trial. * Narrow-spectrum penicillin derivatives (flucloxacillin and dicloxacillin) can cause elevation of hepatic enzymes and should not be used as first line therapies. * There is no good evidence that routine microbiological tests improve treatment outcomes.14

Impetigo * Treat isolated lesions with topical antibiotic preparations, such as mupirocin, neomycin or soframycin. * Widespread lesions: give oral antibiotics, e.g. cephalexin or erythromycin. * Reduce the skin bacterial load to limit the extent of the current infection and recurrences. Try regular baths using an antiseptic solution or soap.

FUNGAL INFECTIONS

* Reduce the nasal and perianal carry rates of staphylococcus by the use of topical antibacterial agents (again mupirocin, neomycin or soframycin). * Treat all family members simultaneously if cross infection is a problem. Children should not attend school until the lesions have crusted over and there is no discharge.

Other bacterial infections * Boils should be drained. Daily washes with chlorhexidine will prevent spread. Oral flucloxacillin is needed for ill patients. * Recurrent staphylococcal skin infections. Patients with recurrent staphylococcal infections often carry staphylococci in sites not cleared by systemic antibiotics, especially the nose and the perineum. Bath daily with triclosan bath concentrate. Prescribe chlorhexidine and neomycin nasal cream or mupirocin nasal ointment. * Hidmdenitis suppuritiva. If the axilla is infected, give: (a) erythromycin 500 mg b.d. or oxytetracycline 500 mg b.d. for 1 month in the first instance, but 6 months treatment may be needed; or (b) topical clindamycin for 3 months. * Erythrasma is due to a corynebacterium, and is often mistaken for axillary tinea. Use a topical imidazole or oral tetracycline or erythromycin for 2 weeks. * Hair follicles: (a) folliculitis: use topical antiseptics or topical antibiotics; (b) furunculosis: use oral antibiotics (e.g. flucloxacillin). * Caruncle. Refer for surgical excision and drainage. The patient will also need antibiotics if unwell, or if there is associated cellulitis. Reduce the risk of recurrence by clearing the skin and nose of pathogenic staphylococci as above.

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Ketoconazole is slightly more expensive. Terbinafine is yet more expensive but appears to be slightly more effective15 and need only be applied for 1 week. • Use imidazoles for 2 weeks after clinical healing to avoid recurrence. • Nystatin is effective against yeasts, but not against other fungi. • Oral ketoconazole, fluconazole, itraconazole and terbinafine should only be used for severe fungal infections.

Candidiasis • Occurs in napkin dermatitis, intertrigo and angular stomatitis. Use nystatin cream, or an imidazole, with a steroid if there is inflammation.

Pityriasis versicolor * Explain that: (a) it does not need treatment if the patient does not mind the appearance; (b) killing the organism, which can be done by means of topical or even systemic antifungals, will not solve the cosmetic issue until old discoloured skin has been desquamated in the usual way; and will not prevent recurrence in susceptible people if treatment is stopped. * If the patient wishes treatment, use a low toxicity fungal poison such as zinc piridinthione, selenium sulfide, or a sulpha compound, often made up into a shampoo, which can be used several times a week indefinitely. * If there is extensive skin involvement, consider using itraconazole 200 mg daily for 5 days.

Seborrhoeic dermatitis and dandruff See p. 368.

FUNGAL INFECTIONS • There is little to choose between the topical imidazoles: clotrimazole, econazole and miconazole.

Tinea capitis Send skin scrapings before starting treatment. Use oral itraconazole or terbinafine. Topical antifungals are ineffective.

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Tinea pedis, cruris and corporis (a) Stress the importance of drying any moist areas. (b) Use an imidazole cream for 3 weeks or terbinafine cream for 1 week. (c) Consider oral terbinafine in resistant cases. (d) Avoid relapse by using dusting powders.

FUNGAL INFECTIONS OF NAILS Guidelines: Denning DW et al. Fungal nail disease: a guide to good practice (report of a Working Group of the British Society for Medical Mycology). BMJ1995; 311:1277-81. MeReC. Fungal nail infections. MeReC Bulletin 1997; 8: 45-8.

* Most people are not troubled by it. Oral antifungals are not without side-effects. * If planning to treat, confirm the diagnosis by sending scrapings, taken with a blunt scalpel, or nail clippings. These should be transported in a folded square of paper. Pus from the nail fold is valuable in chronic paronychia. Specify microscopy and culture for fungi. * Dermatophyte infection (infection with Trichophyton species). Give terbinafine 250 mg daily for 6 weeks (fingernails) or 3 months (toenails). Patients who prefer not to take oral drugs, or whose disease is confined to one or two nails, may use amorolfine nail lacquer or tioconazole nail solution. * Infection with yeasts, non-dermatophyte moulds or mixed infections. Give itraconazole 200 mg daily for 3 months or 'pulsed', i.e. 200 mg b.d. for 1 week every month for 2 (fingernails) or 3 (toenails) months. * Explain that the infection may continue to respond after the end of the course of treatment. Terbinafine and itraconazole persist in nail keratin for up to 9 months after the end of treatment.

LICHEN PLANUS * This condition may follow contact sensitivity to certain chemicals and drugs, or in response to chronic graft-versus-host disease.

* The rash is a collection of dark shiny, pearly papules about 2-4 mm across. They have a lacy pattern on the surface. The papules are distributed most commonly to flexor surfaces of the body, most commonly the wrist. * The lesions are self-limiting and usually resolve in a year. * Examine the mouth and genital mucosa. Asymptomatic white lines across the buccal, anal or genital mucosa confirm the diagnosis. * If drugs are suspected, they should be withdrawn. * Topical steroids may assist in reducing the size of the papules.

ALOPECIA With scarring * Take skin scrapings. Treat with antifungals if positive. If negative, refer for skin biopsy for conditions such as discoid lupus erythematosis.

Patchy without scarring * If there is a rash, scrape off scales and send for microscopy. If positive, treat with oral itraconazole or terbinafine. * Alopecia areata. Reassure the patient that regrowth is likely to occur within 1 year. Steroid creams are often prescribed, but there is little evidence of value. * Trichotillomania. Consider mechanical damage, especially in children.

Diffuse without scarring * 80% of men and 35% of women have frontal/ temporal pattern balding by the age of 60. Men usually progress to baldness of the crown; women do so less often. Work-up when hair loss is unexpected (a) FBC; (b) Ferritin; (c) TFTs; (d) Calcium phosphate.

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* Exclude drugs as a cause, e.g. cytotoxics, anticoagulants, antithyroid drugs, retinoids. * Acute hair loss. Explain that there is a cycle in the growth of each hair. Stress and illness can bring the majority of hairs to the end of the cycle at the same time so that they all fall out. They will then regrow together. * Postpartum acute hair loss. Explain that the hairs have been held back in their natural cycle during pregnancy, and that now that the pregnancy is over they have caught up and come out at the same time. They will regrow, but it may take many months. Exclude anaemia and thyroid dysfunction. * Consider referral for counselling, which may be available through the local dermatology department, and for a NHS wig. Advise men with male pattern baldness that minoxidil lotion will reduce the loss in 70% of cases and promote new hair growth in 30%, with a good cosmetic result in 10%. It is only available in the UK on a private prescription, and should be applied for 3 months before judging benefit. Once stopped the benefit is lost. * Discuss the option of surgery. Self-help groups: Hairline International: the Alopecia Patients' Society, Lyons Court, 1668 High Street, Knowle, Solihull, West Midlands B93 OLY, tel. 01564 775281. Alopecia Areata Support Association (Australia), PO Box 89, Camberwell, Victoria 3124, Melbourne, Australia, tel. 03 9513 8580; www.alopecia.auz.com

* Malathion may be effective, but there is no RCT evidence. * Gamma benzene hexachloride may have rare adverse events - aplastic anaemic and fits in patients treated for extensive disease. * Treat all members of a household and all sexual contacts simultaneously. Where transmission is sexual, screen for other STDs (see p. 42). * Instructions: 1. Apply the selected treatment to all areas below the neck before retiring, and wash off in the morning. Children and those with compromised immunity need to have the cream applied to the neck, face, ears and scalp as well. Wash all bedclothes, bed linen and underclothes in hot water that day. 2. Repeat the treatment in one week to eradicate the hatched mites. * Give appropriate antibiotics (cephalexin or erythromycin) if pustules are present. Note: The itch may persist longer than the mites. Repeated use of antiscabetic lotions does not accelerate resolution. Specific antipruritic measures are more appropriate.

LICE Head lice Guideline: MeReC. Management of head louse infection. MeReC Bulletin 1999; 10:17-20. Systematic review: NHS Centre for Reviews and Dissemination. Treating head lice and scabies. Effectiveness Matters. June 1999; 4 (1).

SKIN INFESTATIONS SCABIES Systematic review: Walker G, Johnstone P. Scabies. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org

• Pyrethrin (permethrin) has been found to cure 90% of affected individuals. It is more effective than gamma benzene hexachloride or crotamiton though both these latter agents are effective in their own right.

• Overuse of insecticides has led to resistance. Current guidelines stress that treatment should only be given if a living, i.e. moving, louse is found. Nits are egg cases stuck to hairs and do not necessarily mean there is active infestation. • Bug-busting (wet-combing of the hair to remove lice) is ineffective as treatment.16 The place of wetcombing is in detection (see box below). • Make sure the whole household is checked and ask the parents to notify the school nurse so that the rest of the class can be examined. UK guidelines stress that the affected child should not be

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excluded from school. Any transmission will already have taken place. * Prescribe the recommended parasiticidal preparation: malathion, permethrin or carbaryl. Health districts will rotate from one preparation to the other every 3 years to avoid resistance. It is important to cooperate with this policy. Local chemists or the local pharmaceutical advisers will know the current recommendations. * Practicalities: two applications are needed 7 days apart; lotions, liquids or creme rinses should be used, not shampoos. Note: Asthmatics or young children should use aqueous malathion. Note: Carbaryl has been found to cause cancer in laboratory animals after prolonged exposure to high doses. There is therefore a theoretical risk that it may be carcinogenic in humans, although the CMO advises that any risk is exceedingly small.17 Detection combing 1. Buy a fine tooth detector comb. 2. Wash the hair and leave it wet. Comb first with an ordinary comb then with the detector comb, beginning at the top of the head. The comb must be touching the scalp and be drawn right to the ends of the hairs. 3. Look for lice on the comb. 4. Continue to comb until the whole scalp and all the hair has been covered - it should take 10-15 minutes. 5. If you find something that could be a louse, stick it to a piece of paper with clear sticky tape and take it to a nurse, doctor or pharmacist for confirmation.

Genital lice (crab lice) * Treat all intimate contacts. It is usually spread by sexual contact in adults, but finding it in a child does not necessarily raise suspicion of sexual abuse. It can survive for 24 hours in clothes or bedding. * Use aqueous preparations of malathion, permethrin, phenothrin or malathion to the whole body, not just the pubic hair, for 12 hours and repeated after 7 days.

Body lice * Wash clothes in hot water. No other treatment is necessary.

PAPULAR URTICARIA Fleas * Deflea the cat, dog or rabbit. * Treat the house. Spray insecticide on carpets and soft furnishings, and around where the pet sleeps.

Bed bugs * Wash bed linen. * Call in the Environmental Health Department to treat the house. The bugs live behind wallpaper and skirting boards, not in the beds themselves.

Animal mites * Treat the pet, who is likely to have signs of the disease (e.g. mange).

Bird mites * Clear birds' nests from outside the bedroom window if bird mites are suspected.

MALIGNANCY There are three main groups of primary skin cancer: 1. basal call carcinoma (BCC), arising from the basal cells of the germinal layer; 2. squamous cell carcinoma (SCC) arising from squamous cells; and 3. melanoma, arising from pigmented cells. Although all are common in white-skinned people living in sunny climates, the prognosis of each is very different.

BASAL CELL CARCINOMAS (BCCs) • These are the most common skin cancers. They are most common on head and upper trunk. • Although they do not metastasize without undergoing squamous transformation, delay in diagnosis can lead to disaster from loss of wide areas of tissue, or underlying organs such as the eye.

MALIGNANCY

* A trial of sun-screen failed to show benefit for preventive BCCs.

Management * If there is any doubt about diagnosis, biopsy is indicated. * Treatment is by excision, or in the elderly, superficial X-ray therapy. * Superficial ones can be treated with local destruction, such as liquid nitrogen cryotherapy or imiquimod cream.

SQUAMOUS CELL CARCINOMAS (SCCs) * These are also common skin cancers. They are most common on sun-exposed areas including extremities. * Referral. In the UK, urgent referral, to be seen within 2 weeks, is indicated for any patient with: (a) an indurated lesion in which there has been documented expansion over the last 1-2 months; or (b) a positive biopsy; or (c) any new or growing skin lesion in a patient who is immunosuppressed. * If there is any doubt about diagnosis, biopsy is indicated. * Treatment is by excision (SCCs are much less radiosensitive to superficial X-ray therapy than BCCs). * Stress the importance of further prevention. Use of a sun-screen has been shown to decrease the later formation of SCCs in a randomized trial. Use of a hat and clothing is even more effective in avoiding solar damage.

MELANOMA * Melanomas are the most dangerous form of skin cancer, and are also related to past sun exposure. * Of several forms, the most common is the superficial spreading melanoma (SSM) for which early diagnosis of thin lesions is critical because it conveys such a better prognosis compared to

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those left to grow thicker. Nodular, and acral forms (on palms and feet) carry a worse prognosis, although this is very variable. • They are pigmented (except in the case of the rare amelanotic form). SSMs especially can be hard to distinguish from benign skin naevi ('moles'), and also flat seborrhoeic keratoses. • Special distinguishing characteristics of melanomas include asymmetry, size greater than 6 mm, irregular edges or surface, or non-uniform pigmentation. Unfortunately, early SSMs are often difficult to diagnose.

Management * If there is any doubt about diagnosis, biopsy. * In the UK, patients qualify for referral urgently, to be seen within 2 weeks, if they have a pigmented lesion which shows one or more of the following: growing in size, changing shape, irregular outline, changing colour, mixed colour, ulceration, or inflammation. * Definitive treatment is by excision, leaving at least 5 mm of healthy skin around the specimen. Lesions with a histology report indicating less than 0.075 mm thickness have a very good prognosis. There is no evidence that any immunotherapy, chemotherapy or radiotherapy provides any significant improvement in mortality or morbidity.

SOLAR KERATOSES • Solar keratoses (SKs) are keratotic lesions on sun-exposed skin caused by solar damage. • They may be precursors of SCCs. However, most SKs spontaneously remit without progress into SCCs. Fewer than 1 in 1000 SKs transform into SCCs.18 Moreover, many SCCs arise from previously normal skin rather than SKs. • Some patients worry about skin cancer (for which SKs are indeed a risk factor), and the hardness of the lesions can be irritating on clothing. * If there is any doubt about the diagnosis of cancer, biopsy. * Destructive treatment of SKs (by cryotherapy, or excision biopsy if there is diagnostic doubt) may be required for local symptomatic treatment.

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* Symptomatic treatment of SKs can be achieved by use of topical applications such as salicylic ointment (5%) or urea cream (10%) or by topical diclofenac. * Treating SKs as a preventive to developing SCCs is unjustified. * Preventive activity in the form of sunscreens has been shown to decrease the later formation of solar keratoses. * 5-fluorouracil cream (5-FU): people with damaged skin can benefit from applying 5-FU daily to affected areas, such as the forehead or forearms. Stop the cream after 2-3 weeks, when the patient experiences an inflammatory reaction. The skin will then regenerate, leaving a cosmetically improved surface. Only prescribe it if reasonably experienced in its use. Before treatment: (a) warn the patient that the treatment is unpleasant; the inflammatory reaction will cause erythema and possibly ulceration; (b) check carefully for malignancy in the area; 5-FU can mask an SCC or a BCC.

HYPERHIDROSIS GENERALIZED HYPERHIDROSIS A cause is not usually found, but consider: (a) autonomic nervous system imbalance, as in the menopause; (b) autonomic neuropathy, with areas of hypohidrosis and compensatory hyperhidrosis elsewhere; (c) hyperthyroidism; (d) hyperpituitarism.

HYPERHIDROSIS OF THE PALMS, SOLES AND AXILLAE * Use daily aluminium chloride hexahydrate applied at night to dry skin and washed off in the morning, until benefit is achieved. Do not apply within 12 hours of shaving or using depilatories. Use hydrocortisone cream if the preparation causes irritation. Reduce to weekly applications once control is achieved.

* For treatment failures, discuss the availability of iontophoresis or injections of botulinum toxin with the local physiotherapy department or dermatologist.19 * Otherwise consider referral for sympathectomy for palmar sweating and axillary skin excision for axillary sweating. Both have their problems; infection following skin excision, and surgical disasters following sympathectomy. * If odour is a problem apply an antibacterial b.d., e.g. chlorhexidine cream or dusting powder.

GRAVITATIONAL ULCERS Systematic reviews: Nelson EA, Cullum N, Jones J. Venous leg ulcers. Clinical Evidence, Issue 5. London. BMJ Publishing Group, 2001. Available: www. clinicalevidence.org Compression therapy for venous leg ulcers. Effective Health Care 1997; 3 (4).

• Firm, but not excessive compression dressings of the legs have been shown to reduce healing time. Compression devices (e.g. TED stockings) also reduce recurrence of venous ulcers. • The type of dressing used does not appear to influence healing time. • Oxpentiphylline and flavinoids improve healing rates but they are not recommended by current guidelines.20,21

Management * Check the arterial supply before compression bandages are applied. The ratio of ankle to brachial systolic blood pressure (see p. 293) should be measured by Doppler. Compression should not be applied if the ratio is <0.8. Palpation of foot pulses may miss significant arterial insufficiency. * Check the Hb and fasting sugar in all patients, and possibly the serum iron in patients with chronic ulcers that fail to heal. * Agree, with the responsible nursing staff, a practice policy for the treatment of gravitational ulcers using multilayer compression bandaging capable of being left on for a week at a time.

REFERENCES 381

* General measures. Encourage mobility. Elevate the leg when sitting; footstools are not sufficient elevation. Raise the foot of the bed. * Infection. Take a swab and clean the ulcer as above. If the ulcer is foul-smelling, start metronidazole 400 mg b.d. for 7 days. If there is evidence of cellulitis, use appropriate oral antibiotics (e.g. erythromycin). A positive swab does not necessarily mean infection.22 Do not use topical antibiotics: they are ineffective and give rise to sensitization. * Prevention. Once the ulcer is healed, the patient must be obsessional about wearing a support stocking of at least medium strength. Even then, one-third of ulcers relapse in the first year after healing.

Referral * Refer all patients with a poor arterial supply (ankle to brachial systolic pressure <0.8) for further investigation by a vascular surgeon. * Consider varicose vein surgery in any patient with prominent incompetent perforators or varicosities: (a) before the skin ulcerates; or (b) after the skin has healed; or (c) while the ulcer is present. Venous surgery has increased healing from 57% to 89%.23 * Consider referral for grafting for any ulcer that is failing to improve after 3 months, or which has not healed after 1 year.

URTICARIA Guideline: Grattan C, Powell S, Humphreys F. Management and diagnostic guidelines for urticaria and angio-oedema. Br J Dermatology 2001; 144: 708-14.

* Distinguish 'ordinary' urticaria from physical urticaria and contact urticaria. The management of the last two is to avoid the stimulus.

Management of ordinary urticaria * Attempt to identify an allergen from the history. Skin prick tests and radioallergosorbent testing (RAST) may be able to confirm a clinical suspicion. Biological allergens are the most common triggers. These include drugs (antibiotics, salicylates), insect stings, ingestion of certain foods (including nuts, fish and shellfish, fruits), and preservatives (e.g. tartrazine). * Avoid drugs which can worsen urticaria, e.g. aspirin and codeine. * Avoid aggravating physical factors, e.g. cold, heat or stress. * Offer soothing lotions, e.g. calamine. * Give a non-sedating antihistamine. Try at least two before abandoning them; individual responses vary. A sedating antihistamine may be more helpful at night. * Consider adding an H2-receptor antagonist if the antihistamine alone is insufficient. * Oral corticosteroids can shorten an episode of acute urticaria. Give prednisolone 50mg/day for 3 days but not long term. * Refer patients whose chronic urticaria is sufficiently troublesome. Even their prognosis is not bad; referred patients have a 50% chance of being symptom free without treatment in 6 months.24 Self-help group: The British Allergy Foundation, Deepdene House, 30 Bellegrove Road, Welling, Kent DA16 SPY, tel. 020 8303 8525; helpline 020 8303 8583 (Mon-Fri 9.00 a.m. to 5.00 p.m.); www.allergyfoundation.com Professional organisation with patient information: The Australasian Society of Clinical Immunology and Allergy (ASCIA). PO Box 450, Balgowlah, NSW 2093, Australia, tel. 02 8900 6402; www.allergy.org.au

REFERENCES 1. Hoare C, Li Wan Po A, Williams H. Systematic review of treatments for atopic eczema. Health Technology Assessment 2000; 4: 37. Online. Available: www.ncchta.org

2. Williams HC, Wuthrich B. The natural history of atopic dermatitis. In: Williams HC (Ed). Atopic dermatitis. Cambridge: Cambridge University Press, 2000.

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3. BNR Topical steroids. Section 13.4. British National Formulary 2001; No. 42. 4. Charman C. Atopic eczema. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. 5. Adami M, Inglott A, Azzopardi L et al. Acne management in community practice. Pharmaceutical Journal 1998; 261: R20. 6. Leyden JJ. Therapy for acne vulgaris. New Engl J Med 1997; 336: 1156-62. 7. MeReC. Acne vulgaris. MeReC Bulletin 1994; 5: 41-4. 8. DTB. Azelaic acid - a new topical treatment for acne. Drug Ther Bull 1993; 31: 50-2. 9. Garner SE, Eady EA, Popescu C et al. Minocycline for acne vulgaris: efficacy and safety (Cochrane Review). In: The Cochrane Library, Issue 4. Oxford: Update Software, 2001. 10. DTB. Tackling warts on the hands and feet. Drug Ther Bull 1998; 36: 22-4. 11. Bandolier. Does treating acute herpes zoster in primary care stop post-herpetic neuralgia? Bandolier 1995; 17: 133. 12. Kost RG, Straus SE. Postherpetic neuralgia - pathogenesis, treatment and prevention. New Engl J Med 1996; 335: 32-42. 13. DTB. Update on drugs for herpes zoster and genital herpes. Drug Ther Bull 1998; 36. 14. Morris A. Cellulitis and erysipelas. Clinical Evidence, Issue 5. London: BMJ Publishing Group, 2001. Available: www.clinicalevidence.org

15. Crawford F, Hart R, Bell-Syer S et al. Topical treatments for fungal infections of the skin and nails of the foot (Cochrane Review). In: The Cochrane Library, Issue 4, Oxford: Update Software, 2001. 16. Roberts RJ, Casey D, Morgan DA et al. Comparison of wet combing with malathion for treatment of head lice in the UK: a pragmatic randomised controlled trial. Lancet 2000; 356: 540-4. 17. Chief Medical Officer. CMO's letter PL/CMO (95)4. 18. Marks R, Rennie G, Selwood T. Malignant transformation of solar keratoses. Lancet 1988; i: 795-7. 19. Simpson N. Treating hyperhidrosis. BMJ 1988; 296: 1345. 20. RCN Institute, Centre for Evidence Based Nursing, University of York and School of Nursing, Midwifery and Health Visiting, University of Manchester. The management of patients with venous leg ulcers. 1998. Online. Available: www.rcn.org.uk 21. British Association of Dermatologists and the Royal College of Physicians of London. Guidelines for the management of chronic venous leg ulceration. Br J Dermatology 1995; 132: 446-52. 22. Thompson PD, Smith DJ. What is infection? Am J Surg 1994; 167 (Suppl. la): 75-115. 23. Wilson E. Prevention and treatment of venous leg ulcers. Health Trends 1989; 21: 97. 24. Champion RH, Roberts SOB, Carpenter RG et al. Urticaria and angio-oedema: a review of 554 patients. Br J Dermatol 1969; 81: 588-97.

CHAPTER CONTENTS Food allergies

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Anaphylaxis 385 Useful contacts References

21 Allergic problems

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387

FOOD ALLERGIES Guidelines/reports: Bruinjzeel-Koomen CA, Ortolani C, Aas K et al. Position paper. Adverse reactions to foods. Allergy 1995; 50: 623-36. Committee on Toxicity of Chemicals in Food, Consumer Products and the Environment. Peanut allergy. London: Department of Health, 1998. Online. Available: www.doh.gov.uk/pub/docs/doh/peanut.pdf

• Adverse reactions to food may result from allergy (hypersensitivity) or intolerance (reactions that are not immunologically mediated).1 • Patients will often use the term 'allergy' to refer to any of a number of food-related adverse reactions; double-blind, placebo-controlled studies, however, show that only a minority of these reactions have an allergic basis.2'3 • There are currently no evidence-based guidelines for the diagnosis and management of food allergies.4'5 • Oral allergy syndrome is an IgE-mediated hypersensitivity to tree fruits and root vegetables that occurs in some patients with pollen allergy (hay fever). Contact urticaria and/or angioedema of the lips and oropharynx occur through a crossreactivity between specific epitopes in pollens and fresh fruit/vegetables manifesting as an itchy oropharynx and swelling of the lips and tongue. More severe reactions may occur but are unusual.1

Diagnosis • Differentiating food allergy from intolerance is important because the former will typically 383

384

ALLERGIC PROBLEMS

Table 21.1 Foods commonly responsible for triggering allergic reactions Children

Adults

Milk Egg white Peanuts Wheat Soya beans

Fish and seafood Tree nuts Peanuts Additives Fruits

require meticulous avoidance of the food(s) in question; continued exposure to the triggering foods in those with IgE-mediated food allergy increases the risks of major systemic allergic reactions such as anaphylaxis.6 * Attempt to differentiate food allergy from intolerance by the following features of the history and examination: (a) Family history: food allergy usually occurs in those with a personal and/or family history of allergic disorders. (b) Type of food: although almost any food may provoke allergic symptoms in sensitized individuals, most reactions occur in relation to exposure to a small group of foods (Table 21.1).7'8 (c) Speed of onset: symptoms soon after food intake (usually <1 hour and often within minutes) are suggestive of allergy. (d) Effect of re-exposure: re-exposure to the same food(s) will produce very similar reactions in those with food allergy; the picture is often much more variable in those with intolerance. (e) The clinical picture: food allergy will typically precipitate symptoms suggestive of inflammation of one or more organs including features of angioedema (particularly of lips and tongue), urticaria, conjunctivitis, rhinitis, bronchospasm, gastrointestinal oedema (cramps, vomiting and diarrhoea) and anaphylaxis.7 Food-related symptoms of tiredness, joint and muscle pains, sleep disturbance and emotional upset are all suggestive of a diagnosis of food intolerance.

Management Symptomatic treatment * Treat symptomatically; if life-threatening features are present (respiratory difficulty or

symptoms suggestive of hypotension) then treat as for anaphylaxis (see p. 385). Further management * Attempt to identify unequivocally food(s) responsible for triggering symptoms, confirming clinical suspicion with objective allergy test (skinprick test, serum-specific IgE) or, in mild cases, challenge testing. * Refer to an allergist if provision for suitable testing is unavailable or in the event of diagnostic uncertainty.9 * Recommend the avoidance of food(s) found to trigger symptoms. * Recommend that patients with oral allergy syndrome avoid the offending fruits and/or vegetables in their raw form; in most cases, the allergen is destroyed by heating and cooked fruits and/or vegetables can be safely consumed. * Enlist the help of a dietician to ensure that patients are fully counselled about the foods that they need to avoid and to ensure their diet is nutritionally adequate. * Issue self-injectable adrenaline to those with a history of anaphylaxis and recommend a Medic Alert bracelet/necklace (see p. 386). * Refer patients with severe food intolerance(s) to a gastroenterologist. A dietician-supervised 'few foods diet' may be beneficial.10 Reintroducing foods after a period of avoidance Children with allergy to milk and eggs will commonly develop tolerance to these foods, as they grow older. In those with a history of reactions that are not considered to be life threatening, the careful reintroduction of these foods at a later date may be appropriate. * Consider reintroducing: (a) milk at the age of 3 years (by which time nine out of ten children with a history of milk allergy will be tolerant); and (b) eggs at the age of 6 years (by which time half of children with a history of egg allergy will be tolerant)7. * Persistent symptoms. Consider rechallenging at yearly intervals thereafter.

ANAPHYLAXIS 385

Note: Peanut and fish/seafood allergy is typically life-long and reintroduction is not recommended.11 Prevention * Encourage lactating mothers of infants with a parent or sibling with an atopic disease to exclusively breast-feed for 4-6 months.12 * Family history of allergic disorders. Advise: (a) pregnant and lactating mothers to avoid peanuts and peanut products in order to reduce the risk to their child of developing peanut allergy in later life.12 (b) that infants should not be exposed to peanuts and peanut products during weaning and until they are at least 3 years of age.12

Useful contacts For patients Anaphylaxis Campaign PO Box 275, Farnborough, Hampshire GU14 6SX Tel. 01252 542029; fax 01252 377140; website: www.anaphylaxis.org.uk British Allergy Foundation Deepdene House, 30 Bellegrove Road, Welling, Kent, DA16 3PY Helpline 020 8303 8583; tel. 020 8303 8525; website: www.allergyfoundation.com MedicAlert Foundation 1 Bridge Wharf, 156 Caledonian Road, London N19UU Tel. 020 7833 3034; fax 020 7278 0647; website: wwTv.medicalert.co.uk Supermarkets can provide information on lists of products that are 'free from' certain ingredients (Table 21.2). For professionals British Society for Allergy & Clinical Immunology 66 Weston Park, Thames Ditton, Surrey KT7 OHL Tel. 020 8398 9240; fax 020 8398 2766; website: www.soton.ac.uk/~bsaci

Table 21.2 Consumer telephone numbers for major UK supermarket chains Supermarket

Telephone number

Asda Co-op Marks & Spencer Safeway Sainsbury Somerfield Tesco Waitrose

Freephone 0500 1000 55 Freephone 0800 0686727 02072681234 020 8970 3622 Freephone 0800 636262 01179356584 Freephone 0800 505555 01344824975

National Asthma & Respiratory Training Centre The Athenaeum, 10 Church Street, Warwick CV34 4AB Tel. 01926 493313; fax 01926 493224; website: www.nartc.org.uk The Leatherhead Food Intolerance Databank Leatherhead Food Research Association, Randalls Road, Leatherhead, Surrey KT22 7RY Tel. 01372 376761; fax 01372 386228; website: www.lfra.co.uk

ANAPHYLAXIS Guidelines: Project team of the Resuscitation Council (UK). Emergency medical treatment of anaphylactic reactions. J Accid Emerg Med 1999; 16: 243-8. Project team of the Resuscitation Council (UK). Update on the emergency medical treatment of anaphylaxis reactions for first medical responders and for community nurses. Resuscitation 2001; 48: 241-3. Chief Medical Officer's Update. Anaphylaxis guidance. London: Department of Health, PL/CMO/2001/1. Online. Available: www.doh.gov.uk/pdfs/cmo0101 .pdf

• Anaphylaxis is a potentially life-threatening emergency. It is estimated that there are between 20 and 30 deaths from anaphylaxis in the UK each year, many of which are preventable.13 • The UK incidence of anaphylaxis is believed to be increasing rapidly.14 • The classification into anaphylactic (IgEmediated hypersensitivity reactions) and anaphylactoid (non-IgE-mediated mast cell degranulation)

386

ALLERGIC PROBLEMS

is of little practical relevance to the generalist management of anaphylaxis. • Guidelines for the recommendation of anaphylaxis are consensus-based. Randomized controlled trials of commonly recommended treatments have not been conducted.15'16

Table 21.3 Route of administration and drug dosage for agents used in the emergency treatment of anaphylaxis Drug (Route of administration)

Age-related dosage

Adrenaline 1 : 1000 (im)

<6 months: 50 (xg (0.05ml) > 6 months-6 years: 120|xg (0.12ml) 6-12 years: 250 jxg (0.25ml) >12 years (small or prepubertal child): 250 jig (0.25 ml) >12 years: 500 (xg (0.5ml) 1-6 years: 2.5-5mg 6-1 2 years: 5-1 Omg >12 years 10-20mg

Diagnosis • There is no uniformly accepted objective definition of anaphylaxis.15 • Diagnosis depends on the identification of a constellation of symptoms and signs which may involve a number of organ systems: (a) skin: erythema, pruritis, urticaria and angioedema; (b) eyes: injection of conjunctiva, tearing and itching; (c) airways: sneezing, rhinorrhoea, laryngeal oedema, cough, wheeze and dyspnoea; (d) gastrointestinal: tongue swelling, abdominal cramps, vomiting and diarrhoea; (e) cardiovascular: palpitations, light-headedness and collapse. • A working diagnosis of anaphylaxis can be made by identifying features of a systemic allergic reaction accompanied by either respiratory difficulty or symptoms/signs suggestive of hypotension.17

Chlorpheniramine (im or slow iv) Hydrocortisone (im or slow iv) Crystalloid fluid (iv)

1-6 years: 50 mg 6-1 2 years: 100mg >12 years: 1 00-500 mg Children: 20 ml/kg of body weight Adults: 1-2 litres

4. Give inhaled beta2-agonist if there is severe bronchospasm. 5. Repeat adrenaline 5 minutes after first dose if there is no clinical improvement. 6. Give chlorpheniramine im (or by slow iv injection). 7. Give iv crystalloid fluid infusion if symptoms of hypotension persist (a repeat dose may be necessary).20 8. Give hydrocortisone im (or by slow iv injection). 9. Admit to hospital as a later relapse in symptoms may occur.21

Management • Management is best considered in two stages: treatment of the acute attack and follow-up care.18 Acute management (see Table 21.3) 1. Commence life support (basic and advanced) if indicated. 2. Give oxygen. 3. Give adrenaline (epinephrine) 1:1000 solution (im) if not already administered (many patients with a history of anaphylaxis will have been issued with adrenaline for self-injection). The Chief Medical Officer cautions against the use of the iv route except by experienced practitioners treating profound shock.19 A 10-fold dilution is needed.

Follow-up care * Trigger. Refer to an allergist in order to objectively identify the trigger and for consideration of desensitization therapy.22 This will involve a detailed history and subsequent investigations, which may include skin-prick testing, serumspecific IgE tests and allergen challenge. * Allergen avoidance. Reinforce any allergen advice issued; dietetic referral may be indicated in cases of food allergy. * Adrenaline. Issue adrenaline for self-administration (for example, EpiPen, Anapen or Ana-Guard) and educate the patient on when and how to use it. * Alert bracelet. Recommend purchase of a MedicAlert bracelet/necklace documenting history of anaphylaxis, noting where adrenaline is

REFERENCES

kept should the patient be found in a collapsed state. * Asthma control. Optimize asthma control in those with a history of asthma as the risk of fatality is increased in this group.22

387

* Liaise with nursery/school/work as appropriate.23

USEFUL CONTACTS For patients Anaphylaxis Campaign PO Box 275, Farnborough, Hampshire GU14 6SX Tel. 01252 542029; fax 01252 377140; website: www.anaphylaxis.org.uk British Allergy Foundation Deepdene House, 30 Bellegrove Road, Welling, Kent, DA16 SPY Helpline 020 8303 8583; tel. 020 8303 8525; website: www.allergyfoundation.com MedicAlert Foundation 1 Bridge Wharf, 156 Caledonian Road, London Nl 9UU

Tel. 020 7833 3034; fax 020 7278 0647; website: www.medicalert.co.uk For professionals British Society for Allergy & Clinical Immunology 66 Weston Park, Thames Ditton, Surrey KT7 DHL Tel. 020 8398 9240; fax 020 8398 2766; website: www.soton.ac.uk/~bsaci National Asthma & Respiratory Training Centre The Athenaeum, 10 Church Street, Warwick CV34 4AB Tel. 01926 493313; fax 01926 493224; website: www.nartc.org.uk

REFERENCES 1. Sampson HA. Food allergy. In: Kay AB (Ed). Allergy and allergic diseases. Oxford: Blackwell Science, 1997: 1517-49. 2. Clough J. Allergies at your fingertips. London: Class, 1997: 120-49. 3. Young E, Stoneham MD, Petruckevitch et al. A population study of food intolerance. Lancet 1994; 343: 1127-30. 4. Bruinjzeel-Koomen CA, Ortolani C, Aas K et al. Position paper. Adverse reactions to foods. Allergy 1995; 50: 623-36. 5. Committee on Toxicity of Chemicals in Food, Consumer Products and the Environment. Peanut allergy. London: Department of Health, 1998. 6. Wood SF. CP guide to the diagnosis and management of allergic disorders in children. London: Mosby, 1999: 33-8. 7. Bindslev-Jensen C. Food allergy. In: Durham SR (Ed). ABC of allergies. London: BMJ Books, 1998: 44-7. 8. Joint Task Force on Practice Parameters for Allergy and Immunology. Practice parameters for allergy diagnostic testing. Ann Allergy Asthma Immunol 1995; 75: 543-625. 9. Royal College of Physicians Committee on Clinical Immunology and Allergy. Allergy: conventional and alternative concepts. London: RCP, 1992. 10. National Asthma & Respiratory Training Centre. Management of allergy in primary care. Warwick: NARTC, 2001: Unit 8. 11. Radcliffe MJ. Food allergy and intolerance. In: Jackson WJ (Ed). Allergic disorders. London: Mosby-Wolfe, 1997: 91-107. 12. Chief Medical Officer. Health advice on peanut allergy. CEM/CMO/98/9. Online. Available: www.doh.gov.uk/cmo/cmo989.htm

13. Pumphrey RS. Lessons for management of anaphylaxis from a study of fatal reactions. Clin Exp Allergy 2000; 308: 1144-50. 14. Sheikh A, Alves B. Hospital admissions for acute anaphylaxis: time trend study. BMJ 2000; 320: 1441. 15. Project team of the Resuscitation Council (UK). Emergency medical treatment of anaphylactic reactions. / Accid Emerg Med 1999; 16: 243-8. 16. Project team of the Resuscitation Council (UK). Update on the emergency medical treatment of anaphylaxis reactions for first medical responders and for community nurses. Resuscitation 2001: 48: 241-3. 17. Ewan PW. Anaphylaxis. BMJ 1998; 316: 1442-5. 18. Sheikh A. Anaphylaxis. Update 1999; 58: 984-8. 19. Professional letter - Chief Medical Officer. Current vaccine and immunisation issues. PLCMO(2001)1. 20. Schierhout G, Roberts I. Fluid resuscitation with colloid or crystalloid solutions in critically ill patients: a systematic review of randomised trials. BMJ 1998: 316: 961-4. 21. Joint Task Force on Practice Parameters, Work Group on Diagnosis and Management of Anaphylaxis. The diagnosis and management of anaphylaxis. / Allergy Clin Immunol 1998; 101: S465-S528. 22. Lane SJ, Lee TH. Anaphylaxis. In: Kay AB (Ed). Allergy and allergic diseases. Oxford: Blackwell Science, 1997: 1550-72. 23. Vickers DW, Maynard L, Ewan PW. Management of children with potential anaphylactic reactions in the community: a training package and proposal for good practice. Clin Exp Allergy 1997; 27: 898-903.

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22

CHAPTER CONTENTS Keeping older people healthy

389

Older people

Managing frailty 390 Supporting older people at home 390 Acute confusion, agitation, loss of mobility

391

Subacute deterioration without acute illness: depression or dementia 393 Dementia

394

Falls 396 Lower limb problems 398 Night cramps 398 Restless legs syndrome 398 Postural ankle oedema 399 Elder abuse Legal aspects

KEEPING OLDER PEOPLE HEALTHY

399 399

References 400

Screening and prevention * Continue screening programmes for mammography and cervical screening until older age. There is little evidence that screening is no longer worthwhile and most programmes recommend continuing to age 75 years, although in the UK routine screening ends at 65. * Screening for and management of hypertension in particular and cardiovascular disease in general is more important for older people1 as they are at higher absolute risk. Ten older people with hypertension and diabetes or twenty older people treated for hypertension for 5 years will prevent one cardiovascular event. * Influenza vaccination is indicated for all persons age 65 years and over and in some countries (although not the UK) pneumococcal vaccination as well.

Smoking * Advise smoking cessation for all older people as stopping reduces mortality by 50%.l * Simple advice from primary care doctors is effective in smoking cessation.2

Alcohol • Although alcohol consumption decreases with age, 17% of men and 7% of older women exceed limits of regular consumption of alcohol. 1-5% of 389

390

OLDER PEOPLE

older people who drink more than occasionally report that they are 'problem drinkers' and males more so than females.3 • Alcohol misuse in the elderly contributes towards falls, stroke, depression and osteoporosis. • Simple advice from the GP is effective in reducing alcohol consumption.4

Exercise • 30 to 60 minutes of sustained low level activity, such as walking, on at least 3 days per week5 will result in moderate health benefits such as reduced fatigue, weight loss, increased socialization, improved control of type II diabetes, less shortness of breath on exertion.6 • No adverse effects of low to moderate activity have been observed in trials promoting physical activity for community dwelling older people.7 • Advise regular, safe, physical activity as part of daily activities. Simple advice delivered in primary care is effective in increasing activity for older patients.8 • Recommend exercise which mimics the activities of daily living (ADL) such as repetitive sit to stand and walking. This is more acceptable and beneficial for older people. • Referral to physiotherapy for strength and balance training will reduce falls by 40% in women over age 80 years.9

MANAGING FRAILTY • Frailty is best regarded as 'a condition or syndrome which results from a multi-system reduction in reserve capacity to the extent that a number of physiological systems are close to, or passed the threshold of symptomatic clinical failure. As a consequence, the frail person is at increased risk of disability and death from minor external stresses'.10 • General practice, as part of the primary healthcare network, is well placed to detect and treat emerging frailty and prevent further deterioration. • The Nottingham Extended Activities of Daily Living (EADL; see Appendix 25) scale may be

useful in detecting unsuspected functional decline (see p. 405).

Early intervention * Preventive home visits with health professionals or lay people trained in case-finding reduce mortality and admission to residential care and may promote independence and improve quality of life for frail older people.11 * Effective management of minor ailments, such as painful foot problems, may halt declining mobility and improve long term outcomes. * Maximizing management of chronic problems and minimizing polypharmacy may halt functional decline. The UK National Service Framework12 recommends an annual review of medication for people aged 75 and over, with a 6-monthly review for those taking four or more medicines.

SUPPORTING OLDER PEOPLE AT HOME • There is considerable variation in availability of services for older people internationally and within countries, although care can be broadly divided into formal and informal provision. Generally, there is a multitude of services available aimed at either maintaining function or providing rehabilitation. • Services can be accessed either by direct referral or through specialist geriatric services. • A comprehensive assessment is recommended before referring to services. • In general, services are accessed in the UK through a social worker; Australia, by direct referral or through Geriatric Assessment Services; and New Zealand, the Needs Assessment Coordinator.

Difficulty maintaining living arrangements • Refer to an occupational therapist for assessment of activities of daily living (ADL) function at home and provision of equipment.

ACUTE CONFUSION, AGITATION, LOSS OF MOBILITY

* Refer to a social worker to set up home carer for housework. * Refer to a social worker for Meals-on-Wheels (daily hot food or weekly frozen meal deliveries). * Refer to a podiatrist/chiropodist for foot care.

391

(c) the occupational therapist, physiotherapist for functional assessment and provision of equipment; (d) the social worker for temporary provision of personal care and housework.

Isolation * Refer to Age Concern for provision of a volunteer/befriending service. * Refer to a social worker for day centre placement. * Refer to Dial-a-driver or social worker to access subsidised taxi service for transport. Carer stress (see p. 408) * Ascertain reason for stress. * Refer for access to respite services, including day and night respite, and residential home placement for short periods. * Refer for home care for personal assistance. * Refer to occupational therapist, for assessment of ADL function and equipment.

Functional deterioration at home Refer to: (a) a geriatrician for a comprehensive assessment; (b) a day hospital, for multidisciplinary assessment and treatment; (c) physiotherapy for musculoskeletal assessment and exercises; (d) occupational therapy for ADL functional assessment, provision of equipment and treatment; (e) a social worker for personal home care; (f) the district nurses for bathing service; (g) a speech language therapist if the condition affects communication or swallowing; (h) a dietician.

Poor recovery after an acute episode * Consider referral to: (a) the appropriate hospital service; (b) the district nursing service;

ACUTE CONFUSION, AGITATION, LOSS OF MOBILITY • A change in mental or physical status of usually well independent older people should be taken seriously and treated urgently. Symptoms of serious illness are often masked and fever is seldom higher than 37.5°C even in overwhelming infection. • Acute change can be due to serious disease without overt symptomatology related to that disease. Confusion can be as simple as 'the patient has suddenly changed, something is wrong, the level of function has deteriorated'. • Agitation may occur and can be defined as 'inappropriate verbal, vocal, or motor activity that is not explained by needs' and may be due to undiagnosed pain, acute illness, or simply the inability to communicate needs. • A loss of mobility or 'taken to the bed' may be due to serious illness. • Establishing the onset of deterioration and prior function is essential to identify delirium characterized by a fluctuating level of consciousness, disorientation, global cognitive deficit. This is almost always due to organic cause, usually infection. • The classic triad of confusion, incontinence and falls should alert the clinician to the presence of serious illness.13

Diagnosis A diagnosis of acute serious illness or medication toxicity should be assumed until proven otherwise. There are many causes for acute confusion including:14 (a) infection, most commonly, pneumonia and urinary tract infection;

392 OLDER PEOPLE

(b) cardiovascular disorder, especially myocardial infarction and congestive heart failure; (c) neurological disorder, most commonly stroke; (d) medication interaction or toxicity, particularly psychotropic medications or cardiovascular medications; (e) acute alcohol withdrawal; (f) acute psychiatric disorder, including psychosis; (g) electrolyte disturbance, dehydration, hyponatremia; (h) endocrine disorder, thyroid disease, diabetes; (i) acute change in hearing or vision; (j) faecal impaction or retention of urine; (k) neoplasia; (1) acute surgical emergency, such as peritonitis from appendicitis or gall bladder disease; (m) undiagnosed fracture.

Specific evaluation A thorough history and physical examination are necessary including: (a) History of prior functional level, social support, medications and medical problems. Involving caregivers or family member to get a history may be necessary. (b) Examination of cardiovascular and neurological systems. (c) Musculo-skeletal examination, especially for those with reduced mobility. Suspect undiagnosed fracture after apparent minor injury with excessive disability. (d) Abdominal examination to exclude an acute abdomen. (e) Rectal examination. (f) Examination of the fundi. (g) Investigations will be guided by the history and physical (see box below).

Possible investigations FBC and ESR; U&Es; LFTs; TFTs; MSU; blood glucose; calcium;

blood cultures; ECG; CXR and other X-rays as suggested by the examination.

Management Delirium, acute confusion * Refer for admission to acute medical ward or geriatric assessment ward if the diagnosis cannot be clearly identified and the patient cannot be managed at home. * Refer for admission to acute hospital if pneumonia or stroke is evident and the patient cannot be cared for at home. If treating the patient at home: * Treat any infection with an appropriate antibiotic. * Stop medications with potential toxicity, especially sedative/hypnotics if they are suspected of causing acute confusion. * Treat dehydration with fluids with attention to cardiovascular function. * Deal with faecal impaction in the usual way, with attention to follow up bowel function. Fluids, exercise and fibre are all needed for adequate bowel function in older people. Agitation * Treat the cause as above, especially undiagnosed pain. * Refer for admission to geriatric or psychogeriatric assessment ward if the patient cannot be managed at home. * Refer to a psychiatrist for evaluation if psychosis is suspected. * If no cause is found and the patient has underlying dementia, see below (p. 394). * Behavioural management is useful for any agitated patient including: (a) allowing wandering in a safe environment; (b) relocation to alternative living arrangements if agitation becomes a constant problem and cannot be managed in current living arrangement;

SUBACUTE DETERIORATION WITHOUT ACUTE ILLNESS: DEPRESSION OR DEMENTIA

(c) encouraging participation in usual activities; (d) avoiding confrontation if aggression is a feature; (e) physical restraint should be avoided as it causes injury. * See p. 395 for services available. * Medication. Treat with haloperidol if no cause is found and behavioural management fails. * Benzodiazepines should be avoided. Even short-acting benzodiazepines accumulate in the older person and worsen the confusion. Loss of mobility

* Treat acute illness as for acute confusion above. * If minor injury is the cause, and significant disability has resulted and there is no fracture: (a) give adequate pain relief; (b) mobilize as soon as possible; (c) refer to community physiotherapy or outpatients department or day hospital services for rehabilitation.15 * Consider giving vitamin D with calcium co-supplementation. This may reduce the risk of hip fracture in frail older people16 (see p. 180). * Refer the patient to a day hospital, which can improve overall functional outcome and service utilization when compared with no comprehensive care.15 * Refer for comprehensive inpatient geriatric assessment and management if overall function has deteriorated without apparent cause.

SUBACUTE DETERIORATION WITHOUT ACUTE ILLNESS: DEPRESSION OR DEMENTIA • Deterioration in mental or physical status can be slow and progressive and not apparently due to acute illness (i.e. not delirium). • If the onset is slow and workup has not shown a treatable acute cause then the differential diagnoses of dementia or depression must be considered.

393

Diagnosis * The diagnosis of depression is often missed in older people as the prominence of physical symptoms compounds the diagnosis. * Dementia is of more insidious onset than depression. * Cognition and physical deterioration in depression is worse in the mornings. * In depression insight is present, whereas those with dementia rarely have insight. * Orientation is poor in dementia and reasonable in depression. * Memory loss is characteristically worse for recent events in dementia, but can be similar for recent and remote events in depression.

Specific evaluation * Ask about a family and personal history of depression. * Ask about recent significant events. Bereavement and relocation predispose to social isolation and depression. * Check functional status and independence in living activities. * Check the patient's social and financial supports. * Ask about driving. Specific driving assessment may be necessary later. * The geriatric depression scale is useful in diagnosing depression in those without severe dementia.17 * Assess the patient's cognitive state formally using one or both of the following tests: • Clock drawing is a useful, non-threatening test of mental function. Simply draw a circle and ask the patient to 'make it into a clock with the time at 10 to two'. If numbers are not spread throughout all four quadrants then the test is positive. • A standardized mental test score, such as the Abbreviated Mental Test Score, see box. It is only reliable if used precisely.18 Abbreviated Mental Test Score Each question scores 1. A score of 6 or less suggests dementia. 1. Age (exact number of years); 2. Time (to the nearest hour);

394

OLDER PEOPLE

3. A simple address, e.g. 42 West Street, to be repeated by the patient at the end of the test; 4. Year (the current year); 5. The place (exact address or the name of the surgery or hospital); 6. Recognition of two persons present (by name or role); 7. Date of birth (correct day and month); 8. Year of the First World War (1914 or 1918 is enough); 9. Name of the monarch (must be current monarch); 10. Count backwards from 20 to 1 (with no mistakes, or with mistakes which the patient corrects without prompting).

Management * Treatment of dementia is outlined in the next section. * Refer for geriatric medical or geriatric psychiatric assessment if the diagnosis is not clear. * Treat depression, if present, with: (a) a SSRI or a TCA (see p. 311);19 and/or (b) psychological therapy including cognitive behaviour therapy. Access to publicly funded counselling services may limit availability. * Follow-up is essential to ensure recovery of function.

DEMENTIA Guidelines: Practice parameter: Diagnosis of dementia (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2001; 56:1143-53. Practice parameter: Management of dementia (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2W\; 56:1154-66.

• Dementia affects 10% of those over age 65 years and 20% of those over age 80 years. • Absolute numbers of those with dementia will increase exponentially in the next two decades as the world population ages. • New therapies recently available and currently under investigation mean that identification of early dementia will be more important in the future.

Diagnosis * Suspect dementia when the patient has: (a) impairment in short and long-term memory, abstract thinking, judgement, other higher cortical function or personality change; and (b) the disturbance is severe enough to interfere significantly with work, social activities or relationships; and (c) delirium is absent.20 * Confirm it with a formal assessment using the Abbreviated Mental Test Score or the clock drawing test (see above). * Determine, from the history and examination, whether it is possible to decide on the cause of the dementia (see below). This entails examination of the patient's mental state and cognition, a neurological and cardiovascular examination and a screen for depression. However, many patients have atypical or non-specific presentations. * Exclude the few cases of reversible cognitive impairment as detailed below. The box lists the clinical features of the types of dementia.

Types of dementia listed in order of prevalence Dementia - Alzheimer's type • Accounts for the majority of dementia cases • Insidious onset over several years • Global deficits Vascular dementia • Accounts for 10% of dementia cases, although 29-41% of dementia cases autopsied have some vascular pathology • Stepwise progression • Bilateral neurological signs • A history of cardiovascular disease such as HTN suggests vascular dementia • Risk factors, or a history of risk factors for cardiovascular make vascular dementia more likely Dementia with Lewy bodies Dementia plus Balance and gait disorder Prominent hallucinations and delusions Sensitivity to antipsychotics Fluctuations in alertness Frontotemporal dementia • Early loss of personal awareness • Early loss of social awareness • Hyperorality • Stereotyped, perseverative behaviours

DEMENTIA

Prion disease (Creutzfeld-Jakob disease; CJD) • Rapidly progressive symptoms • Characteristic EEG pattern of periodic sharp wave complexes • Pathological brain tissue diagnosis • CSF 14-3-3 protein (high sensitivity and specificity for diagnosis of CJD)

Specific evaluation * History from caregiver or source other than the patient. * Screen for depression. * Examination of mental state, cognition, neurological system. * Examination of cardiovascular system. * Investigations: (a) vitamin B12 level; (b) thyroid function; (c) FBC, U&Es, LFTs, calcium, glucose, ESR; (d) CXR, MSU; (e) Syphilis screening may no longer be needed unless there is a specific risk factor or they are from certain parts of the USA. * Imaging with CT or MRI. Availability will limit access in some regions. * Imaging with single photon emission computerized tomography (SPECT) or positron emission tomography (PET) is not recommended for routine use. * Genetic testing and genotyping is not currently recommended. * The CSF 14-3-3 protein may become available and is useful for confirming or rejecting the diagnosis of CJD in clinically appropriate circumstances.

Management General * It is widely accepted that most patients with dementia should be assessed by a psychogeriatrician or geriatrician. * Cognition, function, mood and behaviour as well as general health should be evaluated in patients with dementia every 6 months.

395

* Refer for intensive long-term education and support for caregivers (if available) to delay time to residential care placement. * Offer or refer for education for family caregivers to reduce caregiver stress. * Refer for home support (see supporting older people at home). * Refer for placement in dementia specific longterm care. This reduces behavioural problems and use of chemical and physical restraints. For patients in residential care * Offer residential care staff education about the behavioural management patients with Alzheimer's disease (AD). * Explain that a safe wandering space is essential for the older person with dementia to avoid agitation and unnecessary restraint, although there is no direct evidence of benefit.21 This is especially useful for 'sundowning' (increased agitation in the late afternoon). * Scheduled toileting and prompted voiding reduces urinary incontinence. * Music therapy, simulated natural sounds and intensive multimodality group training may improve overall function. Medications for cognitive symptoms Alzheimer's disease22 * Treatment with cholinesterase inhibitors should be considered in patients with mild to moderate AD. Accessibility to subsidized medications will limit availability in some countries. The UK National Institute for Clinical Excellence23 recommends that any of the drugs below (except tacrine) may be given to people with mild to moderate AD, but only if the Mini-Mental State Examination (MMSE) score is above 12, the diagnosis of AD has been made in a specialist clinic and treatment has been initiated by a specialist. Studies show a small average degree of improvement in cognition and behavioural control but no effect on the eventual progression of the disease. These include: (a) Tacrine: 10 mg four times daily for 4 weeks, then 20 mg four times daily for 4 weeks, then

396 OLDER PEOPLE

30 mg four times daily for 4 weeks then 40 mg four times daily with dose escalation as tolerated; (b) Donepezil: 5mg daily increased to 10 mg daily after 4 weeks; (c) Rivastigmine: 1.5 mg twice daily for 2 weeks, then 3 mg twice daily for 2 weeks, then 4.5 mg twice daily for 2 weeks to 6 mg twice daily; (d) Galantamine: 16 to 24 mg daily. * Vitamin E may slow the progression of AD, although evidence is limited.24 Ischemic dementia

vascular

dementia

and

mixed

* Evidence-based data supporting pharmacological efficacy of agents to treat non-AD dementia is lacking. However, the patient with vascular dementia would benefit from measures used in other cardiovascular diseases, e.g. aspirin, if the quality of life makes such measures appropriate. Medications for non-cognitive symptoms Behavioural disturbance Treatment of delirium, an acute change in mental status characterized by fluctuation in level of consciousness, is empirical. It is more complex in those with dementia.25 * Look for an organic cause, most commonly undiagnosed pain, infection and cardiovascular disorder as with well older people. * Use behavioural and environmental modification. * Treat agitation and psychosis with psychotropic medications. Risperidone or haloperidol are the drugs of choice. Start at the lowest dose and titrate to the lowest possible effective dose. Financial and legal aspects (UK) * Assess whether the patient is eligible for exemption from the Council tax (see p. 3). * Assess whether it is appropriate for someone else to be given power of attorney (see p. 14 and 399). Support groups: Alzheimer's New Zealand, PO Box 2808, Christchurch, New Zealand, tel. 64 3 365 1590; www.alzheimers.org.nz

Alzheimer's Association Australia, PO Box 108, Higgins 2615, tel. 61 2 6254 4233; www.alzheimers.org.au Alzheimer's Society, Gordon House, 10 Greencoat Place, London SW1P 1PH, tel. 020 7306 0606; www.alzheimers.org.uk Details of Alzheimer's Associations in other countries can be found on the website of the Alzheimer's Disease International. Website: www.alz.co.uk Age Concern New Zealand, PO Box 10-688, Wellington, New Zealand, tel. 64 4 471 2709; www.ageconcern.org.nz Age Concern England, Astral House, 1268 London Road, London SW16 4ER, tel. 020 8765 7200; www.ageconcern.org.uk Details of other UK branches of Age Concern can be found on the UK website above.

FALLS Guideline: American Geriatrics Society, British Geriatrics Society, American Academy of Orthopaedic Surgeons Panel on Falls Prevention. Guideline for the prevention of falls in older persons. J Am Geriatric Soc 2001; 49: 664-72.

Falls are serious and common for those over age 75 years. Risk factors have been identified and specific interventions proven to reduce falls.

Risk factors (a) age over 80 years; (b) cognitive impairment; (c) history of fall and fall-related injury; (d) arthritis; (e) depression; (f) use of mobility aid; (g) gait and balance impairment; (h) lower limb muscle weakness; (i) visual deficit; (j) impaired daily functioning, or a low score on the Nottingham EADL scale (see p. 405); (k) use of psychotropic medications. Those with multiple risk factors are at a higher risk of falls.

FALLS

Diagnosis * Distinguish between 'hot' and 'cold' falls. (a) 'Hot' falls result from major medical conditions such as stroke, myocardial infarction or seizure. Treatment of the acute illness usually entails admission to hospital. (b) 'Cold' falls occur in the absence of serious acute illness. * Case-finding in primary health care for those with a high risk of falls is recommended although clear evidence of benefit is lacking. Case-finding could include a process where every adult over the age of 75 years is asked if they have sustained a fall over the previous year. This part of the chapter deals with management of cold falls and those with high risk of falls. The UK National Service Framework for Older People12 recommends that those who have fallen and those at high risk of falling are referred to a Falls Centre. Specific evaluation Those presenting with a single or recurrent falls or those who have a gait and balance deficit should undergo a fall assessment including: (a) history of fall circumstances; (b) medication review; (c) review of chronic medical problems including alcohol misuse; (d) examination of: - vision; - gait and balance (see below); - lower leg strength; -neurological system, especially proprioceptive and coordination function and including mental status; -cardiovascular system, especially heart rate and rhythm, lying and standing blood pressure and the murmurs of valvular disease. (e) the environment in which the falls occurred, with attention to: -hazards such as loose mats, cords and unstable furniture; - lighting levels; (f) investigations including FBC and ECG.

397

Simple gait assessment Ask the patient to stand from a chair, without using the arms, walk several paces, turn around and return (the Get Up and Go test26). Unsteadiness or difficulty completing this in less than 30 seconds shows a gait and balance deficit and further evaluation is needed.

Management * Results of the evaluation will guide specific management. In general, multiple risk factors are present. * Multifactorial intervention is more effective than single intervention in preventing future falls.27 * All older people over age 75 years should be asked whether they have had a fall in the last year. * Refer all with a history of loss of consciousness to a physician. Investigations, such as a 24-hour ECG, may reveal a cause. * Treat any injury resulting from the fall in the usual way * Treat those at high risk of recurrent falls as follows: Community-dwelling older people at high risk of recurrent falls27 * Refer for a programme of muscle strengthening and balance retraining and advice on use of assistive devices. This depends on the local availability of a falls clinic or falls-related exercise groups. * Encourage increased exercise such as walking or refer to an exercise programme with a balance component. * Refer for Tai Chi C'uan exercise. A 15-week course reduces falls by 50%.28 The availability of classes may be limited. * Review medications and reduce psychotropics. * Home environment. Refer for modification of environmental hazards according to the availability of occupational therapy. * Review all medical conditions. Treat cardiovascular disorders, including postural hypotension and any cardiac arrhythmia.

398

OLDER PEOPLE

* Osteoporosis. Consider giving vitamin D3 with calcium co-supplementation to reduce the risk of vertebral fracture.16 Older people in residential care and assisted living settings at high risk of recurrent falls * Refer to physiotherapy for gait and balance training and advice on use of assistive devices. This depends on the availability of physiotherapy services in long-term care. * Review medications and reduce medication use, especially psychotropics. * Educate staff about falls reduction. * Consider giving vitamin D3 with calcium co-supplementation to reduce the risk of hip fracture.16 * Offer hip protective devices and garments to reduce hip fracture.29

LOWER LIMB PROBLEMS NIGHT CRAMPS Nocturnal leg cramps are common occurrences among older, generally healthy adults.

Diagnosis The history, physical exam, and laboratory testing can uncover treatable causes: (a) (b) (c) (d) (e)

endocrine; neurological; vascular disorders; treatment with certain drugs; occupational factors.

However, a significant portion of cases are idiopathic. Possible investigations FBC and ESR; U & Es; LFTs; blood glucose; calcium; TFTs.

Management * Treat the underlying cause whenever possible. * Massaging and stretching is the recommended first-line treatment for idiopathic cases. * Quinine sulfate also appears to offer safe and effective symptom management, although its efficacy has not been definitively established in clinical trials.30 RESTLESS LEGS SYNDROME Review: Chaudhuri KR, Appiah-Kubi LS, Trenkwalder C. Restless legs syndrome. J A/euro/ Neurosurg Psychiatry 2001; 71:143-6.

• The minimum diagnostic criteria proposed by the International Restless Legs Syndrome Study Group31 are: (a) a desire to move limbs, usually associated with para/dysaesthesia; (b) motor restlessness; (c) symptoms worse or exclusively present at rest: partial/temporary relief with activity; (d) symptoms worse in the evening or at night. • Iron deficiency with or without anaemia is found in a third of elderly patients with restless legs. • A positive family history is present in 63% to 92% of patients with the idiopathic syndrome. Work-up (a) RBC; (b) serum ferritin; (c) folic acid; (d) B12; (e) U & Es.

* Examine to exclude peripheral neuropathy. * Review the patient's drugs. Possible causes are neuroleptics, lithium, beta-blockers, TCAs, anticonvulsants, H2-blockers. * Consider drug treatment for patients with symptoms that are sufficiently severe. Dopaminergic drugs are effective but prone to causing adverse effects. Levodopa itself appears to cause augmentation, that is, symptoms appear to start earlier in the day and may spread to the upper

LEGAL ASPECTS 399

limbs. Start each drug with a low dose at first, administered at least 2 hours before the patient goes to bed. If one drug fails, try one from another group. Options are: (a) levodopa (+decarboxylase inhibitor) starting with a low dose (100-200 mg); (b) bromocriptine 7.5 mg daily; (c) clonazepam 0.5-2 mg daily; (d) carbamazepine 100-300 mg daily.

POSTURAL ANKLE OEDEMA Many patients are given diuretics inappropriately for postural oedema. * Assess carefully to exclude a cardiac, renal or hepatic cause. * Treat postural oedema by advising the patient to: (a) keep active; (b) elevate legs when sitting; and (c) use support hosiery. * Give a diuretic only where the oedema is too severe to permit the patient to pull on a support stocking, and even then only give it for a maximum of 3 weeks. * Consider a trial without diuretics for those already started on them. In one study, 85% of those suitable were successfully withdrawn from diuretics.32 Be aware that even patients who do not need their diuretics are likely to suffer an initial increase in oedema as a rebound phenomenon, which may take 6 weeks to settle.

ELDER ABUSE • A UK study has shown the prevalence of abuse in the patient's own home to be significant, with physical abuse in 2%, verbal in 5% and financial in 2%.33 Forty-five per cent of carers of the elderly in respite care admitted to some form of abuse.34 • There are no statutory guidelines or legislation. Intervention should always be interdisciplinary. • Abuse may take the form of: (a) physical: hitting, pushing, restraining; (b) psychological: threats, insults, left alone unaware of when the caregiver will return;

(c) sexual: unwanted advances, rape; (d) financial: theft of money or property; (e) neglect: failure of adequate personal care / food / medication/ continence /pressure relief etc.; (f) lack of privacy: especially intimate functions; (g) misuse of medications: especially sedatives; (h) routines: e.g. rigid meal, bed and bath times; (i) overstimulation. * The following may suggest abuse: (a) delay in seeking medical help; (b) differing histories from patient and carer, especially if explanations are implausible; (c) inconsistencies on examination; (d) frequent calls for visits by the GP or A & E attendances; (e) carer not accompanying patient when that would be expected; (f) abnormal behaviour in the presence of the carer, e.g. fear or withdrawal. * Discuss the situation with the patient, carer and involved care agencies. Further action will depend on the wishes and competence of the patient and the nature and severity of the abuse. * Discuss the case with Action on Elder Abuse (see box below). Professional and patient information: Action on Elder Abuse, Astral House, 1268 London Road, London SW16 4ER, tel. 020 8765 7000; helpline 080 8808 8141; www.elderabuse.org.uk

LEGAL ASPECTS Power of attorney At an early stage of declining mental function, suggest to the family that they consult a solicitor about an Enduring Power of Attorney (EPA) before the patient is incapable of signing the form. In order to sign, the patient must be capable of understanding the implications of so doing, and may be capable of this even if incapable of managing his or her affairs. If it is left too late, it is then necessary to apply to the Court of Protection to appoint a receiver, and this is much more

400 OLDER PEOPLE

cumbersome. Note that this is different from the registration of an EPA with the Court of Protection. This becomes necessary when the person who signed it becomes incapable of managing his or her own affairs (see p. 14).

Detention of an older person Guideline: UK: Section 47 of the National Assistance Act and Article 10, The Human Rights Act 1999.

The National Assistance Act allows for the 'detention and maintenance in suitable premises of persons in need of care and attention'. It enables a local authority to seek an order from a magistrates court for the removal of a person from his or her home on the grounds that: (a) He is suffering from a grave chronic disease or, being aged, infirm or physically incapacitated, is living in insanitary conditions; (b) He is unable to look after himself and is not receiving proper care and attention from anyone else; and (c) His removal is necessary in his own interests or for preventing injury to the health or, serious nuisance to, other persons. Following the court hearing, an order can be made to remove a person by a specified officer of the appropriate authority. The order authorizes the person's detention and maintenance, but does not provide for medical treatment to be given without the person's consent. The initial order can

last up to 3 months although it can be extended. If after 6 weeks an appeal is successful, the order can be revoked. There is now some discussion that The Human Rights Act 1999, which came into force on 2 October 2000, will prove contrary to the National Assistance Act, as Section 6 (1) of the former makes it unlawful for a public authority to act in a way which goes against the European Convention on Human Rights, such as everyone has the right to 'liberty and security of person' and 'respect for his private and family life, his home and his correspondence.' However, if the person is removed under Section 47, this can be undertaken under 'protection for health,' although it remains a possibility that removals may be appealed at some stage in the future. * Refer for comprehensive assessment by social worker, geriatrician, or psychogeriatrician, as part of application to the magistrate. This is not technically required under the Act. Note: The Act should not be used for patients with mental disorder, who are covered by the Mental Health Act. * In Australia and New Zealand, refer to the local geriatric assessment team if there is a need for legal intervention to ensure patient safety and wellbeing.

Mental capacity See p. 13.

REFERENCES 1. Curb }, Pressel S, Cutler } et al. Effect of diuretic-based antihypertensive treatment on cardiovascular disease risk in older diabetic patients with isolated systolic hypertension. Systolic Hypertension in the Elderly Program Cooperative Research Group. / Am Med Assoc 1996; 276: 1886-92. 2. Vetter N, Ford D. Smoking prevention among people aged 60 and over: a randomized controlled trial. Age Ageing 1990; 19: 164-8. 3. DHSS. Living in Britain: Results from the 1994 General Household Survey. Norwich: The Stationery Office, 1994.

4. Anderson P. Effectiveness of general practice interventions for patients with harmful alcohol consumption. Br J Gen Prac 1993; 43: 386-9. 5. Manley A. Physical activity and health. A report of the Surgeon General. Pittsburgh: US Department of Health and Human Services, 1996. 6. Rooney E. Exercise for older patients: Why it's worth the effort. Geriatrics 1993; 48: 68-72. 7. Elward K, Larson E. Benefits of exercise for older adults. A review of existing evidence and current recommendations for the general population. Clin Geriatric Med 1992; 8: 35-50.

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8. Kerse N, Jolley D, Arroll B et al. Improving health behaviours of the elderly: a randomised controlled trial of a general practice educational intervention. Br Med J 1999; 319: 683-7. 9. Campbell A, Robertson M, Gardner M et al. Randomised controlled trial of a general practice programme of home based exercise to prevent falls in elderly women. Br Med J 1997; 315: 1065-9. 10. Campbell A, Buchner D. Unstable disability and the fluctuation of frailty. Age Ageing 1997; 26: 315-18. 11. Elkan R, Kendrick D, Dewey M et al. Effectiveness of home based support for older people: systematic review and meta-analysis. Commentary: When, where, and why do preventive home visits. Br Med J 2001; 323: 719. 12. Department of Health. National Service Framework for Older People. London: Department of Health, 2001. Online. Available: www.doh.gov.uk/nsf/olderpeople.htm 13. Mold J. Principles of geriatric care. American Health Consultants. Primary Care Reports 1996; 2: 2-9. 14. O'Keefe K, Sanson T. Elderly patients with altered mental status. Emerg Med Clin N Am 1998; 16: 701-15. 15. Forster A, Young J, Langhorne P. Medical day hospital care for the elderly versus alternative forms of care (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. 16. Gillespie W, Avenell A, Henry D et al. Vitamin D and vitamin D analogues for preventing fractures associated with involutional and post-menopausal osteoporosis (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. 17. Montorio I, Izal M. The Geriatric Depression Scale: a review of its development and utility. International Psychogeriatrics 1996; 8:103-12. 18. Holmes J, Gilbody S. Differences in use of the Abbreviated Mental Test Score by geriatricians and psychiatrists. BMJ 1996; 313: 465. 19. Wilson K, Mottram P, Sivanranthan A et al. Antidepressants versus placebo for the depressed elderly (Cochrane review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. 20. American Psychiatric Association. Diagnostic and Statistical manual of mental disorder, 3rd edition, revised 1987. Washington DC: American Psychiatric Association, 1987. 21. Price J, Hermans D, Grimley Evans J. Subjective barriers to prevent wandering of cognitively impaired people

22.

23.

24. 25.

26. 27.

28.

29.

30. 31. 32. 33. 34.

(Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. Doody R, Stevens I, Beck C et al. Practice parameter: management of dementia (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2001; 56: 1154-66. UK National Institute for Clinical Excellence, Technology appraisal guidance No. 19. London: National Institute for Clinical Excellence, 2001. Online. Available: www.nice.org.uk Tabet N, Birks J, Grimley Evans J et al. Vitamin E for Alzheimer's disease (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. Britton A, Russell R. Multidisciplinary team interventions for delirium in patients with chronic cognitive impairment (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. Mathias S, Nayak U, Isaacs B. Balance in elderly patients: the 'Get up and Go' test. Arch Phys Med Rehabil 1986; 67: 387-9. Gillespie L, Gillespie W, Robertson M et al. Interventions for preventing falls in elderly people (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. Wolf S, Barnhart H, Kutner N et al. Reducing frailty and falls in older persons: an investigation of Tai Chi and computerized balance training. Atlanta FICSIT Group. Frailty and Injuries: Cooperative Studies of Intervention Techniques. / Am Geriatric Soc 1996; 44: 489-97. Parker M, Gillespie L, Gillespie W. Hip protectors for preventing hip fractures in the elderly (Cochrane Review). In: The Cochrane Library, Issue 1. Oxford: Update Software, 2002. Kanaan N, Sawaya R. Nocturnal leg cramps. Clinically mysterious and painful - but manageable. Geriatrics 2001; 56: 39-42. Walters A. Towards a better definition of restless legs syndrome. The International Restless Legs Syndrome Study Group. Movement Disord 1995; 10: 634-42. de Jonge J et al. Short term effect of withdrawal of diuretic drugs prescribed for ankle oedema. BMJ 1994; 308: 511-13. Ogg J, Bennett G. Elder abuse in Britain. BMJ 1992; 305: 998-9. Homer A, Gilliard C. Abuse of elderly people by their carers. BMJ 1990; 301: 1359-62.

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CHAPTER CONTENTS

23

Physical disability and rehabilitation 403 Carers

Disability

408

People with learning disability (UK) or intellectual disability (Australia) 409 Down syndrome References

411

413

PHYSICAL DISABILITY AND REHABILITATION • A person is defined as disabled if blind, deaf, dumb, or suffering from mental disorder, or substantially and permanently handicapped by illness, injury or congenital deformity or other such disabilities (The Chronically Sick and Disabled Persons Act 1970). • Clear evidence exists for the benefit of rehabilitation in patients with stroke,1 heart failure1 and falls due to poor balance. • Physiotherapy can reduce pain and improve mobility.2 • The provision of equipment and alterations to the home can improve outcomes.3 Every patient with a significant disability needs: (a) a key worker, who may be a GP, who will coordinate all aspects of care; (b) an assessment of overall disability, by the occupational therapist (OT) as a minimum; (c) an identification of a person who is a carer. The GP needs to become familiar with the medical condition that has caused the disability. Where the condition is a rarity, the GP will not gain the confidence of the family without this knowledge and will not be able to take a proactive role. Check the following: (a) Can the patient's physical state be improved? (b) Can the patient's mental state, including self-esteem, be improved? 403

404

DISABILITY

(c) Can the patient's ability to live independently be improved? (d) Can the patient's mobility inside and outside be improved? (e) Is there any work that the patient may be able to undertake? (f) Can the patient be helped to enjoy leisure and social activities? (g) Would the carer benefit from more support? (h) Can the patient's or carer's financial state be improved (see Chapter 1)? Patient information and organizations: Department of Health. A practical guide for disabled people: where to find information, services and equipment (HB6). London: Department of Health, 2002. Royal Association for Disability and Rehabilitation (RADAR), 12 City Forum, 250 City Road, London EC1V 8AF, tel. 020 7250 3222; e-mail: [email protected] Disablement Information and Advice Line (DIAL), St Catherine's, Tickhill Road, Doncaster, South Yorkshire DN4 8QN, tel. 01302 310123; e-mail: [email protected]

Referral Consider referral to the appropriate agency if: (a) the cause of the disability is unknown; or (b) there is new impairment, disability or handicap; or (c) there is deterioration in existing impairment, disability and handicap; or (d) the disabled person and/or their carer are barely coping.

Eligibility Section 2 of the Chronically Sick and Disabled Persons Act 1970 lists the arrangements that the Local Authority has a duty to make: (a) the provision of practical assistance for that person in his or her home; (b) the provision for that person of, or assistance to that person in obtaining wireless, television, library or similar recreational facilities; (c) the provision for that person of lectures, games, outings ... and taking advantage of educational facilities that are available; (d) the provision of facilities or assistance in travelling to and from his or her home for the purposes of participating in any services provided; (e) the provision of assistance for that person in arranging for the carrying out of any works of adaptation in the home or the provision of additional facilities designed to secure his or her greater safety, comfort or convenience; (f) facilitating the taking of holidays; (g) the provision of meals for that person in his or her home or elsewhere; (h) the provision for that person in obtaining a telephone and any special equipment necessary to enable him or her to use a telephone.4

Where rehabilitation services can be located

Consider:

With the overall reduction of hospital bed numbers there has been expansion of rehabilitation services accessible to the GP and provided in community settings. GPs need to be familiar with the provision in their local area. Awareness of the eligibility criteria of the different services are essential in managing patients' expectations.

(a) unidentified medical problems, e.g. anaemia, heart failure, hypothyroidism, Parkinson's disease; (b) occult depression; (c) occult dementia that may be masked by intact social skills; (d) communication problems, e.g. poor vision or hearing.

(a) Social Services for the OT team, and visual and hearing impairment teams. (b) Intermediate care: often integrated health and social services multidisciplinary teams for defined periods of rehabilitation either up to 2 weeks (rapid response) or 6 weeks. They may provide home-based services or alternative beds outside the acute hospital setting.

Poor progress

PHYSICAL DISABILITY AND REHABILITATION 405

(c) Community rehabilitation teams: for specific clinical conditions such as stroke, physical disability, learning disability, etc.

Assessment of level of disability and dependency In addition to the assessment of the underlying condition a broader picture of how the person is coping with managing daily tasks can assist in deciding where referrals need to be made. Activities of daily living can be categorized into: (a) personal care: feeding, dressing, toiletting, grooming and indoor mobility; (b) extended care: managing domestic tasks and the wider environment in cooking, shopping, working, and socializing. The Community Dependency Index (CDI) (see Appendix 24) is a standardized assessment of personal care and is recommended to use when: (a) there is high level dependency, the patient is predominantly housebound, reliant on informal carers; (b) when a package of care from statutory services appears to be indicated. The CDI will help decide on the frequency and extent of support needed. A score of less than 75 usually represents a moderate disability and a score of less than 50 represents a severe disability.5'6 The Nottingham Extended Activities of Daily Living Questionnaire (see Appendix 25) is more appropriate when there are low or moderate levels of dependency and the person is able to access the wider environment.7

Assessment and improvement of the home environment Refer for an OT assessment if the home environment is presenting a barrier to the person managing daily tasks independently. The following are the most common areas that the GP can observe, or ask questions about, to identify difficulties: • Access Are the steps and stairs at the entrance way and within the house negotiable by the person?

Is there level access or do thresholds or door widths present a barrier? Is there adequate space to manoeuvre in corridors and in rooms with mobility equipment? Do the floor coverings present a hazard? • Controls Can the person reach sockets and light switches? Is the home adequately heated? Is the lighting, door and window security adequate? Can the person get to the door to respond to callers? • Kitchen Can the person reach the oven and hob to handle hot items safely? Can the person reach into both high and low cupboards? Is the sink and work surface a suitable height? Can the person open jars and tins, and prepare vegetables? • Toilet and bathroom Is the person able to get in and out of the bath/ shower? Can the person get on and off the toilet? Can the person manage taps? • Bedroom and living rooms Can the person get in and out of his or her bed and chair? Can the person easily reach the telephone from his or her bed or chair? Self assessment for patients on reducing home hazards: Clemson L. Westmead Home Safety Assessment.8 Forms can be purchased through Winslow, tel. 0845 921 1777; e-mail: [email protected]

Role of GP in rehabilitation assessment To enable the OT or rehabilitation team to plan appropriate short- and long-term intervention they often require detailed information on the client's disability, diagnosis and prognosis. In complicated cases it is useful to discuss the issues to reach realistic long-term solutions and to ensure resources are appropriately allocated for major equipment and adaptation.

406

DISABILITY

Community interventions Following assessment the OT or rehabilitation team may: 1. plan interventions to reduce the impairment and prevent complications through goal-focused, patient-centred home activity programmes that are reviewed and progressed; 2. reduce the disability through advice on, and in some cases supply of, specialist equipment; 3. recommend environmental adaptations both minor and major or if necessary provide rehousing reports. These three broad areas of intervention are generally considered to form a hierarchy and are covered in more detail in the rest of this section. Throughout, advice, support and training can be given to formal and informal carers alongside liaison with other involved parties. Community rehabilitation The following areas of rehabilitation may be included in a patient's programme. Referrals for specific services may be made either by the GP or the therapist, or for some services the therapist may need to approach the GP for authorization. (a) Physical rehabilitation: to improve balance, muscle power and joint range of movement, reduce pain, maintain positioning, seating, the management of tone and spasms, referral for splints or prosthetics. Passive movements need to be completed regularly by a carer trained by the therapist to prevent contractures. GPs and therapists will need to work closely together to achieve optimum dosage of medication for spasticity in order to reduce spasm without reducing tone to such an extent that function is adversely effected. (b) Cognitive rehabilitation: problem solving, perceptual difficulties, memory strategies, compensation techniques. (c) Vocational rehabilitation: assessment of vocational aptitudes, referral to the Disability Employment Advisor, sources of equipment to support employment (see Chapter 1). (d) Community access: refer for shop mobility schemes, taxi-card schemes, dial-a-ride, driving

assessment centres, motability scheme for adapted vehicles for a disabled person as a driver or as a passenger (see Chapter 1). (e) Leisure: the importance of leisure activities for self-esteem and motivation should not be overlooked; accessing clubs and community education classes, socializing and a balance of active and sedentary pastimes. Community equipment services A wide range of large-scale equipment can be loaned to patients for use both in the long term and short term. Increasingly, health and social services are required to integrate their provision into a single service.9 There may be some charges for loans. Small-scale equipment such as dressing, feeding and kitchen equipment may not be available for loan. GPs can encourage patients to return equipment that they no longer use. Commonly available equipment includes: (a) nursing equipment: hoists, beds, pressurerelieving equipment, commodes, urinals, palliative care equipment; (b) mobility and positioning equipment: sticks, crutches, frames, wheelchairs, cushions, wedges, transfer belts and boards, multiglide sheets; (c) daily living equipment: toilet seats and frames, bath boards, seats and hoists, trolleys, perching stools, bed and chair raisers, modified cutlery, tap turners. Alternative sources of equipment Retail outlets: Look up Yellow Pages index under 'disabled equipment and vehicles' Mail order catalogues for equipment: • Ways and Means, Novara House, Excelsior Road, Ashby Park, Ashby de la Zouch, Leicestershire LE65 1NG, tel. 01530 418916. • Smith and Nephew, PO Box 5665, Kirkby-in-Ashfield, Notts NG17 7QX, tel. 01623 722337; http://www.snrehab.com Mail order catalogues for special shoes: • Hotter Comfort Concept, tel. 0800 525 893. • Cosyfeet, 5 The Tanyard, Leigh Road, Street, Somerset, BA16 OHR, tel. 01458 447275. Mail order catalogues for adapted clothes: • Rolli Moden, tel. 0049 6226 960 208; www.rolli-moden.de

PHYSICAL DISABILITY AND REHABILITATION

407

• Silvalea Textiles, Unit 3-4, Silverhills Buildings, Decoy Industrial Estate, Newton Abbot, DevonTQ12 5LZ, tel. 01626 333793; www.silvaleatextiles.co.uk

• All patients who use powered vehicles should be advised to take out at least third party liability insurance.

Assist!ve technologies for profound disability.

For patients who depend on these assistive technologies, a 24-hour call-out service for breakdown maintenance and servicing arrangement is essential.12

GPs and community therapists will need to consider the need for, and know where to refer for, the following: (a) (b) (c) (d)

communication aids; environmental control systems; sensory alarm systems; wheelchairs.

Wheelchairs. GPs and therapists can requisition the issuing of a manual wheelchair from a standard limited range. Assessment needs to include the frequency and location of use, height and weight of the patient, whether the patient will self propel, fitness of the main carer for attendantpropelled wheelchairs, whether the wheelchair will be transported in a car and, if being used in the home, whether any adaptations to the house are required. If a patient has a very clear idea of a nonstandard type of wheelchair he or she requires, the patient should be advised about the voucher scheme. There are two options: (a) The independent option to contribute to the cost of a more expensive wheelchair of the patient's choice. The patient will own the wheelchair and be responsible for its maintenance and repair. (b) The partnership option to contribute to the cost of a more expensive wheelchair of the patient's choice from a range selected by the local wheelchair service. The NHS will own the wheelchair and be responsible for its maintenance and repair.10 Powered wheelchairs

• Referral should be made to the local wheelchair service for electrically powered indoor chairs (EPIC), electrically powered indoor/outdoor chairs (EPIOC) and special seating clinics for wheelchair postural supports. • OTs can advise on suitable electric scooters. Patients who receive mobility allowance can use this to hire or hire/purchase a scooter through the Motability scheme11 (www.motability.co.uk).

Home adaptation: grants Assistance towards housing adaptations for people with permanent and substantial physical disability is available via the Disabled Facilities Grant, for which there is a means test. The OT's role is to assess and advise on what is necessary and appropriate.13 If the disabled person wishes, the OT can make a referral to the local council for a grant. The grant process is lengthy and works cannot be started prior to grant approval. At the present time grants can be mandatory or discretionary. Indications for mandatory grants (a) Access in, out and around the property to the principal rooms: kitchen, bathroom, toilet, living room, bedroom. (b) To ensure the safety of the disabled person and his or her carer. (c) Access to essential facilities: bathing, cooking, lighting, heating. (d) To enable the disabled person to care for dependants or visa versa. Discretionary grants. In some circumstances, a grant may be available for a disabled person's accommodation, welfare or employment needs, e.g. a safe play area for a disabled child or to enable a housebound disabled person to work from home. Other grants. Renovation grants are available for properties in serious disrepair. Home assistance grants are for smaller works. The disabled person should be advised to apply direct to the council. In all cases, the council will need to check that proposed works are necessary, appropriate, reasonable and practical. There are plans to phase out the discretionary grants, renovation grants and home assistance grants. New options include assistance to move to a new property, preferential loans to top-up

408 DISABILITY

the mandatory grant for meeting the costs of a home owner's contribution and equity release loans. Councils will be required to publish their local housing strategy.14 For information on the grants and schemes in operation, apply to the local council or the Home Improvement Agency. Other sources of information for adaptations and equipment Patients who have above the financial savings threshold for grants or compensation cases may wish to seek assistance privately to avoid waiting for assessment and advice. Alternatives could include the following: (a) Private OT: lists obtainable through College of Occupational Therapists (UK), 106-114 Borough High Street, Southwark, London, SE1 1LB, tel. 020 7357 6480; www.cot.co.uk (b) Independent Living Centres and Disability Resource Centres. (c) National exhibitions: Naidex, Independent Living, Care and Rehabilitation. (d) Centre for Accessible Environments for architectural advice, consultancy, training and publications for new build homes, public buildings and spaces. Nutmeg House, 60 Gainsford Street, London SE1 2NY, tel. 020 7357 8182; e-mail cae@ globalnet.co.uk (e) Disabled Living Foundation - a registered charity that provides information on equipment that promotes independence. It runs an equipment centre, telephone helpline, training events and publishes the Hamilton Index, a comprehensive database of equipment and suppliers (available on subscription), tel. 020 7289 6111; e-mail info@ dlf.org.uk; www.dlf.org.uk (f) Care and Repair England supports a network of home improvement agencies dedicated to offering home repair and adaptations for older and disabled people to live independently in their own homes for as long as they wish. 3rd Floor, Bridgford House, Pavilion Road, West Bridgford, Nottingham NG2 5GJ, tel. 0115 982 1527; e-mail [email protected]; www.careandrepair-england.org.uk (g) Joseph Rowntree Foundation for research on housing and social issues. Details of Part M

building standards, lifetime home standards and smart homes are on their website. The Homestead, 40 Water End, York, North Yorkshire YO30 6WP, tel. 01904 629241; e-mail: info® jrf.org.uk; www.jrf.org.uk (h) Mandelstram M. How to get equipment for disability, 3rd edition. London: Jessica Kingsley and Kogan Page for the Disabled Living Foundation, 1993.

Supports for home living Refer to Social Services for: (a) home care assessment, identification of needs, commissioning care through private care agencies for personal care tasks only, 24-hour live-in carer schemes; (b) meals; (c) respite care including sitting service, day care and short or long stays in residential/nursing homes; (d) personal alarm systems; (e) assessment of carer's needs.

CARERS Guideline: The National Strategy for Carers. London: Department of Health, 2001. Online. Available: www.carers.gov.uk

• Six million people in the UK are carers, of these 1.5 million care for more than 20 hours per week. Family and friends provide 70% of the care. The Australian Bureau of Statistics15 estimates that there are 2.3 million carers in Australia, or one in every five households. Of these carers, about 500,000 are providing substantial or full-time care. • Caring imposes a strain on the health of the carer. Twenty per cent have never had a break from caring, and 65% admit that their own health has suffered.16 • Carers need good information on the needs and treatment of the person they are caring for. One study found that only a third of carers had received any information, training or guidance in

PEOPLE WITH LEARNING DISABILITY (UK) OR INTELLECTUAL DISABILITY (AUSTRALIA)

relation to the patient's medication, dressings or other health-care needs.17

Services UK local services have a duty to assess the ability of the carer to provide care, and to increase support services they provide to the household if the carer cannot cope (Carers (Recognition and Services) Act 1995). Parents who care for disabled children can ask for a separate assessment under the Children Act or Section 2 of The Chronically Sick and Disabled Persons' Act. Young people under 18 often act as carers and have the same right to an assessment and to provision of services as an older carer.18

Common problems for carers (a) Poor health and exhaustion. (b) A restricted social life. (c) Damage to their relationships with the person with care needs or with their partner. (d) Emotional problems: -anger with the situation, with the person they are caring for, and with those who provide services; -feeling undervalued, especially by health professionals, if they think they are being patronized; (e) Financial limitations: -loss of earnings; -increased wear and tear on clothing, upholstery, etc; - the need to purchase special items; - increased heating and laundry bills. * Consider whether keeping an index of carers would help to foster an awareness of their needs. * During every consultation with a patient or carer consider: (a) whether the carer and patient know everything they need to know about the illness. Information can, however, only be passed to the carer with the patient's consent;19 (b) whether more is expected of the carer than he or she has been educated for, e.g. simple nursing procedures;

409

(c) the impact of caring under the categories above. * Discuss with every carer the need for extra support, with a view to referral to Social Services for assessment. * Direct the carer to appropriate local or national voluntary organizations to provide emotional support as well as practical advice (e.g. eligibility for financial benefits or the provision of breaks). Support and advice for carers Australia: Carers Australia: www.carers.asn.au Carer Resource Centres: freecall 1800 242 636 (10 a.m.-4 p.m. Monday to Friday). Provide support and information for carers. Carer Respite Centres: freecall 1800 059 059. Provide emergency assistance 24 hours per day. UK: Carers' National Association: tel. 0345 573369; www.carersuk.demon.co.uk; Helpline 0808 808 7777 (10 a.m.-12 noon and 2 p.m.-4 p.m.).

Princess Royal Trust for Carers: 020 7480 7788; www.carers.org Crossroads - Caring for Carers: tel. 01788 573653; www.crossroads.org.uk Contact a Family: tel. 020 7383 3555.

PEOPLE WITH LEARNING DISABILITY (UK) OR INTELLECTUAL DISABILITY (AUSTRALIA) • Poor communication is a major barrier to care. It can be minimized by people with an intellectual disability, where able, or their carers, providing high quality health information. Clinicians can only make informed assessments and management recommendations if symptoms or signs, such as records of seizures, behaviour, bowel chart or menstruation, and past history, are clearly documented. It is the responsibility of paid carers and their organizations to provide such information.20 • The GP is the professional most in contact with people with an intellectual disability and the one responsible for the provision of medical care. Proactive care is especially important as, at times,

410

DISABILITY

such patients will not seek care or be able to describe subjective experience crucial to assessment and diagnosis. Investigations may be indicated where a clear history is lacking. • The possibility that the aetiology of an individual's disability can be identified becomes more likely as genetic knowledge increases. Accurate diagnosis may have major ramifications for the family and for the person with the disability. • There are high levels of undiagnosed and undermanaged conditions.16 The RCGP has recommended regular medical review21 and some evidence exists to support yearly health review byGPs.18'22 * Refer when the suspicion of learning disability arises. Re-refer if there is weight change, change in behaviour or if the patient's carer or a close relative dies.23 * Check the aetiology of the patient's condition, if it is unknown: (a) Send blood for karyotyping and screening for fragile X. (b) Send urine for screening for inborn errors of metabolism. (c) Consider review by a genetics service.

Areas of concern24 (a) Gastrointestinal disorders -Constipation is present in up to 70%, with the attendant risk of faecal impaction, atonic bowel and obstruction. -Reflux oesophagitis is present in 30% of people with an intellectual disability with an IQ <50. The use of anticonvulsants and cerebral palsy appear to predispose patients to develop reflux oesophagitis.25 The prevalence of H. pylori is high, with up to 90% in institutional populations affected. (b) Dental care: is often poor. Dental disease is found in up to 86%. (c) Visual impairment: is present in 19-44% of adults. Eye pathology is common.22 (d) Hearing impairment: is present in 12-36% of adults, yet regular screening is not commonly performed. Wax impaction is common.

(e) Epilepsy: • The risk of death is increased 3.6 times, when compared to non-disabled people with epilepsy. Under- and overdiagnosis occurs. • Assessment should include past history of treatment, investigation, identification of seizure type and assessment of the standard of support that is needed. In particular, the service must ensure that: - the prescribed medication is given; -carers are adequately trained to provide rescue medication; -an experienced staff or family member attends the consultation and provides an adequate history and can describe the impact of the epilepsy on the person's quality of life; - the risk of bathing or showering has been assessed; -a management plan for acute seizures has been agreed; - the impact of the epilepsy in the patient's social setting has been assessed. (f) Nutrition, inactivity and obesity: obesity in this population has been found to be between 10 and 40%.22'24'25 * Ensure that adequate exercise is made available. * Check for nutritional deficiencies associated with difficulties with feeding and swallowing. (g) Psychiatric disorders: the lifetime prevalence is probably 30^10%. Unrecognized grief reaction, post-traumatic stress, depression and anxiety disorders are common. (h) Medication: inadequate review, polypharmacy and side-effects are all common, (i) Behaviour change: may indicate an unrecognized physical or mental health problem per se or be secondary to changes in the environment, (j) Unrecognized pain: with musculoskeletal and other sources of pain unreported. (k) Women's health: menstrual management can often be taught and medications avoided. Cervical smears are indicated where women fulfill the criteria for screening, in particular that they have at some time in their life been sexually active. (1) Men's health: check for undescended testes.

DOWN SYNDROME

(m) Osteoporosis: is more common26 in people with an intellectual disability, and especially those with Down syndrome. Some risk factors for osteoporosis such as immobility, use of anticonvulsants, hypogonadism and poor diet, are more common in this population. (n) Hypothyroidism: is present in 15% of people with Down syndrome and also may be increased in the non-Down syndrome population with intellectual disability. (o) Health screening/promotion activities: may have been omitted. * Check that the patient's immunizations meet national standards. Ensure, especially, that hepatitis A and B, influenza and pneumococcus are covered where indicated. The Australian National Health and Medical Research Council,27 and the UK Department of Health recommend hepatitis A and B immunization where people with learning disability live in residential care facilities, especially in large institutions. * Cervical smears and mammography are all indicated as per national guidelines. Resources for patients and families: Australia: Ideas: tel. 1800 029 904 provides a national database of disability and aged care organizations and links to information lines in each state. UK: Mencap: tel. 020 7454 0454; information line 020 7696 5593; www.mencap.org.uk

SPOD (Association to Aid the Sexual and Personal Relationships of People with a Disability), tel. 020 7607 8851. The Family Fund (able to provide financial help to families caring for a child with severe disabilities) tel. 01904 621115; www.familyfundtrust.org.uk A list of other resources can be found on www.psychiatry.ox.ac/cebmh/whoguidemhpcuk.html (choose 'learning disability').

DOWN SYNDROME Guidelines: Cohen Wl, for the Down Syndrome Medical Interest Group. Health Care Guidelines for Individuals with Down Syndrome: 1999 Revision.

411

Down Syndrome Quarterly, 4(3). Online. Available: http://www.denison.edu/dsq/health99.shtml DSMIG Surveillance Essentials Development Group. Basic medical surveillance essentials for people with Down's syndrome, 1999-2000. Online. Available: www.dsmig.org.uk

Patients with Down syndrome need regular surveillance. This may not occur even if they see a pediatrician. Topics that need attention are: (a) Cardiac problems: have a prevalence of 50%, mostly endocardial cushion defects, with some being life-threatening and treatable. * All babies need a cardiological assessment, as the cardiac abnormality may not be clinically easy to detect. * Refer again at the age of 18; the patient may have developed mitral valve prolapse or aortic regurgitation despite normal findings at an earlier assessment. * Give antibiotics to all adults with Down syndrome who: - are known to have a cardiac defect prone to SBE; or -who have not had an echocardiogram since early childhood. (b) Eye problems: are very common and include stenotic nasolacrimal ducts, blepharitis, conjunctivitis, cataracts (from birth and increasing throughout life), keratoconus and refractory errors. * Routine evaluations should begin at 6 months of age (USA) or in the second year of life (UK), and be performed annually (USA) or every 2 years (UK) thereafter. (c) Hearing and ENT problems: over 50% of people with Down syndrome have a significant hearing impairment. If undetected it is likely to be a significant cause of preventable secondary handicap. * Ensure the patient has a hearing assessment before 6 months of age then yearly until age 3 and 2-5 yearly thereafter (USA) or between 6 and 10 months, yearly until age 5, then every 2 years for life (UK). (d) Thyroid diseases: Up to 40% have thyroid dysfunction at some time in their lives. About 7% have current hypothyroidism, while in others

412 DISABILITY

it is subclinical. The clinical diagnosis can be difficult.28 * Check TFTs every 1 to 2 years. If the TSH is only mildly raised but the thyroxine (T4) is normal and the patient is clinically euthyroid, check antithyroid antibodies and give T4 only if they are raised. (e) Sleep apnoea: is common and may result from excessive lymphoid tissue in the nasopharynx. The usual symptoms of sleep apnoea may be present or just behaviour change. * Refer for sleep and ENT assessment if suspicious that this may be a problem. (f) Growth: The average height at any age is around the 2nd centile for the general population. Deviations from this expected height may indicate congenital heart disease, upper airways obstruction, coeliac disease, hypothyroidism or poor nutrition because of feeding problems. * Chart height and weight on charts specific for Down syndrome. * Refer those who fall significantly below their expected centile. * Manage obesity with advice about exercise and diet. (g) Alzheimer's-type dementia: is increasingly present from middle age. * Exclude depression and other reversible causes of functional deterioration, especially hypothyroidism. (h) Osteoporosis: appears to be more common. * Review dietary calcium and check levels of estradiol, progesterone, FSH , LH, TSH, vitamin D and testosterone. Consider assessing the bone mineral density. (i) Infections and immunodeficiency: are both common. Frequent respiratory infections may be due to low IgG levels. * Check IgG levels if there are recurrent or chronic infections. Levels of IgG subclasses must be measured. In Down syndrome, subclasses 2 and 4 may be low yet the total IgG normal. * Give influenza and pneumococcus immunizations. (j) Atlantoaxial instability: ligamentous laxity occurs in 14% and about 1% suffer from acute or

chronic neurological problems due to instability of the cervical spine. Cervical spine X-rays in children are not helpful in predicting subsequent acute dislocation or subluxation at the atlantoaxial joint. * Do not ban sport: there is no evidence that it increases the risk of cervical spine injury in this group any more than in the general population. * Refer urgently anyone with Down syndrome who develops: -pain behind the ear or elsewhere in the neck; - an abnormal head posture; - torticollis; -a deterioration in gait, manipulative skills or bowel or bladder control, (k) Gastrointestinal disorders: congenital abnormalities, such as duodenal atresia and imperforate anus, partial upper GI obstruction, tracheo-esophageal fistula and pyloric stenosis are all more common as are chronic constipation and Hirschsprung disease. Between 7 and 16% have coeliac disease. (1) Skin: alopecia areata, vitiligo and fungal infections are common. (m) Depression: appears to be more common. Exclude hypothyroidism. (n) Bereavement: many patients with Down syndrome live with elderly parents, and are strongly attached to, and dependent on them. In addition, when a parent dies they may be unable to understand death or their own grief. They may find things even harder if they are sent away in a mistaken attempt to spare their feelings. This problem can be reduced by getting the patient used to other people, and to staying away for short periods.

Resources for patients and families: Australia: Down Syndrome Association of NSW Inc., tel. (02) 9683 4333; www.hartingdale.com.au/~dsansw which includes links to associations in other states. UK: Down's Syndrome Association, tel. 020 8682 4001; www.dsa-uk.com

REFERENCES

413

REFERENCES 1. Sinclair A, Dickinson E. Effective practice in rehabilitation, the evidence of systematic reviews. London: King's Fund Publishing, 1998. 2. Young J. Rehabilitation and older people, BMJ 1996; 313: 677-81. 3. Mountain G. Occupational therapy in social services departments: a revieiv of the literature. London: College of Occupational Therapists, 2000. 4. Dirnond BC. Legal aspects of occupational therapy. Oxford: Blackwell Science, 1997. 5. Eakin P, Baird H. The Community Dependency Index: a standardised assessment of need and measure of outcome for community occupational therapy. Br J Occ Ther 1995; 58: 17-22. 6. Ward G, Macaulay F, Jagger C, Harper W. Standardised assessment: a comparison of the Community Dependency Index and the Barthel Index with an elderly hip fracture population. Br J Occ Ther 1998; 61: 121-126. 7. Gladman JR. The Extended Activities of Daily Living Scale: a further validation. Disabil Rehab 1992; 14: 41-3. 8. Clemson L, Fitzgerald M, Heard R. Content validity of an assessment tool to identify home fall hazards: the Westmead Home Safety Assessment. Br J Occ Ther 1999; 62: 171-9. 9. Department of Health. Community equipment services, HSC 2001/008. London: DoH. 10. Department of Health. Wheelchair voucher scheme, HSG (96)53. London: DoH. 11. The Disability Information Trust, Mary Marlborough Centre. Manual wheelchairs: a practical guide, and Powered wheelchairs and scooters, and Wheelchair accessories. The Cromwell Press, Trowbridge, Wiltshire, 1998. 12. Audit Commission. Fully equipped. London: Audit Commission Publications, 2000. 13. Thorpe S. House adaptations: a working file for occupational therapists. Centre for Accessible Environments, 1999. 14. Department for Transport, Local Government and the Regions, New powers to help home owners, News release 538. 13 December 2001. Online. Available: www.press.dtlr.gov.uk

15. Disability, Ageing and Carers, Australia. Summary of findings. Canberra: Australian Bureau of Statistics, 1998, p. 25. 16. Beange H, McElduff A, Baker W. Medical disorders of adults with mental retardation: A population study. Am J Mental Retard 1995; 99: 595-604. 17. The National Strategy for Carers, Chapter 4, p. 41. London: Department of Health, 2001. Online. Available: www.carers.gov.uk 18. Lennox N, Green M, Diggens J, Ugoni A. Audit and comprehensive health assessment programme in the primary healthcare of adults with intellectual disability: a pilot study. / Intell Disabil Res 2001; 45: 226-32. 19. The National Strategy for Carers. London: Department of Health, 2001. Online. Available: www.carers.gov.uk 20. Lennox N, Diggens J, Ugoni A. The general practice care of people with intellectual disability: barriers and solutions. / Intell Disabil Res 1997; 41: 380-90. 21. Primary care for people with a mental handicap. London: Royal College of General Practitioners, Occasional Paper 47, 1990. 22. Webb O, Rogers L. Health screening for people with intellectual disability: the New Zealand experience. / Intell Disabil Res 1999; 43: 497-503. 23. WHO Guide to Mental Health in Primary Care - Learning Disability. Online. Available: www.psychiatry.ox.ac/cebmh/whoguidemhpcuk.html 24. Beange H, Lennox N, Parmenter T. Health targets for people with intellectual disability. / Intell Dev Disabil 1999; 24: 283-97. 25. Bohmer CJ, Klinkenberg-Knol EC, Niezen-de Boer RC, Meuwissen SG. The prevalence of gastro-oesophageal reflux disease based on non-specific symptoms in institutionalized, intellectually disabled individuals. Eur J Gastroenterol Hepatol 1997; 9: 187-90. 26. Centre J, McElduff A, Beange H. Osteoporosis in groups with intellectual disability. Aust N ZJ Dev Disabil 1994; 19: 251-8. 27. NH&MRC. The Australian immunisation handbook, 7th edition. Canberra: National Health and Medical Research Council, Australian Government Publishing Service, 2000. 28. Dinani S, Carpenter S. Down's syndrome and thyroid disorder. / Mental Defic Res 1990; 34: 187-93.

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CHAPTER CONTENTS Anaemias 415 Hypochromic microcytic anaemia 415 Normochromic normocytic anaemia 416 Macrocytic anaemia 416

24 Haematology

Haematological problems in ethnic minorities 417 Haemoglobinopathies 417 Sickle-cell disease 417 Venous thromboembolism 418 References

420

ANAEMIAS HYPOCHROMIC MICROCYTIC ANAEMIA Diagnosis of iron deficiency (a) Hb 12.5 g/dl (male); <11.5g/dl (female); (b) MCV <76fl, MCHC <32g/dl; (c) serum ferritin <10|jLg/L but for premenopausal women <5 |xg/L; or (d) serum iron <14|xmol/L (male), <11 (xmol/L (female) and TIBC >75 (xmol/L; (for iron assays check local laboratory's normal range).

Work-up of established iron deficiency anaemia (a) History and examination including evidence of Gl blood loss, menstrual blood loss, malabsorption, dietary deficiency and drug history (especially NSAIDS). (b) Three faecal occult bloods, although false negatives are common. It is only 50-75% sensitive in the detection of colorectal carcinoma.

Management * Refer to a gastroenterologist, gynaecologist or haematologist if the work-up points in a specific direction. * Refer to a gastroenterologist patients in whom there is no obvious cause for the iron deficiency. In one study, 84% of patients with no obvious cause from the history had a GI cause (28% upper, 24% lower and 29% upper and lower).1

Iron supplementation * Give a ferrous compound, e.g. ferrous sulfate 200 mg b.d. or t.d.s., after food, to reduce the GI 415

416 HAEMATOLOGY

side-effects. Avoid modified-release preparations; they may release their iron beyond the duodenum and upper jejunum, where absorption is best. * Warn patients that their stools will turn black. * Check the haemoglobin after 3 weeks. It should rise by 0.1 g/dl per day starting 10 days after the start of treatment. * Continue treatment for 3 months after the haemoglobin has returned to normal.

Transfusion

Failure to respond

NORMOCHROMIC NORMOCYTIC ANAEMIA

* Check that the patient is taking the tablets. * Review the diagnosis. Was the ferritin really low? If it was, are there other deficiencies, e.g. folic acid, as well? * Look for underlying chronic disease, which is inhibiting a response to the iron. * Look for continuing blood loss or malabsorption. * Refer if no reason is found.

Intramuscular iron * Give iron sorbitol im to: (a) those unable to tolerate oral iron because of side-effects; (b) those unable to absorb oral iron. * Stop oral iron at least 24 hours before. Calculate the dose according to the patient's weight, and the number of injections according to the degree of anaemia. * Give l.Smg per kilo in each injection, up to a maximum of 100 mg. * Give a total of 12 injections if the haemoglobin at the start of the course is 10 g/dl. * Give 24 injections if the starting haemoglobin is 5 g/dl. For haemoglobin values in between, give a proportionate number of injections as detailed in the manufacturer's chart. * Give the injections daily, or less frequently if convenience or the frailty of the patient dictates. * Injection technique. Pull the skin sideways before inserting the needle. Give the injection deep into muscle. Withdraw the needle and release the skin immediately. The Z-shaped track will reduce the risk of staining of the skin.

* Admit for transfusion only if the patient is decompensating, or if blood loss is continuing at a rate that is too fast for supplementation to keep up. There is no absolute level at which patients require transfusion. Every patient will have a different requirement according to the time over which the anaemia has developed and the ability of the heart and lungs to cope.

Diagnosis (a) Normochromic, normocytic or mildly hypochromic (MCV rarely <75fl); (b) Mild and non-progressive anaemia rarely <9.0g/dl_; (c) Reduced serum iron and TIBC; (d) Serum ferritin normal or raised.

Causes (a) Chronic infection; (b) Chronic inflammation, e.g. rheumatoid arthritis or polymyalgia rheumatica (PMR); (c) Malignancy; (d) Hypothyroidism; (e) Chronic renal failure; (f) Mild iron deficiency. Work-up (a) FBC and ESR; (b) Serum ferritin; (c) U & Es; (d) TFTs; (e) CXR.

MACROCYTIC ANAEMIA (MCV>100fl) Review: Hopffbrand V, Prowan D. Macrocytic anaemias. BMJ 1997; 314: 430-3.

* Check: (a) Diet. Only a diet free of all animal produce, i.e. a vegan diet, can lead to vitamin B12 deficiency, but folic acid deficiency can occur in those who eat few green vegetables and cereals

HAEMATOLOGICAL PROBLEMS IN ETHNIC MINORITIES

and little liver, especially if there is increased cell turnover or during pregnancy. (b) Alcohol intake. (c) Evidence of hypothyoidism or liver disease. (d) Bowel symptoms. Malabsorption may cause folic acid deficiency and, rarely, B12 deficiency. (e) Previous surgery. Gastrectomy or ileal resection will lead to B12 deficiency. (f) A personal or family history of conditions related to pernicious anaemia: Hashimoto's thyroiditis, Addison's disease or diabetes. Note: Macrocytosis without anaemia is most likely to be due to excess alcohol intake. Work-up (a) Serum vitamin B12 and folate; (b) LFTs, including GGT; (c) TFTs; (d) Reticulocyte count; (e) Serum ferritin (to exclude coexistent iron deficiency).

Management

417

* People anaemic because of dietary deficiency should be encouraged to eat bread fortified with B12, or to take oral B12 supplements. Folic acid deficiency * Refer to a gastroenterologist unless a dietary cause is obvious or there is increased cell turnover, e.g. haemolysis or pregnancy. * Give 5 mg daily for 4 months and continue for life unless the cause has been corrected. One tablet (5 mg) per week is enough in the prophylaxis of haemolytic anaemia. * Check that a serum B12 has been organized. It is customary to caution against giving folic acid to a patient with macrocytic anaemia until B12 deficiency has been excluded lest the folic acid precipitate the neurological effects of B12 deficiency. A full diagnosis of the cause of the macrocytosis is clearly essential, but the fear of precipitating neurological damage is probably unfounded.3 * Give iron supplements in all patients with a haemoglobin of <8g/dl or in those where the serum ferritin is low.

Vitamin B12 deficiency * Refer urgently all patients with dyspepsia and pernicious anaemia to a gastroenterologist or directly for endoscopy.2 * Intrinsic factor. Check autoantibodies. * Refer young patients to a haematologist, whether or not intrinsic factor antibodies are positive. * Refer older patients if the intrinsic factor antibodies are negative and they are fit enough for further investigations. * Give B12 without referral to older patients with positive intrinsic factor or in whom a Schilling test would be inappropriate. * Treat with at least six injections of hydroxycobalamin 1 mg over 2 weeks. There is no value in more frequent injections. Continue life-long prophylaxis with hydroxycobalamin 1 mg im every 3 months. Improvement in appetite and wellbeing occur within days, the reticulocyte count peaks at about 7 days and the haemoglobin should rise by 2-3 g a fortnight. Check the Hb fortnightly until normal.

HAEMATOLOGICAL PROBLEMS IN ETHNIC MINORITIES The following conditions may be found in people whose family originates from an area where malaria is or was once common. HAEMOGLOBINOPATHIES * The basic screen is: FBC, haemoglobin electrophoresis and sickle test. (See table on next page.) SICKLE-CELL DISEASE • It is estimated that there are about 12,000 people with sickle-cell disease in the UK.4 * Screen potential carriers antenatally, see p. 254.

418 HAEMATOLOGY

Condition

Origins of those at risk

Thalassaemia disease

Mediterranean, Middle East, Indian subcontinent, South East Asia

Sickle-cell trait

Transfusion and desferrioxamine

Self-help groups UK Thalassaemia Society, 1 9 The Broadway, Southgate Circus, London, N14 6PH, tel. 02088820011; www.ukts.org

Genetic counselling and prenatal diagnosis

Thalassaemia trait Sickle-cell disease

Management

Central and West Africa, Caribbean and, occasionally, Mediterranean Middle East and Indian subcontinent

Penicillin and folic acid Pneumococcal and HIB vaccine Avoid factors which precipitate crises Strong analgesia for crises

Sickle-cell Society, Green Lodge, Barretts Green Road, London NW10 7AR tel. 020 8961 7795; www.sicklecellsociety.org

Genetic counselling and prenatal diagnosis

* Refer to hospital urgently, any patient with sickle-cell disease who presents with: (a) severe pain; (b) systemic illness (fever, generally unwell, dehydration, unable to drink because of vomiting); (c) abdominal pain, especially if associated with rapid enlargement of the spleen; (d) worsening anaemia; (e) acute chest symptoms (breathlessness, cough, chest pain); (f) neurological or ocular event; (g) sustained priapism. * manage less severe attacks at home with: (a) parenteral morphine ± an antiemetic; and (b) anNSAID;and (c) oral hydration. * Be on the look-out for chronic complications: pulmonary fibrosis, renal impairment, proliferative retinopathy, avascular necrosis, especially of the hip, and leg ulcers. * Advise all parents of children with sickle-cell disease to take steps to avoid crises by making sure that they guard against cold and dehydration, and seek early treatment for infection. * Ensure that the child: (a) is taking prophylactic penicillin from the age of 3 months. This is usually given in a b.d. dosage, e.g. a 6-12-year-old would receive 250 mg b.d. The prophylaxis is maintained until leaving school or college; (b) has received the pneumococcal vaccination appropriate for his or her age, i.e. conjugated

vaccine up to the age of two and unconjugated vaccines thereafter. Repeat it 3-yearly until the child reaches the age of ten, and then 5-10 yearly; (c) receives all other childhood vaccines; (d) is immunized every autumn against influenza. * Consider giving folic acid supplements, according to the local policy.

VENOUS THROMBOEMBOLISM (VTE) • Five per cent of the UK population is heterozygous for the commonest hereditary form of thrombophilia, Factor V Leiden. It carries a fourfold increased risk of VTE and accounts for 20-40% of VTE in this country.5 • Acquired causes of VIE are common, e.g. cancer, myeloproliferative diseases, SLE. • In both hereditary and acquired forms, a trigger is usually also present, e.g. immobility, pregnancy, combined oral contraception, the post-operative state.

Patients at risk of VTE * Give all patients at high risk of VTE, lifestyle advice to reduce that risk. * Consider referring the following. They may have thrombophilia:6 (a) DVT aged under 40; (b) recurrent DVT or superficial thrombophlebitis;

HAEMATOLOGICAL PROBLEMS IN ETHNIC MINORITIES 419

(c) unusual DVT, e.g. mesenteric vein thrombosis; (d) skin necrosis in association with venous thrombosis; (e) family history of venous thrombosis <45; (f) recurrent fetal loss (>3); (g) unexplained neonatal thrombosis. * In borderline cases the GP may choose to screen the patient with a FBC, prothrombin time, APTT and a fibrinogen concentration.

weekly checks for 4 weeks, even in patients previously well controlled. * Aim for an INR of 2.5 (acceptable range 2-3). * Aim for an INR of 3.5 (acceptable range 3-4), in patients with: (a) a mechanical prosthetic heart valve; or (b) recurrent thromboembolism while on warfarin; or (c) antiphospholipid syndrome. The management of poor control10

Implications of a diagnosis of thrombophilia: (a) should a thrombosis occur, anticoagulants are needed for the same duration and intensity as in patients without the condition; (b) prophylactic anticoagulants are not indicated; (c) identification of an inherited disorder may prejudice life insurance. Population screening is therefore not justified and screening of family members may not be, unless a female relative is considering starting the combined oral contraceptive.

Management of VTE * Initial management of confirmed VTE at home with low molecular weight heparin appears to be cost effective.7 Routine monitoring is not needed. * Refer patients with suspected VTE for specialist assessment. Clinical diagnosis is unreliable. * Continue the specialist's treatment if VTE is confirmed; either low molecular weight heparin or warfarin. * Prescribe a calf-length elastic compression stocking. If the patient is prepared to wear it for 2 years it will reduce the risk of post-thrombotic syndrome, at least in patients with proximal DVTs.8

(a) INR 3.0 to 6.0 with no or minor bleeding: reduce or stop warfarin for 1-2 days and recheck INR. (b) INR 6.0 to 8.0 with no or minor bleeding: stop warfarin for 1-2 days and recheck INR. Restart when INR <5.0. (c) INR >8.0 with no or minor bleeding: stop warfarin. Restart when INR <5.0. Give vitamin K 0.5-2.5 mg orally if there are other risk factors for bleeding. (d) INR >8.0 with major bleeding: give vitamin K 5 mg iv or orally and admit. (e) INR less than 4.5 with bleeding: look for a local cause. Note: Reversal of warfarin with vitamin K can cause prolonged warfarin resistance. In patients at high risk of thromboembolism (e.g. with mechanical heart valves) assess the need for vitamin K on an individual basis. Dosage. If the dose goes above 9mg a day, suspect poor compliance, drug interaction or abnormal warfarin handling. * Check warfarin level. * Check the INR 3-5 days after starting a new drug unless it is known not to affect the INR. Duration of therapy10

Warfarin9 Timing and frequency of prothrombin times.

Patients should take their warfarin in the evening and have blood taken in the morning. Prothrombin time should be checked weekly for 3-4 weeks, then every 6-8 weeks if control is good. Warfarin needs often change with the change of lifestyle following discharge from hospital. Revert to

(a) Uncomplicated DVT with a precipitating factor (now resolved): 6 weeks. (b) VTE where there is no precipitating factor: -proximal DVT or PE: 6 months; -calf vein thrombosis only: 3 months. (c) second DVT: 12 months; (d) further DVTs: possibly indefinitely. There is no need to tail-off warfarin.

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REFERENCES 1. Hardwick RH, Armstrong CP. Synchronous upper and lower gastrointestinal endoscopy is an effective method of investigating iron deficiency anaemia. London: BrJSurg 1997; 84: 1725-8. . 2. Department of Health. Referral guidelines for suspected cancer. London: DoH, 2000. Online. Available: www.doh.gov.uk/cancer/referral.htm 3. Dickinson CJ. Does folic acid harm people with vitamin B12 deficiency? QJM 1995; 88 (5): 357-64. 4. Yardumian A, Crawley C. Sickle cell disease. Clin Med 2001; 1: 441-6. 5. Greaves M. Thrombophilia. Clin Med 2001; 1: 432-5. 6. DTB. Management of patients with thrombophilia. Drug Ther Bull 1995; 33: 6-8.

7. Schraibman IG, Milne AA, Royle EM. Home versus in-patient treatment for deep vein thrombosis (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2001. 8. Brandjes DP, Buller HR, Heijboer H et al. Randomised trial of effect of compression stockings in patients with symptomatic proximal-vein thrombosis. Lancet 1997; 349: 759-62. 9. DTB. How to anticoagulate. Drug Ther Bull 1992; 30: 77-80. 10. British Committee for Standards in Haematology. Guidelines on oral anticoagulation: third edition. Br J Haematol 1998; 101: 374-87.

CHAPTER CONTENTS Management of pain 421 Acute pain 421 Chronic malignant pain 422 Chronic non-malignant pain 422 Nociceptive pain 423 Neuropathic pain 423 Post-herpetic neuralgia 424 Trigeminal neuralgia 424 Reflex sympathetic dystrophy 424 Central pain 424

25

Pain, palliative care and bereavement

Palliative care and bereavement 425 Symptom control in the dying patient 426 Special types of pain 430 Syringe drivers 431 Nausea and vomiting 431 Other symptoms 432 Bereavement 436 Cot death 437 Bereaved children 438 Suicide 438 Death rituals of different cultures 438 Aspects of death 439 Donation of eyes and bodies 439 References 440

MANAGEMENT OF PAIN ACUTE PAIN Adults Use the WHO 'analgesic ladder': • Step 1: paracetamol, or an NSAID, or both. • Step 2: codeine or dihydrocodeine or dextropropoxyphene. Use doses that are significantly more effective than paracetamol alone, e.g. 30-60 mg codeine or dihydrocodeine. Do not use dextropropoxyphene p.r.n. In single doses it is no better than paracetamol.1 Its long half-life means that 3 days are needed for it to reach therapeutic levels. • Step 3: a strong opioid. Once the decision to use an opioid has been made, morphine or diamorphine in adequate doses are usually most appropriate. The place of tramadol is not yet clear. It may find a place between the weak and the strong opioids and may have fewer side-effects than morphine and with less tendency to tolerance or dependence. It may have a place on the ladder between codeine and morphine.2 Strong opioids are controversial in non-malignant pain and are not normally given for more than 4 days. • An adjuvant analgesic (a tricyclic antidepressant or an anticonvulsant) may be added to any of the three steps in pain of any type, although they are most helpful in neuropathic pain (see p. 423). NSAIDs NSAIDs are useful analgesics in pain of any cause. At doses of ibuprofen 600-800 mg t.d.s. 421

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PAIN, PALLIATIVE CARE AND BEREAVEMENT

they are more powerful than paracetamol.3 Given parenterally or rectally, they are first-line treatment for renal and biliary colic and are very helpful in acute severe musculoskeletal pain, e.g. diclofenac 75 mg im or 100 mg rectally or ketorolac 10-30 mg im. Diclofenac injection may be repeated after half an hour, ketorolac after 2 hours. Even the suppositories have been shown to be superior to im pethidine in renal colic.4 Suppositories have the advantage over injections of not causing sterile abscesses.

Infants and young children Review: Treating moderate and severe pain in infants. Drug Ther Bull March 1994; 32: 21-4.

• Infants, even neonates, feel pain and need analgesia in the same way as older children.5 • Explain to the child the cause of the pain and likely duration. Pain and fear are closely related, especially in children. It is important to be honest and not pretend that all pain will be removed. • Paracetamol is only licensed for children aged 2 months for vaccinations and from 3 months on the recommendation of a doctor. The rectal route is effective and safe. • Ibuprofen is effective as an antipyretic and analgesic in children6 although only licensed for children aged 1 and over. Paracetamol can be coprescribed. Doses • Paracetamol (maximum dose, neonates 60mg/kg per 24 hours, others 90mg/kg per 24 hours): oral: 15mg/kg 4-6-hourly; rectal: 20mg/kg 6-hourly. • Ibuprofen oral: 20mg/kg/day in 3—4 divided doses. • Dihydrocodeine oral solution (10mg/5ml) age <4, 500 |xg/kg 4-6 hourly; age 4-12, 500-1000 fig/kg 4-6 hourly. • Morphine: oral: age <1, 150|xg/kg 4-hourly; and age 1-12, 200-400 |xg/kg 4-hourly; subcutaneous: age <1 month, 150(xg/kg 4-hourly; age 1-12 months, 200pig/kg 4-hourly; age 1-5 years, 2.5-5mg 4-hourly; age 6-12 years, 5-1 Omg 4-hourly.

CHRONIC MALIGNANT PAIN See p. 426.

CHRONIC NON-MALIGNANT PAIN Three concepts are crucial to successful management: • Relating the drug treatment to the type of pain. The treatment of neuropathic pain is different from the treatment of tissue-damage (nociceptive) pain. Even within nociceptive pain, some types respond better to NSAIDs than to analgesics, e.g. dysmenorrhoea, dental pain and arthritic pain. This is independent of their anti-inflammatory effect.7 • Addressing the psychological component. There is always a psychological component in chronic pain, be it the understandable anger, fear and misery likely to be found in anyone with chronic pain, or the significant psychiatric illness, especially depression, present in up to 50% of patients. This will intensify the pain that is felt. In addition, pain can lead to changes in behaviour, which in turn alter the family dynamics. All these components of the 'chronic pain syndrome' must be addressed. Psychological treatment, especially cognitive behaviour therapy, has been shown to be helpful.2 • Measurement of pain intensity. Formal measurement of perceived pain intensity can guide the physician in the tailoring of medication as well as demonstrating improvement to the patient and carer, if there has been any. The simplest way to assess and document the severity of pain is to ask the patient to score it on a scale of 1 to 10, either verbally or by drawing a 10cm line, marked from 1 to 10 and asking the patient to mark the position of the pain on the line, where 0 is no pain and 10 the worse possible pain imaginable. Patients tend to score mild pain as <3, moderate pain between 3 and 5.5 and severe pain above 5.5.8 Pain scores above 5 usually interfere with the patient's functioning.9 Professionals tend to underestimate pain, especially severe pain, while carers tend to overestimate it.10

MANAGEMENT OF PAIN

General principles of management * Explain the reason for the pain. Pain clinics sometimes find that patients are more concerned about this than about pain relief. * Explain that treatment is unlikely to abolish pain completely, but that the aim is to stop it from affecting the quality of the patient's life. * Explore the psychological component with the patient and the family. If the patient seems to think you are implying that it's all 'in the mind', make it clear that you know the pain is real.

NOCICEPTIVE PAIN (PAIN DUE TO TISSUE DAMAGE) * Use the analgesic ladder as above, but not usually beyond dihydrocodeine 120 mg per day. Most pain specialists will not use strong opioids for this condition because of tolerance, addiction and side-effects. Others feel there is a place for opioids in patients with intractable pain which is interfering with life, where the diagnosis is proven, the pain is opioid-sensitive, and where there are no psychological contraindications. For the practicalities of opioid use, see p. 427. * NSAID. Consider adding an NSAID to the chosen analgesic (see p. 421). * Adjuvant analgesics. Consider adding a tricyclic antidepressant. Adjuvant analgesics are sometimes helpful (see below), although not as helpful as in neuropathic pain. * Pain clinic. Consider referral to a pain clinic for further physical, as well as psychological, interventions. Transcutaneous electrical neural stimulation (TENS) is widely used in pain clinics, although evidence for benefit is not strong.2 Nerve blocks may be extremely helpful in certain situations, e.g. epidural injection for low back pain, caudal injection for coccidynia, intercostal block for rib pain and brachial plexus block for arm pain. Nerve destruction is little used because of the problem of deafferentation pain that it can cause.

NEUROPATHIC PAIN (PAIN DUE TO NERVE DAMAGE) Examples of neuropathic pain are nerve injury, compression or infiltration of nerve by tumour,

423

shingles, and diabetic neuropathy. It is characterized by one or more of the following: (a) a burning character; (b) an element of 'shooting' pain; (c) autonomic instability; vasoconstriction or vasodilation; (d) allodynia (severe pain from mild stimuli) this is diagnostic; (e) sensory loss. Neuropathic pain responds only partially to analgesics, including opioids.11 While some studies show no benefit, two trials of tramadol in painful neuropathy achieved an NNT of 3.4,11 i.e. one patient achieved 50% pain relief for every 3.4 patients treated.

Principles of management * Simple analgesic. Give paracetamol and/or an NSAID. * Adjuvant analgesic. Add a tricyclic antidepressant and/or an anticonvulsant. The evidence of benefit from other antidepressants is poor. * Opioid. If still in pain, institute a trial of a weak opioid. * Pain clinic. Refer to a pain clinic. Tricylic antidepressants, e.g. amitriptyline * Start at 25 mg o.n. (10 mg in the elderly) as soon as the diagnosis of neuropathic pain is made. * Warn the patient that it will be 1-7 days before there is benefit. The NNT is 3; i.e. one in three patients will have at least 50% relief which they would not have had with placebo.11 * Warn the patient that side-effects may occur, especially dry mouth and drowsiness. * Increase the dose fortnightly until a good response is achieved. This is likely to be at 75 mg or below, although occasionally it is worth continuing up to 150 mg daily. The effective dose is likely to be lower than that required for depression. * Continue treatment for 2 months after the pain has stopped, then tail it off over a month. Do not change to another tricyclic if amitriptyline fails; most are likely to be less successful.11

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PAIN, PALLIATIVE CARE AND BEREAVEMENT

Anticonvulsants * Use carbamazepine, valproate or gabapentin. Carbamazepine and gabapentin have overall NNTs of approximately 3. The evidence for phenytoin is less clear.11 * Start with low doses, and increase until relief of symptoms is achieved or until blood levels are in the therapeutic range for epilepsy (see Appendix 21). * Continue this dose for 6 weeks before abandoning as ineffective. If one drug fails it is not helpful to try another.12 Only carbamazepine and phenytoin are licensed for treating the pain of trigeminal neuralgia. Only gabapentin is licensed for all neuropathic pain.

POST-HERPETIC NEURALGIA (PHN) See also p. 374. * Give conventional analgesics, e.g. paracetamol. Most will not respond, in which case stop the drug. Do not proceed up the analgesic ladder beyond a trial of a weak opioid. * Give tricyclic antidepressants (see above) to all patients with severe pain and to others who are at high risk of PHN. Starting amitriptyline early appears to reduce the severity of PHN should it develop later.13 * If tricyclic antidepressants do not give adequate relief, consider: (a) an anti-convulsant (see above); (b) a local anaesthetic cream (e.g. Emla®) to be applied thickly at night and covered with clingfilm. It may be removed in the morning, and benefit will last for some of the day;14 (c) capsaicin cream q.d.s. for up to 8 weeks. In one trial 39% reported at least moderate relief after 6 weeks against 6% on placebo.11 An unpleasant burning sensation is likely for the first 1-2 weeks, and can be reduced by applying Emla® cream before the capsaicin. * Refer patients still in pain after 8 weeks. Stellate ganglion block may reduce pain. In one uncontrolled study it was associated with freedom from pain in 40% of those referred before

1 year although in only 22% of those referred after 1 year.15

TRIGEMINAL NEURALGIA * Use carbamazepine first-line, then, if necessary, phenytoin (see above). 70% will achieve significant relief from the former.16 * Refer, if no benefit, for consideration for: (a) local anaesthetic injection into trigger points; (b) cryoprobe nerve block to the trigeminal nerve to preserve sensation; (c) neurosurgical injection or section of the trigeminal ganglion, which will lose sensation and may cause deafferentation pain worse than the original pain.

REFLEX SYMPATHETIC DYSTROPHY * This is a pain syndrome, usually affecting an extremity, following trauma (even minimal trauma). It is characterized by swelling, discoloration, temperature changes, abnormal sensitivity, sweating and loss of function. X-rays may show osteopenia. Early recognition, pain control and mobilization are thought to be associated with better prognosis.17 * Refer to a pain clinic as soon as the diagnosis is made. A trial of mild analgesics is worthwhile but usually unhelpful. A pain clinic is likely to try intravenous regional sympathetic blockade but evidence of benefit is poor.18

CENTRAL PAIN This may be of brain or spinal cord origin. Stroke is the commonest cause. The onset of pain may be delayed for up to 3 years after the event. It is particularly difficult to treat, and early referral to a pain clinic would be wise.

* Use: (a) amitriptyline (see above); (b) anticonvulsants (see above); (c) a neuroleptic, e.g. perphenazine. In a patient already on low-dose amitriptyline, the compound preparation Triptafen is convenient.

PALLIATIVE CARE AND BEREAVEMENT

PALLIATIVE CARE AND BEREAVEMENT Review: O'Neill B, Fallon M. ABC of Palliative Care. 6/WJ1997;315:801-4. Guidelines: Scottish Intercollegiate Guidelines Network and the Scottish Cancer Therapy Network. Control of pain in patients with cancer. SIGN, June 2000. Online. Available: www.sign.ac.uk National Council for Hospice and Specialist Palliative Care Services. Guidelines for managing cancer pain in adults, 2nd edition, 1998. Online. Available: www.hospice-spc-council.org.uk

The World Health Organization defines palliative care19 as the 'active total care of patients whose disease is not responsive to curative treatment'. Control of pain, of other symptoms and of psychological, social and spiritual problems is paramount. The goal of palliative care is the achievement of the best quality of life for patients and their families. Palliative care should not be reserved for the terminal stages; it may be appropriate as soon as an incurable disease is diagnosed. Palliative care is necessarily multidisciplinary and requires effective communication with patients, their families and others involved in their care.

Talking to the patient * Breaking bad news. Patients will dictate the pace at which they want to receive details of the diagnosis. A step wise approach beginning with a 'warning shot' may be helpful. They may not wish to hear the whole diagnosis straight away. At any stage they may be more concerned with practical aspects of their care. * Give patients every opportunity to ask about their illness. Useful questions to ask are: 'is there anything you want to ask me about your illness?' and 'have you any particular worries about your illness?' * Denial may be a valid coping mechanism for those who are unable or not yet ready to adapt to the reality of a terminal illness. Denial is rarely complete or fixed. Empathetic listening and

425

responding to a patient's concerns may allow the patient to feel safe enough to let the painful reality into their conscious thinking. * 'Please don't tell him he has cancer'. Collusion is a means of protecting another from pain. It is most often seen between patients and relatives but may also occur between professionals. Honest discussion allows patients to be reassured and to readjust hopes and aims for the future. * Anger, guilt and blame. Acknowledging the emotion and exploring the issue with a patient and his or her family will help them to move on. * Fears. Explore patients' fears about their illness. It will be possible to reassure them about the degree to which many of their symptoms can be relieved. * False reassurance can undermine the relationship in the future. Do not guarantee to control their pain all the time. Five per cent of patients have uncontrollable pain. Advice: CancerBACUP (British Association for Cancer United Patients), 3 Bath Place, Rivington Street, London EC2A 3RJ, tel. 0808 800 1234; www.cancerbacup.org.uk National Cancer Information Service, tel. 0800 181199. Cancer advice: www.canceradvice.co.uk The National Electronic Library; cancer. www.nelc.org.uk Cancer Research Campaign, www.crc.org.uk

The home * Try to keep the patient from adopting an invalid role before it is essential. Encourage the family to eat, watch television, etc. with the patient. * Some rearrangement of the home may be necessary, especially sleeping near the toilet or providing a commode. District nursing services or Social Services may be able to help.

Financial aspects * Free prescriptions are available for patients once they are confined to the house (Form FP92A for patients under retirement age).

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* Disability Living Allowance and Attendance Allowance under the Special Rules is available for patients unlikely to live more than 6 months (see p. 5). * Macmillan Cancer Relief Grants are available for people with cancer who have a low income and little capital. Application must be by a professional on a form available from: 15-19 Britten Street, London SW3 3TZ or 9 Castle Terrace, Edinburgh, EH1 2DP.

Support * Psychological support has been shown to improve emotional and physical symptoms although not to affect the medical outcome.20 This may be available as part of the hospital oncology service. Alternatively, the national cancer self-help groups have details of local branches. * The Palliative Care/Community Hospice team should be involved early in the illness if this facility exists locally. An early meeting of all the professionals involved in the patient's care is necessary in order to establish one person as the key worker, with responsibility for ensuring that all the different aspects of the patient's care are being covered. * Hospice admission may be needed at some stage, for symptom control, as a respite for the carer or for the terminal stage. Encourage patients and relatives to visit the hospice early in the illness. Remember, however, that most patients in the UK wish to die at home, although only a quarter do so.21

Provision of palliative care drugs out of hours * In the terminal stages, deterioration in the clinical condition may be rapid. The GP needs to think proactively about medication. Sufficient supplies of drugs to cover the out of hours period (possibly allowing for an increased dose) should be available. Drugs not previously used (e.g. hyoscine and midazolam) may be required, and it may be appropriate to leave these in the patient's home for administration by a visiting doctor.

• Despite difficulties with controlled drugs, there is a place for assembling palliative care bags which might include drugs, palliative care equipment (e.g. syringe driver, catheter, needles and gloves) and useful information on crisis management, palliative support telephone numbers etc. The palliative care bag22 Drugs to consider include: Non-controlled drugs

Controlled drugs

Midazolam Haloperidol Cyclizine Hyoscine hydrobromide/ butylbromide Methotrimeprazine Rectal diazepam Water for injection

Diamorphine

Local conditions will determine where these bags are kept.

SYMPTOM CONTROL IN THE DYING PATIENT • Patients with cancer experience psychological as well as physical symptoms. Most patients will have more than one symptom. • Look for as many physical and psychological symptoms as possible (Table 25.1).

Pain • Most patients with cancer pain have more than one pain. The pains may be from different sources, and the pains from any one source may be of several different types, e.g. neuropathic or due to Table 25.1 Common symptoms in cancer patients23 Physical

%

Psychological

%

Lack of energy Pain Drowsiness Dry mouth Nausea Anorexia

73 63 60 55 45 45

Worrying Feeling sad Feeling nervous Difficulty sleeping Feeling irritable Difficulty concentrating

72 67 62 53 47 40

PALLIATIVE CARE AND BEREAVEMENT

tissue damage (nociceptive). The treatment of each of these is different. * Depression, anxiety, fear and anger are important factors that contribute to the perception of pain. The failure to deal with the patient's emotional state can lead to inappropriately high but ineffective doses of morphine. * With effective assessment and a systematic approach to the choice of analgesics, over 80% of cancer pain can be controlled with the use of inexpensive drugs administered by mouth. Consideration must always be given to treating the underlying cause of the pain by means of surgery, radiotherapy, chemotherapy or other appropriate measures. * Fully assess the cause and type of each pain experienced by the patient. * Assess the severity of the pain, or rather, accept the patient's assessment of it. Professionals tend to underestimate pain, especially severe pain.24 Record the severity formally, e.g. by asking the patient to score it on a scale of 1 to 10 and record the score, the medication that was being given and any changes made. * Assess the impact of the pain on the patient's daily life. * Assess the way that the patient's emotional and spiritual state is contributing to their perception of pain. * Explain the cause(s) of the pain and your treatment plan to the patient and family. Leave a written pain management plan with the patient, even if it just gives the patient instructions about how to use the medication for breakthrough pain.25 * Enlist the help of anyone available to combat loneliness, boredom and despair.

Principles of analgesic use Use an effective drug in adequate doses and at regular intervals. Route of administration

(a) give drugs orally by choice; if this is not possible then (b) give drugs rectally or transdermally; or

427

(c) give drugs intramuscularly in the very shortterm, but a subcutaneous syringe driver is preferable for anything longer than a few days. Choice of drug

* Use the WHO analgesic ladder: Step 1: non opioid analgesia, e.g. paracetamol ± a NSAID; Step 2: Opioid for mild to moderate pain, e.g. codeine 30-60 mg or dihydrocodeine 30-60 mg ± paracetamol and an NSAID; Step 3: Opioid for moderate to severe pain, e.g. morphine ± paracetamol and an NSAID. * If a patient's pain is not controlled on steps 1 or 2 move up the ladder. * If the patient needs a NSAID but is at high risk of gastrointestinal ulceration, coprescribe misoprostol or omeprazole. * Do not hesitate to start a strong opioid immediately for severe pain. * Adjuvant analgesics may be added to the ladder at any step (see p. 423).

Strong opioids Guidelines: Expert Working Group of the European Association for Palliative Care. Morphine in cancer pain: modes of administration. BMJ1996; 312: 823-6. Working Group of the Ethical Issues in Medicine Committee of the Royal College of Physicians. Principles of pain control in palliative care for adults. London: Royal College of Physicians, 2000. Online. Available: www.rcplondon.ac.uk

Tolerance and addiction

• Both patients and professionals may have fears about the use of strong opioids. These should be discussed. • Tolerance to opioids is rarely seen in managing cancer pain. A need for increasing doses is usually due to progression of the disease. • Psychological dependence or addiction is not a problem except in patients with pre-existing addiction. • Titmtion of dose. Titrate the dose required using a formulation with rapid onset and short duration.

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The dose may need to be changed daily until control is achieved (see oral morphine below). * Maintenance. Once stabilized, change to a controlled-release drug. * Reduction of dose. If a patient on large doses of morphine is given DXR or a nerve block, watch for evidence that the pain is subsiding. If it does, reduce the dose by one-third in the first instance, once it is clear that the intervention has succeeded. Deprived of pain the patient will become more sensitive to morphine, and may suffer respiratory depression. Side effects of opioids * Warn patients of the side-effects of opioids and treat as necessary: (a) sedation: this usually resolves within a few days; (b) nausea and vomiting: an antiemetic, e.g. metoclopramide or haloperidol, should be prescribed routinely, to be taken as needed, when opioids are started or when their dose is increased. It can usually be stopped 5-10 days later; (c) constipation: prescribe a laxative prophylactically. Combine a stimulant and/or a softening agent, and/or an osmotic laxative; (d) dry mouth: this is often the most troublesome symptom. Recommend simple measures, e.g. cool drinks, sucking sweets, etc.; (e) cerebral toxicity (see below). Oral morphine • Morphine is the most commonly used strong opioid analgesic. • Immediate-release preparations are better when more intensive titration of the dose is needed. They can be given as tablets or solution. • Start at 5-1 Omg 4 hourly, or higher if the pain was uncontrolled on high doses of weak opioids. Note that: (a) the dose needed may be 5-150 mg or more 4-hourly; (b) a double dose at night may avoid waking at 2 a.m.;

(c) a responsible person is needed to ensure the 4-hourly dosage is maintained; (d) if pain returns before the next dose, increase the strength and not the frequency of doses. Make increases of 30 to 50% of the previous dose each time; (e) breakthrough pain: authorize, for every patient on morphine, extra doses for breakthrough pain, with each extra dose equal to the patient's 4-hourly dose. The patient should wait 30 minutes and take a further dose if the response is insufficient. If breakthrough pain is frequent, increase the 24-hour dosage; (f) painful procedures: if cover is needed for a painful procedure, use a drug that is rapid in onset and short acting, e.g. dextromoramide. This works in 10 minutes if given sublingually or rectally. Five milligrams is equivalent to 15 mg of oral morphine (see also incident pain, below). Alternatively, if there is sufficient time, give an extra dose of morphine 30 to 60 minutes before, as for breakthrough pain; (g) other opioid analgesics: pentazocine and buprenorphine can act as antagonists to morphine and are probably best avoided in a patient on morphine. Pethidine leads to the accumulation of the toxic metabolite norpethidine and it should not be used. Modified-release (MR) morphine is best for the patient with stable requirements; it gives steady blood levels and good compliance. Tablets should not be crushed. Where there is a difficulty in swallowing, liquid controlled-release formulations can be used. (a) The dose needed may be from 20-400 mg daily. (b) Preparations designed for 12-hourly and 24-hourly use are available. (c) Continue to use normal release morphine for breakthrough pain. Morphine in the elderly * Start at 2.5 to 5 mg 4-hourly. * Morphine may be metabolized more slowly than in the younger patient. Dosage intervals may be lengthened to 6 hours, but changes of dose

PALLIATIVE CARE AND BEREAVEMENT

should only be made after 3 days, since the drug may accumulate over that time before reaching a steady state. Other routes In the patient unable to take oral drugs, use: (a) suppositories: either morphine 15 mg 4-hourly or oxycodone 8-hourly (see below for equivalence); (b) diamorphine injection: which is over 10 times more soluble than morphine. Give subcutaneously injections 4-hourly in the very short term; im injections are more painful. In the longer term, use a syringe driver (see p. 431); (c) tmnsdermal drugs: fentanyl patches should only be used for patients whose pain is stable because of the time taken to titrate the dose (peak plasma levels are not achieved for 24-48 hours).

429

(e) confusion; (f) myoclonic jerks. Note: Agitated confusion may be interpreted as being due to pain and higher doses of opioids given. * Consider: (a) reduction of the dose of opioids; (b) change to an alternative opioid; (c) whether the patient is well hydrated; (d) using haloperidol for agitation. It may be possible to increase the dose of opioid once symptoms have resolved.

Adjuvant drugs • Adjuvants are most often used for neuropathic pain but they may help in pain of any type. Tricyclic antidepressants, e.g. amitriptyline or dothiepin

Opioid equivalents Warning: use Tables 25.2 and 25.3 as a rough guide only. Equivalents vary from one individual to another as absorption varies. Opioid toxicity Opioid toxicity may present with: (a) agitation; (b) seeing shadows in the periphery of the visual field; (c) vivid dreams; (d) visual and auditory hallucinations;

1. Start at 25 mg o.n. (10 mg in the elderly). 2. Warn the patient that it will be several days before any benefit is felt; 50-90% of patients achieve at least 50% reduction in the severity of the pain.26 3. Warn the patient that side-effects may occur, especially dry mouth and drowsiness. 4. Increase the dose fortnightly until a good response is achieved. This is likely to be at 75 mg or below, although occasionally it is worth continuing up to 150 mg daily. The effective dose is likely to be lower than that required for depression.

Table 25.2 Morphine equivalents Oral morphine

Rectal morphine

Oral diamorphine

Parenteral diamorphine

Oral oxycodone

Rectal oxycodone

Oral hydromorphone

15mg

15mg

10mg

5mg

10mg

30 mg

2mg

Table 25.3 Sustained release equivalents 24 hour dose of oral 4-hourly morphine

MR morphine 12-hour action

MR morphine 24-hour action

Fentanyl patch, every 72 hours

Hydromorphone S/R 12-hourly

90 mg

45 mg

90 mg

25|xg

6mg

430

PAIN, PALLIATIVE CARE AND BEREAVEMENT

5. Continue treatment for 2 months after the pain has stopped, then tail it off over a month. Anticonvulsants 1. Use sodium valproate, carbamazepine, clonazepam or gabapentin. Start with low doses, and increase until relief of symptoms is achieved or until blood levels are in the therapeutic range for epilepsy (see Appendix 21). 2. Continue this dose for 6 weeks before abandoning as ineffective. 3. If one drug fails it is not helpful to try another.12

SPECIAL TYPES OF PAIN Bone pain * Radiotherapy is the most effective treatment for localized bony secondaries. It will take 2-3 weeks to work with almost half of patients obtaining at least 50% pain relief at some stage. As the pain starts to ease, remember to reduce the dose of morphine as above. * NSAIDs should be used in high doses, e.g. naproxen 500 mg t.d.s. orally or rectally. * Opioids are often less effective than NSAIDs. Do not persevere if side-effects occur without benefit. * Nerve blocks are helpful in the medium term if a nerve is accessible, e.g. intercostal block for rib pain or epidural block for vertebral pain. * Bisphosphonates. Admit for intravenous infusion of bisphosphonate, e.g. sodium pamidronate in patients with myeloma and breast cancer. * Pathological fractures. The pain may be greatly reduced by internal fixation followed by radiotherapy.

Incident pain (pain on movement but absent at rest) * Do not increase the 24-hour opioid dose. The increase in side-effects may outweigh the benefit. Consider giving breakthrough analgesia as above before the patient is moved. * Consider adjuvant analgesics. * Physiotherapy. Refer to improve muscle tone and rehabilitation.

* Orthopaedic surgeon. Refer for surgical stabilization of the affected area. Internal stabilization of long bones may produce considerable benefit, even in advanced disease.

Neuropathic pain (pain due to nerve damage) Examples are nerve injury, and compression or infiltration of nerve by tumour. Neuropathic pain may be improved but is often not completely relieved by non-opioid or opioid analgesics. Adjuvant analgesics are often required (see p. 423). * TCAs. Start a tricyclic antidepressants as soon as the diagnosis of neuropathic pain is made. Add, or change to, an anticonvulsant if necessary (see Adjuvant drugs, above). * Use conventional analgesics in addition. A consensus is emerging that they can be of benefit in neuropathic pain.11 * Pain clinic. Refer to a pain clinic. * Consider steroids, e.g. dexamethasone 8 mg daily if inflammation is contributing to the nerve damage. * Consider using non-drug methods, e.g. TENS, acupuncture, physiotherapy and occupational therapy. Some patients find them helpful despite the lack of solid evidence to support their use, whether for pain, dyspnoea, nausea or vomiting at the end of life.27

Visceral pain Although initially managed by analgesics, invasive techniques should be considered at an early stage: (a) Epidural and intrathecal catheters can deliver opioids and local anaesthetics direct to the spinal cord to give effective analgesia in lower limb and abdominal pain with few adverse effects. (b) Pancreatic carcinoma or other upper abdominal tumours. Coeliac axis block may have a place early in the disease. (c) Pelvic tumours. These may give rise to bladder and rectal tenesmus and severe perineal pain. Response to opioids is poor, although muscle relaxants and anticholinergics may have some value. Sacral nerve root block may prove effective.

PALLIATIVE CARE AND BEREAVEMENT

SYRINGE DRIVERS • Indications include: (a) persistent nausea or vomiting; (b) difficulty swallowing; (c) intestinal obstruction; (d) comatose patient. • Timescale. No individual infusion solution should be used for more than 24 hours (to minimize the risk of infection). • Skin reactions: (a) Do not use chlorpromazine, prochlorperazine or diazepam; (b) Be cautious with cyclizine and methotrimeprazine; both can cause local irritation; (c) Add a small dose of dexamethasone (0.5 mg daily) to the solution if irritation is a problem.

Diamorphine • Diamorphine can be given by subcutaneous infusion in a strength of up to 250 mg/ml. In doses of up to 40 mg/ml, either water or physiological saline can be used as a diluent. Above 40 mg/ml, only water can be used. If the dose needs to be increased, increase it by 30-50% in a fresh syringe for a new 24-hour period. • Breakthrough pain. Give a subcutaneous bolus injection roughly one-sixth of the total daily dose of diamorphine. • Combinations. The following can be mixed with diamorphine: cyclizine, dexamethasone, haloperidol, hyoscine, methotrimeprazine and metoclopramide, but see Appendix 28 for details.

NAUSEA AND VOMITING Differentiation of the likely cause is vital so that reversible factors may be addressed and the right type of antiemetic chosen. 1. Evaluation of the patient * Consider particularly: (a) the timing of symptoms (with smell of food or after large meal); (b) the type of vomiting (regurgitation, or vomiting);

431

(c) the content of the vomit (faeculent, bile stained, undigested food, etc.); (d) the drug regimen. Several drugs may be implicated by a variety of mechanisms including: -GI irritation (e.g. NSAIDs, antibiotics); -gastric stasis (e.g. opioids, tricyclics, phenothiazines); -chemoreceptor trigger zone stimulation (e.g. opioids, digoxin, antibiotics, cytotoxics); -5-HT3 receptor stimulation (e.g. cytotoxics, SSRIs). * Check the fundi for papilloedema. * Examine the mouth, pharynx and abdomen. * Consider checking plasma biochemistry, including calcium. 2. Correct any reversible causes: (a) hypercalcaemia: rehydrate and give a bisphosphate; (b) infection: consider antibiotics; (c) raised intracranial pressure: give dexamethasone; (d) gastric irritation/ulceration: stop NSAIDs, give an H2-antagonist or proton pump inhibitor; (e) constipation: treat with laxatives; (f) anxiety: empathize and give explanation and reassurance. 3. Non-drug measures might include: (a) control of malodorous discharge from fungating tumour; (b) calm environment away from food; (c) avoidance of exposure to foods causing nausea; (d) small meals; (e) acupuncture/acupressure over the P6 point at the wrist. 4. Drug treatment. There is no universal antiemetic. Instead the choice of drug depends upon the postulated cause of the problem. In practice, the initial decision is a choice between three first-line antiemetics depending upon the clinical problem (Table 25.4). Remember that: (a) The final common pathway for the action of prokinetic drugs is cholinergic. Anticholinergic drugs (including cyclizine) block their action.

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PAIN, PALLIATIVE CARE AND BEREAVEMENT

Table 25.4

First-line antiemetics

Class of drug

Clinical problem

Drug

Dose Orally

Subcutaneous infusion

Prokinetic

Gastritis, gastric stasis, functional bowel obstruction

Metoclopramide

10mg q.d.s.

40-100mg/24 hours

Acting principally in area postrema

'Chemical' causes of vomiting, e.g. hypercalcaemia, morphine

Haloperidol

1-2mg nocte or 1-2mg b.d.

5 mg/24 hours

Acting principally on the vomiting centre

Mechanical bowel obstruction, raised intracranial pressure, motion sickness

Cyclizine

50 mg t.d.s. (or 100mg t.d.s. rectal ly)

150mg/24 hours

(b) If more than one antiemetic is required, choose a combination with complementary actions and effects (e.g. cyclizine and haloperidol). Drugs such as methotrimeprazine have an action at multiple receptors and may replace drug combinations. (c) If the patient is vomiting or nauseated for most of the time then the drug should be given by a non-oral route (continuous sc infusion or rectally). If there is still inadequate symptom control, other drugs may need to be considered: (a) Dexamethasone is most often used in combination with other antiemetics. Dose: 4-20 mg daily (orally or sc). (b) Methotrimeprazine is the most broad-spectrum antiemetic available. May be sedating, particularly in higher doses and causes postural hypotension in the elderly. Dose: 6.25-12.5 mg as a stat dose (orally or sc) then 12.5-50 mg over 24 hours. If preferred,the dose may be given once daily at bedtime. (c) 5-HT3 antagonists (ondansetron, granisetron and tropisetron). They have similar efficacy and tolerability and few side-effects. Their effects are enhanced by corticosteroids, but they are expensive. (d) Benzodiazepines may be particularly useful in anticipatory vomiting. Lorazepam is useful as it is available in a 1 mg sublingual tablet obviating the need for swallowing. (e) Other phenothiazines. Prochlorperazine may be given buccally but not subcutaneously. There is a risk of dystonic reactions particularly in adolescents and those with AIDS. Chlorpromazine

has a wide spectrum of receptor affinity but does not share the potent 5-HT2 antagonistic properties of methotrimeprazine. (f) Domperidone does not cross the blood-brain barrier so has fewer central effects than metoclopramide. Whichever drug regime is chosen, if a subcutaneous infusion is used, consider changing to an oral regime once good control has been achieved and maintained for 72 hours. Continue antiemetics indefinitely unless the cause resolves.

In malignant bowel obstruction * Surgery. Never exclude referral for palliative surgery as an option in patients with advanced cancer who develop obstruction. * Nasogastric suction should only be used in those being considered for surgery and those with high obstruction who respond poorly to drug treatment. The latter may benefit from a gastrostomy.

OTHER SYMPTOMS Anorexia and weakness * Exclude reversible causes, e.g. drug-related, depression. Ensure pain is controlled. * Good mouth care is essential. * Consider metoclopramide if the patient feels full after eating only a small amount. * Consider appetite stimulants e.g. dexamethasone 2-4 mg daily (if the prognosis is felt to be short) or a progestogen such as megestrol

PALLIATIVE CARE AND BEREAVEMENT

320^480 mg daily or medroxyprogesterone acetate 40-800 mg daily if the prognosis is longer.

Constipation Constipation can lead to abdominal pain and increased use of constipating analgesics. Overflow diarrhoea, urinary retention or 'overflow' incontinence may occur. * Prevention. Use stimulant and softening laxatives in combination (see p. 138). * Avoid bulking agents, which are especially ineffective in constipation due to opioids. * Well-timed suppositories and enemas are better than a painful manual removal. * Be prepared to give high doses of laxatives to patients on high doses of opioids, e.g. senna 10 tablets at night or bisacodyl 4 tablets at night, or co-danthrusate 3 capsules q.d.s.

Diarrhoea * Exclude impaction with faecal overflow. These patients require rectal laxatives with a stool softener. Stimulant laxatives may cause colic. * Check that the patient is not taking a high dose of laxative intermittently. * Use loperamide 2-16 mg a day, or codeine 10-60 mg every 4 hours. * Malabsorption due to pancreatic carcinoma - use pancreatic enzyme supplements with an H2receptor antagonist or proton pump inhibitor. * Radiotherapy-induced or chologenic diarrhoea - use cholestyramine 12-24 g daily.

Psychiatric reactions Depression Patients with malignant disease suffering from major depression respond as well to antidepressants as those with no physical illness. * Check that poorly treated symptoms are not contributing to the depression. Look for specific problems, e.g. low sodium, which may be causing depression. * Check that the issues covered on p. 425 have been addressed. * Treat with an antidepressant see p. 311.

433

Anxiety * Physical cause. Look for a physical cause, e.g. pain, pulmonary embolism, internal haemorrhage, drug or alcohol withdrawal. * Treat with: (a) a benzodiazepine; or (b) haloperidol, which may be of particular value if the patient is confused; or (c) a tricyclic antidepressant for chronic lowgrade anxiety. * Severe acute anxiety is more likely to be secondary to a physical cause, and may need lorazepam 1-3 mg orally or midazolam 5-10 mg sc. Agitated confusion * Consider causes that need treatment in their own right: infection, constipation, retention of urine, poorly treated physical symptoms, oversedation, opioid toxicity, hypercalcaemia or cerebral oedema. * Use: (a) haloperidol 1.5-10 mg orally t.d.s. or 5mg b.d. im; (b) methotrimeprazine 25-50 mg orally or 12.5-25 mg im, 4-hourly; (c) midazolam 20-60 mg per 24 hours via syringe driver or 2.5-5 mg sc every 6-8 hours; or (d) diazepam, orally or rectally. * If the confusion is primarily nocturnal, use a single night-time dose. * Consider withdrawing the sedative after a few days if the cause was treatable, e.g. dehydration or infection. * Long-term heavy sedation. Give haloperidol 10-30mg with midazolam 30-60 mg over 24 hours, by syringe driver.

Respiratory symptoms Cough * Infection. Treat infection if present. * Cough suppressants. Give pholcodine linctus 10 ml 4-hourly. If the patient is already on a strong opioid, increase the dose of that instead. Severe cough may justify the use of diamorphine for that indication alone. Methadone is traditional, but

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PAIN, PALLIATIVE CARE AND BEREAVEMENT

has an excessively long half-life. It can be of value at night, but there is a risk of accumulation. * Antimuscarinics. These can be used to reduce salivary secretions. Hyoscine hydrobromide 0.20.4 mg sc stat. or 1.2-2.4 mg sc over 24 hours. Breathlessness * Give a benzodiazepine as an anxiolytic (e.g. lorazepam 0.5-2 mg sublingually) to achieve rapid control, followed by oral diazepam 5-1 Omg daily; or * Give an opioid (e.g. diamorphine 2.5 mg 4-hourly). If the patient is already taking an opioid, increase the dose. Other respiratory symptoms * Hiccup. Use chlorpromazine 25-50 mg orally or im q.d.s. In severe cases, 25 mg iv is effective. * Death rattle. Give hyoscine hydrobromide 200-600 |xg 4-6-hourly sc, or hyoscine butylbromide 20-80 mg per 24 hours via syringe driver. * Haemoptysis: (a) Consider referral for radiotherapy for bronchial tumours; (b) Consider tranexamic acid to reduce bleeding; (c) Plan a course of action in case massive haemoptysis occurs. Emergency admission might be appropriate for the patient who is not otherwise terminal. Otherwise, lie the patient on the side of the tumour and give intravenous diamorphine and benzodiazepine (for anxiety). * Stridor from obstruction of the larynx or airway. Give high-dose dexamethasone (16 mg daily). * Pleural or chest wall pain. Consider the need for radiotherapy or intercostal nerve block. * Severe breathlessness and a sensation of drowning may be due to superior vena caval obstruction. (a) Give high-dose oral steroids (stop after 5 days if not effective); (b) Admit for radiotherapy if appropriate; (c) Use low dose morphine, with or without a benzodiazepine, to relieve dyspnoea.

Spinal cord compression Spinal cord compression may present as back pain, nerve root irritation and weakness of the legs. * Commence dexamethasone 16 mg/day. * Refer urgently for radiotherapy or laminectomy if the patient's condition warrants it. If treatment is commenced within 24-48 hours of the onset of symptoms, neurological damage may be reversible.

Itch * Dry skin. Use any moisturizing agent, e.g. emulsifying ointment. Apply it copiously and often. A sedative antihistamine may help, e.g. hydroxyzine. * Obstructive jaundice. Use stanozolol 5 mg daily. Cholestyramine is less helpful and harder to take.

Insomnia * Use: (a) a short- to medium-acting benzodiazepine or similar (e.g. zolpidem, zopiclone or zaleplon); (b) amitriptyline 25-50 mg at night, which may help pain as well; or (c) a neuroleptic, e.g. methotrimeprazine. It will help concurrent agitation or nausea.

Mouth and throat problems * Dysphagia. If there is also oral Candida, assume that dysphagia is due to oesophageal Candida and use fluconazole 50 mg daily for 7 days. Also consider obstructive tumour or neuromuscular causes. * Aphthous ulcers. Treat with triamcinolone in carmellose paste, or betamethasone mouthwash (0.5 mg in 5ml of water) or tetracycline mouthwash every 8 hours (250 mg capsule dissolved in 5 ml of water). * Dryness. Use crushed ice, semifrozen juices or tonic water, pineapple chunks or artificial saliva, e.g. Glandosane spray. Use petroleum jelly for cracked lips, but avoid lemon glycerine combinations that increase the dryness overall.

PALLIATIVE CARE AND BEREAVEMENT 435

* Pain/soreness. Use: (a) local anaesthetic, e.g. benzydamine oral rinse or local anaesthetic lozenges or spray; (b) coating agents, e.g. sucralfate suspension as a mouthwash, carmellose paste or carbenoxolone mouthwash. * Dirty mouth. Encourage: (a) regular brushing with a soft toothbrush; (b) mouthwashes to remove debris; (c) chewing unsweetened pineapple chunks (fresh or tinned) if the tongue is coated. The proteolytic enzyme annanase will clear the tongue in days. * Foul smell. Use oral metronidazole 400 mg b.d. or rectal 500 mg b.d.

Raised intracranial pressure * Dexamethasone 16mg daily for 5 days, reducing to 4 mg daily over 2 weeks. Avoid an evening dose, which can cause insomnia.

Muscle spasm * Use: (a) dantrolene 25 mg daily increasing to a maximum of 400 mg a day; (b) baclofen 5mg t.d.s. increasing to a maximum of lOOmg a day; or (c) diazepam 2-10 mg t.d.s.

Bladder spasm * Treat any infection. * Give: (a) oxybutynin5mgb.d./t.d.s.; or (b) amitriptyline 25 mg at night increasing to t.d.s.

Malignant ulcer * If smelly: (a) give metronidazole topically or orally. Topical gel is much more expensive than tablets, and is only indicated in those who cannot tolerate the latter;28 (b) clean the ulcer with antiseptic, e.g. povidone iodine; and

(c) dress it with a charcoal cloth dressing or sprinkle oxychlorodene (Ostobon) between layers of dressings. Neither is available on the NHS. * If bleeding: (a) Acute. Pack with gauze soaked in adrenaline 1 in 1000; (b) Long-term. Consider referral for radiotherapy. Apply topically: - sucralfate suspension (tightly applied on a non-adherent dressing); or -tranexamic acid (use the injection solution on a non-adherent dressing). * If dirty: consider debridement with a polysaccharide, hydrocolloid or hydrogel dressing. * Excessive discharge. Use dressings with high absorbency, e.g. calcium alginate. A watery discharge may respond to high-dose topical steroids once daily for a week.

Lymphoedema * Active or passive movements of the limb should be performed regularly, i.e. 2-hourly in the daytime. * Skin dry ness. Maintain skin hydration with a moisturizer. * Breaks in the skin. Protect them from infection with antiseptic cream. * Cellulitis. Treat with elevation and antibiotics. Use penicillin; infection is usually due to streptococcus. A patient with repeated infections should have a supply of penicillin at home. Antibiotics may need to be continued for several months.

Reducing lymphoedema * Consider: (a) Massage. This can be done by the patient or carer, or by an electrical massager, for about 20 minutes twice a day. It is contraindicated if the skin is damaged, for instance by tumour or infection; (b) Support should be from high-compression, low-stretch materials. A single layer is usually adequate. Do not apply compression to the lower limb if the ankle brachial pressure index is <0.8 (see p. 293).

436

PAIN, PALLIATIVE CARE AND BEREAVEMENT

Self-help group: Lymphoedema Support Network, St Luke's Crypt, Sydney Street, London SW3 6NH, tel. 0207 351 4480; www.lymphoedema.org

(b) encourage the bereaved to begin life again after an appropriate time; (c) detect and treat pathological grief.

Pathological grief Living will (advance directive)

Expect it where:

Patients may wish to state their wishes about medical treatment should they develop:

(a) death was sudden or stigmatized (e.g. suicide or AIDS); (b) death was, or seen by the survivor to have been, avoidable; (c) death is unexplained, as in cot death, miscarriage or stillbirth; (d) the bereaved is socially isolated or has economic difficulties; (e) the relationship was of either a dependent or ambivalent nature. Excessive pining may follow the former, and excessive guilt the latter; (f) the person is vulnerable by reason of personality, previous mental illness or bereavement, or poor physical health.

(a) a life-threatening condition; or (b) permanent mental impairment; or (c) permanent unconsciousness. For this to be valid the patient must: (a) Be competent at the time of the declaration; (b) Be informed in broad terms about the nature and effect of the procedure; (c) Have anticipated and intended the refusal to apply to the circumstances that subsequently arise; (d) Be free from undue influence when issuing the declaration. Patient information: The Natural Death Centre, 20 Heber Road, London NW2 6AA, tel. 0208 208 2853; www.naturaldeath.org.uk The Terence Higgins Trust, 52-54 Gray's Inn Road, London WC1X 8JU, tel. 0207 831 0330; www.tht.org.uk

BEREAVEMENT

Systematic review: Woof WR, Carter YH. The grieving adult and the general practitioner: a literature review in two parts. Br J Gen Pract 1997; 47: 443-8, 509-14.

The GP is in a unique position to: (a) help the bereaved to express their feelings and come to terms with their loss at a pace that is appropriate for that person. At times, even the avoidance of emotion may be part of that adaptation;

Consider it when: (a) the bereaved appears to be stuck in a state of denial, anger or depression, rather than moving towards acceptance. Most people will have overcome denial by 2 months, anger by 6 months and depression by 12 months, although these timings are a crude guide only; (b) guilt is anything more than transitory; (c) the depth of grief makes the patient unable to discuss his or her feelings; (d) the bereaved is suicidal, rather than just 'looking forward to joining' the deceased; (e) the bereaved is using alcohol or drugs to cope with the loss. Management Before the death

If the family is willing and time permits, it is helpful for them to discuss openly their feelings with the dying, especially sharing any resentments they have about the past which, if not expressed now, might lead to prolonged guilt or anger later. Love, too, can be expressed at this stage, and the bereaved will not feel when it is too late that the

BEREAVEMENT

dead person 'never knew how much I loved him/her'. At the time of death

* Encourage the family to see the dead person and express any unfinished business. * Inform the family about the registration of the death, and how to contact a funeral director (see p. 439). After the death

After a bereavement, the minimum should be one visit with an invitation to talk further at a later stage. * Show concern and talk through the last illness. Encourage the patient to express any anger or guilt about what happened. Acknowledge anything you think could have been done better, without waiting for the relatives to bring it up. Where possible, reassure the bereaved that they did everything possible to help. * Explain that many different emotions may follow bereavement, and that none is abnormal. Specifically, explain that it is common to continue to hear and look for the deceased, and that this is not a sign of madness. * Pathological grief. If pathological grief is present, the GP should arrange for support (from a counsellor, psychologist or psychiatrist as appropriate). * Drugs. Avoid using regular tranquillizers and discourage alcohol (a number of people given benzodiazepines at bereavement continue longterm use). Antidepressants will be needed where bereavement has triggered a depressive illness. * Tell the bereaved about people or organizations who can help, e.g. counsellor, health visitor, Cruse, the clergy (addresses of the main organizations are given below). * Recommend reading, e.g. A Death in the Family by Jean Richardson (1979), published by Lion Publishing, or the leaflet Bereavement, by the Royal College of Psychiatrists. * Give an invitation for review some 6 weeks later.

437

* Note the event in the notes of the whole family. Record the actual date on the summary card of the spouse or consort, in order to spot the significance of symptoms presenting at that time in future years. Patient organizations: Cruse Bereavement Care, 126 Sheen Road, Richmond, Surrey TW9 1UR, tel. 020 8939 9530; helpline 0870 167 1677; www.crusebereavementcare.org.uk Literature: Royal College of Psychiatry. Bereavement. Online. Available: www.rcpsych.ac.uk/info/index.htm For parents who have lost a child: The Child Death Helpline, tel. 0800 282986; www.childdeathhelpline.org.uk Compassionate Friends, 53 North Street, Bristol BS3 1 EN, tel. 0117 953 9639; www.tcf.org.uk The National Association of Bereavement Services, 2 Plough Yard, London, EC2A, tel. 020 7247 1080/0617. For professionals: The WHO Guide to mental health in primary care: bereavement. Online. Available: www.psychiatry.ox.ac.uk/cebmh/whoguidemhpcuk/ index.html

COT DEATH Guideline: Guidelines for GPs when cot death occurs. Foundation for the Study of Infant Deaths, 1996.

Early contact and follow-up will help reduce feelings of isolation and misdirected anger. Close liaison with the health visitor is essential. * Explain that many reactions may occur, including hearing the baby cry, having aching arms and waking at night as though to attend to the baby. * Post-mortem. Explain that a post-mortem examination will be necessary, and that the parents will have to make a statement to the police. Explain the role of the Coroner, and that there may have to be an inquest. * Breast-feeding. Give advice about stopping lactation (see p. 270). * Hypnotics. Offer a small number of benzodiazepines to help sleep if the patient is becoming exhausted. * Identical twins. The survivor is at higher risk, and short-term admission should be considered.

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PAIN, PALLIATIVE CARE AND BEREAVEMENT

* Other siblings will need support. Give guidance on the emotional needs of brothers and sisters. * Follow-up. Ensure that the parents have been given an appointment to discuss the death with a paediatrician, and arrange to see them yourself afterwards. Professional and patient information: The Foundation for the Study of Infant Deaths, 35 Belgrave Square, London SW1X 8QB, tel. 0207 233 2090; www.sids.org.uk/fsid/ Parent 24-hour Cot Death helpline, tel. 0207 235 1721. SANDS (Stillbirth and Neonatal Death Society), 28 Portland Place, London W1N 4DE, tel. 0207 436 5881; www-uk-sands.org

BEREAVED CHILDREN * Children are 'concrete' thinkers, and do not develop the adult concept of death until the age of 9 or 10. This means that they do not realize that it happens to everyone, and that the dead person will never come back. Between the ages of 3 and 6 they are egocentric and go through a phase of antagonism to the parent of the same sex, and if that parent dies they may feel they caused the death. * Bereaved children have a high morbidity; 40-50% have behavioural symptoms at 1 year, and up to 10% develop quite severe mental illness later in life. * Try to match the explanation to the child's level of understanding. Explain that, although doctors are able to help with most illnesses, some are too severe to cure. * Encourage the whole family to talk about the dead person in front of the child, thereby giving the child permission to express his or her feelings. * Explain to the parents that children are more likely to express their feeling through play and art than verbally. They should make sure that play leaders and teachers are alert to what has happened. * Viewing the body and attending the funeral can help the child to realize that the person is really dead.

Books for children: Couldrick, A. When your mum or dad has cancer. Sobell Publications, 1991. Varley S. Badger's Parting Gifts. Picture Lions. For teenagers: Hollins S, Sireling L. When mum died and when dad died. London: Royal College of Psychiatrists, 1989. Online. Available: www.rcpsych.ac.uk/info/index.htm Williams G, Ross J. When people die. London: Macdonald Publishers, 1983.

SUICIDE • Because the death is sudden, perhaps unexpected and possibly violent, particularly intense feelings of shock and distress may occur in survivors. • Suicide carries a stigma, which may make those bereaved reluctant to seek support. • Guilt is a common reaction to suicide. A GP may feel that he or she has failed their patient. A relative may feel guilt that they were unable to prevent the death, or guilt at their relief after years of caring for a mentally ill relative. Anger against 'helping' professions is a common response to bereavement. Support organizations: The Samaritans, tel. 08457 909090. www.samaritans.org.uk SAMM (Support after Murder and Manslaughter), Cranmer House, 39 Brixton Road, London SW9 6SZ, tel. 0207 735 3838; www.samm-se.freeserve.co.uk Literature Hill K, Hawton K, Malmberg A, Simkin S. Bereavement information pack for those bereaved by suicide or other sudden death. London: The Royal College of Psychiatrists, 1997. Online. Available: www.rcpsych.ac.uk/info/index.htm Wertheimer A. A special scar: the experiences of people bereaved by suicide. London: Routledge, 2001.

DEATH RITUALS OF DIFFERENT CULTURES • Buddhism: cremation is arranged at a time determined by monks; no fundamental objections to post-mortem examination. • Christianity, cremation or burial; no fundamental objections to post-mortem examination.

BEREAVEMENT

• Hinduism: body is cremated; post-mortem examination is felt to be disrespectful. • Islam: the body should be touched only by Muslims; non-Muslims should wear disposable gloves when touching the body. Burial should be carried out within 24 hours. • Judaism: burial within 24 hours; post-mortem only in exceptional cases. • Sikhism: cremation within 24 hours.

ASPECTS OF DEATH Advice: Office for National Statistics. Death certification and referral to the coroner. London: HMSO, 1996.

Certification Refer to the coroner cases where: (a) The cause of the death is unknown; (b) The deceased was not seen by the certifying doctor either after death or within the 14 days before death; (c) The death was violent, unnatural or suspicious; (d) The death may be due to an accident (whenever it occurred); (e) The death may be due to self-neglect or neglect by others; (f) The death may be due to an industrial disease, or related to the deceased's employment; (g) The death may be due to an abortion; (h) The death may have occurred during an operation or before recovery from the effects of an anaesthetic, or was related to a medical treatment or procedure; (i) The death may be a suicide or was otherwise self-induced; (j) The death occurred during or shortly after detention in police or prison custody or while detained under the Mental Health Act. Referral is not obligatory but is helpful when: (a) The relatives are considering a complaint; (b) The deceased was in receipt of a service or industrial disability pension and where the cause

439

of death is related to that disability, or where the pensioner was in receipt of a constant attendance allowance. It will assist a claim for a funeral grant and widow's pension. If the death is to be a coroner's case or if the GP is uncertain: (a) Do not allow the body to be moved from the place of death. The police will do this; (b) Contact the coroner's officer (out of normal hours, ring the police station). Uniformed officers will attend the scene of death. Warn the relatives of this, and explain that this does not (necessarily) mean that something has gone wrong. The coroner may organize a post-mortem and/or issue a death certificate; or advise the GP to issue a certificate (the GP should initial the back of the certificate). If the death is not a coroner's case: (a) Discuss the death with the relatives, and explain that they can now telephone the funeral directors; (b) Issue the death certificate or, if another doctor will be signing it, tell the relatives to contact that doctor; (c) Recommend the leaflets What to do After a Death (available from Social Security offices or from DSS Leaflets Unit, PO Box 21, Stanmore HA71 AY) and What to do when someone dies (available from Which? Castlemead, Gascoyne Way, Hertford SG14 1LH). In Scotland, for 'coroner' read 'procurator fiscal'.

DONATION OF EYES AND BODIES Donation of eyes * Telephone the local ophthalmology unit. The eyes should be removed within 24 hours of death. Most people who die could become corneal donors. The only medical conditions restricting donation are viral diseases, motor neurone disease, active leukaemias or lymphomas, previous intraocular surgery, ocular infection or malignancy.

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Donation of bodies for dissection * Telephone the anatomy office of the nearest medical school or, in London, the London Anatomy Office (0208 741 2198), who will arrange

for removal of the body. If death occurs outside office hours, telephone HM Inspector of Anatomy (0208 407 5522).

REFERENCES 1. Li Wan Po A, Zhang WY. Systematic overview of co-proxamol to assess analgesic effects of addition of dextropropoxyphene to paracetamol. BMJ 1997; 315: 1565-71. 2. McQuay HJ et al. Systematic review of outpatient services for chronic pain control. Health Technology Assessment 1997; 1 (6). 3. The Oxford Pain Internet Site: www.jr2.ox.ac.uk/ bandolier/painres/painpag/index.html 4. Thompson JF et al. Rectal diclofenac compared with pethidine injection in acute renal colic. BMJ 1989; 299: 1140-1. 5. Anonymous. Pain relief in babies - a not so simple problem. Mat Child Health April 1989: 121-2. 6. Marriott SC et al. A dose-ranging study of ibuprofen syrup as an antipyretic. Arch Dis Child 1991; 66 (Suppl.): 1037-42. 7. MeReC. Combination Analgesics. MeReC Bulletin 1993; 4: 45-7. 8. Collins SL, Moore RA, McQuay HJ. The visual analogue pain intensity scale: what is moderate pain in millimetres? Pain 1997; 72: 95-7. 9. Serlin RC, Mendoza TR, Nakamura Y, Cleeland CS. When is cancer pain mild, moderate or severe? Grading pain severity by its interference with function. Pain 1995; 61: 277-84. 10. Grossman SA, Sheidler VR, Sweeden K et al. Correlation of patient and caregiver ratings of cancer pain. / Pain Sympt Management 1991; 6: 53-7. 11. DTB. Drug treatment of neuropathic pain. Drug Ther Bull Dec 2000; 38: 89-93. 12. McQuay HJ et al. Anticonvulsant drugs for the management of pain: a systematic review. BMJ 1995; 311: 1047-52. 13. Bowsher D. Post-herpetic neuralgia in older patients. Incidence and optimal treatment. Drugs Aging 1994; 5: 411-18. 14. Rowbotham MC et al. Topical lidocaine reduces pain in post-herpetic neuralgia. Pain 1989; 38: 297-301. 15. Milligan NS, Nash TP. Treatment of post-herpetic neuralgia: a review of 77 consecutive cases. Pain 1985; 23: 381-6.

16. Wiffen P, Collins SL, McQuay HJ et al. Anticonvulsant drugs for acute and chronic pain (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2001. 17. Paice E. Reflex sympathetic dystrophy. BMJ 1989; 310: 1645-8. 18. Jadad AR, Carroll D, Glynn CJ, McQuay HJ. Intravenous regional sympathetic blockade for pain relief in reflex sympathetic dystrophy: a systematic review and a randomised, double-blind crossover study. / Pain Sympt Management 1995; 10: 13-20. 19. WHO. Cancer pain relief and palliative care. Geneva: World Health Organization, 1990. 20. Fallowfield L. Psychosocial interventions in cancer. BMJ 1995; 311: 1316-17. 21. Thorpe G. Enabling more dying people to remain at home. BMJ 1993; 307: 915-18. 22. Thomas K. Macmillan Cancer Relief Report: Out of Hours Palliative Care in the Community, Mar 2001. 23. Portnoy RK, Thaler HT, Kornblith AB et al. Memorial symptom assessment scale: an instrument for the evaluation of symptom prevalence, characteristics and distress. Eur J Cancer 1994; 30A: 1326-36. 24. Scottish Intercollegiate Guidelines Network and the Scottish Cancer Therapy Network. Control of pain in patients with cancer. SIGN, 2000. Online. Available: www.sign.ac.uk 25. National Council for Hospice and Specialist Palliative Care Services. Guidelines for managing cancer pain in adults, 2nd edition. 1998. 26. McQuay HJ, Moore RA. Antidepressants and chronic pain. BMJ 1997; 314: 763^. 27. Pan CX et al. Complementary and alternative medicine in the management of pain, dyspnoea, and nausea and vomiting near the end of life: a systematic review. / Pain Sympt Management 2000; 20: 374-87. 28. DTB. Metronidazole gel for smelly tumours. Drug Ther Bull 1992; 30: 18-19.

CHAPTER CONTENTS

26

Lifestyle advice 442 Screening 447 References 454

Health promotion and screening

441

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HEALTH PROMOTION AND SCREENING

LIFESTYLE ADVICE Exercise

Exercise reduces the risk of:

Overview: Exercise Referral Systems: A National Quality Assurance Framework.1 www.doh.gov.uk/ exercisereferrals There is considerable evidence that moving from a sedentary to a more active lifestyle improves health. Exercise advice is more likely to be effective if appropriate to the individual; for example, although some will join a gym, many more will be able to adapt to walking to work or walking the children to school.

(a) CHD. Physically inactive people have about double the risk of CHD.1 Exercise must be vigorous (for 20 minutes, three times a week) or moderate, e.g. brisk walking (for 30 minutes five times a week).2 The exercise may be broken into separate bouts as long as they are for at least 10 minutes each. In the short term, the risk of myocardial infarction is halved3 with less risk of death if it does occur, but this benefit is lost if exercise is stopped. Exercise continued for over 1 year improves the coronary collateral circulation enough to be demonstrable on a thallium scan. There is a strong inverse association between physical activity and the risk of coronary events. Regular physical activity prevents or delays the development of high blood pressure and reduces blood pressure in people with hypertension.4 (b) Diabetes. Time spent in a sedentary manner, e.g. watching television, significantly increases the risk of developing type 2 diabetes.5 However, the risk of developing diabetes may be reduced by more than 50% in those who undertake regular moderate exercise.6'7 (c) Cancer. It is postulated that adoption of an active lifestyle could reduce all-cause cancer by 45%.8 There is strong evidence that physically active men and women have about half the risk of colonic cancer, and women possibly 30% of breast cancer, than sedentary counterparts.9 (d) Osteoporosis. Exercise will increase bone density, even in women over 70. It should be weight bearing, for at least 30 minutes per day. If practised at least three times a week, fracture rates are halved. Conversely, immobilization produces irreversible bone loss. (e) Depression. There is evidence that exercise reduces the intensity of depression,10 and that depression is more common in those who are inactive physically.11 Both short- and long-term exercise reduces the intensity of depression.12 (f) Disability in older people. Those who exercise develop disability at a quarter of the rate of those who don't exercise, even though the exercisers have a higher rate of fractures and short-term disability related to their exercise behaviour. This difference is still present after 9 years. Medical care costs were a quarter less in the exercising group. Even the very old benefit, with improvements in muscle strength, gait velocity and stair-climbing ability.13 (g) Falls in older people are reduced by a multifactorial intervention approach that includes exercise and balance training.14 Exercise of moderate intensity equates to 60-85% of the maximum age-adjusted heart rate. Maximum age-adjusted heart rate = 220 - age in years. The patient will feel slightly breathless and warmer than usual.

LIFESTYLE ADVICE 443

Diet The COMA report recommends major changes in the national pattern of food consumption.15

A higher fruit and vegetable intake (six or more servings daily) helps provide protection against: (a) cardiovascular disease. Increasing fruit and vegetables by two servings per day may decrease cardiovascular risk by 30%; (b) macular degeneration and cataract; (c) cancers, including breast, colorectal and stomach; (d) diverticular disease; (e) diabetes. A higher intake of starch carbohydrates and non-starch polysaccharides (e.g. potatoes, bread, pulses and pasta) helps provide protection against: (a) cardiovascular disease; (b) cancers, including breast and colorectal. Reduced intake of saturated fat (by an average of 11 g per day) and total fat (by 15 g per day). The COMA report recommends simple measures, e.g. reduction in fatty meat and meat products, high-fat dairy products and full-fat spreads, with increased use of skimmed milks and low-fat spreads. High fat intake increases the risk of: (a) CHD: modest reductions in saturated fat and salt intake may reduce cardiovascular disease; (b) cancers: including colorectal, prostate and breast; (c) large bowel disease; (d) osteoarthritis. Altered fat intake. Eating fatty fish may reduce CHD deaths by up to 30%. The COMA report recommends eating at least two portions of fish per week, at least one of which should be fatty, and replacing fats rich in saturated fatty acids with fats and oils low in saturates and rich in monounsaturates. Reduced sodium. The COMA report recommends an average reduction of salt intake of about 3 g per day. They recommend not adding salt to food and consuming low-salt foods where possible. Common sources of salt are nuts, crisps, canned foods and bread. High sodium intake increases the risk of: (a) stroke; (b) hypertension; (c) stomach cancer; (d) osteoporosis.

Obesity

Obesity increases the risk of:

The COMA report recommends that people should maintain a desirable body weight (BMI between 20 and 25).

(a) CHD. Central obesity (rather than BMI) carries the greater cardiovascular risk, and can be assessed by measuring the waist circumference. Men with a waist circumference over 94cm (women over 80 cm) are likely to have other risk factors for cardiovascular disease, and men with a waist circumference over

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102cm (women over 88cm) are two-and-a-half to four-and-a-half times as likely to have other major cardiovascular risk factors;16 (b) diabetes: the risk of diabetes increases with BMI, with no evidence of a threshold;6 (c) hypertension; (d) stroke; (e) gall bladder disease; (f) gout; (g) breast cancer (postmenopause).

Alcohol

Modest alcohol intake (1-3 units a day) protects against CHD,17 heart failure18 and ischaemic stroke,19'20 but promoting alcohol for this purpose could be hazardous and needs to be balanced against the adverse effects of alcohol. (a) CHD. Keep alcohol intake within safe limits - up to four units per day for men and three for women, with a daily tally rather than a weekly one21 (see also binge drinking, below22, and p. 321). A higher intake raises LDL cholesterol, triglyceride, blood pressure and calorie intake. (b) Cancer. Several cancers show evidence of a dose-response relation with alcohol intake, e.g. mouth, pharynx, larynx, oesophagus and liver. In others there is a possible relationship, e.g. breast and rectum (the latter beer only). Studies indicate that the cancer risk of alcohol multiplies, instead of merely adding to, the risk from smoking.23 (c) Binge drinking has been shown to be associated with a seven times higher risk of 'external death' (violence, suicides, injuries, poisonings), and a five times higher risk of fatal myocardial infarction.22

Smoking

Smoking increases the risk of: (a) Cancers, including lung (90% are smokers, people smoking 25 per day have 25 times the risk), stomach, colon, bladder and renal pelvis (increased five times). (b) CHD. Men under the age of 65 who smoke >25 cigarettes a day have three times the risk of dying from CHD. There is evidence that women who smoke have a 50% higher risk of dying from CHD than men.24 Someone who smokes 20 a day or less and stops has a risk of CHD 10 years later almost the same as one who has never smoked. (c) Stroke and vascular dementia. Stroke risk is increased almost four times in women smoking >25 cigarettes a day. (d) Peripheral vascular disease, which is up to twice as common as in non-smokers. (e) Diabetes. Current smoking roughly doubles the risk of developing diabetes.6'25

LIFESTYLE ADVICE 445

(f) COPD. Almost all patients with COPD are (or have been) smokers. The rate of fall of FEY^ is reduced if a patient stops smoking. (g) Osteoporotic fractures. Postmenopausal bone loss is greater in smokers than non-smokers. The risk of hip fracture is estimated to be 17% greater at age 60,41% greater at age 70 and 71% greater at age 80. The data on men is limited, but the effect appears to be similar.26 (h) Gastric ulcers, which are up to twice as common in smokers. (i) A poorer outcome of pregnancy. There is 60-70% increased risk of ectopic pregnancy, placental abruption, placenta praevia and premature rupture of membranes.27 There is a significantly increased risk of miscarriage, low birthweight, admission to neonatal intensive care units, perinatal deaths and sudden infant death syndrome.28 Cancers may also be more prevalent in infants of mothers who smoke through their pregnancy.29

Passive smoking30

Passive smoking increases the risk of: (a) CHD; the risk is increased by about 25%;31 (b) lung cancer; the risk is increased by about 15-25%;32'33 (c) respiratory illness and decreased lung function;34 (d) low birthweight infants: Infants born to non-smoking women exposed to environmental tobacco smoke are 2-4 times more likely to be of low birthweight.35 In children: (a) Respiratory illness. Children who grow up in households where one or both parents smoke have a significantly higher risk of:36 - aged 0-2: wheeze, lower respiratory tract infection, hospital admission for lower respiratory tract infection. -aged 5—16: asthma, other respiratory symptoms (cough, phlegm, breathlessness), decreased FEV^ -recurrent otitis media and middle ear effusion; (b) Sudden infant death-37 (c) Cancer. The results of exposure to paternal smoke suggest an increased risk of brain tumours and lymphomas.38

Sun exposure3>9,40

• Basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and melanoma are all caused by sun exposure. • The incidence rate is higher in fair-skinned, sun-sensitive, rather than darker-skinned less sun-sensitive people. • The risk increases with increasing solar radiation. • The highest incidences are on the most sun exposed parts of the body and lowest densities on the least sun exposed parts. • Occupational exposure is associated mainly with SCC and recreational sun exposure is associated mainly with melanoma and BCC.

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HEALTH PROMOTION AND SCREENING

• Risk is increased if there is a history of sunburn or the presence of benign skin damage. • There is evidence that sunscreens reduce the incidence of squamous cell carcinoma and solar keratosis. It is more effective if applied to exposed areas every morning and reapplied after heavy sweating, bathing or prolonged sun exposure.41 • The sunscreen should protect against ultraviolet A and B. In patients at high risk, suggest a sun protection factor (SPF) of at least 15.42 Sunscreens should be liberally applied, and reapplied every 2 hours if exposure to the sun continues. • Fine-woven cotton clothing and hats also screen the skin. • Exposure to sunlight should be rationed and strong midday sun avoided. • Children should be protected against strong sunlight at all times. Use a sunscreen with a high sun protection factor (>15), and additional protection against ultraviolet A radiation. • Avoid sunbeds and sunlamps.

Osteoporosis See p. 180.

The following measures are recommended for the whole population regardless of risk: (a) Stopping smoking before the menopause reduces the later fracture rate by 25%. Heavy smokers could halve their risk by stopping. (b) Exercise will increase bone density, even in women over 70. Exercise should be weight bearing for at least 30 minutes per day. If practised at least three times a week, fracture rates are halved. Conversely, immobilization produces irreversible bone loss. (c) Alcohol. Weekly intake should be kept within sensible limits. (d) Diet. Should contain at least 700 mg of calcium (=1 pint of milk) and adequate vitamin D per day (400 iu/day). Women with a low calcium diet (less than 400 mg a day) are likely to benefit from supplements. Studies on those whose diet is already adequate are conflicting. Recommended doses vary if supplements are to be given. One consensus is lOOOmg daily, but 1500 mg for postmenopausal women and 1200 mg for adolescents.43

Avoidance of falls4445

The National Service Framework (NSF) recommendations for assessing the risk of falls and guidance on referral: Patient risk factors: (a) balance, gait or mobility problems (including joint disease, stroke and Parkinson's disease); (b) polypharmacy, i.e. taking four or more medicines, particularly centrally sedating or blood-pressure-lowering drugs; (c) visual impairment; (d) impaired cognition or depression; (e) postural hypotension.

SCREENING

447

Environmental risk factors: (a) poor lighting, particularly on the stairs; (b) loose carpets or rugs; (c) badly fitting footwear or clothing; (d) lack of safety equipment, e.g. grab rails; (e) steep stairs; (f) slippery floors; (g) inaccessible lights or windows. Older people who fall should be referred to a specialist falls service?5 particularly those who: (a) have had previous fragility fractures; (b) have attended the accident and emergency department following a fall; (c) have called an ambulance following a fall; (d) have two or more patient risk factors; (e) have frequent unexplained falls; (f) fall in hospital or in a nursing or residential home; (g) live in unsafe housing conditions; (h) are very afraid of falling. Interventions to reduce the risk of falls and damage from falling: (a) assessment and correction of vision, if possible; (b) medication review and discontinuation of inappropriate medication; (c) rehabilitation, including physiotherapy, to improve confidence; (d) occupational therapy to identify and correct hazards in the home; (e) exercise and balance training; (f) use of hip protectors; (g) treatment of osteoporosis.

Blood pressure See CHD below

• Ten per cent of all adults in the UK have raised blood pressure, using UK definitions. US definitions give a prevalence of 1 in 4 of the population with hypertension.46 • The rule of halves, described in the US in 197247 was still operating in Scotland in the late 1980s,48 although there may have been some improvement since then.49 The rule states that half of hypertensives are not known to have a raised blood pressure; of those with known hypertension, half are not on treatment and half of those on treatment are poorly controlled. • Even in mild hypertension, a reduction of systolic pressure by lOmmHg and diastolic pressure by 6mmHg reduces the risk of stroke by one-third and the risk of a major coronary event by one-sixth.50 The benefits of treatment of hypertension in the elderly are greater than in the younger patient, at least up to the age of 80.51

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HEALTH PROMOTION AND SCREENING

* Measure the blood pressure of all adults at least every 5 years until age 80. * Search for other risk factors for cardiovascular disease if hypertension is found.

Lipids See CHD below

• A10% reduction in cholesterol concentration will reduce the risk of coronary heart disease by 30%.52 However, the advice on whom to test varies according to the healthcare system (see p. 107). • The UK NSF53 does not recommend the screening of the whole population for hypercholesterolaemia but urges identification, advising and treating those most at risk: (a) with clinical evidence of CHD, e.g. past history of heart attack, angina, coronary revascularization, or other clinical manifestations of occlusive arterial disease, e.g. PVD, TIA or ischaemic stroke. (b) without clinical evidence of CHD or other occlusive arterial disease but who have a risk of a cardiac event of more than 30% in the next 10 years (calculate the 10-year risk of coronary heart disease using the Cardiac Risk Assessor computer program (available on www.hyp.ac.uk/bhs). Familial hypercholesterolaemia is present in 1 in 500 of the population in the UK and for men carries a 50% risk of a major coronary event by the age of 60; in women the risk is 30%.54 Suspect it if: (a) the total cholesterol is >7.5 mmol/L (>6.7 in children <16) or the LDL-C is >4.9 mmol/L (>4.0 in children <16); and (b) the patient or a first- or second-degree relative has tendon xanthomas; or (c) a first-degree relative had a myocardial infarction before the age of 60 or a second-degree relative before the age of 50; or (d) a first- or second-degree relative has a total cholesterol >7.5 mmol/L. * Refer all suspected cases to a lipid clinic.

Coronary heart disease (CHD)54

Risk factors for CHD cluster. The finding of one should prompt a search for others. They are:

See p. 107

(a) (b) (c) (d) (e) (f) (g) (h) (i) (j)

smoking; central obesity; poor diet; high alcohol intake; low levels of exercise; hyperlipidaemia; hypertension; diabetes; a family history; ethnicity, especially South Asians.

SCREENING

ColoreCtal Cancer55-56

449

• 90% of colorectal cancers in the UK are not diagnosed until the cancer has penetrated through the bowel wall (Dukes B, C and D). These carry 5-year survival rates of 64%, 38% and 3%, respectively. • 70-90% of colorectal cancer arises from premalignant adenomatous polyps. • 10% of colorectal cancers are due to genetic causes but 90% are accounted for by environmental factors, especially diet (high in red meat and fat, and low in vegetables). There is evidence from three studies that screening for faecal occult blood (FOB) reduces overall mortality by 15-30%, with fewer patients being diagnosed at an advanced stage. To be cost-effective, the number of false positives needs to be low: FOB needs to be taken after 3 days on a diet that is meat-free and low in vegetables with high peroxidase, e.g. tomatoes. At least three samples from 3 separate days are needed. Case control studies have demonstrated a 60% reduction in mortality of tumours within reach of the sigmoidoscope (about 60% of all colorectal cancers if a flexible sigmoidoscope is used). High-risk individuals • Refer for screening those who have: (a) a first-degree relative who developed colorectal cancer before the age of 45 (their lifetime risk is 1 in 10); (b) two first-degree relatives with colorectal cancer (their lifetime risk is 1 in 6); (c) a family history of familial adenomatous polyposis (FAP) or hereditary non-polyposis colon cancer HNPCC (see below). Screening will probably be by total colonoscopy, 5-yearly, starting 5-10 years younger than the age at cancer presentation of the relative. Screening is likely to be more frequent in the case of FAP and HNPCC. Family history of FAP FAP in a first-degree relative carries a risk of 1:2. It accounts for 0.5% of all colorectal cancers. Patients with the condition develop cancer by the age of 40. Family history suggestive of HNPCC The Amsterdam criteria are: (a) three or more family members with colorectal cancer, one of them a first-degree relative of one other; and (b) at least two generations affected; and (c) one family member less than 50 years old at diagnosis. Such patients have a 40% lifetime risk of colorectal cancer, and colonoscopy every 12 to 18 months is recommended.

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HEALTH PROMOTION AND SCREENING

Inflammatory bowel disease or previous carcinoma (a) Patients with a personal history of long-standing ulcerative colitis and, to a lesser extent, Crohn's disease, are at increased risk of carcinoma (see p. 141); (b) Patients who have had one colorectal cancer are at increased risk of a second primary. Normal risk individuals who request screening 'Screening on demand' is increasingly common practice for patients who are anxious about the risk of colorectal cancer. In general this consists of three faecal occult blood tests and, if positive, referral for colonoscopy.

Breast cancer

Risk in women with a family history57'58

Criteria determining level of risk

Recommended management

High risk (more than four times the average age-related risk)

Four or more relatives affected at any age by breast or ovarian cancer Three relatives affected with breast or ovarian cancer, with an average age of diagnosis of the breast cancer of less than 60 years Two relatives affected by breast cancer, with an average age at diagnosis of less than 40 years One relative with both breast and ovarian cancer at any age

Referral for specialist genetic counselling at a regional genetic centre Genetic testing may be appropriate for some of these patients

Moderate risk (at least three times the average age-related risk)

One first-degree relative* with breast cancer diagnosed under the age of 40 years

Current sparsity of evidence to support intervention - referral for management within clinical studies can be considered Genetic testing is not appropriate for these patients

Two first- or second-degree** relatives with breast cancer diagnosed under age 60, or ovarian cancer at any age Three first- or second-degree relatives with breast or ovarian cancer diagnosed at any age One first-degree relative with bilateral breast cancer under age 60 One first-degree male relative with breast cancer at any age Low risk

Women not falling into the above categories

Reassurance that the individual's risk is not significantly elevated, with discussion of the difference between familial and non-familial cancer Advice on breast awareness (because

SCREENING

451

the risk of non-familial breast cancer remains) Encourage participation in the National Breast Screening Programme at age 50 • A first-degree female relative is a mother/sister/daughter; **a second-degree female relative is a grand-daughter/grandmother/aunt/niece.

Breast and Ovarian

cancers

NHS breast-screening programme59 • Women over the age of 50 are offered breast screening every 3 years until (at present) the age of 64. However, it is intended to extend this until age 70 by 2004. There is no upper limit of screening 'on request' and women over 64 are encouraged to make their own appointments. (The NHS Breast Cancer Screening Programme www.cancerscreening. nhs.uk). • Thp NHS breast-screening programme was introduced in 1988. By 1998 there was a reduction in mortality of around 21% attributable to improvements in treatment, presentations outside screening* and screening itself. It is estimated that the effect of improvements in treatment is around 15% and the direct effect of screening around 6.5%.60 • About 70-80% of breast cancers detected on screening have a good prognosis. At an initial screen, up to 20% will be in situ, 20-25% will be invasive lesions under 1 cm in diameter, and 25% will be invasive lesions between 1 and 2 cm in diameter. • Regular screening reduces mortality of the order of 40% among women aged over 50, if screened every 2-3 years. No improvement in mortality has been demonstrated in younger women.61 Recommendations for GPs (a) Women under 50. There is insufficient evidence to demonstrate that screening women under 50 is effective in reducing mortality from breast cancer. The risks may be greater than any potential benefit. (b) Women with a family history of breast cancer: - Women under 50 with a strong history of breast cancer are not included in the screening programme. General practitioners should be aware of the local provision for the management of these women. - Women aged 50 and over with a family history should not be offered screening more frequently than once every 3 years unless there are mammographic or clinical reasons for doing so. (c) Women with symptoms between routine mammography. Women should continue to be aware of minimal symptoms and to report these without delay to their GP, even if they have been recently screened. A woman with symptoms should be examined by her GP and, if necessary, referred for investigation to a surgeon with a special interest

452

HEALTH PROMOTION AND SCREENING

in breast disease working in a hospital that can provide a multidisciplinary approach to it. (d) Women with breast cancer. Women who have undergone surgery for breast cancer should have mammography yearly for the first 5 years, and then 2-yearly.62 (e) Women receiving hormone replacement therapy. Women aged 50-64 on HRT do not need more frequent screening. Women do not need a baseline mammogram before receiving HRT. Ovarian cancer There is no national ovarian cancer screening programme but the following are at higher risk of developing ovarian cancer and may wish to be referred for inclusion in research screening (annual CA125 and ultrasound examination): (a) two first-degree relatives on the same side of the family with ovarian cancer; or (b) one first-degree relative with ovarian cancer and one first-degree relative with breast cancer on the same side of the family diagnosed under 50; or (c) one first-degree relative with ovarian cancer and two first- or second-degree relatives with breast cancer on the same side of the family diagnosed under 60.

Cervical cancer screening6364

• The Intercollegiate Working Party's recommendation63 is that all women aged 20-64 who have ever had sexual intercourse should be offered a smear every 3 years, with no upper age limit for women who have never had a smear. Women reaching the age of 65 who have had at least two normal smears in the last 10 years need not have further smears.65 • Screening every 3 years, in the 20-64 age group, reduces the incidence of cervical cancer by 91%, whereas screening every 5 years reduces the incidence by 84%. Screening every year reduces the incidence by 93%, but puts a considerable burden on resources.65 • Screening within a year of starting sexual intercourse may encourage future compliance, but is unlikely to pick up abnormal cytology.

Prostate cancer6667

There is no evidence to support the screening of asymptomatic men, either by digital rectal examination, by PSA level or transrectal ultrasound. However, the advice of the National Screening Committee is that men who ask for the PSA test, after counselling, should receive it and any follow-up necessary. All men requesting the test should be offered the opportunity to discuss the information using the more detailed information in one of the leaflets available (www.nelc.org.uk).

SCREENING

453

Explain that: (a) a rectal examination is needed as well as the blood test; (b) screening can miss a quarter of prostate cancers; (c) if screening is positive, a biopsy will be needed. Only about one in three who have a positive screen turn out to have cancer; (d) It is not clear that early treatment saves lives; and the treatments that may be offered have potentially severe adverse effects; (e) the stress of knowing that you have cancer is considerable and will affect insurance applications.

Diabetes6869

There is no national screening programme but GPs should regularly test those at higher risk: (a) women who have developed gestational diabetes; (b) women with polycystic ovary syndrome (PCOS); (c) men or women with evidence of impaired glucose tolerance (IGT) or with impaired fasting glycaemia (IFG); (d) people with coronary heart disease or those with multiple risk factors for it. GPs should have a high level of awareness and low threshold for testing those at multiple risk: (a) where there is a family history; (b) those overweight or obese; (c) because of ethnic background (especially those of South Asian, African, African-Carribean and Middle Eastern descent); (d) because of age (the prevalence is 1 in 20 at 65, and 1 in 5 at 85).

Glaucoma70 See p. 361

In the UK there is no formal national screening programme but, to facilitate opportunistic surveillance, free sight tests are available for certain sections of the community (see p. 6). • The incidence of glaucoma increases with age from 0.08/1000/year in white people in their early 40s to 1.46/1000/year in the over 80s. • The General Household Survey71 indicates that the subgroup most at risk is least likely to attend for sight tests. It also indicates that sight tests are much less common in unskilled men and women. • Optometrists check intraocular pressure in only half of their customers and test the visual field in only one-tenth.72 • Advise all patients, when attending for a sight test, to request tests of intraocular pressure and visual fields. • Advise particularly the following to be screened for glaucoma: (a) the elderly;73 (b) Africans or African-Caribbeans;74 (c) those with a family history;

454

HEALTH PROMOTION AND SCREENING

(d) high myopes; (e) diabetics; (f) males.

Osteoporosis75

The frail, with increased risk of falls whether housebound or not. Those with major risk factors: (a) untreated hypogonadism: -premature menopause; - secondary amenorrhoea; -primary hypogonadism in women; -primary or secondary hypogonadism in men. (b) prolonged corticosteroid therapy (6 months or more): -those on prednisolone 15mg daily or more; -those on prednisolone 7.5 to 15mg daily plus one or more risk factors; (c) diseases associated with osteoporosis, e.g. Crohn's disease, ulcerative colitis, chronic liver disease, hyperparathyroidism, hyperthyroidism; (d) radiological osteopenia (both vertebral fractures and apparent loss of bone density). Other risk factors include family history of osteoporosis, low body weight, and smoking. Those with a previous fragility fracture.

REFERENCES 1. Exercise Referral Systems: A National Quality Assurance Framework. Online. Available: www.doh.gov.uk/ exerciserefer rals / 2. Powell KE, Pratt M. Physical activity and health. BMJ 1996; 313: 126-7. 3. British Heart Foundation. Working Group Report. British Heart Foundation, 1991. 4. Manson JE, Hu FB, Rich-Edwards JW et al. A prospective study of walking as compared with vigorous exercise in the prevention of coronary heart disease in women. New Engl} Med 1999; 341: 650-8. 5. Hu FB, Letzmann MF, Stampfer MJ et al. Physical activity and television watching in relation to risk for type 2 diabetes mellitus in men. Arch Intern Med 2001; 161:1542-8. 6. Perry IJ et al. Prospective study of risk factors for development of non-insulin dependent diabetes in middle-aged British men. BMJ 1995; 310: 560-4. 7. Hu FB, Stampfer MJ, Solomon C et al. Walking compared to vigorous physical activity and risk of type 2 diabetes in women. JAMA 1999; 282: 1433-9. 8. Shephard RJ, Futcher R. Physical activity and cancer: how may protection be maximized? Crit Rev Oncog 1997; 8: 219-72. 9. Batty D. Does physical activity prevent cancer? BMJ 2000; 321: 1424-5.

10. Martinsen EW et al. Effects of aerobic exercise on depression: a controlled study. BMJ 1985; 291: 109. 11. Farmer ME et al. Physical activity and depressive symptoms: the NHANES 1 epidemiologic follow-up study. Am J Epidemiol 1985; 128: 1340-51. 12. North TC, McCullagh P, Zung vu Tran A. The effect of exercise on depression. Exercise Sports Sci Rev 1990; 80: 379-416. 13. Elon RD. Geriatric medicine. BMJ 1996; 312: 561-3. 14. Tinetti ME, Baker DI et al. A multifactorial intervention to reduce the risk of falling among elderly people living in the community. New Engl J Med 1994; 330: 1769-73. 15. Report of the Cardiovascular Review Group of the Committee on Medical Aspects of Food Policy (COMA). Nutritional aspects of cardiovascular disease. Report of Health and Social Subjects, 46. London: DoH, 1994. 16. Han TS et al. Waist circumference action levels in the identification of cardiovascular risk factors: prevalence study in a random sample. BMJ 1995; 311: 1401-5. 17. Meister KA, Whelan EM, Kava R. The health effects of moderate alcohol intake in humans: an epidemiologic review. Crit Rev Clin Lab Sci 2000; 37: 261-96. 18. Abramson JL, Williams SA, Krumholz HM et al. Moderate alcohol consumption and risk of heart failure among older persons. JAMA 2001 285: 1971-7.

REFERENCES

19. Sacco R, Elkind M, Boden-Albala B et al. The protective effect of moderate alcohol consumption on ischemic stroke. JAMA 1999 281: 53-60. 20. Berger K, Arjani UA, Kase CS et al. Light to moderate alcohol consumption and the risk of stroke among U.S. male physicians. New Engl J Med 1999; 341: 1557-64. 21. Gaziano JM, Hennekens C. Royal Colleges' advice on alcohol consumption. BMJ 1995; 311: 3-4. 22. Kauhanen J, Kaplan GA et al. Beer hinging and mortality: results from the Kuopio ischaemic heart disease risk factor study, a prospective population based study. BMJ 1997; 315: 846-51. 23. Austoker J. Cancer prevention in primary care: reducing alcohol intake. BMJ 1994; 308:1549-52. 24. Prescott E et al. Smoking and the risk of myocardial infarction in women and men: longitudinal population study. BMJ 1998; 316: 1043-7. 25. Rimm EB et al. Prospective study of cigarette smoking, alcohol use, and the risk of diabetes in men. BMJ 1995; 310:555-9. 26. Law MR, Hackshaw AK. A meta-analysis of cigarette smoking, bone mineral density and risk of hip fracture: recognition of a major effect. BMJ 1997; 315: 841-6. 27. Castles A, Adams EK et al. Effects of smoking during pregnancy. Five meta-analyses. Am J Prev Med 1999; 3: 208-15. 28. DiFranza JR, Lew RA. Effect of maternal cigarette smoking on pregnancy complications and sudden infant death syndrome. / Fam Pract 1995; 40: 385-94. 29. Boffetta P, Tredaniel J, Greco A. Risk of childhood cancer and adult lung cancer after childhood exposure to passive smoke: a meta-analysis. Environ Health Perspect 2000; 108: 73-82. 30. Davis RM. Passive smoking: history repeats itself. BMJ 1994; 315: 961-2. 31. He J, Vupputuri S, Allen K et al. Passive smoking and the risk of coronary heart disease - a meta-analysis of epidemiologic studies. New Engl J Med 1999; 340: 920-6. 32. Copas JB, Shi JQ. Reanalysis of epidemiological evidence on lung cancer and passive smoking. BMJ 2000; 320: 417-18. 33. Taylor R, Gumming R, Woodward A et al. Passive smoking and lung cancer: a cumulative meta-analysis. Aust NZ J Public Health 2001; 3: 203-11. 34. Spitzer WO, Lawrence V, Dales R et al. Links between passive smoking and disease: a best-evidence synthesis. A report of the Working Group on Passive Smoking. Clin Invest Med 1990; 1: 17-42. 35. Misra DP, Nguyen RH. Environmental tobacco smoke and low birth weight: a hazard in the workplace? Environ Health Perspect 1999; 107 Suppl. 6: 897-904. 36. Cook DG, Strachan DP. Summary of effects of parental smoking on the respiratory health of children and implications for research. Thorax 1999; 54: 357-66. 37. Cook DG, Strachan DP. Summary of effects of parental smoking on the respiratory health of children and implications for research. Thorax 1999; 54: 357-66. 38. Boffetta P, Tredaniel J, Greco A. Risk of childhood cancer and adult lung cancer after childhood exposure to passive smoke: a meta-analysis. Environ Health Perspect 2000; 108: 73-82. 39. Austoker J. Melanoma: prevention and early diagnosis. BMJ 1994; 308: 1682-6.

455

40. Armstrong BK, Kricker A. The epidemiology of UV induced skin cancer. / Photochem Photobiol B 2001; 63: 8-18. 41. Green A. Squamous cell carcinoma of the skin: nonmetastatic. Does the use of sunscreen help to prevent cutateous squamous cell carcinoma? Clinical Evidence, Issue 5. Oxford: Update Software, 2001. 42. DTB. Topical sunscreens. Drug Ther Bull 1990; 28: 61-2. 43. MeReC. Treatment and prevention of osteoporosis. MeReC Bulletin 1994; 5: 9-12. 44. National Service Framework (NSF)for older people. London: Department of Health, March 2001. Online. Available: www.doh.gov.uk/nsf/olderpeople.htm 45. MeReC. Common issues in osteoporosis. MeReC Bulletin 2001; 12: 5-8. 46. American Heart Association. 2999 heart and stroke statistical update. Dallas: American Heart Association, 1999. 47. Wilber JA, Barrow JG. Hypertension: community problem. Am J Med 1972; 52: 653-63. 48. Smith WCS, Lee AJ, Cromby IK et al. Control of blood pressure in Scotland: the rule of halves. BMJ 1990; 300: 981-3. 49. Cranney M, Barton S, Walley T. The management of hypertension in the elderly by GPs in Mersey side: the rule of halves revisited. Br J Gen Pract 1998; 48:1146-50. 50. Collins R et al. Blood pressure, stroke and coronary artery disease. Part 2: short-term reductions in blood pressure: overview of randomized drug trials in their epidemiological context. Lancet 1990; 335: 827-38. 51. Simon JA. Treating hypertension: the evidence from clinical trials. BMJ 1996; 313: 437-8. 52. Law MR et al. By how much and how quickly does reduction in serum cholesterol concentration lower the risk of ischaemic heart disease? BMJ 1994; 308: 367-72. 53. Department of Health. The National Service Framework for Coronary Heart Disease. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/nsf/ coronary.htm 54. Neil HAW et al. Extent of underdiagnosis of familial hypercholesterolaemia in routine practice: prospective registry study. BMJ 2000; 321: 148. 55. Colorectal cancer screening in the UK: joint position statement of the British Society of Gastroenterology, The Royal College of Physicians, and the Association of Coloproctology of Great Britain and Ireland. Gut 2000; 46: 746-8. 56. DoH. Referral guidelines for suspected cancer. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/cancer 57. British Association of Surgical Oncology. Guidelines. Eur J Surg Oncol 1998; 24: 464-76. 58. Interim advice to GPs on familial breast cancer. London: Department of Health, 2000. Online. Available: www.doh.gov.uk/publications/ 59. Austoker J. Screening and self-examination for breast cancer. BMJ 1994; 309: 168-74. 60. Blanks RG, Moss SM et al. Effect of NHS breast screening programme on mortality from breast cancer in England and Wales, 1990-8: comparison of observed with predicted mortality. BMJ 2000; 321: 665-9.

456

HEALTH PROMOTION AND SCREENING

61. Day NE. Screening for breast cancer. Br Med Bull 1991; 47 (2): 400-15. 62. Clinical Outcomes Group. Improving outcomes in breast cancer: guidance for general practitioners and primary care teams. London: DoH, 1998. 63. IARC Working Party Group on evaluation of cervical cancer screening programmes. Screening for squamous cell cancer: duration of low risk after negative results of cervical cytology and its implications for screening policies. BMJ 1986; 293: 659-64. 64. Austoker J. Screening for cervical cancer. BMJ 1994; 309: 241-8. 65. Royal College of Obstetricians and Gynaecologists. Report on the Intercollegiate Working Party on Cytology Screening. London: RCOG, 1987. 66. NHS Centre for Reviews and Dissemination. Screening for Prostate Cancer. Effectiveness Matters 1997; 2 (2). 67. The NHS Prostate Cancer campaign. Online. Available: www.doh.gov.uk/cancer/prostate.htm 68. Scottish Intercollegiate Guidelines Network (SIGN). Online. Available: http://www.sign.ac.uk/signweb/ guidelines/published/index.html#diabetes

69. Diabetes National Service Framework. London: Department of Health. Online. Available: www.doh. gov.uk / nsf / diabetes / index .htm 70. Wormald R, Fraser S, Bunce C. Time to look again at sight tests. BMJ 1997; 314: 246. 71. General household survey: analysis of ophthalmic data 1990-91 to 1993-94. London: Government Statistical Service, 1995. 72. Crick RP, Tuck MW. How can we improve the detection of glaucoma? BMJ 1995; 310: 546-7. 73. Tielsch JM. The epidemiology of primary open angle glaucoma. Ophthalmol Clin N Am 1991; 4: 649-657. 74. Wormald RPL, Basauri E, Wright LA, Evans J. The African Caribbean eye survey: risk factors for glaucoma in a sample of African Caribbean people living in London. Eye 1994; 8: 315-20. 75. Royal College of Physicians and The Bone and Tooth Society of Great Britain. Osteoporosis: clinical guidelines for prevention and treatment. Update on pharmacological interventions and an algorithm for management. London: RCP, 2000. Online. Available: www.rcplondon.ac.uk

Appendices

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APPENDIX 1,2 459

APPENDIX 1 ROUTINE SCHEDULE OF IMMUNIZATIONS Age

Immunization

2 months 3 months 4 months 12-15 months 3-5 years 10-14 years 15-19 years 20-24 years 65 onwards

DTwP DTwP DTwP MMR DTaP BCG (if tuberculin negative) Td

Hib Hib Hib Men C if not already given MMR Men C if not already given MMR if not already given

polio polio polio

Men C Men C Men C

polio polio

Men C if not already given Men C if not already given

flu annually

aP, acellular pertussis; D, diphtheria; d, low dose diphtheria; flu, influenza; Hib, H. influenzae b; Men C, meningococcal C conjugate vaccine; MMR, mumps, measles and rubella; T, tetanus; wP, wholecell pertussis. Note: 3 doses of Meningococcal C conjugate vaccine should be given to anyone age <25 not already immunized with the conjugate vaccine or immunized with the polysaccharide vaccine over 3 years ago.

APPENDIX 2 INCUBATION PERIOD AND INFECTIVITY OF COMMON DISEASES Disease

Incubation period

Period of infectivity/ exclusion from school

Bacillary dysentery Scarlet fever

1-7 days 1-8 days

Chickenpox Malaria

Measles Mumps

7-21 days 10-14 days (may be longer, especially if the patient is taking prophylactic drugs) 7-14 days 12-21 days

While present in stools 10-21 days after the onset of the rash; 1 day after penicillin started 5 days before to 6 days after the last crop of spots appeared

Whooping cough

7-10 days

Hepatitis A

2-6 weeks

Hepatitis B Rabies

6 weeks to 6 months or longer 9 days-9 weeks (possibly up to 2 years) 14 days-21 days 4-21 days 2-10 days 4—12 weeks

Rubella Tetanus Meningococcal infection Tuberculosis Impetigo Ringworm Scabies Conjunctivitis

From prodromal symptoms to 4 days after the onset of the rash 3 days before to 7 days after the start of the swelling (provided the swelling has gone) 7 days after exposure to 3 weeks after the onset of typical cough (shortened to 7 days with antibiotics) From late in the incubation period until 10 days after the appearance of jaundice From onset to appearance of anti-HBe

1 week before the rash appears to 1 week after Not contagious Until 48 hours after starting treatment Until 2 weeks after starting treatment Until effectively treated Until effectively treated Until effectively treated Until effectively treated

460 APPENDICES

APPENDIX 3 A SUGGESTED SCHEDULE OF IMMUNIZATION FOR TRAVELLERS Week

Normal schedule

Rapid schedule

0

Typhoid, tetanus, poli(3, (diphtheria, rabies, Japanese encephalitis)

1

(Rabies, Japanese encephalitis)

Typhoid, tetanus, polio, hepatitis A (diphtheria, yellow fever, rabies, Japanese encephalitis) (Rabies, Japanese encephalitis) Tetanus, (diphtheria), polio,

2 3 4

(Yellow fever) (Rabies, Japanese encephalitis)

6

Typhoid, tetanus, (diphtheria), polio, hepatitis A Tetanus, polio, (diphtheria)

10

Polio, tetanus, typhoid, (diphtheria, rabies, Japanese encephalitis)

Italic type indicates that immunization is unnecessary if a primary course has been given previously. Entries in brackets indicates those immunizations that are needed only in special situations. Meningococcal vaccine can be given at any stage. Diphtheria can be added to a tetanus immunization as the combined dose. For hepatitis A and B, polio, diphtheria, meningococcal infection and BCG, see Chapter 2.

APPENDIX 5

APPENDIX 4 NOTIFICATION OF INFECTIOUS DISEASES The notification of the following diseases is required by law in England and Wales, and the doctor is not excused from notification by considerations of confidentiality. Acute encephalitis* Acute meningitis Acute poliomyelitis Anthrax Cholera Diphtheria Dysentery (amoebic or bacillary) Food poisoning Suspected food poisoning Leprosy*1' Leptospirosis+ Malaria* Measles Meningococcal septicaemia Mumps Ophthalmia neonatorum*f Paratyphoid fever

Plague Rabies Relapsing fever Rubella Scarlet fever Smallpox Tetanus* Tuberculosis Typhoid fever Typhus Viral haemorrhagic fever Viral hepatitis Whooping cough Yellow fever

*Not notifiable in Scotland, where chicken pox, continued fever, erysipelas, Legionella infection, membranous croup, meningococcal infection and puerperal fever are notifiable. f Not notifiable in Northern Ireland.

CHILD HEALTH PROMOTION

(Adapted from annex to DOH letter PL/CMO(92)3, PL/CNO(92)3.) History and examination

Health education

Neonatal examination (a) Elicit and consider concerns expressed by the parents. (b) Review family history, pregnancy and birth. (c) Assess risk of hearing defect and refer accordingly. (d) Full physical examination, including weight and head circumference. (e) Check for CDH and testicular descent. (f) Inspect eyes, check red reflex. (g) Check PKU and thyroid tests have been done, (h) Screen for haemoglobinopathy, if relevant. First 2 weeks In addition to the neonatal examination: (a) Assess the level of support and assistance that each new parent is likely to require.

6-8 weeks (a) Check history, review growth and development and ask about parental concerns.

(a) Feeding and nutrition. (b) Sleeping position. (c) Baby care. (d) Sibling management. (e) Crying and sleep problems. (f) Transport in a car.

(a) (b) (c) (d)

Nutrition. The effects of passive smoking. Accident prevention - bathing scalding by feeds, fires. Immunization.

(a) Immunization. (b) Nutrition.

APPENDIX 6 461

History and examination

Health education

(b) Physical examination, weight, head circumference (+ and length, if indicated). (c) Check for CDH. (d) Enquire about concerns regarding vision, squint and hearing. (e) Check whether the baby is in the high-risk category for hearing loss and refer if necessary. (f) Discuss and perform immunizations. 2, 3 and 4 months (a) Primary immunizations. 6-9 months (a) Enquire about parental concerns regarding health and development, vision and hearing. (b) Look for evidence of CDH. (c) Check for testicular descent. (d) Observe visual behaviour and look for squint. (e) Distraction test for hearing (HV).

(c) Dangers of fires, falls, overheating and scalds. (d) Recognition of illness and what to do.

18-24 months (a) Enquire about parental concerns particularly regarding behaviour, vision and hearing. (b) Confirm that the child is walking with a normal gait, and that speech and comprehension are appropriate for age. (c) Arrange detailed vision, hearing or language assessment if indicated. (d) Remember the prevalence of iron deficiency anaemia. (e) Measure height. Note: Inform the community paediatric services if there is any anxiety about a child's educational potential.

(a) Accident prevention - choking, scalds and burns (including sunburn), falls. (b) Anticipate increased mobility (e.g. safety gates, guards etc.). (c) Nutrition. (d) Dental prophylaxis. (e) Reinforce advice about safety in cars and passive smoking. (f) Developmental needs. (a) Accident prevention - falls from heights, drowning, poisoning, road safety. (b) Nutrition. (c) Developmental needs - language and play. (d) Need to mix with other children - playgroup etc. (e) Avoidance and management of behaviour problems.

36-54 months (a) Enquiry and discussion about vision, squint, hearing and behaviour, language acquisition and development. (b) Discuss, if appropriate, whether the child is likely to have special educational problems and refer as appropriate. (c) Measure the height and chart it. (d) Refer for hearing test if concerned.

APPENDIX 6

(a) (b) (c) (d)

Accidents - fires, roads, drowning. Road safety. Preparation for school. Nutrition and dental care.

INJURY PREVENTION STRATEGIES

(ref. Hall, DM Health for All Children. OUP, 1996. By permission of Oxford University Press.) Injury type

Implications

MVA"- passenger

Children should travel in car seats or restraints. Legislation combined with programmes to promote car safety by loan schemes results in increased use, but many children still travel without any restraints. Needs: loan schemes, advertising campaigns and personal education Educating young children (1-5 age group) is unlikely to be effective. Parent education may play a small role; some parents overestimate a young child's capacity for road sense. Needs: Traffic-calming schemes, traffic-free areas, safe play areas, safer journeys to school (Note: parents often take their children to school prompted by fear of abduction as well as anxiety about traffic)

MVA - pedestrian

(continued)

462 APPENDICES

Bicycle injuries, horse-riding accidents

Fire and flame

Drowning

Suffocation* and strangulation Poisoning

Falls

Glass injuries (doors, windows etc.) Dog attacks Farm accidents Sunburn and heat stroke

Not common in under 5s. Cycle helmet use reduces injury severity. Possibility of rear-pedalling brake for under 5s. Needs: legislation combined with education. Few data on horse-riding, but importance of safe helmets is recognized Burns: house fires are an important cause. Various causes: smoking, paraffin stoves, electrical and battery fires. Social class gradient - higher risk in inner cities, with multiple occupancy, etc. Smoke alarms may reduce morbidity and mortality. If issued free by health authority, need to consider who should receive them and how they are fitted and maintained (battery replacement). Other measures, e.g. flame-proofing of nightdresses. Scalds: coiled flexes on kettles, reducing thermostat setting on hot water tanks. Needs: supply and fitting of smoke alarms (consider as joint responsibility with housing departments). Check hot water temperature Three main hazards: baths, ponds and swimming pools. Public swimming bath accidents are uncommon. Needs: parent education about bath dangers*, ponds to be covered, fenced, or drained; swimming pools should be fenced. Swimming lessons are unlikely to prevent drowning accidents in the preschool age group Examples include inhalation of peanuts, sweets etc., plastic bags over the head, being trapped in disused refrigerators, playing games involving 'hanging' etc. Child-resistant packaging for drugs and medicines has reduced deaths and admissions from poisonings. Still a hazard - some children learn to open 'child-proof containers. Domestic materials dangers - bleach, paraffin, detergent, paint stripper etc. Needs: parent education, continuing improvements in packaging, reminders to parents about potentially lethal toxicity of products that might be thought relatively harmless (e.g. dishwasher powder, iron tablets, aspirin, children's chemistry sets) Falls from high-rise flats (windows and balconies) are potentially fatal. Prevention programmes involve window catches, bars, or guards, making balconies safe, and providing other safer play areas. Playground injuries risk reduction by use of safer materials and good design. Needs: collaboration with local authority-housing department and parks/playgrounds to initiate actions; health visitors need access to carpenter/locksmith/fitter Preventable by use of safety glass, membranes etc., and by good design at planning stage. Little evidence regarding interventions. Needs: parent education about hazards; proper training of dogs; educating children about dog attacks. Little research available in health sector Farm environment is hazardous for young children: animals, machinery, muck heaps, etc. Severe sunburn in early childhood is a risk factor for skin cancer. 'Heat stroke' can occur if children are left in cars in full sun. Needs: parent education

MVA, motor vehicle accident. * Drowning in baths (especially over the age of 2) and suffocation without clear explanation should be viewed with some suspicion - it may not be accidental.

APPENDIX 7

STAGES OF CHILD DEVELOPMENT

Summary of development: birth to 16 weeks

Social Motor Ventral suspension

0-4 Weeks

6-8 Weeks

12-26 Weeks

Watches mother and may smile

Responsive smile by 6 weeks and vocalizes

Recognizes family, shows pleasure

Head hangs down until 3-4 weeks then up momentarily

Head held in horizontal plane, and briefly up by 8 weeks

Head maintained well above plane of body by 12 weeks

APPENDIX 7 463

0-4 Weeks

6-8 Weeks

22-26 Weeks

Prone

Head to side, pelvis high, knees drawn up under abdomen

Head and shoulders up, and chest by 16 weeks Weight on forearms

Supine

Head to side, limbs flexed or ATNR posture Complete head lag Very round back Head drops forward Walking and placing reflexes present until 6 weeks Strong grasp reflex Hands often closed

Chin up intermittently at 6 weeks, well up at 8 weeks Pelvis flat ATNR posture common but head to midline by 8 weeks Less head lag Back rounded Head briefly up Sags at hips and knees, getting head up by 8 weeks Grasp reflex present but slight by 8 weeks. Fingers extend more often Smoother conjugate eye movements. Fixates on face/objects and follows through 45°-90° Eyes turn to sounds Starts vocalizing

Pull-to-sit Held sitting Held standing Hands

Vision

Vocalization / hearing

Blink and pupil reflexes Eye righting reflex Random movements but can fixate Cries, stills or startles to sounds

ATNR declining. Head and hands now to midline Slight head lag Back straighter and head up Increasingly bears weight on legs Grasp reflex fades between 12 and 16 weeks. Can hold rattle briefly Follows through 130°. Hand regard common (12-20 weeks) Varied coos, squeals and laughs. Turns to sounds

ATNR: asymmetric tonic neck reflex Summary of development: 4-10 months 4-6 Months

6-8 Months

8-20 Months

Personal and social behaviour

Responsive to all comers

Wary of strangers

Gross motor

Smiles at self in mirror Excited at approach of food No head lag in traction Rolls prone to supine Back straight in supported sitting

Discriminates between family and strangers Attracts attention Hand feeds biscuit Lifts head up in supine Rolls supine to prone /creeps Sits without lateral support Bears weight on feet (5-8 months) Transfers cube hand to hand (5-7 months). Can hold two cubes

Fine motor and vision

Reaches and grasps toys (4-6 months). Plays with toes Very alert visually

Language and hearing

Varied sounds and squeals. Consonants such as ba or da

Any squint reported after 6 months is abnormal Starts to babble da-da. Turns to sounds (4-8 months)

Waves bye-bye Attempts to use spoon Sits steadily, pivots and leans Can get from prone to sitting Pulls self to stand and crawls

Pincer grasp of pellet (8-12 months) Releases object and looks for it (7-11 months) Points at 1 mm sweet Varied babble ma-ma, ba-ba, da-da. Indicates and understands 'No' Locates sounds well

Summary of development: 12-24 months

Personal and social behaviour

12-15 Months

18-24 Months

Shows affection and may be shy Indicates wants, points, claps hands (10-18 months) Mouthing stops (12-15 months) Enjoys casting (12-15 months)

Becoming egocentric, clinging and resistant Loves domestic mimicry Definitely stopped mouthing and casting (by 18 months) Helps undress

464 APPENDICES

Gross motor Fine motor and vision

Language and hearing

22-15 Months

18-24 Months

May manage cup and spoon with spills (10-17 months) Walks holding on (8-12 months) Walks alone (11-15 months) Fine pincer grasp. Bangs bricks together (8-14 months) Holds two cubes Scribbles (12-18 months) Mama, Dada, with meaning (9-15 months) Three to four clear words (12-18 months)

Independent with cup and spoon (15-24 months) Walks well (12-18 months) Climbs stairs, kneels (14-22 months) May show hand preference (after 15 months) Builds 2 to 3 cubes. Turns pages (15-24 months) Can point to 3 parts of body, has 6 to 20 words and jargon (15-24 months)

Summary of development: 2-5 years 2-2/4 years Personal and social skills Enjoys solitary play, alongside peers, not sharing Possessive: tantrums if thwarted Feeds quite neatly, using spoon and fork or fingers. May be clean and dry by day, with supervision, or may refuse to cooperate

3-4 years

5 years old

Plays with peers, sharing toys. Enjoys make-believe play. Shows concern and sympathy for others, and able to take turns by 4 years Easily manages spoon and fork and then knife. Mostly dry day and night. Can wash hands, dress and undress by 4 years, except fastenings

Plays complicated cooperative games. Makes friends. Comforts playmates and siblings in distress Almost completely independent in self help-skills now. Can carry out simple domestic tasks and run errands

Gross motor

Now very mobile. Runs, kicks ball, tries to throw. Walks up and down stairs two feet to a step. Propels tricycle by pushing with feet on floor

Up stairs one foot per step at 3, and down by 4 years. Can walk, then run on tip-toe, and hop, by 4 years. Enjoys climbing, pedals a tricycle skilfully

Enjoys running, jumping, climbing, swings and slides, and starting to play ball games. Can stand on one leg, hop 10 times and heel-toe walk a narrow line.

Fine motor and vision

Neat prehension, and controlled release. Tower of 6-8 bricks. Holds pencil in fist. Circular scribble (24 months) copies vertical line, imitates circle, (30 months). Simple puzzles and can thread large beads

Tower of 9-10 and imitates 3 cube bridge at 3 years, steps or gate by 4 years. Awkward tripod grasp of pencil at 3 years - copies circle and imitates cross. Dynamic tripod after 4 years; draws man with head, trunk and legs Can do letter-matching vision tests, using linear charts, each eye separately, by 3M to 4 years

Can write name, copy a square and a triangle. Draws man with detailed features and limbs. Can fold paper and use scissors to cut out shapes

Intelligible but immature speech, 3-5 word sentences, knows name and sex, at 3 years. Long stories, constant more abstract questions, grammar mostly correct, and speech clear, by 4 years. Knows age and address, 6+ colours and can count to 4+ Can do cooperative (conditioned) hearing tests

Enjoys riddles and jokes. Understands negatives and complex questions and instructions. Gives long descriptions and explanations. Speech easily intelligible with few errors

Difficult age to test vision. Recognizes two-dimensional symbols and may match letters at 30 months Language and hearing

Listens to simple stories and understands two-part instructions. Can say 50-100 single words and join two to three: (Daddy gone car). Many questions - what? and who? Long monologues, still some jargon, enjoys nursery rhymes and jingles Toy tests of hearing or may point to named pictures

Performs Snellen chart type of vision test

Manages full audiometry and speech discrimination now

APPENDIX 8 465

APPENDIX 8

STAGES OF PUBERTY

(b) (a) The stages of male genital and pubic hair development. (b) The stages of breast and pubic hair development in a female, (a) Boys: genital development: Stage 1: Preadolescent: testes, scrotum and penis are of about the same size and proportion as in early childhood. Stage 2: Enlargement of scrotum and testes. Skin of scrotum reddens and changes in texture. Little or no enlargement of penis at this stage. Stage 3: Enlargement of penis, which occurs at first mainly in length. Further growth of testes and scrotum. Stage 4: Increased size of penis with growth in breadth and development of glans. Testes and scrotum larger; scrotal skin darkened. Stage 5: Genitalia adult in size and shape. (The volume of the adult testis varies in size from 12 to 25ml). (b) Girls: breast development: Stage 1: Preadolescent: elevation in papilla only. Stage 2: Breast bud stage: elevation of breast and papilla as a small mound. Enlargement of areolar diameter. Stage 3: Further enlargement and elevation of breast and areola, with no separation of their contours. Stage 4: Projection of areola and papilla to form a secondary mound above the level of the breast. Stage 5: Mature stage; projection of papilla only, due to

recession of the areola to the general contour of the breast. (This last stage may not be reached in women until after their first pregnancy.) (a,b) Both sexes: pubic hair: Stage 1: Preadolescent. The vellus over the pubes is not further developed than that over the abdominal wall, i.e. no pubic hair. Stage 2: Sparse growth of long, slightly pigmented downy hair, straight or slightly curled, chiefly at the base of the penis or along the labia. Stage 3: Considerably darker, coarser and more curled. The hair spreads sparsely over the junction of the pubes. Stage 4: Hair now adult in type, but area covered is still considerably smaller than in the adult. No spread to the medial surface of the thighs. Stage 5: Adult in quantity and type with distribution of the horizontal (or classically feminine) pattern. Spread to medial surface of thighs but not up linea alba or elsewhere above the base of the inverse triangle (spread up linea alba occurs later and is rated Stage 6). Both sexes: axillary hair: Stage 1: Preadolescent. No axillary hair. Stage 2: Scanty growth of slightly pigmented hair. Stage 3: Hair adult in quality and quantity.

466 APPENDICES

APPENDIX 9 PREDICTED NORMAL PEAK FLOW VALUES IN CHILDREN (UNDER 15 YEARS OF AGE) Height (cm)

(ft in)

Peakflow (1/min)

91 99 107 114 122 130 137 145 152 160 168 175

3-0 3-3 3-6 3-9 4-0 4-3 4-6 4-9 5-0 5-3 5-6 5-9

100 120 140 170 210 250 285 325 360 400 440 480

APPENDIX 10

APPENDIX 10

467

PEAK EXPIRATORY FLOW IN NORMAL SUBJECTS

Source: Gregg, I. and Nunn, A. J. (1973) Peak expiratory flow in normal subjects. British Medical Journal, 3, 282. Reproduced by kind permission of the British Medical Journal.

468 APPENDICES

APPENDIX

11

FEVi/FVC CHARTS Girls

Females

Age N (yeairs; 25 30 35 40 45 50 55 60 65 70

Height (m)

Height

1.45

1.50

1.55

1.60

1.65 1.70 1.75

1.80

metres

inches

FVC FEVi FVC FEVi FVC FEVi FVC FEV! FVC FEVi

2.5 2.9 2.4 2.8 2.3 2.6 2.1 2.5 2.0 2.4 1.9

2.7 3.1 2.6 3.0 2.5 2.9 2.3 2.7 2.2 2.6 2.1

2.9 3.3 2.8 3.2 2.7 3.1 2.6 2.9 2.4 2.8 2.3

3.1 3.6 3.0 3.4 2.9 3.3 2.8 3.2 2.6 3.0 2.5

3.4 3.8 3.2 3.6 3.1 3.5 3.0 3.4 2.9 3.3 2.7

3.6 4.0 3.4 3.9 3.3 3.7 3.2 3.6 3.1 3.5 2.9

3.8 4.2 3.7 4.1 3.5 4.0 3.4 3.8 3.3 3.7 3.2

4.0 4.4 3.9 4.3 3.7 4.2 3.6 4.0 3.5 3.9 3.4

0.80 0.90 1.00 1.10 1.20 1.30 1.40 1.50 1.60 1.70 1.80

32 35 39 43 47 51 55 59 63 67 71

CVP r vv^.

99 Z.Z

9R Z.O

97 Z./

9Q Z.7

•3 1 J.I

•3 0 J.J

0 f. J.O

Q Q J.O

FEVi

1.8 2.1 1.6 2.0 1.5 1.8 1.4 1.7

2.0 2.3 1.8 2.2 1.7 2.1 1.6 1.9

2.2 2.6 2.1 2.4 1.9 2.3 1.8 2.2

2.4 2.8 2.3 2.6 2.1 2.5 2.0 2.4

2.6 3.0 2.5 2.9 2.4 2.7 2.2 2.6

2.8 3.2 2.7 3.1 2.6 3.0 2.4 2.8

3.0 3.4 2.9 3.3 2.8 3.2 2.7 3.0

3.2 3.7 3.1 3.5 3.0 3.4 2.9 3.3

FEVJ

FVC FEVi FVC FEVi FVC FEVi FVC

25 30 35 40 45 50 55 60 65 70

FEV! FVC FEV! FVC FEVi FVC FEVi FVC FEVi FVC FEV! FVC FEVj FVC FEVj FVC FEVj FVC FEVj FVC

FVC

0.41 0.56 0.75 0.98 1.24 1.55 1.90 2.29 2.73 3.23 3.77

0.46 0.64 0.86 1.11 1.43 1.79 2.20 2.68 3.22 3.82 4.48

FEVi

FVC

0.40 0.56 0.76 1.00 1.28 1.61 2.00 2.43 2.93 3.49 4.11

0.46 0.65 0.89 1.17 1.52 1.92 2.38 2.92 3.53 4.22 4.99

Boys

Males

Age (years)

FEV!

Height (m)

Height

1.55

1.60

1.65

1.70

1.75

1.80 1.85 1.90

3.4 3.9 3.3 3.8 3.1 3.7 3.0 3.6 2.8 3.4 2.7 3.3 2.5 3.2 2.4 3.0 2.2 2.9 2.1 2.8

3.6 4.2 3.5 4.1 3.3 4.0 3.2 3.8 3.0 3.7 2.9 3.6 2.8 3.5 2.6 3.3 2.5 3.2 2.3 3.1

3.8 4.5 3.7 4.4 3.6 4.3 3.4 4.1 3.3 4.0 3.1 3.9 3.0 3.7 2.8 3.6 2.7 3.5 2.5 3.3

4.1 4.8 3.9 4.7 3.8 4.5 3.6 4.4 3.5 4.3 3.3 4.2 3.2 4.0 3.1 3.9 2.9 3.8 2.8 3.6

4.3 5.1 4.2 5.0 4.0 4.8 3.9 4.7 3.7 4.6 3.6 4.4 3.4 4.3 3.3 4.2 3.1 4.1 3.0 3.9

4.5 5.4 4.4 5.3 4.2 5.1 4.1 5.0 3.9 4.9 3.8 4.7 3.7 4.6 3.5 4.5 3.4 4.3 3.2 4.2

4.7 5.7 4.6 5.5 4.5 5.4 4.3 5.3 4.2 5.2 4.0 5.0 3.9 4.9 3.7 4.8 3.6 4.6 3.4 4.4

5.0 6.0 4.8 5.8 4.7 5.7 4.5 5.6 4.4 5.4 4.2 5.3 4.1 5.2 4.0 5.0 3.8 4.9 3.7 4.8

metres

inches

0.80 0.90 1.00 1.10 1.20 1.30 1.40 1.50 1.60 1.70 1.80

32 35 39 43 47 51 55 59 63 67 71

APPENDIX 12A 469

APPENDIX 12A MANAGEMENT OF CHRONIC ASTHMA IN SCHOOLCHILDREN AND YOUNG ADULTS • Starting treatment. Patients should start treatment at the step most appropriate to the initial severity. The aim is to achieve early control of the condition and then to reduce treatment (stepping down). Starting at a moderately high level of inhaled steroid or with a rescue course of prednisolone, will allow rapid control. • Inhaled steroids: (a) School children Until growth is complete, any child requiring more than 400 jxg of beclomethasone or budesonide, or 200 (xg of fluticasone daily, should be referred to a paediatrician with an interest in asthma. (b) Inhaled steroids at high doses (at or above 1000 (jig of beclomethasone or budesonide, or 500 jxg of fluticasone) should be inhaled using a large volume spacing device (see below). (c) The dose delivered to the lung varies considerably between devices, e.g. the Turbohaler delivers about twice the dose as other dry powder inhalers. (d) Osteoporosis. Inhaled steroids at high doses (at or above 1000 jxg of beclomethasone or budesonide, or 500 |xg of fluticasone) and patients on oral steroids should be advised on general measures to reduce osteoporosis and those on >7.5mg prednisolone daily, should be started on prophylaxis.

• Stepping up. Before stepping up: (a) ensure that the patient is taking the treatment; (b) the inhaler and use of a large volume spacer is appropriate; (c) the inhaler technique is good. • Stepping down: (a) Review treatment every 3-6 months. If control is achieved then a stepwise reduction in treatment may be possible. (b) In patients recently started at step 4 or 5, or who have included steroid tablets to gain control, this reduction can take place after a short interval. (c) In patients with chronic severe asthma a period or 3-6 months stability should be shown before reduction is undertaken. • 'Rescue' courses of steroids. Prednisolone 30-40 mg a day (60 mg in those already on steroids) should be given until the patient has improved. In patients not already on steroids, courses of 3 weeks can be stopped suddenly or tailed down quickly.

Step 1 Occasional use of relief bronchodilators

Step 2 Regular low-dose inhaled steroid

Step 3 Low-dose inhaled steroids plus long-acting beta agonist bronchodilators

Step 4 High-dose inhaled steroids

Step 5 Regular oral steroids or steroid sparing agents

Inhaled short-acting beta agonist as required

Inhaled short-acting beta agonist as required plus beclomethasone or budesonide 200-800 |ig daily or fluticasone 50^100 (jig daily

Inhaled short-acting beta agonist as required plus an inhaled long-acting beta agonist

Inhaled short-acting beta agonist as required plus beclomethasone or budesonide increased to 800-2000 (jig daily or fluticasone 500-1000 (jig

Inhaled short-acting beta agonist as required plus beclomethasone or budesonide maintained at 2000 jxg daily or fluticasone 1000 fxg daily via a large volume spacer

daily via a large volume spacer

plus if necessary beclomethasone or budesonide increased to 800 |xg daily or fluticasone 500 (jug daily

plus an inhaled long-acting beta agonist (if effective)

plus an inhaled long-acting beta agonist (if effective)

plus a sequential therapeutic trial of one or more of: (a) leukotriene receptor antagonist; (b) a sustained-release theophylline (c) beta agonist tablet

plus regular prednisolone tablets in a single daily dose and /or steroid sparing agents

via a large volume spacer

If there is insufficient benefit from the above, consider trials of: (a) leukotriene receptor antagonist; (b) a sustained-release theophylline (c) beta agonist tablet

Outcome of steps 1-3: asthma is controlled if there are: no chronic symptoms; infrequent exacerbations; minimal need for relieving bronchodilators; no limitations on activities including exercise; peak flow variation over the day of <20%; PEF ^ 80% of predicted or best; minimal or no adverse effects of medicines.

Outcome of steps 4-5: asthma is controlled if there are the: least possible symptoms; least possible need for relieving bronchodilators; least possible limitation of activity; least possible variation in PEF; best PEF; least adverse effects from the medicine.

Based on The British Guidelines on Asthma Management: 1995 Review and Position Statement. Thorax 1997; 52 (Suppl. 1): S9-S10, with permission of the BMJ Publishing Group, and modified in the light of advice from the Scottish Intercollegiate Guidelines Network, 2002.

APPENDIX 12B

MANAGEMENT OF ASTHMA IN CHILDREN UNDER 5

• Starting treatment: The child should start treatment at the step most appropriate to the initial severity. Starting at a moderately high level of inhaled steroid will allow rapid control but regular review is necessary and the dose brought down after symptoms improve. • Stepping up: Before stepping up: (a) ensure that the patient is taking the treatment; (b) the inhaler is appropriate; (c) the inhaler technique is good. • Stepping down: If symptoms are controlled consider the possibility of decreasing the inhaled steroid dose. Review after 1-2 months. • 'Rescue' course of steroids: Prednisolone (or soluble prednesol) l-2mg/kg under 1 year, 1-5 years 20 mg, daily for 5 days. • Inhaled steroid dose: The higher steroid doses carry a risk of growth retardation and are outside the manufacturers' recommendations. The advice of a paediatrician is essential if a child advances to step 4 and is recommended for a child on step 3 for more than 3 weeks Stepl

Step 2

Step3

Step 4

Occasional use of bronchodilators

Regular inhaled preventer therapy

Increased dose of inhaled steroids

High-dose inhaled steroids and bronchodilator

Beta agonist: use short-acting beta agonists for symptom relief, no more than once a day.

Beta agonist: use inhaled short-acting beta agonists as required

Beta agonist: use inhaled short-acting beta agonists as required

Inhaled steroids (via a large volume spacer): beclomethasone or budesonide increased to 2000 (Jig or fluticasone to 1000 (Jig daily

plus

plus

plus

cromoglycate: (a) as powder 20 mg 3-4 times a day; or (b) via metered dose inhaler and large volume spacer - 10 mg t.d.s.

inhaled steroid (via a large volume spacer): beclomethasone or budesonide increased to 800 (jug or fluticasone to 500 (jug daily

other treatment as in step 3.

or

Consider a short prednisolone course.

inhaled steroid: beclomethasone or budesonide 100-400 |xg or fluticasone 50-200 |jig daily * To gain rapid control. Consider a 5-day course of soluble prednisolone or doubling the dose of inhaled steroids.

Consider adding regular inhaled long-acting beta agonist or oral slow-release xanthine.

• Cromoglycate is safe and helpful in many children and is still recommended as an optional first line preventative treatment. A trial of 4-6 weeks is necessary. • Inhaled steroids should be started at a dosage appropriate to the child's age, size and the severity of asthma.

• Inhaled steroids. See the • Inhaled long-acting beta agonists advice about referral to produce bronchodilatation in children a paediatrician above. for up to 12 hours and inhibit exercise induced bronchoconstriction. They should only be given to children aged 4 or more, receiving preventer therapy. • Sustained-release xanthines produce effective bronchodilatation but a number of side-effects. They may be of particular value in night time symptoms. Monitoring of serum or salivary concentrations is recommended.

• Use inhaled drugs wherever possible. Mildest cases may respond to oral beta agonists. However, bronchodilator syrups are much less effective than inhaled therapy and have more systemic side-effects.

Based on The British Guidelines on Asthma Management: 1995 Review and Position Statement. Thorax 1997; 52 (Suppl. 1): S9-S10, with permission of the BMJ Publishing Group.

472 APPENDICES

APPENDIX 13

PILL LADDERS

Ladder A: C, Cilest Ladder B: F, Femodene; M, Minulet; T, Triadene; T-M, Tri-Minulet; Ft, Femodette Ladder C: Ma, Marvelon; Me, Mercilon Ladder D: Ov, Ovran; O30, Ovran 30; E30, Eugynon 30; Oe, Ovranette; M30, Microgynon 30; T, Trinordiol; L, Logynon; Ne, Neogest; M, Microval; No, Norgeston Ladder E: N-l, Norinyl 1; No, Norimin; L30, Loestrin 30; Bi, BiNovum; T, TriNovum; S, Synphase; Br, Brevinor; O, Ovysmen; L20, Loestrin 20; Nr, Noriday; M, Micronor; F, Femulen Ladder F: Y, Yasmin Adapted from Your questions answered, Guilleband J, 1999, by permission of the publisher Churchill Livingstone.

APPENDIX 14 January October February November March December April January May February June March July April August May September June October July November August December September

1 8 1 8 1 6 1 6 1 5 1 8 1 7 1 8 1 8 1 8 1 8 1 7

2 9 2 9 2 7 2 7 2 6 2 9 2 8 2 9 2 9 2 9 2 9 2 8

3 10 3 10 3 8 3 8 3 7 3 10 3 9 3 10 3 10 3 10 3 10 3 9

OBSTETRICAL TABLE 4 11 4 11 4 9 4 9 4 8 4 11 4 10 4 11 4 11 4 11 4 11 4 10

5 12 5 12 5 10 5 10 5 9 5 12 5 11 5 12 5 12 5 12 5 12 5 11

6 13 6 13 6 11 6 11 6 10 6 13 6 12 6 13 6 13 6 13 6 13 6 12

7 14 7 14 7 12 7 12 7 11 7 14 7 13 7 14 7 14 7 14 7 14 7 13

8 15 8 15 8 13 8 13 8 12 8 15 8 14 8 15 8 15 8 15 8 15 8 14

9 16 9 16 9 14 9 14 9 13 9 16 9 15 9 16 9 16 9 16 9 16 9 15

10 17 10 17 10 15 10 15 10 14 10 17 10 16 10 17 10 17 10 17 10 17 10 16

11 18 11 18 11 16 11 16 11 15 11 18 11 17 11 18 11 18 11 18 11 18 11 17

12 19 12 19 12 17 12 17 12 16 12 19 12 18 12 19 12 19 12 19 12 19 12 18

13 20 13 20 13 18 13 18 13 17 13 20 13 19 13 20 13 20 13 20 13 20 13 19

14 21 14 21 14 19 14 19 14 18 14 21 14 20 14 21 14 21 14 21 14 21 14 20

15 22 15 22 15 20 15 20 15 19 15 22 15 21 15 22 15 22 15 22 15 22 15 21

16 23 16 23 16 21 16 21 16 20 16 23 16 22 16 23 16 23 16 23 16 23 16 22

17 24 17 24 17 22 17 22 17 21 17 24 17 23 17 24 17 24 17 24 17 24 17 23

18 25 18 25 18 23 18 23 18 22 18 25 18 24 18 25 18 25 18 25 18 25 18 24

19 26 19 26 19 24 19 24 19 23 19 26 19 25 19 26 19 26 19 26 19 26 19 25

20 27 20 27 20 25 20 25 20 24 20 27 20 26 20 27 20 27 20 27 20 27 20 26

21 28 21 28 21 26 21 26 21 25 21 28 21 27 21 28 21 28 21 28 21 28 21 27

22 29 22 29 22 27 22 27 22 26 22 29 22 28 22 29 22 29 22 29 22 29 22 28

23 30 23 30 23 28 23 28 23 27 23 30 23 29 23 30 23 30 23 30 23 30 23 29

24 31 24 1 24 29 24 29 24 28 24 31 24 30 24 31 24 1 24 31 24 31 24 30

25 1 25 2 25 30 25 30 25 1 25 1 25 1 25 1 25 2 25 1 25 1 25 1

26 2 26 3 26 31 26 31 26 2 26 2 26 2 26 2 26 3 26 2 26 2 26 2

27 3 27 4 27 1 27 1 27 3 27 3 27 3 27 3 27 4 27 3 27 3 27 3

28 4 28 5 28 2 28 2 28 4 28 4 28 4 28 4 28 5 28 4 28 4 28 4

29 5 29 3 29 3 29 5 29 5 29 5 29 5 29 6 29 5 29 5 29 5

30 6 30 4 30 4 30 6 30 6 30 6 30 6 30 7 30 6 30 6 30 6

31 7 31 5 31 7 31 7 31 7 -

January November February December March January April February May March June April July May August June September

- July 31 7 31 7

October August November September December October

474

APPENDICES

APPENDIX 15

EDINBURGH POSTNATAL DEPRESSION SCALE

(Reproduced by permission of the Royal College of Psychiatrists from Cox JL, Holden JM, Sagorsky R. Detection of postnatal depression: development of the 10-item Edinburgh Postnatal Depression Scale. Br J Psychiatry 1987; 150: 782-786.)

Instructions for users

Scoring the EPDS

1. The mother is asked to underline the response which comes closest to how she has been feeling in the previous 7 days. 2. All 10 items must be completed. 3. Care should be taken to avoid the possibility of the mother discussing her answers with others. 4. The mother should complete the scale herself, unless she has limited English or has difficulty with reading. 5. The EPDS may be used at 6-8 weeks to screen postnatal women. The child health clinic, postnatal check-up or a home visit may provide suitable opportunities for its completion.

Response categories are scored 0, 1, 2, and 3 according to increased severity of the symptom. Items marked with an asterisk are reverse scored (i.e. 3, 2, 1, and 0). The total score is calculated by adding together the scores for each of the 10 items. Mothers who score above a threshold 12/13 are likely to be suffering from a depressive illness of varying severity. Nevertheless the EPDS score should not override clinical judgement. A careful clinical assessment should be carried out to confirm the diagnosis. The scale indicates how the mother has felt during the previous week, and in doubtful cases it may be usefully repeated after 2 weeks. The scale will not detect mothers with anxiety neuroses, phobias or personality disorders.

As you have recently had a baby, we would like to know how you are feeling. Please UNDERLINE the answer which comes closest to how you have felt IN THE PAST 7 DAYS, not just how you feel today. Here is an example, already completed. I have felt happy: Yes, all the time Yes, most of the time No, not very often No, not at all This would mean: "I have felt happy most of the time" during the past week. Please complete the other questions in the same way. In the past 7 days: 1. I have been able to laugh and see the funny side of things: As much as I always could Not quite so much now Definitely not so much now Not at all 2. I have looked forward with enjoyment to things: As much as I ever did Rather less than I used to Definitely less than I used to Hardly at all

f

7* I have been so unhappy that I have had difficulty sleeping: when things went wrong: Yes, most of the time Yes, most of the time Yes, sometimes Yes, some of the time Not very often Not very often No, not at all No, never o * I have been anxious or worried for I have felt sad or miserable: Yes, most of the time no good reason: Yes, quite often No, not at all Not very often Hardly ever No, not at all Yes, sometimes I have been so unhappy that I have Yes, very often been crying: I hhave felt scared or panicky for no Yes, most of the time very good reason: Yes, quite often Yes, quite a lot Only occasionally Yes, sometimes No, never No, not much -^Q f The thought of harming myself No, not at all has occurred to me: h Things have been getting on top Yes, quite often Sometimes of me: Hardly ever Yes, most of the time I haven't Never been able to cope at all Yes, sometimes I haven't been coping as well as usual No, most of the time I have coped quite well No, I have been coping as well as ever

I have blamed myself unnecessarily

APPENDIX 16

APPENDIX 16

475

INTERNATIONAL PROSTATE SYMPTOM SCORE (IPSS) Not at all

Less than one time in five

Less than half the time

About half the time

More than half the time

Almost always

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

None

Once

Twice

Three times

Four times

Five times or more

7. NOCTURIA Over the past month, how many times did you typically get up to urinate from the time you went to bed at night to the time you got up in the morning?

0

1

2

3

4

5

Quality of life

Pleased

Mostly satisfied

No strong feelings either way

Mostly dissatisfied

Unhappy

Terrible

1

2

3

4

5

6

1. INCOMPLETE EMPTYING Over the past month, how often have you had a sensation of not emptying your bladder completely after you have finished urinating? 2. FREQUENCY Over the past month, how often have you had to urinate again less than 2 hours after you finished urinating? 3. INTERMITTENCY Over the past month, how often have you found you stopped and started again several times when you urinated? 4. URGENCY Over the past month, how often have you found it difficult to postpone urination or felt sudden urges to urinate? 5. WEAK STREAM Over the past month, how often have you had a weak urinary stream? 6. STRAINING Over the past month, how often have you had to push or strain to begin urination?

Delighted

If you were to spend the rest of 0 your life with urinary conditions just the way they are now, how would you feel about it?

476

APPENDICES

APPENDIX 17

BODY MASS INDEX

APPENDIX 18

APPENDIX 18

477

BLOOD DATA

Normal values

Immunoglobulins:

Serum or plasma

Acid phosphatase: total prostatic ACTH Alkaline phosphatase Alanine aminotransferase Amylase Asparate aminotransferase Bicarbonate Bilirubin Calcium Chloride Cholesterol Complement: C3 C4

Cortisol: midnight 9.00 a.m. Creatinine Creatine kinase a-fetoprotein "y-glutamyl transferase: men

women Glucose (fasting) Growth hormone

l-5iu/l 0-1 iu/1 < 10-80 ng/1 30-300 iu/1 5-35 iu/1 70-300 iu/1 5-35 iu/1 22-30 mmol/1 <17mmol/l 2.26-2.60 mmol/1 95-105 mmol/1 <5.5 mmol/1 0.69-1. 5 mg/1 0.12-0.27 mg/1 80-320 nmol/1 280-700 nmol/1 70-130 n-mol/1 <200iu/l <10ku/l

IgG IgM IgA

Lactate dehydrogenase Magnesium Osmolality Phosphate (inorganic) Potassium Prolactin Protein: total albumin Sodium Thyroxine: total free T4 Tri-iodothyronine TSH

Triglycerides Urea Uric acid: men

11-51 iu/1 7-33 iu/1 4.5-6.0 mmol/1 <20mu/l

women

6-13 g/1 0.5-2.0 g/1 1.0-4.0 g/1 70-250 iu/1 0.7-1.0 mmol/1 275-300 mosmol/kg 0.8-1. 4 mmol/1 3.4-5.2 mmol/1 male <450 female <600 60-80 g/1 35-50 g/1 133-145 mmol/1 70-140 nmol/1 10-26 pmol/1 1.2-3.0 nmol/1 0.3-3.8 mu/1 <0.55-1.90 mmol/1 2.5-6.7 mmol/1 0.15-0.42 mmol/1 0.10-0.36 mmol/1

Arterial blood gases

pH

PCO2

7.35-7.45 35-45 mmHg (4.6-6.0 kPa)

478 APPENDICES

APPENDIX 19

DERMATOMES AND MYOTOMES

Myotomes Muscle group Diaphragm Shoulder abductors Elbow flexors Supinators/pronators Wrist extensors Wrist flexors El bow extensors Finger extensors Finger flexors Intrinsic hand muscles Hip flexors Hip adductors Knee extensors Ankle dorsiflexors Toe extensors Knee flexors Ankle plantar flexors Toe flexors Anal sphincter

Reflexes Nerve supply C(3),4, (5) C5 C5, 6 Biceps jerk C5, 6 Supinator jerk C6 C6 C6 C7 C7 Triceps jerk C7 C7 C8 Abdominal reflex T1 T8-12 LI.2 L2, 3 L3,4 Knee jerk L3, 4 L4, 5 L5 L4 5S1 Ankle jerk S1, 2 S1 2 S1 2 Bulbocavernosus S2 3,4 reflex S3, 4 Anal reflex S5 Plantar reflex

APPENDIX 20 479

APPENDIX 20

TESTING PERIPHERAL NERVES

Nerve root:

Muscle:

Test - by asking the patient to:

C3,4 C4,5 C5, 6, 7

trapezius rhomboids serratus anterior pectoralis major (clavicular head) pectoralis major (sternocostal head) supraspinatus infraspinatus latissimus dorsi biceps deltoid

Shrug shoulder, adduct scapula Brace shoulder back Push forward against resistance Adduct arm from above horizontal and forward Adduct arm below horizontal Abduct arm the first 15° Externally rotate arm, elbow at side Adduct horizontal and lateral arm Flex supinated forearm Abduct arm between 15° and 90°

triceps brachioradialis

Extend elbow against resistance Flex elbow with forearm half way between pronation and supination Extend wrist to radial side with fingers extended Arm by side, resist hand pronation Keep fingers extended at MCP joint Extend wrist to ulnar side Abduct thumb at 90° to palm Extend thumb at MCP joint Resist thumb flexion at IP joint

C5, 6, 7, 8 C6, 7, 8 Tl C5 C5, 6 C6, 7, 8 C5, 6 C5, 6 Radial nerve C7, 8 C5, 6 C6, 7 C5,6 C7, 8 C7, 8 C7,8 C7,8 C7, 8 Median nerve

extensor carpi radialis longus supinator extensor digitorum extensor carpi ulnaris abductor pollicis longus extensor pollicis brevis extensor pollicis longus

C6,7 C6, 7, 8 C7, 8 Tl C8T1 C8T1

pronator teres flexor carpi radialis flexor digitorum sublimis flexor digitorum profundus I and II flexor pollicis longus

C8T1 C8T1 C8T1

abductor pollicis brevis opponens pollicis 1st and 2nd lumbricals

Ulnar nerve C7,8 C8T1

flexor carpi ulnaris flexor digitorum profundus III and IV

C8T1 C8T1 C8T1 C8T1 C8T1 Nerve root L4,5 SI L5 Sl,2 L2, 3, 4 Femoral nerve

dorsal interossei palmar interossei adductor pollicis abductor digiti minimi opponens digiti minimi gluteus medius and minimus (superior gluteal nerve) gluteus maximus (inferior eluteal nerve) adductors (obturator nerve)

Keep arm pronated against resistance Flex wrist towards radial side Resist extension at PIP joint (while you fix the proximal phalanx) Resist extension at the DIP joint Resist thumb extension at interphalangeal joint (fix proximal phalanx) Abduct thumb (nail at 90° to palm) Thumb touches 5th finger-tip (nail parallel to palm) Extend PIP joint against resistance with MCP joint held hyperextended Abducting little finger, see tendon when all fingers extended Fix middle phalanx of little finger, resisting extension of distal phalanx Abduct fingers (use index finger) Adduct fingers (use index finger) Adduct thumb (nail at 90° to palm) Abduct little finger With fingers extended, carry little finger in front of other fingers Internal rotation at hip, hip abduction Extension at hip (lie prone) Adduct leg against resistance

LI, 2, 3

ilio-psoas

L2,3 L2, 3, 4

sartorius quadriceps femoris

Flex hip with knee flexed and lower leg supported (patient lies on back) Flex knee with hip externally rotated Extend knee against resistance

Sciatic nerve L4, 5 SI, 2 L4,5

hamstrings tibialis posterior

Flex knee against resistance Invert plantar-flexed foot (continued)

480 APPENDICES

Nerve root:

Muscle:

Test - by asking the patient to:

L4,5 L5,S1 L5, SI L5, SI SI SI, 2 SI, 2 SI, 2

tibialis anterior extensor digitorum longus extensor hallucis longus peroneus longus and brevis extensor digitorum brevis gastrocnemius flexor digitorum longus small muscles of foot

Dorsiflex ankle Dorsiflex toes against resistance Dorsiflex hallux against resistance Exert foot against resistance Dorsiflex hallux (muscle of foot) Plantar flex ankle joint Flex terminal joints of toes Make sole of foot into a cup

Source: Aids to the Examination of the Peripheral Nervous System (1976) HMSO

APPENDIX 21

DRUG LEVELS

Drug

Therapeutic range

For the following drugs, blood should be taken pre-dose: Carbamazepine Ethosuximide Phenobarbitone Phenytoin Primidone Theophylline

4-12 mg/1 40-100 mg/1 20-40 mg/1 10-20 mg/1 (child: 6-14 mg/1) as for phenobarbitone 10-20 mg/1

For the following drugs, blood should be taken at the times indicated: Digoxin (8-12 hours after last dose) Lithium (12 hours after last dose) Valproate (2 hours after last dose)

0.6-2.0 (jug/1 toxicity 1.8-3.0 |xg/l 0.4-1.0 mmol/1 50-100 mg/1

APPENDIX 22 SUGGESTIONS FOR MONITORING PATIENTS TAKING DISEASE-MODIFYING ANTI-RHEUMATIC DRUGS This table has been compiled from information to be found in the British Rheumatology Society Guidelines, which are currently available only to members of the society. Responsibility for the compilation rests with the author of Chapter 9, K Hopayian. Drug

Cautions/adverse effects

Pretreatment

Monitoring

Stop drug and inform specialist if:

Managing adverse reactions

Azathioprine

1. Common: nausea, anorexia, abdominal discomfort, headaches 2. Less common: rash, myelosuppression causing thrombocytopenia, neutropenia and rarely anaemia, Hepatotoxicity 3. Potential impairment of fertility 4. Potential teratogenicity, avoid if possible in pregnancy

1. Order varicella serology. Advise non-immune patients to consult urgently if they have close contact with chicken pox or shingles 2. Warn patients to report sore throat, abnormal bleeding/ bruising

1. FBC and LFTs fortnightly for the first 4 weeks and then monthly 2. ESR monthly

1. WBC <3.5 X 109/L 2. Platelets <150 X 109/L 3. ALT > twice upper limit of normal

1. Nausea, anorexia, abdominal discomfort, headaches. Reduce dose or, if symptoms persist, stop it 2. Sore throat, abnormal bleeding/bruising - check FBC and stop azathioprine if abnormal 3. Close contact with chicken pox or shingles non-immune patients require acyclovir or zoster immunoglobulin (ZIG)

Methotrexate

1. Common: nausea, anorexia, oral ulceration, minor hair thinning, abdominal discomfort, diarrhoea, headaches 2. Less common: rash, myelosuppression causing thrombocytopenia, neutropenia and rarely anaemia 3. Rare but important: (a) hepatotoxicity, liver fibrosis/ cirrhosis (rare if alcohol is avoided), avoid if pre-existing liver disease (b) Pulmonary toxicity. Acute pneumonitis or chronic pulmonary fibrosis not dose related). Symptoms: dry cough, dyspnoea and fever 4. Teratogenic to ova and sperm. Counsel men and women about contraception, during treatment and for 3 months after stopping methotrexate 5. Close contact with chicken pox or shingles - non-immune patients require acyclovir or ZIG

1. FBC, LFTs, U&E 2. CXR 3. Order varicella serology. Advise non-immune patients to consult urgently if they have close contact with chicken pox or shingles 4. Warn patients to report a sore throat and abnormal bleeding/bruising 5. Warn patients to avoid alcohol

1. FBC and LFTs fortnightly for the first 4 weeks and then monthly 2. ESR monthly 3. U&E periodically at hospital review

1. WBC <3.5 X 109/L 2. Platelets <150 X 109/L 3. ALT > twice upper limit of normal or falling albumin 4. Symptoms of pneumonitis

1. Nausea, abdominal discomfort, diarrhoea and anorexia may be helped by the addition of folk acid 5 mg 2 days per week, avoiding the methotrexate day 2. Sore throat, abnormal bleeding or bruising - check FBC and stop methotrexate if abnormal 3. Close contact with chicken pox or shingles non-immune patients require acyclovir or zoster immunoglobulin

Gold

1. Rashes /pruritus /mouth ulcers 2. Bone marrow suppression causing thrombocytopenia, neutropenia and rarely anaemia 3. Renal damage indicated by proteinuria/haematuria on urinalysis

Warn patients to report a sore throat and abnormal bleeding/bruising

1. Check FBC at the time of each injection. Give injection only if previous result is available and satisfactory

1. Rash or mouth ulcers 2. WBC <3.5 X 109/L 3. Platelets <150 X 109/L

1. Proteinuria+ or haematuria+ on two or more occasions inform specialist, order MSU and 24-hour urinary protein (continued)

Drug

Cautions /adverse effects

Pretreatment

Monitoring

Stop drug and inform specialist if:

2. ESR monthly 3. Urinalysis before each injection

Managing adverse reactions 2. Eosinophilia may precede skin rash; reduce gold dose or frequency and observe patient more closely 3. Sore throat, abnormal bleeding /bruising check FBC and stop gold if abnormal

Sulphasalazine

1. Common: nausea, abdominal discomfort, malaise, headache, anorexia, dizziness, depression 2. Less common: rash, bone marrow suppression, thrombocytopenia, leucopenia, megaloblastic anaemia

1. FBC, ESR 2. Warn patients to report a sore throat and abnormal bleeding/bruising 3. LFTs

FBC, ESR, LFTs fortnightly for the first month, then monthly for the second and third months and then once every 3 months

1. WBC <3.5 X 109/L 2. Platelets <150 X 109/L 3. ALT > twice upper limit of normal

1. Common side effects: reducing dose and/or use enteric coated preparations 2. Macrocytosis - check B12 and folate 3. Sore throat, abnormal bleeding or bruising check FBC and stop sulphasalazine if abnormal

Penicillamine

1. Rashes /anorexia /taste disturbance/nausea 2. Bone marrow suppression, causing thrombocytopenia, neutropenia and rarely anaemia 3. Renal damage: proteinuria/ haematuria on urinalysis 4. Rarely febrile reactions, myasthenia, drug-induced lupus

Warn patients to report a sore throat and abnormal bleeding/bruising

1. FBC fortnightly for the first 4 weeks and thereafter monthly 2. ESR monthly 3. Urinalysis monthly

1. WBC <3.5 X 109/L 2. Platelets <150 X 109/L

1. Proteinuria+ or haematuria+ on two or more occasions, inform rheumatologist, order MSU and 24-hour urinary protein collection 2. Sore throat, abnormal bleeding or bruising - check FBC and, if abnormal, stop penicillamine

1. FBC, creatinine 2. Urine testing for blood and protein 3. Record varicella status. Advise non-immune patients to consult urgently if they have close contact with chicken pox or shingles

1. FBC and urine testing weekly for the first 4 weeks and thereafter monthly 2. Creatinine periodically at hospital review

1. Baseline = average of three recent serum creatinines

2-weekly for 3 months, then monthly: 1. FBC, differential

1. Nausea and minor hair thinning Oral cyclophosphamide 2. Haemorrhagic cystitis and hence risk of bladder cancer 3. Leucopenia and thrombocytopenia 4. Teratogenic. Counsel about contraception 5. Infertility. Consider sperm banking

Ciclosporin

1. Common: (mild) nausea, tremor, paraesthesia, headache and abdominal discomfort

Stop drug and inform specialist if: 1. WBC <3.5 X 109/L 2. Platelets <150 X 109/L 3. Haematuria > trace Sore throat, abnormal bleeding or bruising check FBC and stop cyclophosphamide if abnormal 4. Close contact with chicken pox or shingles non-immune patients require aciclovir or ZIG 1. Common side-effects subside with time. Treat hirsutism with depilatories

Leflunomide

Hydroxychloroquine

2. Less common: hyperuricaemia, hirsutism or gum hyperplasia 3. Nephrotoxicity. Caution with concomitant NSAIDs, in particular diclofenac. Halve the dose of diclofenac if used concomitantly Avoid grapefruit juice 4. Rare: other malignancies and lymphoproliferative disorders

2. Creatinine clearance orGFR 3. Blood pressure. Treat hypertension before commencing ciclosporin 4. Urinalysis

2. Electrolytes, creatinine 3. Blood pressure 4. Urinalysis

1. Common: diarrhoea, nausea, vomiting, anorexia, oral mucosal disorders, abdominal pain, increase in blood pressure, elevation of ALT or AST. Weight loss and increased hair loss. Mild allergic reaction including maculopapular rash. Leucopenia 2. Less common: severe disturbances in liver function, taste disturbances, urticaria, anaemia, mild thrombocytopenia and ulcerative stomatitis. Concomitant or consecutive use of potentially myelotoxic or hepatotoxic preparation may be associated with a higher risk of haematological effects. Alcohol intake - best to avoid 3. Rare: pancytopenia, severe leucopenia WBC <2 X 109/L

1. FBC 2. LFTs 3. BP

1. Check BP every 3 months 2. FBC and LFTs every 2 weeks for first 6 months and thereafter every 2 months (ignore GGT results)

1. Minor: nausea, abdominal discomfort, headache, anorexia, rash, alopecia 2. Ocular retinopathy: exceedingly rare at the doses recommended. Early retinopathy is reversible on stopping the drug 3. Hydroxychloroquine should be avoided in pregnancy, if possible

1. FBCandESR 2. U&E 3. Ask about visual impairment and record near visual acuity, with glasses if appropriate

Where ESR monitoring is recommended, the aim is to assess response to treatment. When monitoring FBC, look out for downward trends as well as absolute levels.

Annual: enquire about visual symptomatology. Check visual acuity using test type

and gum hyperplasia with oral hygiene 2. Rise in serum creatinine: s=30% baseline, seek specialist advice 3=50% seek specialist advice URGENTLY stop therapy if serum creatinine level does not decrease to acceptable levels within 1 month. Refer to rheumatologist. 3. Treat hypertension with antihypertensive therapy Stop ciclosporin if hypertension remains uncontrolled 1. WBC <3.5 X 109/L 2. Platelets <150 X 109/L 3. ALT > twice upper limit of normal on two consecutive occasions

Note: In cases of severe haematological reactions, leflunomide and concomitant myelosuppressives must be discontinued and a leflunomide wash-out procedure initiated

Change in acuity or blurred vision, refer to ophthalmologist

APPENDIX 23

PROBLEMS ASSOCIATED WITH SPECIFIC CAUSES OF Audiovisual

Cerebral palsy 1:500

Visual impairment Hearing impairment

Down syndrome 1:700

Visual impairment (multifactorial), cataracts Hearing impairment (multifactorial) Annual assessments recommended Strabismus Myopia

Prader-Willi 1:10,000-25,000

Fragile X 1:6000

Endocrine

Psychiatric/psychological

CNS

Depression Variable intellectual capacity

Epilepsy

Hypothyroidism Annual TFT recommended

Depression Alzheimer's type dementia (clinical onset uncommon before 40 years)

Epilepsy Usually clonic/tonic

NIDDM (secondary to obesity) Hypogonadism Delayed puberty

Hyperphagia Impulse control difficulties Self-injury

Attention deficit/ hyperactivity Variable intellectual capacity Disabled in social functioning Variable intellectual capacity Phobic anxiety Disabled in social functioning

Visual impairment (multifactorial) Hearing impairment, recurrent ear infections

Phenyl ketonuria 1:10,000-20,000

Epilepsy Usually clonk/tonic, complex partial Epilepsy Hyperactivity Tremor and pyramidal tract signs Extrapyramidal syndromes Severe developmental delay Epilepsy Perceptual and motor function reduced

Angelmann syndrome <1:10,000

Glaucoma

Easily excitable Hyperactive

Williams ?<1:20,000

Hyperacusis Strabismus

Rett 1:14,000 Females

Refractory errors

Variable intellectual capacity Attention deficit problems in childhood Severe intellectual disability

Noonan <1:10,000

Strabismus, refractive errors Vision/hearing impairments

Mild intellectual disability

Epilepsy

Tuberous sclerosis 1:6000-17,000

Retinal tumours Eye rhabdomyomatas

Variable intellectual capacity Behavioural difficulties Sleep problems

Cerebral astrocytomas Epilepsy

Neurofibromatosis 1:3000

Hearing impairment (glioma affecting auditory nerve)

Variable intellectual capacity

Variable clinical phenomena depending on site of the tumours Epilepsy

Various endocrine abnormalities

Epilepsy Vasomotor instability

Adapted from an original unpublished version by Michael Kew and Glyn Jones and reproduced with the kind permission of the

DISABILITY Cardiovascular

Congenital heart defects (present in 40 to 50%)

Muscular/skeletal and skin

Other

Inheritance

Orthopaedic problems Neuromuscular problems

Genitourinary problems Incontinence Constipation Dental problems Recurrent aspiration Oesophagitis, gastroesophageal reflux ± bleeding/anaemia Swallowing/eating difficulties Blood dyscrasias Childhood leukaemia Sleep apnoea Increased susceptibility to infections Coeliac disease

Most cases are sporadic; 4% due to translocation involving chromosome 21 or rarely parental mosaicism

Atlantoaxial instability Skin disorders, alopecia, eczema

Scoliosis, kyphosis Hypotonia Skin picking

Aortic dilatation, mitral valve prolapse (related to connective tissue dysplasia)

Connective tissue dysplasia Scoliosis Congenital hip dislocation

Joint contractures and scoliosis (in adults) Cardiac abnormalities Hypertension, CVAs Chronic hemiparesis Prolonged QT interval

Joint contractures Scoliosis Hypotonia Osteopenia Fractures Scoliosis

Pulmonary valvular stenosis ASD, VSD, PDA

Scoliosis Talipes equinovarus Pectus carina turn/excava turn

Rhabdomyomatas Hypertension

Bone Rhabdomyomata

Skeletal abnormalities especially kyphoscoliosis

University of Queensland.

Infantile failure to thrive, then hyperphagia and severe obesity High tolerance to pain Decreased ability to vomit Sleep apnoea Osteoporosis Undescended testes Dental abnormalities Herniae (CT related) Abnormalities of speech and language

Atypical. Most cases are sporadic

Eczema

Autosomal recessive

Speech impairment Movement and balance disorder Characteristic EEC changes Renal abnormalities

Variety of genetic mechanisms on chromosome 15 Microdeletion on chromosome 7

Hyperventilation Apnoea Reflux Feeding difficulties Growth failure Abnormal clotting factors, platelet dysfunction Undescended testes, deficient spermatogenesis Lymphangiectasia Hepatosplenomegaly Cubitus valgus, hand abnormalities Kidney and lung hamartomata Polycystic kidneys Liver rhabdomyomata Dental abnormalities Skin lesions Variable clinical phenomena depending on the location of the neurofibroma Tumours are susceptible to malignant change Other varieties of tumours may be associated

Usually sporadic X-linked

X-linked

Autosomal dominant may be sporadic

Autosomal dominant

Autosomal dominant

486 APPENDICES

APPENDIX 24 THE COMMUNITY DEPENDENCY INDEX © Professor Pamela Eakin, reproduced with permission. Score the patient under the following nine headings. A score <75 suggests moderate disability; <50 suggests severe disability.

Personal toilet 5 = Client can wash hands and face, comb hair, clean teeth and shave. Must be able to get to water, brushes, without help and operate them independently. 0 = Any help or supervision needed or difficulty with personal toilet.

Feeding 10 = Independent. The client can feed himself a meal from a tray or table when someone puts the food within reach. He must put on his own assistive device if this is needed, cut up food, spread butter and so on. He must accomplish this in a reasonable time (that is acceptable to the client). 5 = Food must be cut for the client or some help is necessary with the items above. Unreasonable time or effort required if feeds independently. 0 = Client unable to feed himself.

Moving from (wheel)chair to bed and return 15 = Independent in all phases of this activity. Chair: client can safely stand up from sitting in his chair (high chair allowed) without help from another person, and sit down again. Client must be able to get in/out bed without help, and, once in bed be able to turn and move up and down in the bed as necessary. Or wheelchair: client can safely approach the bed in his wheelchair, lock brakes, lift footrests, transfer safely to the bed and lie down. Once in bed he must be able to turn and move up and down the bed necessary. Client must be able to transfer back into the wheelchair safely including changing the position of the wheelchair for the return transfer. 10 = Client can independently sit down and stand up from chair, or transfer in and out of a wheelchair, but still has difficulty or needs help in bed. 5 = Help or supervision needed to ensure client's safety in all parts of this activity; or client performs all or parts of this activity with difficulty. 0 = Client unable to perform this activity.

Getting on and off the toilet or commode (during day and night) 10 = Client is able to reach the toilet/commode area unassisted. He is able to transfer on and off the commode, fasten and unfasten clothes, prevent spoiling of clothes, use toilet paper without help. He may use equipment or stable fittings for support if needed (e.g. rail, raised toilet seat, or side of

bath). If he uses a commode he must be able to position it for use, empty it and clean it out. 5 = Client has difficulty with part of this activity or client needs help because of imbalance or in handling clothes or in using toilet paper or in flushing the toilet. 0 = Client needs help to empty the commode or is not able to transfer. Note: If the client can use the toilet independently during the day but has the commode at night, which someone else empties, then score = 5.

Walking 50 yards outside the house or using a wheelchair Walking 15 = Client gets in/out of the house unassisted. He can walk at least 50 yards without help or supervision outside his home. He may wear braces and prostheses and use walking aids. He must be able to reach and operate aids without help. 10 = Client has difficulty or needs minimal help or supervision in any of the above but can walk at least 50 yards. Wheelchair 5 = Client cannot walk but can propel a wheelchair independently. He must be able to go round corners and turn around. He must be able to get in and out of the house independently (access). He must be able to push the wheelchair at least 50 yards. If the wheelchair is used indoors he must be able to manoeuvre himself to a table, bed or toilet. Do not score for wheelchair use if the client gets a score for walking. 0 = Client unable to walk or propel a wheelchair for 50 yards.

Dressing and undressing 10 = Client is able to put on and remove and fasten all clothing and tie shoelaces (adaptations/aids allowed). This activity includes putting on and removing prostheses, braces and corsets where these are prescribed. Special clothing, such as slip-on shoes or dresses that open down the front may be used where necessary. 5 = Client has difficulty or needs help in putting on and removing or fastening any clothing. Where helped he must do at least half the work himself. He must accomplish this in a reasonable time. 0 = Client needs help with all or most of dressing.

Bathing self 5 = Client can use a bath or shower. He must be able to do all the steps involved in whichever method is employed without another person helping him. Verbal supervision is allowed. 0 = Client has difficulty or needs help. Note: Where the client's home does not have bathing/ shower facilities, score 5 for using a bath/shower in another facility or having an all-over wash if independent. Where client has an all over wash because he is unable to use the bath/shower then score = 0.

Ascending and descending stairs 10 = Client is able to get up and down stairs safely, without help or supervision. He may and should use the handrails

APPENDIX 25,26 487

and walking aids when needed. He must be able to carry walking aids up and down the stairs if needed. If a stair lift or vertical lift is used, the client must be able to use it without help or supervision (including transfers). 5 = Client has difficulty, needs help or supervision in any one of the above items. 0 — Client unable to climb the stairs. Note: If the client does not have stairs in the house, count as 10 because they are not an obstacle to independence in the home, even if they are obstacles in the community.

Leisure

Continence of bowels

APPENDIX 26 ADMISSION PROCEDURES FOR PATIENTS WITH MENTAL HEALTH PROBLEMS Compulsory admission

10 = Client is able to control bowels and has no accidents. He can use a suppository or take an enema when necessary (e.g. spinal cord injury). He can manage external devices (e.g. colostomy). 5 = Client needs help with any of the above. 0 = Client does not have bowel control.

Continence of bladder 10 = Client able to control his bladder day and night. Clients who wear external device and leg bag must put them on independently, clean and empty the bag and stay dry day and night. 5 = Client has control of bladder, but cannot get to the toilet or commode in time (e.g. due to poor mobility) or needs help with an external device. 0 = Client does not have bladder control.

APPENDIX 25 NOTTINGHAM EXTENDED ACTIVITIES OF DAILY LIVING QUESTIONNAIRE (EADL) Score the patient on each item on a scale of 0 to 3 where '3' represents independent function, '2' represents alone with difficulty, '!' represents alone with help, and '0' represents unable.

Subscale Item Mobility

Kitchen

1. Do you 2. Do you 3. Do you 4. Do you 5. Do you 6. Do you

walk around outside? climb stairs? get in and out of the car? walk over uneven ground? cross roads? travel on public transport?

7. Do you manage to feed yourself? 8. Do you manage to make yourself a hot snack? 9. Do you take hot drinks from one room to another? 10. Do you do the washing up? 11. Do you make yourself a hot drink?

Domestic 12. Do you manage your own money when you are out? 13. Do you wash small items of clothing? 14. Do you do your own housework? 15. Do you do your own shopping? 16. Do you do a full clothes wash?

17. Do you 18. Do you 19. Do you 20. Do you 21. Do you 22. Do you

read newspapers or books? use the telephone? write letters? go out socially? manage your own garden? drive a car?

Total (0-66)

The team needed to complete a Section 2 or Section 3 consists of: (a) theGP; (b) an approved social worker (ASW); (c) an approved psychiatrist (duty consultant or specialist registrar). The procedure to follow between 9 a.m.-5 p.m. for assessment of a patient who may need to be sectioned from home might be as follows: * Obtain relevant information from a partner who may know the patient better. * Review the records to assess: (a) risk of violence; (b) past history of outcomes of previous sections; (c) previous responses to treatment. * Phone the family or carer to obtain their assessment of the: (a) current situation and urgency; (b) need for police support; (c) risk of violence, access to weapons; * Ensure the patient is at home and someone is in to allow access. * Explain the procedure and arrange a time to visit. * Contact the duty ASW and duty psychiatrist: (a) provide basic information: name, date of birth, address, past history, current problem, reason for assessment, phone number of patient, name of carer or relative at home, how to contact you in the next few hours, name of key worker, any known risk of violence or self harm; (b) decide on the need for police support; (c) arrange a time to meet together at the home. * If a joint visit is not possible decide: (a) when each will visit; (b) how to discuss assessments and decide on need for sectioning; (c) where to leave the section form for GP to sign, e.g. will it be left with relative or brought to surgery? * If the GP visits first, take the section forms and complete, if appropriate. * Avoid sedating patient as this makes subsequent assessment difficult. * Avoid asking the relative to sign Section 2 or 4, as this increases guilt and may disrupt future relationships. * If the two doctors cannot do a joint assessment, they must examine the patient within 5 days of each other.

488 APPENDICES

* Organize a hospital bed (the ASW is responsible for arranging transport). * If the GP and ASW disagree on the need for sectioning: (a) record the basis of each decision in writing; (b) identify a home care plan; (c) decide who will reassess and when; (d) instruct the carer in what to do if the situation changes. * Ask family or carer to make a GP follow-up appointment to find out how they are coping and what has happened to the patient, and future plans. * Complete item of service claim form.

Voluntary admission * If patient is known to hospital staff and a bed is available and the patient accepts admission: arrange admission, write a letter, organize transport. * If patient is known to hospital staff but there are no beds available: contact the bed manager or psychiatric nurse manager. 1. If a bed is found, arrange admission as above. 2. If a bed is not immediately available and a 2-hour delay is acceptable, if supported by CPN or crisis team: (a) arrange for the hospital to phone you and family when bed available;

(b) write letter and give to patient or relative; (c) ask the family to phone the GP if they have not heard from hospital in 2 hours. 3. If a bed is not immediately available and a delay is not acceptable: (a) phone the A&E psychiatric registrar or A & E mental health nurse; (b) consider referral to A&E; (c) other local options (to be entered here). * If the patient not known to hospital staff: if Mon-Fri 9-5 p.m. then: (a) check catchment area and relevant consultant (in London phone Emergency Bed Service 020 7407 7181 to obtain this information); (b) phone the consultant on pager or mobile or contact his or her secretary to ask the psychiatrist to phone you ASAP: - if the consultant is available arrange for domiciliary visit or admission; -if consultant not available, phone the SHO or registrar and proceed as above; -if out of hours, follow the local protocol for voluntary admission if available. Note: The out of hours service should ensure that the protocol is in the doctor's bag.

APPENDIX 27 THE EARLY WARNING FORM FOR USE IN PSYCHOTIC ILLNESS EARLY WARNING SIGNS Name: I am at risk of developing episodes of:

My early warning signs are (e.g. changes in sleep, eating/drinking or mood, becoming quiet or loud or more withdrawn):

1 2. 3 Whenever I have any of these signs I will respond by:

My health worker is:

Phone

My home contact is:

Phone

My advocacy contact is:

Phone

If I have any concerns about my illness I will contact: immediately.

APPENDIX 28 489

APPENDIX 28

DRUG STABILITIES

Notes on using tables of drug mixture stabilities • The following tables are separated into mixtures containing two or three drugs, ordered by diamorphine first, then the other drugs in alphabetical order. • The maximum dose for each drug in each syringe size is given. Provided the doses for every drug in the combination is less than or equal to these maximum values, then the mixture is stable for 24 hours. Above the maximum doses stated the solution is either unstable or has not been tested and it is not possible to say whether it is stable or not. • The following combinations are not stable: (a) diamorphine, dexamethasone and methotrimeprazine; (b) diamorphine, dexamethasone and midazolam; (c) diamorphine, cyclizine and metoclopramide; (d) octreotide and methotrimeprazine; (e) octreotide and cyclizine; (f) octreotide and dexamethasone; (g) diamorphine, metoclopramide and ondansetron.

Two drug combinations for subcutaneous infusion which are stable for 24 hours Diluent: Water for Injections BP Drug combination

Diamorphine and cyclizine

Maximum dose (nig) known to be stable in:

Comments

8ml in a 10ml syringe

14 ml in a 20 ml syringe

17ml in a 30ml syringe

160

280

340

160*

If diamorphine dose >160, cyclizine dose must be no more than 80

280*

If diamorphine dose >280, cyclizine dose must be no more than 140

If diamorphine dose >340, 340* cyclizine dose must be no more than 170

If exceed these doses then likely to get precipitate *Maximum recommended daily dose 150

Diamorphine and dexamethasone

400

700

850

3.2

5.6

6.8

Diamorphine and haloperidol

800

400

—

—

24

32

Diamorphine and hyoscine HBr

1200

—

—

~

Diamorphine and hyoscine butylbromide (Buscopan)

1200

Diamorphine and ketorolac

47

82

90

—

40

74

90

400

700

850

80

140

170

Diamorphine and methotrimeprazine (Nozinan)

Can precipitate if undiluted drugs are mixed during preparation If exceed these doses then likely to get precipitate

3.2

160

Mixture can be irritant, dilute to largest possible volume (continued)

490 APPENDICES

Maximum dose (mg) known to be stable in:

Drug combination

8ml in a 10ml syringe

14ml in a 20ml syringe

Comments

17ml in a 30ml syringe

1200

2100

2550

40

70

85

Diamorphine and midazolam

400

700

850

16

28

34

Diamorphine and octreotide

200

350

425

0.9

1.6

1.9

Diamorphine and ondansetron

40

70

85

5

9

11

Diamorphine and metoclopramide

Mixture can be irritant dilute to largest possible volume

-

Three drug combinations for subcutaneous infusion which are stable for 24 hours Diluent: Water for Injections BP Drug combination

Maximum dose (mg) known to be stable in: 8 ml in a 10 ml syringe

Comments

14ml in a 20ml syringe

17ml in a 30ml syringe

160

280

340

160

280

340

16

28

34

Diamorphine and dexamethasone and haloperidol

400

700

850

3.2

5.6

6.8

8

14

17

Diamorphine and dexamethasone and metoclopramide

400

700

850

3.2

5.6

6.8

24

42

51

Diamorphine and haloperidol and midazolam

560

980

1190

4

7

8.5

32

56

68

Diamorphine and hyoscine butylbromide (Buscopan)

560

980

1190

4

7

8.5

Diamorphine and cyclizine and haloperidol

Above these doses the mixture is likely to precipitate

Only stable if diamorphine and haloperidol are well diluted before dexamethasone is added. Use only if no other options

Hyoscine butylbromide is usually used at doses of 60-120 mg. (continued)

APPENDIX 29 491

Drug combination

and midazolam

Diamorphine and methotrimeprazine and metoclopramide

Comments

Maximum dose (mg) known to be stable in: 8 ml in a 10 ml syringe

14ml in a 20ml syringe

17ml in a 30 ml syringe

22

39

48

400

700

850

80

140

170

24

42

51

Stability data at these concentrations is not known in three drug combinations

Reproduced with permission from SIGN. Control of symptoms in patients with cancer. Available from Scottish Intercollegiate Guidelines Network, 9 Queen Street, Edinburgh EH2 1JQ. Online. Available: http://www.sign.ac.uk

APPENDIX 29 GUIDELINES FOR THE URGENT REFERRAL OF PATIENTS WITH SUSPECTED CANCER Cancer type

Referral guidelines

Lung cancer

Urgent referral for chest X-ray • Haemoptysis • Unexplained or persistent (more than 3 weeks): -cough -chest/shoulder pain -dyspnoea -weight loss -chest signs -hoarseness - finger clubbing -features suggestive of metastasis from a lung cancer (e.g. brain, bone, liver or skin) -persistent cervical/supraclavicular lymphadenopathy Urgent referral to a chest physician Any of the following:

Upper GI cancer

• Chest X-ray suggestive/suspicious of lung cancer (including pleural effusion and slowly resolving consolidation) • Persistent haemoptysis in smokers/ex-smokers over 40 years of age • Signs of superior vena caval obstruction (swelling of face/neck with fixed elevation of jugular venous pressure) • Stridor (consider emergency referral) Urgent referral • Dysphagia - food sticking on swallowing (any age) • Dyspepsia at any age combined with one or more of the following 'alarm' symptoms: - weight loss -proven anaemia -vomiting • Dyspepsia in a patient aged 55 years* or more with at least one of the following 'high risk' features: - onset of dyspepsia less than 1 year ago -continuous symptoms since onset (continued)

492 APPENDICES

Cancer type

Referral guidelines • Dyspepsia combined with at least one of the following known risk factors: - family history of upper GI cancer in more than two first-degree relatives - Barrett's oesophagus -pernicious anaemia -peptic ulcer surgery over 20 years ago - known dysplasia, atrophic gastritis, intestinal metaplasia • Jaundice • Upper abdominal mass *Age 55 years is considered to be the maximum age threshold. Local Cancer Networks may elect to set a lower age threshold (e.g. 50 years or 45 years)

Lower GI cancer

Urgent referral It is recommended that these symptom and sign combinations when occurring for the first time should be used to identify patients for urgent referral under the two week standard: All ages • A definite palpable right-sided abdominal mass • A definite palpable rectal (not pelvic) mass • Rectal bleeding with a change in bowel habit to looser stools and/or increased frequency of defecation persistent for 6 weeks Over 60 years • Rectal bleeding persistently without anal symptoms* • Change of bowel habit to looser stools and/or increased frequency of defecation, without rectal bleeding and persistent for 6 weeks

Any age • Iron deficiency anaemia without an obvious cause (Hb <11 g/dl in men or <10g/dl in postmenopausal women) Note: Patients with the following symptoms and no abdominal or rectal mass, are at very low risk of cancer: • Rectal bleeding with anal symptoms* • Change in bowel habit to decreased frequency of defecation and harder stools • Abdominal pain without clear evidence of intestinal obstruction *Age 60 years is considered to be the maximum age threshold. Local Cancer Networks may elect to set a lower age threshold (e.g. 55 years or 50 years) f Anal symptoms include soreness, discomfort, itching, lumps and prolapse as well as pain Breast cancer

Urgent referral • Patients with a discrete lump in the appropriate age group (e.g. age <30) • Signs which are highly suggestive of cancer such as: -Ulceration - Skin nodule - Skin distortion - Nipple eczema -Recent nipple retraction or distortion (<3 months) Conditions that require referral - but not necessarily urgently Lump • • • •

Discrete lump in a younger women (e.g. age <30 years) Asymmetrical nodularity that persists at review after menstruation Abscess Persistently refilling or recurrent cyst

Pain • Intractable pain not responding to reassurance, simple measures such as wearing a well-supporting bra and common drugs (continued)

APPENDIX 29 493

Cancer type

Referral guidelines Nipple discharge • Age <50 with bilateral discharge sufficient to stain clothes • Age <50 with bloodstained discharge • Age >50 with any nipple discharge

Gynaecological cancer

Urgent referrals • Lesion suspicious of cancer on cervix or vagina on speculum examination • Lesion suspicious of cancer on clinical examination of the vulva • Palpable pelvic mass not obviously fibroids • Suspicious pelvic mass on pelvic ultrasound • More than one or a single heavy episode of postmenopausal bleeding (PMB) in women aged >55 years who are not on HRT • Postcoital bleeding (PCB) age >35 years that persists for more than 4 weeks • HRT: unexpected or prolonged bleeding persisting for more than 4 weeks after stopping HRT Early referral Indications for 'early' referral (i.e. within 4-6 weeks) but not 'urgent' referral • Any other women with postmenopausal bleeding not on HRT • Repeated unexplained postcoital bleeding Note: In women over 45 years with persistent abdominal pain or distension, ovarian cancer should be considered and a pelvic examination performed

Urological cancers

Urgent referral Macroscopic haematuria in adults Microscopic haematuria in adults over 50 years Swellings in the body of the testis Palpable renal masses Solid renal masses found on imaging An elevated age specific PSA in men with a ten year life expectancy A high PSA (>20ng/ml) in men with a clinically malignant prostate or bone pain Any suspected penile cancer PSA testing of asymptomatic men or screening for prostate cancer is not national policy. It is recommended that a PSA test, except in men clinically suspicious of prostate cancer, should only be performed after full counselling and provision of written information

Haematological cancers

Urgent referral Blood count/film reported as suggestive of acute leukaemia or chronic myeloid leukaemia Lymphadenopathy (>1 cm) persisting for 6 weeks Hepatosplenomegaly Bone pain associated with anaemia and a raised ESR (or plasma viscosity) Bone X-rays reported as being suggestive of myeloma Constellation of three or more of the following symptoms: fatigue, night sweats, weight loss, itching, breathlessness, bruising, recurrent infections, bone pain

Skin cancers

Urgent referral Melanoma • Pigmented lesions on any part of the body which have one or more of the following features - growing in size - changing shape -irregular outline -changing colour -mixed colour - ulceration - inflammation Note: Melanomas are usually 5 mm or greater at the time of diagnosis, but a small number of patients with very early melanoma may have lesions of a smaller diameter (continued)

494 APPENDICES

Cancer type

Referral guidelines Squamous cell carcinoma • Slowly growing, non-healing lesions with a significant induration on palpation (commonly on face, scalp, back of hand) - with documented expansion over a period of 1-2 months • Patients in whom squamous cell carcinoma has been diagnosed from a biopsy undertaken in general practice • Patients who are therapeutically immunosuppressed after an organ transplant have a high incidence of skin cancers, mainly squamous cell carcinoma. These tumours can be unusually aggressive and metastasize. It is therefore strongly recommended that transplant patients who develop new or growing cutaneous lesions should be referred under the 2-week standard

Head and neck cancer

Urgent referral Hoarseness persisting for >6 weeks Ulceration of oral mucosa persisting for >3 weeks Oral swellings persisting for >3 weeks All red or red and white patches of the oral mucosa Dysphagia persisting for 3 weeks Unilateral nasal obstruction particularly when associated with purulent discharge Unexplained tooth mobility not associated with periodontal disease Unresolving neck masses for >3 weeks Cranial neuropathies Orbital masses Note: The level of suspicion is further increased if the patient is a heavy smoker or heavy alcohol drinker and is aged over 45 years and male. Other forms of tobacco use (chewing Betel, Gutkha, Pan) should also arouse suspicion

Brain tumours

Urgent referral • Subacute progressive neurological deficit developing over days to weeks (e.g. weakness, sensory loss, dysphasia, ataxia) • New onset seizures characterized by one or more of the following: -focal seizures -prolonged postictal focal deficit (longer than 1 hour) -status epilepticus -associated interictal focal deficit • Patients with headache, vomiting and papilloedema • Cranial nerve palsy (e.g. diplopia, visual failure including optician defined visual field loss, unilateral sensorineural deafness) Consider urgent referral for • Patients with non-migrainous headaches of recent onset, present for at least 1 month, when accompanied by features suggestive of raised intracranial pressure (e.g. woken by headache; vomiting; drowsiness) Note: This last guideline is intended to provide the primary care physician with the discretion to decline urgent referral if there are other known features (e.g. depression, somatization disorder) making a diagnosis of brain tumour very unlikely

Sarcoma

Urgent referral • A soft tissue mass with one or more of the following characteristics: - size >5 cm -painful - increasing in size - deep to fascia - recurrence after previous excision • Patients with radiological suspicion of a primary bone tumour based on evidence of bone destruction, new bone formation, soft tissue swelling and periosteal elevation

Children's cancers

Urgent referral • Abnormal blood count: If reported as requiring urgent further investigation • Petechiae/purpura: These findings are always an indication for urgent investigation (continued)

APPENDIX 29

Cancer type

495

Referral guidelines • Fatigue: in a previously healthy child when combined with either of the following: - generalized lymphadenopathy - hepatosplenomegaly • Bone pain: especially if it is: - diffuse or involves the back -persistently localized at any site - requiring analgesia -limiting activity • Lymphadenopathy: is more frequently benign in younger children but referral is advised if one or more of the following characteristics are present, particularly if there is no evidence of previous local infection -non tender, firm/hard and >3cm in maximum diameter -progressively enlarging -associated with other signs of general ill health, fever and/or weight loss - involves axillary nodes (in the absence of any local infection or dermatitis) or supraclavicular nodes - seen as a mediastinal or hilar mass on chest X-ray • Headache: of recent origin with one or more of the following features: - increasing in severity or frequency - noted to be worse in the mornings or causing early wakening - associated with vomiting -associated with neurological signs (e.g. squint, ataxia) - associated with behavioural change or deterioration in school performance • Soft tissue mass: any mass which occurs in an unusual location should be considered suspicious particularly if associated with one or more of the following characteristics: - shows rapid or progressive growth -size >3cm in maximum diameter - fixed or deep to fascia - associated with regional lymph node enlargement

Reproduced with the permission of HMSO. More details of the guidelines are available on www.doh.gov.uk/cancer/referral.htm Proformas for referrals of patients with suspected cancer in the UK are available on www.doh.gov.uk/cancer/proforma.htm

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Index

Note - To save space in the index, certain abbreviations have been used: COPD:

chronic obstructive pulmonary disease GP: general practitioner HRT: hormone replacement therapy IUD: intrauterine devices NSAIDs: nonsteroidal anti-inflammatory drugs

A Abbreviated Mental Test Score 393–394 Abdominal aortic aneurysm (AAA) 294 Abdominal pain hormonal contraceptives and 235 in pregnancy 261 recurrent, in children 82–83 Abdominal wounds 298 Abortion 245–246 Abscess, amoebic liver, in returning travellers 38 Absent withdrawal bleeds (WB), oral contraceptive-induced 234 Accident victims 16–17 Acne 368–370 Acne rosacea 370 Acute otitis media (AOM) 335–336 Adjuvant analgesia 429–30 Admission procedures for mental health patients 487–488 see also Psychotic disorders Adolescents emotional problems 87–88 preventive health care 87–88 Adoption 247 Adrenaline, anaphylaxis emergency treatment 386 Advance directives 15, 436 Affective disorder, bipolar 307–308 Afro-Caribbeans hypertension 92 prepregnancy care 254 sickle cell disease 417–418

Age-related macular degeneration 361 Agitation in elderly 391–393 in palliative care 433 AIDS 45–19 advice for travellers 39 see also HIV Airways lower infections 121–122 upper, obstruction in children 79–80 Akathisia, adverse effect of schizophrenia treatment 306 Alcohol problems 321–324 advice on 444 alcohol-dependent drinkers 323 driving and 9,322,323 elderly consumption 389–390 health risks 321 'problem drinkers' 322 Allergic conjunctivitis 356 Allergies 383–387 anaphylaxis 385–387 to food 383–385 Allopurinol, gout prophylactic drug 174 Alopecia 376–377 Alpha-blockers, hypertension treatment 93 Alzheimer's disease dementia 394 in Down syndrome 421 treatment 395–396 Amantadine, influenza prophylaxis 26 Amenorrhoea 212–213 hormonal contraceptives and 235, 237

Amniocentesis 258–259 Amoebic liver abscess, in returning travellers 38 Amphetamines 330 Anaemia 415–417 in pregnancy 256, 265 Analgesia adjuvant drugs 429–430 rheumatoid arthritis treatment 176 use in dying patient 427 see also Pain management

Anal surgery 298 Anaphylaxis 385–387 Anencephaly, prenatal screening 258

Aneurysm, abdominal aortic 294 Angina 93–95 acute attacks 94 driving and 9 preventive treatment 94–95 unstable 95–96 Vincent's 349 Angioplasty, driving and 9 Angiotensin converting enzyme (ACE) inhibitors congestive cardiac failure treatment 100–101 hypertension treatment 93 stroke treatment 200 Ankle-brachial index 293 Ankle problems 170 acute injury 290 injections 170–171 postural oedema in elderly 399 Ankylosing spondylitis 178 Anogenital warts 44 pregnancy and 256 Anorexia end-stage heart failure 102 palliative care 432–433 Antenatal care 256–259 Antibiotics acne treatment 369 infective endocarditis prophylaxis 106–107 oral contraceptives and 232 osteoarthritis prophylaxis 164 sore throat management 347–348 Anticonvulsants 424 adjuvant analgesia 430 bipolar affective disorder treatment 308 Antidepressants 311–312 anxiety treatment 316 menstrual problems and 216 panic disorder treatment 316 Antiemetics 431–432 myocardial infarction and 96

498

INDEX

Antifibrinolytics, menorrhagia treatment 211 Antihistamin.es eczema and dermatitis treatment 367 rhinitis treatment 345 Antiplatelet therapy angina 94 myocardial infarction 97 peripheral vascular disease 293 stroke 200, 201 Antipsychotics bipolar affective disorder treatment 308 schizophrenia 305–306 Anxiety 315–316 driving and 9 in palliative care 433 Aphthous ulcers 350–351 in palliative care 434 Apnoea, obstructive sleep (OSA) 346–347 in Down syndrome 412 Apomorphine Parkinson's disease 198 sublingual, erectile dysfunction treatment 249 Arc eye 357 Arrhythmias 103–106 atrial fibrillation see Atrial fibrillation (AF) bradycardia 106 driving and 9 ectopic beats in normal heart 103 paroxysmal supraventricular tachycardia 105–106 sick sinus syndrome 106 ventricular tachycardia 106 Arterial disease, risk factors 229, 230 Arterial surgery 298 Arteritis, temporal see Temporal arteritis Asperger's syndrome 58–59 Aspirin myocardial infarction and 97 peripheral vascular disease 293 stroke prevention 200 Asthma 115–120 acute 119–120 in children 77–79,116,119–120 devices 117–118 diagnosis 115–116 differentiation from viral–associated wheeze 116 inhaler choice for different age groups 118 management 116–117 children under 5 years 471 chronic in children and young adults 469–470 follow–up 118 National Asthma Campaign 'Be in Control' action plan 119

of patients not controlled on inhaled steroids 117 self–management plans 118–119 pregnancy and 267 Atlantoaxial instability, in Down syndrome 412 Atrial fibrillation (AF) 104–105 stroke and 200 Atrial flutter 105 Attendance Allowance (AA) 5, 426 Attention deficit hyperactivity disorder (ADHD) 60–61 Attorney, power of 14, 399^100 Autism 58–59 Autonomic dysreflexia, in paraplegic patients 204 Azathioprine, adverse effects and monitoring 481 Azelaic acid cream, acne treatment 369

B Bacillary dysentery, incubation and infectivity periods 459 Back pain, low see Low back pain Bacterial infections of skin see under Skin infections Bacterial vaginosis 214 Bacteriuria, in pregnancy 265 Barbiturate withdrawal 330 Basal cell carcinoma (BCC) 378–379 Bat ears 85 Bedbugs 378 Bedwetting 70–71 Behavioural disorders 58–61 driving and 9 treatment in elderly 396 'Be in Control' asthma action plan 119 Bell's palsy 343–344 Benefits financial 4–7 maternity 4 Benefits Agency Medical Service 3 advice for patients on appeals 7 Benzodiazepines anxiety treatment 316 bipolar affective disorder treatment 308 driving and 9 panic disorder treatment 316 withdrawal 324–325 Benzoyl peroxide, acne treatment 368–369 Bereavement 436–446 in children 438 cot death 437–438 management 436–437 pathological grief 436 suicide 438 Beta2–agonists, asthma treatment 117

Beta-blockers anxiety treatment 316 congestive cardiac failure treatment 101 hypertension treatment 92 myocardial infarction treatment 97 panic disorder treatment 316 Beta interferon, multiple sclerosis treatment 203 Biliary dyskinesia 295 Bipolar affective disorder 307–308 Bisphosphonates, osteoporosis management 182 Bladder problems in children 70–72 in men 281–283 spasm in palliative care 435 Blepharitis 356–357 Blood data 477 Blood pressure control in diabetic patients 150 screening 447–448 see also Hypertension Blue (formerly Orange) Badge 11 Blue breath–holding attacks, in children 75 Body donation for dissection 440 Body lice 378 Body Mass Index (BMI) 476 Bone pain management 430 Bottle feeding 52–53 Bowel problems in children 70–72 malignant obstruction 432 Bow legs, in children 86 Bradycardia 106 Breakthrough bleeding, oral contraceptive–induced 233 Breast cancer genetic risk for familial cases 292 screening 291–292, 450–452 Breast feeding 52, 267–270 Breast problems 290–292 lumps 290 pain without lump 291 referral criteria 290–291 Breath–holding attacks, in children 75 Breathlessness in end–stage heart failure 102 in palliative care 434 British Hypertension Society UK guidelines thresholds 90–91 Bronchiolitis, in children 76 Bronchodilators asthma treatment 117 COPD treatment 124 Buerger's disease 294 Bullous myringitis 336–337 Bupropion, smoking cessation drug aid 332 Burns 296

INDEX

c Calcium dietary intake in osteoporosis management 181 supplements in pregnancy 256 Calcium channel blockers angina treatment 94–95 hypertension treatment 92–93 Cancer pain 426–427 referral guidelines for suspected patients 491–495 risk in ulcerative colitis 141 see also specific cancers Candidiasis 213–214, 375 oral 349 Cannabis 329–330 Cardiac arrhythmias see Arrhythmias Cardiac failure see Heart failure Cardiovascular problems 89–113 in Down syndrome 411 driving and 9 oral contraception and 228–229 see also individual disorders Care Order 63–64 Carpal tunnel syndrome 169 Caruncle 375 Cataracts 361 Catarrhal child 76 Catheters, urinary 281 Cellulitis 374 Central pain 424 Cerebrovascular accident see Stroke Cervical caps 241 Cervical screening 223–224, 452 Chalazion 358 Chest infections, recurrent, in children 77 Chickenpox see Varicella Child health promotion 51–61 cot death risk reduction 51–52 dental and oral disease 54 injury prevention strategies 52, 461–462 nutrition 52–54 screening 54—61, 460–461 see also Children Child protection 61–64 child sexual abuse (CSA) 61–62 management of suspected abuse 62–63 Munchausen's syndrome by proxy (MSBP) abuse 62 neglect and emotional maltreatment 62 physical abuse 61 possible outcomes 63–64 Child protection register 63 Children 51–88 abdominal pain, recurrent 82–83 asthma 77–79, 116, 119–120 management at school age 469–470

management under 5 years old 471 bat ears 85 behaviour disorders 58–61 bereaved 438 bladder and bowel problems 70–72 breath–holding attacks 75 bronchiolitis 76 catarrhal child 76 chest infections, recurrent 77 colds 75–76 congenital dislocation of hip (CDH) 54–55 congenital heart disease 55 conjunctivitis, acute infective 355–356 convulsions 69–70 cough 76–77 croup 80 cystic fibrosis 79 dental problems 54 development disorders 58–61 development stages 462–464 diarrhoea with/without vomiting 67–69 divorce/marital strife effects on child 64 foreskin 84–85 growth problems 56–57, 81–82 headache, recurrent 83 head injuries 288 hearing defects 57 hydrocoeles 84 hypospadias 85 inguinal hernias 84 'innocent' murmurs 55–56 mouth problems 54 Munchausen's syndrome by proxy (MSBP) abuse 62 neglect and emotional maltreatment 62 night cramps 83–84 orthopaedic problems 85–87 otitis media with effusion (OME) 339–340 pain management 422 Parkinson's disease 197 physical abuse 61 puberty 82, 465 respiratory peak flow values 466 respiratory problems 75–79 school refusal 84 screening aspects 54–61, 460–461 sexual abuse 61–62 sleep problems 72–74 squint 58 status epilepticus 70 strawberry naevus 84 surgical problems 84–87 temper tantrums 74–75 umbilical hernia 85 undescended testis 55 upper airways obstruction 79–80 urinary tract infections (UTI) 80–81

499

visual defects 57 vulvovaginitis 81 whooping cough see Whooping cough (pertussis) see also Child health promotion; Child protection; Infancy Child sexual abuse (CSA) 61–62 Chlamydia infection 43 Chlormethiazole withdrawal 330 Chlorpheniramine, anaphylaxis emergency treatment 386 Cholecystitis 295–296 Cholelithiasis 295–296 Cholera 33–34 Cholesterol coronary heart disease and 107, 108 stroke and 200 work–up of patient with raised levels 109 Choriocarcinoma 217 Chorionic villus biopsy (CVB) 258 Chronically ill patients, financial benefits 5–6 see also individual types Chronic hepatitis 24–25 Chronic obstructive pulmonary disease (COPD) 123–127 Chronic paroxysmal hemicrania 192 Chronic suppurative otitis media (CSOM) 337–338 Ciclosporin, adverse effects and monitoring 482–483 Circumcision 85 Claudication, intermittent 292 Club feet, in children 85 Cluster headaches (migrainous neuralgia) 191 Cocaine 330 Cochlea implants 338 Coeliac disease 142–143 Coital headache 192 Colds 345 in children 75–76 Colic, in infants 66 Colorectal cancer 135–136 hereditary non–polyposis (HNPCC) diagnostic criteria 136 screening 136, 449–450 Combined oral contraceptive (COC) 228–234 adverse effects 232 changing pill due to 233–234 choice of pill 230 contraindications 228–230 dysmenorrhoea treatment 209 follow–up 233 hirsutism treatment 158–159 menorrhagia treatment 211 older women 244 postponing a period 233 procedure and advice 231–232 recommendations 230–231 risks 231–232 young users 243

500

INDEX

Common cold see Colds Community Care Grants 6 Community Dependency Index (CDI) 405, 486–487 Compulsory admission see under Psychotic disorders Condoms 241 Confusion in elderly 391–393 in palliative care 433 Congenital dislocation of hip (CDH), screening in children 54–55 Congenital heart disease, screening in children 55 Congestive cardiac failure 100–102 Conjunctivitis acute infective 355–356 allergic 356 incubation and infectivity periods 459 Connective tissue diseases 179–180 Constipation 137–139 acute 138 in children 67, 71–72 chronic 138–139 high fibre diet 138 in multiple sclerosis 204 in palliative care 433 treatment 139 Contact lenses prescribing for patients 362–363 problems 358 Contraception 227–247 barrier methods 241–242 cervical caps 241 condoms 241 diaphragms 241 emergency 242–243 epileptic patients 194 hormonal 228–238 HRTand 222 learning disability and 244 natural family planning 241–242 non–hormonal methods 238–242 older women 244 pill ladders 472 special cases 243–245 sterilization 244–245 young users 243 see also Combined oral contraceptive (COC)

Controlled crying in children 73–74 Convulsions, in children 69–70 Coronary artery bypass graft (CABG), driving and 9 Coronary heart disease (CHD) lipids 110 prevention 107–110 screening 448 work–up of patient with raised cholesterol 109 Corticosteroids osteoarthritis management 162–163 osteoporosis 182

Cot death 437–438 risk reduction 51–52 Cough in children 76–77 HIV patients 47–48 in palliative care 433–434 Council tax benefit 4 Council tax discount 3 Court of Protection 14 COX–2 selective inhibitors 171 in dyspepsia 134 Crime victims 16–17 immediate management 16 Criminal Injuries Compensation 6 Crohn's disease 141–142 dermatitis herpetiformis (DN) 143 Croup, in children 80 Crying, excessive (colic) in infants 66 Cryptosporidium, in children 69 Curly toes, in children 86 Cyclophosphamide, adverse effects and monitoring 482 Cystic fibrosis, in children 79 Cystitis, in postmenopausal women 278 Cytomegalovirus (CMV), pregnancy and 262

D Dacrocystitis, acute 358 Danazol endometriosis treatment 210 menorrhagia treatment 211 Dandruff 368 Deafness 338–340 conductive 339 perceptive 338–339 Death aspects 439 certification 439 donation of body parts 439–440 rituals of different cultures 438–439 see also Bereavement Deep vein thrombosis (DVT) effect on fitness to fly 12–13 management 419 Delirium, in elderly 391–392 Delirium tremens 323 Dementia 393, 394–396 diagnosis 394–395 in Down syndrome 421 driving and 9 frontotemporal 394 ischemic vascular, treatment 396 management 395–396 mixed, treatment 396 prion diseases 395 types 394–395 vascular 394 Dementia with Lewy bodies 394

Dental problems 351 in children 54 free prescriptions 6 Depo–Provera 236 Depot medroxyprogesterone acetate (DMPA) 236 Depression 309–314 counselling/psychotherapy 312 driving and 9 Edinburgh Postnatal Depression Scale 474 in elderly 393–394 management 310–311 medication 311–312 in palliative care 433 postnatal 270, 313–314 De Quervain's tenosynovitis 169 Dermatitis 365–368 Dermatitis herpetiformis (DH) 143 Dermatomes 478 Developmental disorders 58—61 Diabetes mellitus 147–154 annual review 153 diagnosis 148 driving and 9, 154 erectile dysfunction 152 glycaemic control 149–150 glycated haemoglobins 150 hypoglycaemia 153–154 impaired glucose tolerance 153 management 148–149 blood pressure control 150 education 153 exercise 151 eye care 152 foot care 152 lipid lowering 150–151 overweight and obese patients 152 referral for specialist care 154 smoking cessation 151 target values 149 microalbuminuria 151–152 pneumococcal and flu vaccines 154 pregnancy and 152–153, 255, 267 prevention 148 secondary 151 renal disease 151 screening 453 type 2 149 undiagnosed cases 148 visual problems 152 Diabetes UK 154 Diamorphine 431 stability 489–491 Diaphragms (contraceptive) 241 Diarrhoea in children 67–69 HIV patients 48 implications for food handlers 4 in palliative care 433 in returning travellers 38–39 toddler's 69 travellers' 33

INDEX

Diet advice on 443 coeliac disease management 142 coronary heart disease prevention 108, 109 exclusion, eczema and dermatitis treatment 367 high in fibre, constipation management 138 pregnancy and 256 Digoxin atrial fibrillation 105 congestive cardiac failure treatment 101–102 Diphtheria immunization 21–22 Disability 403–413 carers and 408–409 driving fitness 10–11 financial benefits 5–6 learning (intellectual) see Learning disability problems associated with specific causes of 484–485 rehabilitation 403–408 see also Down syndrome Disability Living Allowance (DLA) 5, 426 Disability Working Allowance 6 Disabled persons tax credits 5 Disease–modifying antirheumatic drugs (DMARDs) monitoring suggestions 481–483 rheumatoid arthritis treatment 176–177 Diuretics, congestive cardiac failure treatment 101 Divorce, effects on children 64 Dog bites 296–297 Donation of body parts 439–440 Down syndrome 411–412 hypothyroidism 411 prenatal screening 258 in previous pregnancies 254 Driving fitness 7–11 advice on disagreements with DVLA's decision 8 Blue Badge 11 common medical problems that affectability 8–10 cardiovascular 9 diabetes mellitus 9, 154 drug and alcohol misuse 9, 322, 323 epilepsy and 7, 193 neurological 8, 200 psychiatric 9 stroke 8, 200 visual 10 confidentiality 7–8 disabled drivers 10–11 elderly 10 exemption from wearing a seatbelt 11 psychotic disorders 304

Drug misuse driving and 9, 322, 323 pregnancy and 268 Dry eye 357 Dry mouth, in palliative care 434 Duodenal ulcer 133 Duodenitis 134 Dyskaryosis, cervical 224 Dyslexia 59–60 Dysmenorrhoea 209–210 Dyspareunia 249–250 Dyspepsia 131–134 NSAIDs–induced 133–134, 173 Dysphagia 349 HIV patients 48 in palliative care 434 Dyspnoea see Breathlessness Dyspraxia 60–61 Dysreflexia, autonomic, in paraplegic patients 204 Dystonia, adverse effect of schizophrenia treatment 306 Dystrophy, reflex sympathetic 424 Dysuria 277

E Early Warning Signs Form 488 Ear problems 335–344 discharge 337–338 bloody 336–337 Hallpike manoeuvre 342 painful conditions 335–337 vestibular rehabilitation exercises 341 see also individual conditions Eclampsia 264 Ecstasy 330 Ectopic pregnancy 259 risk factors 257 Eczema 365–368 seborrhoeic 368 Edinburgh Postnatal Depression Scale 474 Ejaculation disorders 250 Elbow problems 169 Elderly 389–401 Abbreviated Mental Test Score 393–394 abuse 399 agitation 391–393 confusion 391–393 delirium 391–392 dementia see Dementia depression 393–394 deterioration without acute illness 393–394 driving fitness 10 falls 396–398 advice 446 gait assessment 397 fragility management 390 hypertension 89

501

incontinence 280 keeping healthy 389–390 legal aspects 399–400 detention of older person 400 mental capacity see Mental capacity power of attorney 14, 399–400 loss of mobility 391–393 low back pain 167 lower limb problems 398–399 macular degeneration 361 morphine use 428–429 night cramps 398 NSAIDuse 171 Parkinson's disease 197 postural ankle oedema 399 restless legs syndrome 398–399 support at home 390–391 Emergency contraceptive 242–243 Emollients, eczema and dermatitis treatment 366 Emotional problems in adolescents 87–88 maltreatment in children 62 Employment rehabilitation 3 Endocarditis, infective, antibiotic prophylaxis 106–107 Endocrine problems 147–160 see also individual disorders Endocrine surgery 298 Endometriosis 210 Endometritis 270 Endoscopy, indications for 132 Epicondylitis 169 Epilepsy 192–195 in children 70 disability and 410 driving and 7, 8, 193 drug treatment 193–195 effect on fitness to fly 12 follow–up 195 management 192–193 pregnancy and 194, 255, 267 status epilepticus 70, 192 Episcleritis 358 Erectile dysfunction 248–249 in diabetic patients 152 Erysipelas 374 Erythrasma 375 Essential tremor 205 Etonogestrel–releasing implant 238 Euthyroid patient, goitre 155–156 Excessive crying (colic) in infants 66 Excessive weight gain during pregnancy 263 Exercise advice on 442 in cardiac failure 99 coronary heart disease prevention 108, 109 in diabetic patients 151 in elderly 390 osteoarthritis management 163 peripheral vascular disease management 293

502 INDEX

Exertional and coital headache 192 Extended Activities of Daily Living questionnaire (EADL) 487 External canal, acute furunculosis 336 Eye problems 355–363 care in diabetic patients 152 contact lenses 358 in Down syndrome 411 free prescriptions 6 gritty irritable red eye 355–358 blepharitis 356–357 conjunctivitis 355–356 dry eye 357 trauma 357–358 instilling drops 356 lash, lids and lacrimal problems 358–359 painful red eye 358 watering eye/discharge 359 infant eye 66–67 see also Visual problems Eyes, donation 439 Eye surgery 298

F Facial palsy 343 Factitious illness by proxy 62 Faecal incontinence 139–140 Falls, in elderly see Elderly, falls Family credit 5 Fatigue, in end–stage heart failure 102 Fetoscopy 259 Fever postnatal 270 in returning travellers 37–38 Fibre in children 53 constipation management 138 Fibromyalgia 185–186 Financial benefits 4–7 Flat feet, in children 86 Fleas 378 Fluoride supplementation 54 Flying fitness 11–13 Folic acid deficiency 417 Food allergies 383–385 Food handlers 4 Foot care, in diabetic patients 152 Foot problems 170 injections 170–171 Foreskin, problems in children 84–85 Form Mat B1 4 Form Med 3 2 Form Med 4 2 Form Med 5 2 Frailty management 390 Frequency dysuria 277 Frontotemporal dementia 394

Fundal height in pregnancy 263 Fungal infections of nails 376 of skin 375–377

G Gait assessment in elderly 397 Gallstones (cholelithiasis) 295–296 Gas expansion difficulties, effect on fitness to fly 12 Gastrectomy, follow–up 143 Gastric ulcer 133 Gastroenteritis 143 Gastroenterological problems 131–145 see also individual disorders Gastrointestinal disorders bleeding 144 disability and 410 in Down syndrome 412 NSAIDs–induced toxicity 172 Gastrointestinal surgery 298 Gastro–oesophageal reflux disease (GORD) 134–135 Genital herpes see Herpes genitalis Genital lice (crabs) 378 Genital warts 44, 256 Gestational diabetes 265 Gestational trophoblastic disease 217 Giddiness 340–341 driving and 8 Glasgow Coma Scale 288 Glatiramer acetate, multiple sclerosis treatment 203 Glaucoma acute closed angle 360 chronic simple 361–362 screening 453–454 Glucose, impaired tolerance 153 Glue ear 339–340 Glycaemic control, in diabetic patients 149–150 Glycated haemoglobins 150 Glycosuria, in pregnancy 265 Goitre, in euthyroid patient 155–156 Gold, adverse effects and monitoring 481 Gonorrhoea 42^43 Gout 173–174 Gravitational ulcers 380–381 Grommets 339–340 Growth disorders in children 56–57, 81–82 in Down syndrome 412 Gum disease 351 Guttate psoriasis 370, 372 Gynaecological problems 209–226 see also individual disorders Gynaecological surgery 298

H Haematemesis 144 Haematology 415–420 blood data 477 problems in ethnic minorities 417–419 see also Anaemia Haematuria 284–285 Haemoglobinopathies 417 Haemophilus influenzae type B (HIB) 22 Haemoptysis, in palliative care 434 Haemorrhage gastrointestinal 144 vitreous 360 Haemorrhagic fevers, in returning travellers 38 Hair follicle infections 375 Halitosis 352 Hallpike manoeuvre 342 Headaches in children, recurrent 83 cluster 191 exertional/coital–induced 192 HIV patients 48 non–migrainous 191–192 tension–type 191 thunderclap 192 see also Migraine Head injury 287–289 in children 288 Glasgow Coma Scale 288 non–referral 288 referral indications for scans 287 rehabilitation 289 treatment 288 Head lice 377–378 Health promotion 441–456 child see Child health promotion Hearing aids 338 Hearing defects in children 57 in Down syndrome 411 tests 340 see also Deafness Heart failure 99–103 acute pulmonary oedema 103 congestive cardiac failure 100–102 end–stage 102–103 GP management 99–100 intractable 102 right 103 Heart murmurs, 'innocent,' in children 55–56 Helicobacter pylori, testing for in dyspepsia 133 Hemicrania, chronic paroxysmal 192 Hepatitis chronic 24–25 viral, in returning travellers 37 see also Hepatitis A; Hepatitis B

INDEX

Hepatitis A immunization 22–23 implications for food handlers 4 incubation and infectivity periods 459 pregnancy and 262 Hepatitis B immunization 23–24 incubation and infectivity periods 459 pregnancy and 256, 262 screening 25 Hepatitis C, screening 25 Hereditary non–polyposis colorectal cancer, diagnostic criteria 136 Hernias inguinal, in children 84 umbilical, in children 85 Herpes genitalis 44–45 pregnancy and 256, 262–263 Herpes simplex labialis 350, 373–374 Herpes stomatitis 350 Herpes zoster 374 immunization 31–32 Ramsay Hunt syndrome 337 Hiccup, in palliative care 434 Hidradenitis suppurativa 375 High fibre diet, constipation management 138 Hip problems 169 congenital dislocation of (CDH), screening in children 54–55 injections 169 Hirsutism 157–159 HIV 45–49 advice for travellers 39 advice on safer sex and avoiding high–risk activities 45^16 counselling 46–47 immunizations for patients 21 management 47 notification and surveillance 49 postexposure prophylaxis 48 pregnancy and 256, 263 prevention 45–46 symptoms needing special consideration 47–48 testing 46 venepuncture 48 Hoarseness 349–350 Home adaptation grants 407–408 Home support for elderly 390–391 Hormone replacement therapy (HRT) 218–223 adverse effects management 221–222 benefits and risks 219–220 contraception and 222 contraindications 218–219 follow–up 222 older women 244 Housing benefit 4 Hydatidiform mole 217 Hydrocoeles, in children 84

Hydrocortisone, anaphylaxis emergency treatment 386 Hydroxychloroquine, adverse effects and monitoring 483 Hyperemesis gravidarum 263 Hyperhidrosis 380 Hypericum extract, depression management 312 Hypertension 89–93 drug treatment 91–93 in elderly 89 non–drug treatment 91 pregnancy and 255, 264–265 stroke and 200 UK BHS guidelines thresholds 90–91 work–up 90 Hyperthyroidism 155 Hypochromic microcytic anaemia 415–416 Hypoglycaemia, in diabetic patients 153–154 Hypospadias, in children 85 Hypothyroidism 156–157 Down syndrome and 411 Hypoxia, effect on fitness to fly 12

I Immigrants malaria 35 screening 40 Immunizations advice for doctors 33 contraindications 20 in diabetic patients 154 diphtheria 21–22 Haemophilus influenzas type B (HIB) 22 hepatitis A 22–23 hepatitis B, 23–24 herpes zoster 31–32 HIV 21 of infectious diseases 19–32 influenza 25–26 lower respiratory tract infections (LRTIs) 122 measles 27 meningococcal infection 27–28, 35 mumps 27 pneumococcal 29, 154 polio 29–30 in preterm infants 64–65 procedure 19–20 rabies 35–36 routine schedules 20, 459 rubella 27 tetanus 30 in travellers see under Travellers tuberculosis 31 varicella 31–32 whooping cough (pertussis) 28–29

503

Impaired glucose tolerance 153 Impetigo 374–375 incubation and infectivity periods 459 Incapacity Benefit 5 Incident pain management 430 Income support 4 Incontinence in elderly 280 faecal 139–140 overflow 280 stress 279 urge 279–280 urinary 278–281 Indecent assault victims 16–17 Independent Living Fund 6 Indians, prepregnancy care 254 Industrial Injuries Disablement Benefit 5 Infancy 64–75 asthma 116 constipation 67 excessive crying (colic) 66 nappy rash 67 pain management 422 preterm see Preterm infants seborrhoeic eczema and dandruff 368 vomiting 65 watering/discharge from eyes 66–67 see also Child health promotion; Child protection; Children Infectious diseases 19^0 contact with during pregnancy 261–263 in Down syndrome 412 immunizations 19–32 live vaccines 20–21 procedures 19–20 routine schedules 20, 459 in travellers see under Travellers vaccine damage payments 20 see also Immunizations incubation and infectivity periods 459 notification 460 skin see Skin infections Infectious mononucleosis 349 Infertility 246–247 Inflammatory bowel disease (IBD) 140–142 Crohn's disease 141–142 ulcerative colitis 140–141 Influenza 25–26 Influenza vaccination 25–26 in diabetic patients 154 Inguinal hernias, in children 84 Inhalers, choice for asthma patients of different ages 118 'Innocent' murmurs, in children 55–56 Insomnia 314 in palliative care 434 Intellectual disability 409^11

504

INDEX

Interferon–beta, multiple sclerosis treatment 203 Intermenstrual bleeding 212 Intermittent claudication 292 International Prostate Symptom Score (IPSS) 475 In–toeing, in children 85–86 Intractable heart failure 102 Intrapartum care 268 Intrauterine devices (IUD) 238–240 Intrauterine system (Mirena) 240 Invalid Care Allowance 5 Iron in children 53 deficiency 415 see also Anaemia supplementation 415–416 Irritable bowel syndrome (IBS) 136–137 Ischemic vascular dementia, treatment 396 Itch see Pruritus (itch)

J Japanese encephalitis 34 Jaundice 144 neonatal 271 in returning travellers 39 Jewish population, prepregnancy care 254

K Kidney disease 284–285 in diabetic patients 151 Knee problems 169–170 acute injury 289 injections 170 Knock knees, in children I

L Lacerations 297 Lactation 52, 267–270 failing 269 suppression of 270 Language disorders, in children 58 Lassa fever, in returning travellers 38 Learning disability 409^11 contraception and 244 driving and 9 Leflunomide, adverse effects and monitoring 483 Legionnaires' disease, in returning travellers 37–38 Leukotriene antagonists, asthma treatment 117

Levodopa adverse effects 197–198 Parkinson's disease treatment 197 Lice 377–378 Lichen planus 376 Lifestyle changes advice 442^147 osteoarthritis management 163 Limping child 86 Lipids coronary heart disease (CHD) prevention 110 in diabetic patients 150–151 screening 448 Lip reading 338 Listeria, pregnancy and 256, 262 Lithium, bipolar affective disorder treatment 308 Liver amoebic abscess, in returning travellers 38 problems, oral contraception and 229 Liver (food) avoidance in pregnancy 256 Live vaccines 20–21 Living wills 15, 436 Locomotor disability, effect on fitness to fly 12 Lofexidine, detoxification 329 Low back pain 164–167 advice and exercises 167 in elderly 167 management 165–166 nerve root compression 166 possible serious spinal pathology 167 psychosocial blocks to recovery 165 referrals 166 x–rays 166–167 Lower limb problems, in elderly 398–399 Lower respiratory tract infections (LRTIs) 121–122 Low income patients, financial benefits 4–5 Lymphoedema, in palliative care 435

M Macmillan Cancer Relief Grants 426 Macrocytic anaemia 416–417 Macular degeneration, age–related 361 Magnesium, dysmenorrhoea treatment 209–210 Malaria 34–35 in immigrants and their children 35 incubation and infectivity periods 459 prophylaxis 34–35

in returning travellers 37 treatment 35 Males, victims of sexual assault 17 Marriage mental capacity to consent 14 strife effects on children 64 Mastitis 270 Mastoiditis 336 Maternity Allowance (MA) 4 Maternity benefits 4 Measles immunization 27 incubation and infectivity periods 459 Measles, mumps and rubella (MMR) vaccine 26–27 Medical certificates 1–4 Benefits Agency Medical Service involvement 3 employment rehabilitation 3 food handlers 4 maternity benefits 4 Statutory Sick Pay qualification 1–2 Medical evidence, under The Children Act 64 Medical treatment, mental capacity to consent 14–15 Melanoma 379 Meniere's disease 341–342 Meningococcal infection immunization 27–28, 35 incubation and infectivity periods 459 prophylaxis 28 Menopause 217–223 urinary tract infections (UTI) after 278 see also Hormone replacement therapy (HRT) Menorrhagia 210–211 Menstrual problems 209–213 amenorrhoea 212–213 dysmenorrhoea 209–210 endometriosis 210 hormonal contraceptives adverse effects 232, 233–234, 235, 236–237 intermenstrual bleeding 212 irregular periods 211–212 IUD adverse effects 239–240 menorrhagia 210–211 migraine 191 premenstrual syndrome 216–217 Mental capacity 13–15 to consent to marry and engage in sexual intercourse 14 to consent to medical treatment 14–15 Court of Protection 14 power of attorney 14, 399–400 testamentary capacity 14 Methadone maintenance 329 reduction programme 328–329

INDEX

Methotrexate, adverse effects and monitoring 481 Microalbuminuria, in diabetic patients 151–152 Migraine 189–191 menstrual 191 recurrent, in children 83 see also Headaches Migrainous neuralgia 191 Miscarriages 259–261 previous 254 recurrent 254 suspected after 14 weeks pregnancy 261 up to 14 weeks pregnancy 259–260 Mites 378 Mixed dementia, treatment 396 MMR vaccine 26–27 Mobility loss in elderly 391–393 Morphine in elderly 428–429 equivalents 429 oral 428 Motor neurone disease 202 Mouth problems 347–352 in children 54 gum disease 351 in palliative care 434–435 sore mouth 350–351 teeth see Dental problems Multiple sclerosis 202–204 Mumps immunization 27 incubation and infectivity periods 459 Munchausen's syndrome by proxy (MSBP) abuse 62 Murmurs, 'innocent/ in children 55–56 Musculoskeletal problems / disorders nonsteroidal anti–inflammatory drugs 171 spasm in palliative care 435 Myocardial infarction (MI) 96–99 6–week assessment 98–99 contraindications to thrombolysis 97 driving and 9 management immediate 96–97 subsequent home treatment 97–98 Myotomes 478 Myringitis bullous 336–337

N Nails, fungal infections 376 Nappy rash 67 Nasal polyps 346

National Asthma Campaign 'Be in Control' action plan 119 National Framework for Mental Health 301–302 National Health Service (NHS), benefits 5 Natural family planning 241–242 Nausea end–stage heart failure 102 palliative care 431–432 Neck pain 167–168 Needle phobia 317 Neglect, in children 62 Neonatal jaundice 271 Neonate, acute infective conjunctivitis 356 Nerve root compression, in low back pain 166 Neural tube defects, in previous pregnancies 255 Neuritis, retrobulbar 360 Neurogenic bladder 278 Neurological problems 189–207 driving and 8 see also individual disorders Neuropathic pain 423–424 management 430 Nicotine replacement therapy (NRT) 332 Night cramps in children 83–84 in elderly 398 Nightmares, in children 74 Night terrors, in children 74 Nociceptive pain 423 Non–migrainous headache 191–192 Non–specific urethritis (NSU) 43 Nonsteroidal anti–inflammatory drugs (NSAIDs) 171–173 adverse drug reactions 171 dyspepsia 173 in elderly 172 gastrointestinal toxicity 172 peptic ulcer 133–134 risk of bleeds by age 172 classes 173 contraindications 172 dysmenorrhoea treatment 209 in elderly 171 menorrhagia treatment 211 musculoskeletal disorder management 171 osteoarthritis management 162 pain management 421–422 prescribing practice 172 rheumatoid arthritis treatment 176 Norethisterone enanthate (NE) 236 Noristerat 236 Normochromic normocytic anaemia 416 Northern Ireland, compulsory admission under Mental Health Act 1986, 304 Nose problems 344–347

505

Notification, infectious diseases 49, 460 diarrhoea in children 68–69 Nottingham Extended Activities of Daily Living questionnaire (EADL) 487 Nutrition, child health promotion 52–54

o Obesity advice on 443–444 diabetic patients 149, 152 Obstetrical table 473 Obstetric problems 253–273 see also Pregnancy Obstructive sleep apnoea (OSA) 346–347 in Down syndrome 412 Occupational disease reporting 17 Oedema acute pulmonary 103 in end–stage heart failure 102–103 postural ankle, in elderly 399 Oestrogen adverse effects 221 in HRT 219, 220 menstrual problems and 216 Opiate misuse 325–329 Opioids adverse effects 428 equivalents 429 tolerance and addiction 427–428 use in dying patient 427–430 Oral allergy syndrome 383 Oral contraceptive see Combined oral contraceptive (COC) Oral thrush (candidiasis) 349 Oral ulcers 350–351 Orgasm disorders 250 Orthopaedic problems in children 85–87 trauma in primary care 289–292 Osgood Schlatter's disease 86 Osteoarthritis 161–164 disability aids 163 management 162–164 referrals to specialist 163 self–management 161–162 x–rays 163 Osteoporosis 180–183 advice on 446 disability and 411 in Down syndrome 412 high–risk patients 180 management 181–182 screening 454 steroid–induced 182 work–up 180–181 Otitis externa 337 Otitis media with effusion (OME) 339–340

506

INDEX

Ottowa Ankle Rules 290 Ottowa Knee Rules 289 Ovarian cancer, screening 451–452 Overflow incontinence 280 Overweight, diabetic patients 152 Oves cap 241

P

Pacemaker, driving and 9 Pain management acute 421–422 bone pain 430 central 424 in children 422 chronic non–malignant 422–423 drug levels 480 drug stabilities 489–491 management 421–424 neuropathic pain 430 nociceptive pain 423 in palliative care 430 neuropathic 423–424 postoperative 297–298 visceral pain 430 see also Analgesia Palliative care 425–436 anorexia and weakness 432–433 bladder spasm 435 bowel obstruction, malignant 432 constipation 433 diarrhoea 433 financial aspects 425–426 home environment 425 insomnia 434 itch 434 lymphoedema 435 mouth and throat problems 434–435 muscle spasm 435 nausea and vomiting 431–432 psychiatric reactions 433 raised intracranial pressure 435 respiratory symptoms 433–434 spinal cord compression 434 support 426 symptom control in dying patient 426–430 pain 423–424, 426–427 strong opioids 427–430 syringe drivers 431 talking to patient 425 ulcer, malignant 435 Panic disorder 316–317 Papular urticaria 378 Paraplegia 204–205 Paratyphoid fever, in returning travellers 37 Parkinsonian symptoms, adverse effect of schizophrenia treatment 306 Parkinson's disease 196–198

Paroxysmal hemicrania, chronic 192 Paroxysmal supraventricular tachycardia 105–106 Patient education in diabetes mellitus 153 in polymyalgia rheumatica 184 in schizophrenia 305 Patients, violent types 317–318 Pelvic infection, lUD–induced 239 Pelvic inflammatory disease (PID) 214–216 Penicillamine, adverse effects and monitoring 482 Pensions, for war disabled and dependents 5–6 Peptic ulcer, NSAIDs–induced 133–134 Periods, problems with see Menstrual problems Peripheral nerve testing 479–480 Peripheral vascular disease (PVD) 292–294 abdominal aortic aneurysm (AAA) 294 Buerger's disease 294 drug treatment 294 intermittent claudication 292 Raynaud's phenomenon 294 Pertussis see Whooping cough (pertussis) Pharyngitis, in returning travellers 39 Phobias 317 Physical abuse, in children 61 Pityriasis versicolor 375 Pneumococcal immunization 29 in diabetic patients 154 Polio immunization 29–30 Polycystic ovary syndrome (PCOS) 213 Polymyalgia rheumatica 183–184 Polyps, nasal 346 Post–cholecystectomy syndrome 295 Postgastrectomy 143 Post–herpetic neuralgia (PHN) 424 Post–laryngectomy 350 Postmenopausal women, osteoporosis management 181–182 Postnatal care 268–271 breast–feeding 52, 269–270 depression 270, 313–314 fever 270 mastitis 270 neonatal jaundice 271 vaginal bleeding after 24 hours 270 Postoperative complications 297–299 Post–traumatic stress disorder (PTSD) 315 Postural ankle oedema, in elderly 399 Power of attorney 14, 399–400 Pre–eclampsia 264 Pregnancy abdominal pain 261 abnormal lie 266

anaemia 256, 265 antenatal care 256–259 asthma and 267 bacteriuria 265 diabetic patients 152–153, 255, 267 gestational diabetes 265 drug misuse 268 eclampsia 264 ectopic pregnancy 257, 259 Edinburgh Postnatal Depression Scale 474 epilepsy and 194, 255, 267 flying fitness 12 glycosuria 265 high head 266 hypertension 255, 264–265 infection or contact with infectious disease 261–263 see also individual infections intrapartum care 268 IUD and 239 miscarriage see Miscarriages obstetrical table 473 post–maturity 266 postnatal care see Postnatal care pre–eclampsia 264 prenatal diagnosis and screening 258–259 prepregnancy care 253–256 problems 259–268 proteinuria 264–265 psychiatric history 255–256 rhesus status 259 smoking cessation 332–333 termination 245–246 thyroid disorders 267 urinary tract infections (UTI) 278 vaginal bleeding 259–261 Premature ejaculation 250 Premenstrual syndrome 216–217 Prescriptions, free entitlements 6 Pressure ulcers, in paraplegic patients 204 Preterm infants 64–67 feeding 64, 65 immunization 64–65 respiratory infection 65 weight and development 64 Prion diseases 395 Progestogen–only pill (POP) 234–235 Progestogens adverse effects 221–222 in HRT 220 injectable contraceptives 236–237 menorrhagia treatment 211 Prostaglandin inhibitors, menstrual problems and 216 Prostate carcinoma 283 counselling 283 International Prostate Symptom Score (IPSS) 475 screening 452–453 Prostate–specific antigen (PSA) 282 Prostatitis 277

INDEX

Proteinuria 284 in pregnancy 264–265 Proton pump inhibitors (PPIs) 133–134, 135 Pruritus (itch) in palliative care 434 in pregnancy 263 Psoriasis 370–373 guttate 370, 372 localized plaque 371–372 pustular 370 scalp 372 widespread plaque 372 Psychiatric problems 301–320 driving and 9 National Framework for Mental Health 301–302 reactions in palliative care 433 unexplained somatic complaints 318 violent patients 317–318 Psychotic disorders 302–304 compulsory admission 303–304, 487–488 compulsory community treatment 304 driving and 304 Early Warning Signs Form 488 follow–up 304 voluntary admission 304, 488 Puberty 82 stages 465 Pulmonary oedema, acute 103 Pustular psoriasis 370 Pyelonephritis 278 Pyrexia see Fever

R Rabies immunization 35–36 incubation and infectivity periods 459 Radiculopathy 168 Radiotherapy, thyroid disease postexposure 156 Raised intracranial pressure (RIP) 192 in palliative care 435 Ramsay Hunt syndrome 337 Rape victims 16–17 Raynaud's phenomenon 294 Reflex sympathetic dystrophy 424 Reflux 133, 134–135 in pregnancy 263 Renal disease 284–285 in diabetic patients 151 Renal insufficiency 285 Respiration FEVj/FVC charts 468 peak expiratory flow in normal subjects 467 peak flow values in children 466

Respiratory problems 115–129 children 75–79 infections in preterm infants 65 in palliative care 433–434 see also individual disorders Restless legs syndrome, in elderly 398–399 Retarded ejaculation 250 Retinal artery/vein occlusion 359 Retinal detachment 360 Retinoids, topical, acne treatment 368–369 Retrobulbar neuritis 360 Rhesus–negative mothers 266 Rheumatoid arthritis 175–178 non–drug management 177 pharmacological treatment 176–177 prereferal work–up 176 surgery 177 Rheumatological problems 161–188 see also individual disorders Rhinitis 345–346 Ringworm, incubation and infectivity periods 459 Rinne's test 340 Rosacea acne 370 Rubella 459 immunization 27 pregnancy and 256, 261

sss Salt, intake children 133, 134–135 in heart failure 100 in hypertension 91 Salt, intake in children 53 Scabies 377, 459 Scalp psoriasis 372 Scarlet fever 459 Schistosomiasis, in returning travellers 38, 39 Schizophrenia 304–307 carers' needs 307 driving and 9 drug treatment 305–307 management 305 referral 305 School refusal 84 Sciatica 166 Scoliosis, in children 86–87 Scotland, compulsory admission under Mental Health Act 1984 304 Screening 447–454 ankylosing spondylitis 178 blood pressure 447–448 breast cancer 291–292, 450–452 cervical cancer 223–224, 452 in children see under Child health promotion colorectal cancer 136, 449–450

507

coronary heart disease 448 deafness 338 diabetes 453 glaucoma 453–454 hepatitis B 25 hepatitis C 25 of immigrants 40 lipids 448 osteoporosis 454 ovarian cancer 451–452 prenatal 258–259 prostate carcinoma 452–453 in returning travellers 39–40 Scuba diving fitness 13 Seatbelt, exemption from wearing one 11 Seborrhoeic eczema 368 Secretory otitis media 339–340 Seizures, driving and 8 Semen, normal values 246 Sexual abuse, in children 61–62 Sexual intercourse, mental capacity to consent 14 Sexually transmitted infections (STIs) 41–49 anogenital warts 44 chlamydia infection 43 contact tracing 42 diagnostic tests 42 gonorrhoea 42–43 herpes genitalis 44–45 HIV and AIDS, 45–49 see also AIDS; HIV non–specific urethritis (NSU) 43 in returning travellers 39 syphilis 45 tests 42 treatment principles 42 Trichomonas vaginalis 43–44 Sexual problems 247–250 dyspareunia 249–250 erectile dysfunction 152, 248–249 lack of desire 248 in multiple sclerosis 204 orgasm disorders 250 vaginismus 249–250 Shoulder problems 169 Sickle–cell disease 254, 417–418 Sick sinus syndrome 106 Sildenafil, oral, erectile dysfunction treatment 248–249 Sinusitis 344–345 Skin cancer 378–380 basal cell carcinoma 378–379 melanoma 379 solar keratoses 379–380 squamous cell carcinoma 379 Skin infections 373–377 bacterial 374–375, 459 fungal 375–377 candidiasis 213–214, 375 seborrhoeic dermatitis and dandruff 368

508

INDEX

Skin infections (contd) viral 373 herpes simplex labialis 350, 373–374 herpes zoster 31–32, 337, 374 warts 373 Skin problems 365–382 alopecia 376–377 gravitational ulcers 380–381 hyperhidrosis 380 infections see Skin infections infestations 377–378 scabies 377, 459 lichen planus 376 malignancy see Skin cancer urticaria 381 see also Acne; Dermatitis; Eczema; Psoriasis Sleep apnoea, obstructive 346–347 in Down syndrome 412 Sleep problems in children 72–74 Sleep walking, in children 74 Smear test 223 Smoking 331 advice on 444–445 in diabetic patients 150–151 in elderly 389 passive 445 in stroke patients 200 Smoking cessation 331–333 peripheral vascular disease management 293 Sniffing substances 330–331 Snoring 346–347 Social fund payments 5 Soiling, in children 71–72 Solar keratoses 379–380 Solvent abuse 330–331 Somatic events, unexplained complaints 318 Spasticity in multiple sclerosis 203 in paraplegic patients 204 Speech disorders, in children 58 Spina bifida, prenatal screening 258 Spinal cord compression, in palliative care 434 Spondyloarthropathies 178–179 Squamous cell carcinoma (SCC) 379 Squint, in children 58 Staphylococcal infection implications for food handlers 4 recurrent skin infection 375 Statins, coronary heart disease (CHD) prevention 110 Status epilepticus 192 in children 70 Statutory Maternity Pay (SMP) 4 Statutory Sick Pay (SSP) 1–2 Sterilization 244–245 reversal 245 Steroids inhaled asthma treatment 117 COPD treatment 124–125

osteoporosis induction 182 polymyalgia rheumatica management 185 rheumatoid arthritis treatment 176–177 temporal arteritis management 185 topical, eczema and dermatitis treatment 366–367 Stimulants 330 St John's wort, depression management 312 Stomach ulcer 133 Strawberry naevus, in children 84 Streptococcal infection, implications for food handlers 4 Stress incontinence 279 Stress reactions 314–315 Stridor, in palliative care 434 Stroke 198–201 acute 199 driving and 8, 200 management 199 minor 200–201 prevention of recurrence 199–200 Strongyloides, screening in returning travellers 39 Styes 358 Substance abuse 321–334 Sugar, in children 54 Suicide 438 depressed patients 309–310 Sulphasalazine, adverse effects and monitoring 482 Sun exposure, advice on 445–446 Superficial spreading melanoma (SSM) 379 Superficial wounds 296–297 Supervision Order 63 Surgery oral contraceptives and 233 problems 287–300 in children 84–87 returning to work 299 Syphilis 45 Syringe drivers 431

T Tachycardia paroxysmal supraventricular 105–106 ventricular 106 Tardive dyskinesia, adverse effect of schizophrenia treatment 306 Tay Sachs trait 254 Temper tantrums 74–75 Temporal arteritis 184–185, 359 management 184–185 Tenosynovitis, De Quervain's 169 Tension–type headache 191 Terminal illness, financial benefits 5 Termination of pregnancy 245–246 Testamentary capacity 14

Testes, undescended 55 Tetanus immunization 30 incubation and infectivity periods 459 Tetanus–prone injury 30–31 Thalassaemia 254, 418 Thiazides, hypertension treatment 92 Throat problems 347–352 lump 350 in palliative care 434–435 sore throat 347–349 Thrombolysis, contraindications in myocardial infarction 97 Thrombophilia 418–419 Thunderclap headache 192 Thyroid disease 154–157 after radiotherapy or partial thyroidectomy 156 in Down syndrome 411–412 goitre in euthyroid patient 155–156 hirsutism 157–159 pregnancy and 267 Thyroidectomy, postsurgical thyroid disease 156 Tick–borne encephalitis 36 Time zones, effect on fitness to fly 12 Tinea capitis 375 Tinea pedis, cruris and corporis 376 Tinnitus 342–343 Toddler's diarrhoea 69 Toilet training 70 Tonsillectomy 298, 349 indications for referral 349 Toothache 351 Toxoplasmosis, pregnancy and 256, 262 Transient ischaemic attack (TIA) 200–201 see also Stroke Traumatic ulcers 351 Travellers advice and immunizations 32–37 cholera 33–34 Japanese encephalitis 34 malaria 34–35 meningococcal immunization 35 rabies 35–36 suggested schedule 33, 460 tick–borne encephalitis 36 travellers' diarrhoea 33 typhoid 36 yellow fever 36–37 diarrhoea 38–39 fever 37–38 jaundice 39 pharyngitis 39 screening returning travellers 39–40 STIs 39 symptoms in those returning from abroad 37–39 Tremor, essential 205 Trichomonas vaginalis infection 43–44 Tricyclic antidepressants (TCAs) 423 adjuvant analgesia 429–430

INDEX

Trigeminal neuralgia 424 Triglycerides, work–up of patient with raised levels 110 Tuberculosis 122–123 immunization 31 incubation and infectivity periods 459 in returning travellers 38 Typhoid 36, 37

u Ulcer(s) aphthous see Aphthous ulcers duodenal 133 gastric 133 gravitational 380–381 malignant, in palliative care 435 oral 350–351 peptic, NSAIDs–induced 133–134 pressure, in paraplegic patients 204 traumatic 351 Ulcerative colitis 140–141 Ultrasonography 259 Umbilical hernia 85 Undescended testis 55 Unstable angina 95–96 Unsteadiness 340 Upper respiratory tract obstruction 79–80 Urethral syndrome 277 Urethritis, non–specific (NSU) 43 Urge incontinence 279–280 Uricosuric drugs, gout prophylaxis 174 Urinary catheters 281 Urinary problems 275–286 incontinence 278–281 retention in women 283 Urinary stones 283–284 Urinary tract infections (UTI) 275–278 in children 80–81, 278 female cases 275–277 frequency dysuria 277 male cases 277 in multiple sclerosis 203 in paraplegic patients 204 in pregnancy 278 pyelonephritis 278 Urosurgery 298 Urticaria 381 papular 378 Uveitis, recurrent 358

V Vaccinations see Immunizations Vaccine damage payments 6, 20 Vaccines, live 20–21

Vaginal bleeding postnatal 270 during pregnancy 259–261 Vaginal discharge 213 Vaginismus 249–250 Vaginosis, bacterial 214 Varicella immunization 31–32 incubation and infectivity periods 459 pregnancy and 262 Vascular dementia 394 ischemic, treatment 396 Venous thromboembolism 418–419 risk factors 228, 230, 418–419 Ventricular tachycardia 106 Vertigo 341 benign positional 342 Vestibular rehabilitation exercises 341 Victims of crime or accidents 16–17 Vincent's angina 349 Violent patients 317–318 Viral hepatitis see Hepatitis Viral infections of skin see under Skin infections Visceral pain management 430 Visual problems acuity testing 359 in children 57 decreased vision with pain 360 in diabetic patients 152 distortion of vision 360 disturbance 359–360 driving effects 10 floaters and flashes 360 gradual loss of vision 360–362 handicap 362–363 HIV patients 48 painless loss of vision 359–360 see also Eye problems Vitamin B6, menstrual problems and 216 Vitamin B12 deficiency 417 Vitamins, in children 53 Vitreous detachment 360 Vitreous haemorrhage 360 Voiding difficulty 281–283 Volatile substance abuse 330–331 Vomiting implications for food handlers 4 in infants 65 in palliative care 431–432 in pregnancy 263 Vulvovaginitis, in children 81

w Wales, compulsory admission under Mental Health Act 1983 303

509

Warfarin, venous thromboembolism management 419 War pensions 5–6 Warts 373 genital 44, 256 Weakness in end–stage heart failure 102 in palliative care 432–433 Weaning 53 Weber's test 340 Weight gain excessive, in pregnancy 263 hormonal contraceptives side effect 237 Wheelchairs 407 Whiplash injury 289 Whisper test 340 White breath–holding attacks, in children 75 Whooping cough (pertussis) in children 76–77 immunization 28–29 incubation and infectivity periods 459 treatment and prophylaxis 29 Wills, living 15 Withdrawal bleeds (WB), absent, oral contraceptive–induced 234 Work, return to after operation 299 World Health Organization analgesic ladder 421 Wounds, superficial 296–297 Wrist problems 169

X Xerostomia, in palliative care 434

Y Yellow fever 36–37

z Zanamivir, influenza prophylaxis 26

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this Agreement by its initial use of the MULTIMEDIA PROGRAM If you transfer the MULTIMEDIA PROGRAM, you must remove the installation files from your hard disk and you may not retain any copies of those files for your own use. 4. Limited Warranty and Limitation of Liability. For a period of sixty (60) days from the date you acquired the MULTIMEDIA PROGRAM from us or our authorised dealer, we warrant that the media containing the MULTIMEDIA PROGRAM will be free from defects that prevent you from installing the MULTIMEDIA PROGRAM on your computer. If the disk fails to conform to this warranty you may as your sole and exclusive remedy, obtain a replacement free of charge if you return the defective disk to us with a dated proof of purchase. Otherwise the MULTIMEDIA PROGRAM is licensed to you on an "AS IS" basis without any warranty of any nature. WE DO NOT WARRANT THAT THE MULTIMEDIA PROGRAM WILL MEET YOUR REQUIREMENTS OR THAT ITS OPERATION WILL BE UNINTERRUPTED OR ERROR–FREE. THE EXPRESS TERMS OF THIS AGREEMENT ARE IN LIEU OF ALL WARRANTIES, CONDITIONS, UNDERTAKINGS, TERMS AND OBLIGATIONS IMPLIED BY STATUTE, COMMON LAW, TRADE USAGE, COURSE OF DEALING OR OTHERWISE ALL OF WHICH ARE HEREBY EXCLUDED TO THE FULLEST EXTENT PERMITTED BY LAW, INCLUDING THE IMPLIED WARRANTIES OF SATISFACTORY QUALITY AND FITNESS FORA PARTICULAR PURPOSE. WE SHALL NOT BE LI ABLE FOR ANY DAMAGE OR LOSS OF .ANY KIND (EXCEPT PERSONAL INJURY OR DEATH RESULTING FROM OUR NEGLIGENCE) ARISING OUT OF OR RESULTING FROM YOUR POSSESSION OR USE OF THE MULTIMEDIA PROGRAM (INCLUDING DATA LOSS OR CORRUPTION), REGARDLESS OF WHETHER SUCH LIABILITY IS BASED IN TORT, CONTRACT OR OTHERWISE AND INCLUDING, BUT NOT LIMITED TO, ACTUAL, SPECIAL, INDIRECT, INCIDENTAL OR CONSEQUENTIAL DAMAGES. IF THE FOREGOING LIMITATION IS HELD TO BE UNENFORCEABLE OUR MAXIMUM LIABILITY TO YOU SHALL NOT EXCEED THE AMOUNT OF THE LICENSE FEE PAID BY YOU FOR THE MULTIMEDIA PROGRAM. THE REMEDIES AVAILABLE TO YOU AGAINST US AND THE LICENSORS OF MATERIALS INCLUDED IN THE MULTIMEDIA PROGRAM ARE EXCLUSIVE. 5. Termination. This license and your right to use this MULTIMEDIA PROGRAM automatically termipate if you fail to comply with any provisions of this Agreement, destroy the copy of the MULTIMEDIA PROGRAM in your possession, or voluntarily returh the MULTIMEDIA PROGRAM to us. Upon termination you will destroy all copies of the MULTIMEDIA PROGRAM and documentation. 6. Miscellaneous Provisions. This Agreement will be governed by and construed in accordance with English law and you hereby submit to the non–exclusive jurisdiction of the English Courts. This is the entire agreement between us relating to the MULTIMEDIA PROGRAM, and supersedes any prior purchase order, communications, advertising or representations concerning the contents of this package. No change or modification of this Agreement will be valid unless it is in writing and is signed by us.

CD-ROM Instructions MINIMUM SYSTEM REQUIREMENTS PC IBM PC or compatible 486SX or higher Microsoft Windows 95 or later Color monitor with SVGA (800 x 600 resolution, 256 colors) or better, 16 MB RAM (32 MB recommended) 2x speed CD-ROM drive or faster Internet Explorer 4.0 or later Anole Mac G3 processor, OS9, 64 MB RAM or better Netscape 4 7 ' 4.7 ' INSTALLATION INSTRUCTIONS PC Insert the CD into the CD-ROM drive. The CD will run automatically. Click the 'Install' button and follow the instructions on screen. Once installation is complete click on the 'Practical General Practice' icon on your desktop to run the program.

Alternatively, from the 'Start' menu, select 'Run' and browse to where the program is installed, selecting the file 'home.htm' to open the application. The Default directory for installations is C:/Program Files/Practical General Practice/home.htm. Mac If you have enabled CD-ROM autoplay on your system (see below) then the CD will run automatically when inserted into your CD drive. If you have not enabled autoplay. click on the CD icon that appearson your desktop, then click on PGP to open the application. Click on the install button and follow the instructions. When installation is complete click on the 'Practical General Practice' icon on your desktop to run the program or navigate to the directory where you installed the program and click on 'home.htm'. ln E.mail: To enable the CD to autoplay, select Control Panels from the Apple menu on your desktop. Select QuickTime Settings, then select Autoplay. Click the Enable CD-ROM Autoplay checkbox then save the settings.

BROWSERS This application is accessed via your web browser. For all functions to work correctly we recommend PC users use Internet Explorer 4.0 or later and Mac users use Netscape 4.7. Internet Explorer 5 (for PC) and Netscape 4.7 (Mac) are available for installation from the 'Browsers' folder on the disk. Please refer to the help files on those programs for problems specific to the browser. You do not need to access the Internet to run the application, For further details on running the CDROM and some answers to frequently asked questions click the 'Read me' button on the opening screen. TECHNICAL SUPPORT theUKcdrorn@es|O co uk Tel• + 44 (0)20 7611 4202 ln the USA: E-mail: [email protected] Tel • + 1 800 692 9010