Management of Cleft Lip and Palate in the Developing World

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Management of Cleft Lip and Palate in the Developing World Edited by

MICHAEL MARS Great Ormond Street Hospital NHS Trust, London

DEBBIE SELL Great Ormond Street Hospital NHS Trust, London

ALEX HABEL Great Ormond Street Hospital NHS Trust, London

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Management of Cleft Lip and Palate in the Developing World

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Management of Cleft Lip and Palate in the Developing World Edited by

MICHAEL MARS Great Ormond Street Hospital NHS Trust, London

DEBBIE SELL Great Ormond Street Hospital NHS Trust, London

ALEX HABEL Great Ormond Street Hospital NHS Trust, London

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C 2008 Copyright

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John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex PO19 8SQ, England Telephone (+44) 1243 779777

Email (for orders and customer service enquiries): [email protected] Visit our Home Page on www.wiley.com 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, scanning or otherwise, except under the terms of the Copyright, Designs and Patents Act 1988 or under the terms of a licence issued by the Copyright Licensing Agency Ltd, 90 Tottenham Court Road, London W1T 4LP, UK, without the permission in writing of the Publisher. Requests to the Publisher should be addressed to the Permissions Department, John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex PO19 8SQ, England, or emailed to [email protected], or faxed to (+44) 1243 770620. Designations used by companies to distinguish their products are often claimed as trademarks. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The Publisher is not associated with any product or vendor mentioned in this book. This publication is designed to provide accurate and authoritative information in regard to the subject matter covered. It is sold on the understanding that the Publisher is not engaged in rendering professional services. If professional advice or other expert assistance is required, the services of a competent professional should be sought. Other Wiley Editorial Offices John Wiley & Sons Inc., 111 River Street, Hoboken, NJ 07030, USA Jossey-Bass, 989 Market Street, San Francisco, CA 94103-1741, USA Wiley-VCH Verlag GmbH, Boschstr. 12, D-69469 Weinheim, Germany John Wiley & Sons Australia Ltd, 42 McDougall Street, Milton, Queensland 4064, Australia John Wiley & Sons (Asia) Pte Ltd, 2 Clementi Loop #02-01, Jin Xing Distripark, Singapore 129809 John Wiley & Sons Canada Ltd, 6045 Freemont Blvd, Mississauga, ONT, L5R 4J3, Canada Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books. Library of Congress Cataloging-in-Publication Data Management of cleft lip and palate in the developing world/edited by Michael Mars, Debbie Sell, and Alex Habel. p. ; cm. Includes bibliographical references and index. ISBN 978-0-470-01968-9 (pbk. : alk. paper) 1. Cleft lip–Developing countries. 2. Cleft palate–Developing countries. I. Mars, Michael. II. Sell, D. A (Debbie A.) III. Habel, Alex. [DNLM: 1. Cleft Lip–surgery. 2. Cleft palate–surgery. 3. Child. 4. Cleft Lip–rehabilitation. 5. Cleft Palate–rehabilitation. 6. Developing Countries. 7. Medical Missions, Official. WV 440 M2665 2008] RD524.M288 2008 617.5 225091724–dc22 2007035540 British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library ISBN 9780470019689 Typeset in 10/12 pt Times by Aptara Inc, New Delhi, India Printed and bound in Great Britain by insert This book is printed on acid-free paper responsibly manufactured from sustainable forestry in which at least two trees are planted for each one used for paper production.

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Contents

List of Contributors vii Foreword xi Introduction 1 Michael Mars, Debbie Sell and Alex Habel Part I

SURGERY AND ANAESTHESIA

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Exporting Plastic Surgical Care to Developing Countries Evan S. Garfein, Jacqueline Hom and John B. Mulliken

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So You Want to Help in a Less Developed Country? Bruce Richard

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Challenges for Cleft Care in the Developing and the Developed World Brian Sommerlad

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Cleft Lip and Palate Management in the Developing World: Primary and Secondary Surgery and Its Delivery 37 James Lehman

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Anaesthesia for Cleft Lip and Palate Surgery in the Developing World Sarah Hodges and Isabeau Walker

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APPROACHES TO ORGANIZATION

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Operation Smile 59 Bill Magee

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Rotaplast International: A Study in Medical Volunteerism Angelo Capozzi

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Logistics and Nursing Issues Priscilla Jurkovich

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CONTENTS

The Sri Lankan Cleft Lip and Palate Project 95 Sanath P. Lamabadusuriya and Michael Mars

Part III 10

FACIAL GROWTH RESEARCH

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Facial Growth in Cleft Lip and Palate Subjects Michael Mars

Part IV

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MEDICAL MANAGEMENT, DISABILITY, PSYCHOLOGICAL AND SOCIAL ASPECTS

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Paediatric Care in Developing Countries: An Integrated (Holistic) Approach 127 Albert C. Goldberg and Alex Habel

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Disability, Culture and Cleft Lip and Palate Mary Wickenden

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Psychological and Social Aspects of CL/P in the Developing World, Including Implications of Late Surgery or No Surgery 159 Eileen Bradbury and Alex Habel

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The Background, Establishment and Function of a Parents/Patients Support Group in Sri Lanka 173 Parakrama Wijekoon

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SPEECH AND AUDIOLOGY

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Speech in the Unoperated or Late Operated Cleft Lip and Palate Patient 179 Debbie Sell

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Speech Therapy Delivery and Cleft Lip and Palate in the Developing World 193 Debbie Sell

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ENT and Audiology Care for Cleft Palate Patients in the Developing World 203 Tony Sirimanna

Index

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List of Contributors

Eileen Bradbury is a consultant psychologist, Associate Fellow of the British Psychological Society and Honorary Senior Clinical Lecturer at the University of London School of Medicine and Dentistry. She has worked for many years with plastic surgeons and in cleft units in the UK, developing approaches to the psychological care of children, adolescents and adults with clefts. In Uganda, in 2002, she collaborated with a surgical team developing ways of assessing individuals prior to cleft repair and measuring psychological and social outcomes. Angelo Capozzi is a plastic surgeon. He was in private practice for 30 years in San Francisco. In 1998, he left private practice and became the Chief of Plastic Surgery at the Shrine Hospital for Children in Sacramento, California. Dr Capozzi has been involved in international service since 1976 and co-founded Rotaplast International Inc. in 1992. In 30 years of international service, he and his colleagues have worked in 16 different countries in South and Central America, as well as China, Vietnam, India, Ethiopia, Romania and the Philippines. Since the first mission in 1993, Rotaplast has operated on over 10,000 children and young adults. Nigel Crisp was Chief Executive of the NHS and Permanent Secretary of the Department of Health from 2000 to 2006. He is leading the international task force on scaling up the training and education of health workers in Developing Countries as a consultant with the Gates Foundation. He published a report for the Prime Minister in February 2007, Global Health Partnerships: The UK Contribution to Health in Developing Countries. He became a member of the House of Lords in 2006. Evan S. Garfein is a Microsurgery and Reconstructive Surgery Fellow at New York University’s Institute for Reconstructive Plastic Surgery. He has worked in Cange, Haiti, as a consultant for Partners in Health, a Boston-based group dedicated to providing comprehensive health-care solutions for the Developing World. Albert C. Goldberg is a paediatrician in private practice with San Rafael Pediatrics in San Rafael, California. For the past 25 years, he has worked in countries such as Argentina, Bolivia, Chile, China, Ecuador, Ethiopia, Honduras, India, Mexico, Venezuela and Vietnam. He is the Director of Pediatrics with Rotaplast International Inc. Alex Habel is consultant paediatrician to the North Thames Regional Cleft Unit based at Great Ormond Street Hospital NHS Trust, London. He has been involved with multidisciplinary cleft teams in Sri Lanka, the Maldives and Venezuela and is

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working in partnership with local medical and dental professionals researching into children’s growth in Sri Lanka and cleft-related genetic conditions in China. Sarah Hodges is an anaesthesiologist in the Children’s Orthopaedic Rehabilitation unit in Kampala. She started her training in anaesthesia in 1990 in the UK before moving to Uganda to work in a rural hospital in the west of Uganda run by the Church. She was involved in training nurses, interns and anaesthetic officers. She and her husband ran a mobile project for over eight months, repairing cleft lips and palates throughout Uganda in 1997–98. She returned to the UK in 1998, to upgrade her training in anaesthesia where she obtained her FRCA. She has now returned to Uganda with her husband where they are both involved in training. Although based in Kampala, they travel around the country doing a variety of reconstructive surgery but especially cleft lip and palate surgery. She also teaches anaesthetic officers and the postgraduate trainees in anaesthesia at Mulago Hospital in Kampala and up country. Jacqueline Hom is a dental student and Presidential Scholar at Harvard School of Dental Medicine, USA. She completed her DMD thesis on the role of traditional Chinese health beliefs on oral health in Beijing, China. In 2007, Jacqueline interned at The Smile Train, United Nations Development Fund for Women (UNIFEM), and the World Health Organization (WHO). Recently, Jacqueline was selected as a Fogarty/ Ellison Global Health and Clinical Research Fellow to research oral manifestations of HIV/AIDS at the GHESKIO Center in Port-au-Prince, Haiti. Priscilla Jurkovich is a clinical nurse educator at Sanford Health in Sioux Falls, South Dakota, USA. She has been involved as the nursing coordinator or a staff registered nurse on surgical mission trips since 1993. Recently, Priscilla has been involved with cleft lip and palate repairs with Rotaplast and Alliance for Smiles in China, Chile, Romania, Colombia and Bolivia. Sanath P. Lamabadusuriya is the Senior Professor of Paediatrics and Chair of the Faculty of Medicine, University of Colombo, Sri Lanka. He is a former Dean of the same faculty. He is also a consultant paediatrician at the Lady Ridgeway Hospital for Children in Colombo. He has been the Co-Director of the Sri Lankan Cleft Lip and Palate Project since its inception when he was the founder Professor of Paediatrics at the University of Ruhuna in Galle. He has over 120 scientific publications to his credit. Jim Lehman is a plastic surgeon in Dacron, Ohio, and an ex-President of the American Craniofacial Cleft Lip and Palate Association. He has worked for many years with Rotaplast teams in South America. Bill Magee founded Operation Smile in 1982, and serves as the organization’s Chief Executive Officer. Dr Magee was awarded the Hays-Fulbright Scholar Grant from the Franco-American Commission where he studied in France with Dr Paul Tessier. He is Director of the Institute for Craniofacial and Plastic Surgery in the Children’s Hospital of The King’s Daughters, Norfolk, VA, and Chair of the Plastic Surgery Department. In addition, Dr Magee is Associate Professor of Plastic Surgery at Eastern Virginia

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Medical School and Professor in the Department of Plastic Surgery at the University of Southern California. Michael Mars is lead consultant orthodontist at the North Thames Regional Cleft Unit, based at Great Ormond Street Hospital NHS Trust, London. In 1984, he founded and continues to direct the Sri Lankan Cleft Lip and Palate Project. He was a joint founder of the first Sri Lankan Speech and Language Therapy training course at the University of Kelaniya, Colombo. He co-founded CLAPA (the Cleft Lip and Palate Association – a UK registered charity) in 1979, and served as its Chairman until 2005. He has worked in multidisciplinary teams in India, the Maldives, and Russia, as well as Sri Lanka. His major research interest is in all aspects of facial growth in cleft lip and palate: its measurement, assessment in cross-centre studies, and the effects of the nature and timing of surgery on the outcome of facial morphology. He has been awarded an honorary D.Sc from Ruhuna University, Sri Lanka, is a visiting professor at the Faculty of Medicine, Peradeniya, and has recently been made an honorary Fellow of the Sri Lankan College of Paediatricians. John B. Mulliken is Director of the Craniofacial Center, Children’s Hospital Boston, and Professor of Surgery at Harvard Medical School. He has written on surgical repair of cleft lip/palate, lectured in England, Italy, Chile, Korea and Taiwan, and worked in Guayaquil and Quito, Ecuador. Bruce Richard is a consultant plastic surgeon in Birmingham, UK, with a 70% primary cleft surgery commitment. He worked as a plastic surgeon, seconded to the Government of Nepal by a Christian aid organization, in a Nepalese government hospital with Nepali colleagues for nine years. He helped establish a regional plastic and burns unit, and trained seven national surgeons. He performed 473 cleft operations, and participated in the development of individual and family support for those with cleft and other congenital abnormalities. He facilitated the creation of a speech and language therapy service for the western half of Nepal, a database for clefts with a research officer, and completed a randomized controlled trial. After returning to the UK, in 2005, he was part of Operation Smile’s inaugural trip to Ethiopia. Debbie Sell is Head of the Speech and Language Therapy Department at Great Ormond Street Hospital NHS Trust, and Lead Speech and Language Therapist, North Thames Regional Cleft Service. She was responsible for the speech and language therapy component of the Sri Lankan Cleft Lip and Palate Project, involving research, treatment and counterpart training. Her PhD was a study of speech outcomes in the unoperated and late operated patient. She was a joint founder of the first Sri Lankan Speech and Language Therapy training course at the University of Kelaniya, Colombo. She has also been involved in projects in China, Russia, the Maldives, and Mount Abu Rajastan. Tony Sirimanna is a consultant audiological physician and the lead clinician for audiology and audiological medicine at Great Ormond Street Hospital NHS Trust, London, where he has worked since 1995. He is a graduate of Colombo Medical

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Faculty and worked as a consultant ENT surgeon in Sri Lanka before becoming an audiological physician. He has been involved with developing audiology services in Sri Lanka over the past decade and also has been a member of the Sri Lankan Cleft Lip and Palate Project. He is a member of the International Association of Audiological Physicians and the International Society of Audiology and has close working relationships with audiologists in a large number of Developing Countries. Brian Sommerlad is consultant plastic surgeon at Great Ormond Street Hospital NHS Trust, London, and ex-Director of the North Thames Regional Cleft Unit. He is on the Medical Advisory Board of Smile Train, and regularly works with surgical teams in Bangladesh, Sri Lanka and Uganda. He is a frequent guest speaker at meetings in India and was the Millard Lecturer at the Indian Cleft Lip and Palate Society meeting in February 2007. Isabeau Walker is consultant anaesthetist at Great Ormond Street Hospital NHS Trust, London. She has worked in Sri Lanka and Uganda. Mary Wickenden teaches on disability and development in the Centre for International Health and Development at University College London. She trained and worked as a speech and language therapist and lectured on undergraduate SLT programmes in the UK. She developed an interest in culture and disability and the ways in which disability services evolve in development settings. She coordinated and taught on the innovative SLT training programme at the University of Kelaniya, Sri Lanka. She has also undertaken teaching and research projects related to disability in India, Uganda, Iran and Bangladesh. She is currently undertaking a PhD in the anthropology of disability at the University of Sheffield. Parakrama Wijekoon is consultant maxillofacial surgeon and currently Head of the Oral and Maxillofacial Surgery Department at the Faculty of Dental Sciences, University of Peradeniya, Sri Lanka. He specializes in cleft lip and palate surgery and is mainly involved with primary surgery. He was the founder of the parent association of Sri Lankan Cleft Lip and Palate Patients, Sanyathya.

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Foreword

This book is about making a difference to people’s lives. It is about dealing with treatable conditions which blight the lives of many millions. While a cleft lip and palate is rarely life-threatening, it causes facial disfigurement (especially if untreated), damages the ability to communicate, and consequentially limits the opportunity for education, employment and the development of relationships. It diminishes the potential of the individual and of the society. In many ways, this is a story of the passion and commitment of individuals who use their skills voluntarily to provide treatment and care, and of the organizations, many of them NGOs, which are making this work possible. But it is also a very practical account of what is being done and what can be done. It reviews and critiques current approaches and draws out the particular issues that need to be addressed in the environments of Developing Countries – which differ so markedly from each other as well as from the Developed Countries where most of the authors live. Moreover, the editors in their Introduction face up to the most difficult social and ethical questions – about cultural differences, about how you can or cannot apply different standards in different countries, about how even the best intended efforts can harm as well as improve, about motivations and about sustainability. They also describe the dilemmas over how much effort should be put into training local people, as opposed to treating local patients. Internationally, there is increasing agreement on the need to help ‘build capacity’ in Developing Countries. This not only means a strong focus on training as well as treatment, but it also requires the development of local health systems alongside specific initiatives such as those on HIV/AIDS, maternal care and, of course, the management of cleft lip and palate. Many of these initiatives currently operate in isolation and are not linked to local priorities and systems. This, in turn, means that donors and Developed Countries need to switch the emphasis from deciding what needs to be done and doing it for people in Developing Countries, to supporting them in doing what they themselves decide needs to be done. Ultimately, leadership is local.

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FOREWORD

This important book brings together current knowledge and ideas. It offers the insights and experiences of many authors from different countries and different disciplines. It shows that a great deal is being done to improve the lives of people who suffer from cleft lip and palate – and provides a valuable platform for doing even more in the future. Nigel Crisp Honorary Professor, London School of Hygiene and Tropical Medicine, Senior Fellow, Institute for Healthcare Improvement; Member of the House of Lords

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Introduction MICHAEL MARS, DEBBIE SELL AND ALEX HABEL

Every year almost a quarter of a million new babies with cleft lip and/or palate are born in the poorest parts of the world where resources are severely limited, scarce or non-existent. Just 17,000 are born every year in the richer world. The majority born in the poorer countries receive very limited or no treatment at all. To add to this burden, it should be stated that this occurs on a cumulative annual basis, resulting in a reservoir of many millions of under-treated and untreated individuals. In India and China alone, this may result in 2.5 million cleft lip and palate subjects for each country over a period of 50 years, assuming this is the minimum average life expectancy. It is estimated that 154 million new babies are born worldwide annually. Of these, 144 million births occur in the so-called Developing or Least Developed World, and just 10 million in the industrial or Developed World. These statistics need to be placed in the context of overall global health care. But, first, the concept of the ‘Developing World’ needs some explanation. The allocation of countries to either the Developing or Developed World is based on data for infant mortality rates. One in 10 children in South Asia dies before their fifth birthday and in Sub-Saharan Africa 175 per thousand children die before they are five years old, compared to six per thousand in industrialized countries. Some 10.8 million children die each year, most from preventable causes, and almost all in poor countries – 30,000 children die each day, one child every three seconds! (Black et al., 2003). Under-nutrition is important as an underlying cause of child death. It is often associated with infectious diseases, multiple concurrent illnesses, pneumonia and diarrhoea, which remain the diseases most associated with child deaths. All the above naturally raises the question, ‘Should we be concerned about cleft lip and palate?’ In the overall picture of global health-care provision, does cleft lip and palate matter? It is rarely life-threatening and, indeed, some consider it to be merely a cosmetic problem. Throughout this book the terms Developed and Developing Countries/World will be used. The terminology used by the Developed World for the Developing World is itself confusing: ‘The Poorest Countries’, ‘the Under-developed World’, ‘the Nonindustrialized Countries’, ‘the Southern Hemisphere’, ‘the Majority World’, ‘the Third World’, ‘Less Economically Developed Countries’ are just some of the labels used to attempt to gain some understanding of the issues involved. Many of these

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Developing Countries consider these terms patronizing and dismissive, perhaps with some justification. For example, modern surgery in the West is considered to have been developed by Hunter just 200 years ago, whereas Sushruta in India wrote texts on surgery, infection control and the use of silk sutures over 2500 years ago. Nevertheless, it is clear that some countries are disproportionately rich and others poor and this at least provides a good starting point for evaluation of an investigation of how the two might share finite resources. The question is further compounded by a common misconception that the Developing World is uniform. Africa, for example, is often referred to as though it were a single country. Neighbouring countries are often grouped together, for example, India and Sri Lanka are both parts of South-East Asia but there are important health-care, social, educational and economic differences. Life expectancy for females in India is 63.4 years compared to 75.4 years in Sri Lanka and 79.9 years in the UK. Under-five mortality in India per thousand is 95 compared to 22.6 in Sri Lanka and 6.5 in the UK. These figures are worse for Sub-Saharan Africa where under-five mortality in some countries is 200 per thousand. Given that so many programmes of care from the Developed World to the Developing World for cleft lip and palate are undertaken by a variety of individuals and organizations, it is clear that at least in the former, cleft lip and palate is accorded some degree of priority for remedial care. A further question now arises: should those in rich countries determine the health-care priorities for poor countries where perhaps the more pressing needs of basic survival pertain? Should they be able to impose their systems of care by virtue of their wealth? In extreme circumstances, does the quality of life have a value when life itself is so frequently challenged? Clinicians and politicians in Developing Countries are increasingly recognizing that the large reservoirs of untreated cleft lip and palate cases in their populations are actually a drain on their economic well-being and a blight on the lives of millions of people. At the basic level of surgical care alone, the cost of repair is small, requiring little in the way of sophisticated technological equipment. Those treated may become accepted members of society, marry, gain an education and employment and attain their full social and economic potential which is denied to those who do not have access to treatment. That both the Developed World and the Developing World are concerned is demonstrated by the large number of collaborative programmes of care presently being undertaken. These programmes in themselves raise many more questions, often more than they can answer. How can we make an ethical significant and sustainable difference? These so-called (and in our view, inappropriately called) cleft lip and palate ‘missions’, which have become a feature of the provision of care from the rich to the poor, are the object of both praise and derision (Mulliken, 2004). Many emphasize ‘head counts’ – the number of clefts per trip. In an attempt to bring ‘healing’ for a variety of congenital and acquired conditions, organizations such as ‘Mercy Ships’ provide surgery performed by non-specialist teams. These are prepared to take on many relatively low-risk procedures but may lack the essential skills to produce an improvement in life experience resulting from poorly performed surgery. There is often little effort

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to involve local surgeons who are left to manage post-operative problems once the visitors have departed. Many teams provide no continuity of care, without offering help in speech, dental care, orthodontics or secondary surgical procedures (Mulliken, 2004). Some teams are politically or religiously motivated, often a cause of concern within the host country. Some take no responsibility for convalescence and the clinicians involved are often very junior with no experience in their own countries of cleft lip and palate work (Natsume, 1998). Visiting surgeons have sometimes used these missions to train their junior staff, and the safety standards have not always been those that are required in their home countries (Dupuis, 2004). Deaths have been reported in which full postmortem investigations would have taken place in the Developed World. ‘Surgical safaris’ and ‘Parachute programmes’ have been the terms used by critics of such visits. Many have argued that the benefits have often been for the visitors rather than the visited. On the other hand, many programmes of care are truly based on the aims of treatment, teaching and training, leading to the goal of self-sustaining care by the host countries. There is a dearth of research into what treatment practices actually work best in the Developing World. The simple export of the same policies of care as provided in the wealthier countries may fail to deliver equivalent results in the Developing World. Where there are no local facilities whatsoever, the goals and aspirations of visiting teams will need to reflect this. Speech therapy and orthodontic provision is often absent even if basic surgery is possible. Palate surgery for older patients may often provide no help for speech and be a misplaced use of precious resources. The Developed World continues to export and promote treatments (Grayson et al., 1999), the benefits of which are questioned, even in their own countries. Non-governmental organizations (NGOs) are significant contributors to the provision of health care, especially in the poorest countries in the Developing World. However, despite their financial power, which some governments may interpret as being politically motivated or religiously slanted, they also compete for available local resources. Clearly, there are many questions and fewer answers to the problems that beset the provision of cleft lip and palate treatment in the Developing World. This book attempts to address these questions. All the contributors have considerable experience in their own countries and in the Developing World. They do not always agree. There is no ‘right answer’. The conditions differ from one country to another and within any one country. Many, including the editors, will admit to having made mistakes in their approach to cleft care in their efforts in the Developing World. They have been guilty of ethnocentricity, patronizing and of being non-cost-effective. They try, but because of cultural misunderstandings even when motivated by the best intentions, they can so easily fail. The question above all still remains ‘whether we have the will to do what needs to be done’ (Lancet, 2003). With goodwill and understanding between the Developing and the Developed World it should be possible to work towards eliminating the scourge of untreated cleft lip and palate from the world. This multiauthor and multidisciplinary book tries to move towards that goal. The management of cleft lip and palate in the Developing World is and will continue to be a challenge

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for both rich and poor countries. With mutual understanding this challenge presents great opportunities for both worlds.

REFERENCES Black RE, Morris SS & Bryce J (2003) ‘Where and why are 10 million children dying every year?’ Lancet 361: 2226–34. Dupuis CC (2004) ‘Humanitarian missions in the Third World: a polite dissent’, Plast Reconstr Surg 113: 433–5. Grayson BH, Santiago PE, Brecht LE & Cutting CB (1999) ‘Presurgical nasoalveolar moulding in infants with cleft lip and palate’, Cleft Pal Craniofac J 36: 486–98. Lancet (2003) ‘Editorial: the world’s forgotten children’, Lancet 361: 1. Mulliken JB (2004) ‘The changing faces of children with cleft lip and palate’, New Engl J Med 351: 745. Natsume N (1998) ‘Safari surgery’, Plast Reconstr Surg 102: 1304–5.

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Surgery and Anaesthesia

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Exporting Plastic Surgical Care to Developing Countries EVAN S. GARFEIN, JACQUELINE HOM AND JOHN B. MULLIKEN

Editorial comment: This chapter is a thought-provoking essay addressing for whom, why, when, where and how plastic surgical support from the Developed to the Developing World should be organized. It limits itself to a surgical review, but nevertheless raises issues that are pertinent to the whole multidisciplinary team.

INTRODUCTION The word ‘surgery’ – from the Greek words cheir, hand, and ergon, work – denotes a branch of medicine that treats disease using the hands. Power is given to those who have the ability to correct deformity and excise disease. For the physician who practices the surgical art, operating is not only the primary therapeutic method, it is also a mindset. This operative inclination and ability have spurred surgeons to go on ‘missions’ to other countries where obvious congenital and acquired deformities often go unoperated. Surgical correction is usually relatively uncomplicated. Groups and individuals travel with different motivations to locales with widely disparate medical capabilities. In this chapter, we evaluate the ‘rules of engagement’ for these humanitarian activities in overseas plastic surgery and discuss the reasons why a single model does not apply in all circumstances. There are several explanations why congenital and acquired deformities are not treated in resource-poor countries. Economic reasons are foremost, i.e., the lack of financial resources in the country’s public health system and often minimal or no payment to surgeons who treat underprivileged patients (Mulliken, 2004). Technological limitations include a lack of modern surgical equipment and operating facilities. There may be no trained surgeons. There are also cultural reasons such as unequal access to surgical care by fringe or minority groups. Government policies may reinforce the economic and the cultural imbalance within a country or minimize the importance of surgical care versus medical care (Mulliken, 2004). Prioritization of resources varies

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from country to country. Repairing congenital anomalies in a small section of the population is often low on the list of priorities. Whatever the cause, or combination of causes, many Developing Countries do not have the resources or infrastructure to provide surgical care for the majority of their population. A stop-gap solution, which has continued to spark debate, is the exportation of surgical care from Developed to Developing Countries. We discuss the different settings that attract surgical teams, past models of exporting care to Developing Countries, and propose new guidelines for future endeavors abroad. We focus on plastic surgery because it is the area with which we are most familiar. Nevertheless, the problems and principles apply to all surgical specialties, and indeed other professional groups.

THE DESTINATION Surgeons, in general, and plastic surgeons, in particular, have a long history of traveling abroad to operate on congenital and acquired deformities in patients who have no access to care. Inherent in this process is a transfer of resources – intellectual, technological, financial, material and psychological. As exportation of surgical care has evolved, so too have the various locations that attract these types of visits. Not all ‘missions’ arrive in the same setting and address a single type of problem with the same resource requirements. Thus, it is inappropriate to set up a standard core of guidelines or principles to govern the conduct of all groups in all places. Nonetheless, there are tenets that apply to all such humanitarian efforts. Some individuals and groups go to remote villages with no access to Developed World medical care. Notwithstanding the multiple medical problems in such places, usually the team’s ability to treat complex or involved surgical conditions is limited. Patients in small towns have access to care by traveling to cities, but usually without the benefit of specialists to treat anomalies like cleft lip/palate. There are cities in Developing Countries where modern but under-staffed, under-supplied hospitals exist. In these urban settings, the public health system is often unable to provide plastic surgical services to the country’s poor.

MODELS OF DELIVERING CARE OVERSEAS Over the past 30 years, plastic surgical care has been transported from Developed to Developing Countries in many ways. The models span a wide range of preparation, organization and sustainability. At one end of the spectrum is the lone surgeon who travels abroad with donated instruments and supplies, and is self-supported. The surgeon performs operations on the patients he feels qualified to help and in the best operative setting available. In the mid-spectrum are the small surgical groups that try to foster long-term relationships with medical communities abroad and make an effort to return to the same resource-poor locale on a regular basis. Further along

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the spectrum are the highly organized and well-financed philanthropic organizations that sponsor large-scale trips. With increased participation of the local medical and surgical community, their efforts may lead to the development of enduring, homegrown care. Some groups assist well-financed, local hospitals and organizations to improve infrastructure and promote a higher level of health-care delivery. At the far end of the ‘missionary’ spectrum are the few established organizations whose goals are to build infrastructure, train personnel, and develop a self-supporting care system, which might also include research, training surgeons, and assisting other centers in need of help. ONE AUTHOR’S EXPERIENCE One author (ESG) has observed the impact of a medical program in the Caribbean. In 2005, he traveled to rural Haiti to assist the full-time general surgeons at Zanmi Lasante, the Haitian arm of the Boston-based organization, Partners in Health. This organization was founded in the mid-1980s by Dr. Paul Farmer, an infectious disease specialist. His original goal was to treat endemic tuberculosis and HIV infection that were ravaging the Plateau Centrale of Haiti. As the clinic grew in size and resources, surgical care came to be recognized as a necessity. The surgical program in Cange, where Zanmi Lasante is based, has expanded considerably over the past decade. Large-scale capital investments for two operating rooms, a surgical ward, anesthetic machines, and assorted equipment were given as resources allowed. The present complement of staff surgeons permits the treatment of most urgent and emergent problems. Nonetheless, specialty care, such as plastic surgery, urology, thoracic surgery, neurosurgery, remains beyond the scope of these talented and dedicated surgeons. Zanmi Lasante has dealt with this deficiency in the usual way. They appealed to foreign groups to donate time and resources for the care of Haitian patients. Plastic surgeons from Miami and neurosurgeons from South Carolina have made several trips. A personal appeal by Dr. Farmer convinced the author to participate. The challenges faced on the Plateau Centrale were typical of many rural hospital settings. Resources were scarce, equipment was of poor quality, and the patients often presented with advanced or complex pathologic conditions. Often there was the temptation to try to do more than was prudent. The importance of responsible patient care in this setting has been underscored in the literature and is addressed elsewhere in this chapter. The major challenge that faces the surgeon after returning home is how the accomplishments during the brief sojourn can be amplified. Delivery of surgical care in the Developing World ideally requires all the elements with which we, in the Developed World, are familiar. Safe and effective operations require doctors, nurses, ancillary staff, equipment, medications, instruments, information systems and records, and buildings. One way is to establish a large, centrally administered non-governmental organization, such as The Smile Train or Operation Smile. Another is to build a ‘grass-roots’ organization, obtaining excess inventory

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from one’s hospital, recruiting volunteers from colleagues, and obtaining donations from sympathetic sources. In our experience, very good intentions are often expressed during the visit without always an understanding of the time commitment required to maintain or build the program in the Developing Country, once back home with all the day-to-day pressures, obligations and interests there. The effort required to establish and support a surgical program from abroad is considerable, and requires vast amounts of discipline and dedication. Surgeons must be disciplined to operate responsibly and within the limitations of training and resources. They must be dedicated to the cause which they have chosen, whatever the constraints of distance and time.

LARGE ORGANIZATIONS Of the many organizations that export surgical care to Developing Countries, a few have garnered massive logistical support, powerful public relations apparatus, and impressive fund-raising machinery. Moreover, each succession of these large-scale organizations learns from the successes and failures of its predecessors. Three of these organizations are highlighted to illustrate this evolution in exportation of surgical care to Developing Countries: Interplast, Operation Smile and The Smile Train. INTERPLAST Interplast (www.interplast.org) was founded in 1969 by Dr. Donald Laub, while he was Chief of Plastic Surgery at Stanford University. According to its mission statement, Interplast’s three goals are: (1) to perform reconstructive surgery; (2) to teach local surgeons the skills of reconstruction; (3) to assist local surgeons on the road to independent function. Today, Interplast organizes approximately twenty trips per year to various locations around the world. Each expedition involves a 12–15-person team, including surgeons, anesthesiologists, pediatricians, nurses, technicians, therapists, and coordinators. As part of its goal to educate local doctors, Interplast also sponsors fellowships for foreign surgeons to train at centers of excellence in the United States. OPERATION SMILE Operation Smile (www.operationsmile.org) was founded in 1982 by Dr. William Magee, Jr, a plastic surgeon in Norfolk, Virginia, specifically to treat cleft lip and palate. Surgeons operating under the auspices of Operation Smile have cared for over 100,000 children in Developing Countries. Operation Smile also helps surgeons in Developing Countries through in-country fellowships and through sponsorship of plastic and reconstructive surgical training for foreign surgeons in the United States. According to the Operation Smile website, 86% of cash and in-kind donations go directly to patient care.

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In 1999, there was a widely publicized report in the New York Times that highlighted the deaths of several children undergoing operations during Operation Smile visits to Developing Countries (Abelson & Rosenthal, 1999). These and other complications led to the criticism that many surgical ‘missionary’ organizations had inadequate safeguards, poor quality assurance mechanisms, and operational philosophies that emphasized the number of procedures over both safety and quality (‘head count’). This negative publicity and the philosophical issues led to changes in Operation Smile’s organization. Processes for safer procedures have evolved. Surgery is a discipline, a branch of medicine, not an act. Operation Smile’s visits have developed to reflect the increasing importance of teaching the local surgeons advanced techniques in order to lessen the reliance on visiting teams. THE SMILE TRAIN The Smile Train (www.thesmiletrain.org) has taken a different tack from Operation Smile. The operational style of The Smile Train emphasizes lean, cost-conscious, and low-overhead procedures; 100% of donations go directly to patient care. The Smile Train began operations in 1999 based on the age-old concept of ‘teach a man to fish’. Instead of sending teams of American doctors on overseas visits, The Smile Train manages cleft lip and palate by upgrading local infrastructure. The Smile Train provides free education, equipment and financial support to hospitals, organizations and medical professionals through partnerships, educational, research and training grants. The organization’s emphasis on teaching local surgeons how to safely and effectively perform certain procedures is also illustrated by the development of a virtual surgery training CD and the internet-based patient record system, The Smile Train Express (http://www.smiletrainexpress.org). All operations supported by The Smile Train are recorded on The Smile Train Express, and the outcomes of cleft lip repair undergo regular quality assurance reviews by a medical advisory board, based on photographs. The first response to surgeons with poor reviews is to provide opportunities for further training and education. Since March 2000, The Smile Train has treated over 230,000 children with cleft lip and palate (website, 28 June 2007). Recently, The Smile Train has introduced a change of policy by having surgeons visiting the Developing World from the Developed World. In a mass-mailing campaign in early 2007, the organization introduced The Smile Train Medical Exchange Program, the aim of which is to recruit volunteer surgeons to travel abroad to perform cleft lip repair. The proposed benefits of this new program include the ability of the surgeon to choose dates, location, team members, and length of trip. Financial support is included. These volunteers are required to enter patients into The Smile Train patient database and the results of procedures are to be audited in accordance with their standards of care. This departure in approach is notable for its emphasis on facilitating trips by foreign plastic surgeons who may not care for cleft children in their home practice. The application for the new program includes only the most cursory questioning on competence to perform cleft lip/palate repair. The Smile Train was founded, in part, as a reaction to the culture that stressed quantity over

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quality in surgery. This new change of approach seems to represent a reversal from other ‘mission’ styles. While The Smile Train has always had high standards, there is concern that their commitment to the highest quality care will be derailed in favor of sending more surgeons out ‘into the field’, irrespective of expertise in cleft lip/palate repair.1

SUMMARY With each iteration of large-scale charities, the type of care and measure of success has evolved. The goal has changed from that of treating the largest number of patients to training and supporting medical professionals and organizations. Accountability grew from none, to personal recollection, to sporadic records, to web-based, formalized quality review. The ratio of productivity to overhead and number of patients treated has also increased dramatically as these groups have adopted the organizational conduct of well-run companies.2

EVOLVING PRINCIPLES The evolution of surgical philanthropic efforts has been influenced by several professional organizations. Guidelines on surgical overseas visits are set out by international bodies, such as the Volunteers in Plastic Surgery program (VIPS), The American Cleft Palate-Craniofacial Association (ACPA), and the International Plastic Reconstructive and Aesthetic Society. Supplementary anecdotes and advice on international surgical volunteer visits are published in over 50 journal articles. Many of the same principles are cited by numerous authors, although their visions of ‘missionary’ surgery vary widely. Certain principles are universally valid, whether one is a single surgeon traveling with limited provisions to an isolated clinic in the jungle or a multidisciplinary team descending on a large city hospital with crates of supplies. Some of the guidelines governing conduct and goals of surgical philanthropy are broad enough to apply to all the various ways in which specialty care can be exported. For example, the mission statement of VIPS is to serve, train, educate and conduct research in areas of the world where there is need and where surgeons from Developed Countries are invited visitors. VIPS guidelines underscore that the visiting surgeons must work in conjunction with local surgeons and must be formally invited to participate in the care of patients in that locale. These guidelines further state that an operation should be done ‘by experts or senior residents or fellows under their supervision’ and that the ‘team leader should be board certified’. Unfortunately, it is not specified whether or not the ‘team leader’ practices the specific type of procedures, e.g., repair of the cleft lip/palate, in their home country. VIPS states the ‘mission’ should not include untried, experimental, or aesthetic surgery. Regulations about appropriate follow-up, team composition requirements (therapists, dentists, orthodontists, etc.) are not included in their guidelines (Volunteers in Plastic Surgery, 2007).

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Natsume (1998) has also suggested broad guidelines. These include a long-term plan for technology transfer, an understanding of local laws, customs, and systems, collaboration with local doctors, and quality assurance for participating doctors. Visits should not be religiously, politically or profit-motivated. Visiting surgeons should act as both teachers and practitioners and should accept responsibility for the convalescence of the patients on whom they operate.

THREE COMMON PRINCIPLES Three common principles to follow when delivering health care to Developing Countries are: 1. Build and support the local infrastructure. 2. Create long-term, self-sufficient care. 3. Adhere to the highest standards. BUILD AND SUPPORT THE LOCAL INFRASTRUCTURE One concept that applies to all locations and varied systems of health-care delivery is the need to support the existing medical infrastructure. This principle requires that the ‘mission’ has as its basic goal the permanent improvement of the local medical establishment in ways that are not directly dependent on outside assistance. As Abenavoli (2005) clearly stated, organizations that empower the local medical community are those that integrate with and attach themselves to the domestic socio-medical community, by working together with physicians and paramedics in a personal and collaborative manner to share professional experiences and by donating medical equipment that allow these professionals to become autonomous in their local efforts.

Also commenting on the important role of the local medical community, Robinson (2006) wrote: If you do not have a support group in that country, then you shouldn’t operate there and leave behind any particular problems, unless of course, you are willing to stay there or come back and take care of them. I attest that providing alternative care to the local health system, even in a long-term and comprehensive manner, sets a precedent for reliance on foreign aid and undermines respect for the local infrastructure, akin to medical imperialism.

CREATE LONG-TERM, SELF-SUFFICIENT CARE A ‘mission’, whatever its purpose, is a finite endeavor. It has been argued that this is its greatest liability. There seems to be a consensus among the medical community that ceasing to provide necessary medical care, or substituting sub-standard care, is

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unacceptable. Mulliken (2004) wrote about surgical trips that focus on cleft lip/palate: ‘Usually, there is no continuity of care. These children need help with speech, dental and orthodontic services, and often secondary surgical procedures, and they must be followed until their facial growth is complete.’ Echoing this sentiment, Wolfberg (2006) stated, ‘Differences in the medical needs of populations, the skills of local health care providers, and the level of international collaboration present a challenge to volunteer organizations, particularly if they send volunteers for short periods.’ Indeed, there is general disapproval of ‘hit-and-run’ care, leaving local surgeons to manage post-operative problems after the foreign team has departed. There are two responses to the argument that surgical ‘missions’ must be sustained until local professionals assume responsibility. The standard answer is that given the circumstances of time and place, it is better to do something – anything – than to do nothing. Furthermore, groups whose resources are limited can, in time, change their modus operandi to provide more longitudinal care, even if it is delivered sporadically. Indeed, many groups aspire to transition to long-term, locally supplied, self-sufficient surgical care. These surgical ‘missions’ rely on community participation, such as a physician or local organization, that is dedicated to continuing care in the absence of the overseas visitors. The challenge of this feat is highlighted throughout the literature. Bermudez (2004) underscored that, ‘It is not easy to find a well-trained professional who is willing to work for free or almost for free, at home, weekly, and who is committed to an excellent result.’ In Zbar et al.’s (2000) model for cleft lip/palate repair in developing nations, the ‘independence’ phase, in which host health-care providers maintain the site during the absence of the guest team, may represent the greatest hurdle to true independence. Indeed, recruiting local participation is the greatest challenge, i.e., to overcome to provide sustainability in communities with an underdeveloped medical infrastructure. Since brief visits have a limited, and oftentimes negligible effect, ‘missions’ designed on this model of export need to carefully evaluate the medical environment into which they go. There are some settings where, on balance, there will be a net benefit from a short visit, but there are many more in which this model is unacceptable. ADHERE TO THE HIGHEST STANDARDS It is no surprise that quality is the major component of optimal standards of international surgical care. Theories of social justice and human rights justifiably criticize many international surgical trips for practicing new operative techniques and training inexperienced medical professionals on resource-poor communities overseas (Ward & James, 1990). Ideally, surgical teams should follow the highest medical standards to ‘guarantee that the quality of the treatment and the safety standards [are] as high as in any other surgical unit in countries with better resources’ (Ward & James, 1990). Zbar et al. (2000) suggested anything less is ‘perhaps irresponsible, or, at best, purely an aesthetic rather than a fundamental undertaking’.

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Dupuis (2004) wrote: There cannot be, for volunteer plastic surgeons, two standards of care: one for their cosmetic surgery patients at home, and another one, a sort of ‘good enough is better than nothing’ approach, for foreign humanitarian missions because there is little risk of malpractice action. There is only one standard of care, and that is one provided by surgeons qualified in that type of surgery, and it has to be met by all surgeons participating in humanitarian missions.

Linda D’Antonio, a speech pathologist at Loma Linda University who spends much of her professional life teaching abroad, echoed this sentiment: Few efforts have included attention to associated rehabilitation areas such as otologic care and speech treatment. Most of us reading these comments would not think of providing such uni-dimensional services for our cleft patients at home. Why are we allowing ourselves to accept this level of care abroad? (D’Antonio, 1990)

Aspects of high quality care for cleft lip/palate that are largely uncontested include: (1) comprehensive and multidisciplinary treatment; (2) experienced healthcare providers. The attempt should be made to establish centers that resemble as closely as possible those in the Developed World. At these centers, longitudinal, multidisciplinary care of the child is the goal. For the surgeon in the Developing World context, this should be the goal as well. A plastic surgeon should perform abroad only procedures performed regularly at home (Dupuis, 2004). Availability of resources, determination, and good fortune will help to determine how successful he/she will be in this endeavor. Adhering to the highest standards of treatment ensures that surgical visits avoid focusing on the number of operations completed. As Fisher states: ‘A team that sets out to teach will certainly put a lower emphasis on turning out large numbers of operations’ (Fisher, 1990: 16). One of the pitfalls of this long-standing debate on quality of care is that it does not take into consideration variations in medical landscape. For those who choose to go to the most remote places to treat patients with the least access to medical facilities, the standard of care will be unlikely to be equal to that delivered at home. Before embarking on such a venture, the team leader must be certain that the differential is as small as possible and that the benefits of delivering care at a lower level truly outweigh the risks. Ideally, surgical skill and knowledge should be the same. The differences are access to supplies, physical plant, absent multidisciplinary team and nature of the disorders one hopes to correct. It is a Panglossian surgeon who thinks that it is possible, necessary, or desirable that all surgical ‘missions’ be held to the standards that exist in the Developed World. Optimizing standards of care will reinforce the others. Co-ordination with the local community to build and support local infrastructure improves treatment and minimizes many issues of cultural sensitivity. As Zbar et al. (2000) wrote, ‘Guaranteeing patient safety, preserving indigenous culture, and teaching local surgeons the multidisciplinary approach to cleft care are key goals.’ Moreover, targeting the local infrastructure is more conducive to permanence and self-sufficiency. Establishing

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sustainable, locally delivered care helps to address the problem of follow-up and improves treatment overall. Finally, adhering to the highest possible standards depends on building a stable infrastructure in the local community.

CHALLENGING THE PRINCIPLES There is a debate as to whether a high quality of treatment can be achieved through a top-down approach or a bottom-up approach. A decision must be made whether one is committed to replicating, as closely as possible, care that is delivered at the highest levels in Developed Countries (top-down approach) or whether one will try to do one’s best with the available resources (bottom-up approach). As a profession, we have an obligation to continuously strive to find the most effective ways to improve delivery of care, regardless of the environment, situation, or setting. If we are successful, the point where the top-down curve and the bottom-up curve meet will continue to approximate, indicating ever-improving standards of care, independent of the availability of resources. It is unlikely that it is efficient for an individual, relatively inexperienced, plastic surgeon to organize, fund, and manage small expeditions to hospitals visited once or infrequently. Nevertheless, for the time being, in certain settings, this may be the only or best available option. Our responsibility is to provide better choices. In an ideal world, only surgeons with ‘acceptable’ levels of training would be allowed to participate in the care of children in Developing Countries; however, sometimes, this is simply not possible. There are many parts of the world where strict adherence to this standard would lead to high morbidity and mortality. The experience of the Italian group Comitato Collaborazione Medica (CCM) highlighted this problem in general and obstetrical surgery. They have been acting in concert with the UN over many years to perform and teach surgical procedures to individuals in the Sudan, where few doctors live and large portions of the population have limited or no access to adequate care. Instead of waiting in vain for experts to arrive, they have chosen to teach basic surgical skills to paramedical personnel. The results are not the same as if a Caesarean section or a hernia repair were performed by master surgeons but they are good enough to save lives (Meo et al., 2006). In the most remote, poorest parts of the African continent, timely access to expert physicians and surgeons is unrealistic. CCM’s solution was to train people to do the basic minimum to save lives in a variety of circumstances. The standard of care is clearly different from that in the Developed World. CCM’s argument is that the ethical and surgical standards of the Developed World should be modified in situations like this. What is the cost–benefit ratio? Can the operations be done safely? What are the minimum standards of care for different operations and how can they be modified under different situations? Should care in Los Angeles and Lesotho be governed by identical standards? If not, how and how much should they be modified? The debate within the plastic surgical community revolves around a single ideological difference. Is it better to do what one can with existing resources, i.e., to repair x

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number of children, or is it more important to build the closest possible approximation of the care that exists in Developed Countries? Certainly, the health-care infrastructure in most Developing Countries makes the achievement of the latter goal unlikely, at least through the work of individuals or foundations. Equally certain is the fact that submitting to the do-what-we-can mentality can be suboptimal, dangerous, and irresponsible. An alternative is a case-by-case analysis of the needs and resources of a given setting overlaid on a group of principles to govern surgical work abroad. The most advanced and well-established cleft centers in the Developed World have an experienced and interdisciplinary team of professionals working towards the single goal of habilitation of the child born with cleft lip/palate. These teams include plastic surgeons, anesthesiologists, audiologists, otorhinolaryngologists, dentists, orthodontists, speech and language therapists, and nurses. The timing of surgical repair and subsequent interventions is closely choreographed and audited. The families of these children are motivated, responsible, and reachable by phone, email and regular mail (Bearn et al., 2001). In contrast, the care of children with cleft lip/palate in most of the Developing World lacks most of these elements. ‘Missions’ have, for decades, filled only part of the surgical void, and only periodically. How closely must a ‘mission’ approximate the best available care for a given condition? What are the minimal elements for appropriate intervention?

A SURGEON FOR ALL SEASONS AND PLACES There is a relationship between the various settings where surgeons go and various modes of exporting surgical care. One could imagine starting at one end of the spectrum and positing that any surgeon, or health-care worker, could close a cleft lip, even though the result was both functionally and cosmetically poor (‘something or anything is better than nothing’). Most plastic surgeons would deem this unacceptable. Nonetheless, the argument could be made that there are some places and some people for whom such a substandard result would be preferable to the alternative of living with an unoperated lip (see Chapter 13, Bradbury and Habel). The next best option might be to have the repair performed by a junior or senior resident who plans to focus a career on cleft care. One might enlist the assistance of surgeons who might never do cleft repairs in their practice but are competent plastic surgeons willing to volunteer to perform this operation in Developing Countries. It is possible to imagine places and patients who would accept this sub-optimal care rather than have no care at all. A higher standard would be to send an active cleft surgeon whose weekly practice closely approximates the caseload in the Developing Country. This experienced surgeon would help a local surgeon who has specialized in cleft lip/nasal deformities and cleft palate repair. The ultimate goal would be a multidisciplinary center staffed by local experts where the personnel closely resemble those found in the finest cleft centers. Certainly, this would be the preferred choice of all patients and families anywhere in the world. Our goal, as a profession, should be to allocate resources to this end.

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In an attempt to address current shortcomings, many groups now place greater emphasis on teaching, adhere to higher standards for visiting surgeons, and are more selective in choosing their destinations than they were a decade ago. There is another possible prescription for a surgeon wishing to contribute to care of underprivileged children in the Developing World. Mulliken’s advice is to become an expert in one’s own country, ‘refining the craft of labiopalatal repair, writing, [and] teaching’, rather than traveling. If a surgeon wishes to travel abroad, the preferred strategy is to pick one country, one hospital, one local health-care system to assist until the local physicians are sufficiently able to perform the operations themselves. Another alternative is to send a local surgeon who is passionate about cleft care in his or her own country to learn by observing a master-surgeon in a Developed Country. In this way, one-to-one training between the surgeon and the pupil is delivered. This results in a lifetime relationship, sustained by exchanges of ideas, questions, techniques, and principles. Such a post-graduate interaction can now also be continued via the internet, including the exchange of digital images. Cases can be discussed both pre- and post-operatively between surgeons in Developed and Developing Countries.

FROM DEPENDENCE TO INDEPENDENCE Rather than simply outlining costs and benefits of different styles and philosophies, some authors offer algorithms for establishing cleft centers in the Developing World. The tripartite plan put forward by Zbar and colleagues (2000) consists of observation, integration, and independence. These phases coincide with many of the principles and suggestions discussed previously. The goal of their algorithm is the progression of both the visiting team and the local doctors from phase I (observation) to phase III (independence). In his discussion of the Zbar plan, Laub (2000) suggests that fourth and fifth phases are possible. The fourth phase entails the evolution of the newly independent local physicians into visiting surgeons in a less developed location. The student becomes the teacher. Phase V involves the continual improvement and refinement of surgical skills by the local team, up to and including the development of new techniques and, ultimately, perhaps even the elucidation of new operative principles. The goal is to develop a major center in that country for treating the particular deformity or disease, equal to the best the Developed World offers. A UNIQUE EXAMPLE OF A SURGICAL MISSION – DR. SAMUEL NOORDHOFF AND CHANG GUNG MEMORIAL HOSPITAL In 1958, Dr. Samuel Noordhoff completed his training in plastic surgery under Dr. Ralph Blocksma at the Butterworth Hospital in Grand Rapids, Michigan, USA. Dr. Noordhoff became aware of the need for a plastic surgeon at the MacKay Hospital in Taipei, and moved to Taiwan with his family. He learned the Taiwanese language and not only worked as a plastic surgeon, he became an administrator. Over the next

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15 years, he established centers for polio patients, children with cleft lip/palate, burn victims, and suicide prevention. He also started the MacKay Nursing School. In 1976, he became the Chief of Plastic Surgery at the new Chang Gung Memorial Hospital. Dr. Noordhoff had a vision and a simple plan: he recruited the brightest young Taiwanese surgeons and sent them abroad to Europe, North America, and Australia for specialized training. Then they returned to Taiwan. Noordhoff also found time to focus on perfecting his own techniques for repairing cleft lip/palate. Today, Chang Gung Memorial Hospital is one of the most modern and largest medical centers in the world (4,000 beds and over 100 operating rooms). Dr. Noordhoff’s prot´eg´es are leaders in the fields of cleft lip/palate, craniofacial, and microsurgery. They are not simply busy practitioners, they are academic surgeons involved in clinical and basic science research who write and publish in prestigious English-language journals. Plastic surgeons from Chang Gung travel around the globe to share their knowledge and skill with Developing Countries, while Chang Gung has become a mecca for young plastic surgeons.

THE NEXT LEVEL OF SURGICAL VISITS We propose that the next step in exporting surgical care overseas should not focus on direct patient therapy. The purpose of international volunteer surgical visits should be the development of the medical infrastructure. Of all the aspects of building a sustainable medical infrastructure in the Developing World, the most important is training local medical professionals. Following the training of local medical professionals, the next responsibility is the efficient, cost-effective provision of surgical equipment, such as sutures and operating room facilities. Most medical and philanthropic communities now largely agree that educating local medical professionals is the critical next level. Dupuis (2004) argued that ‘twenty operations performed perfectly for the purpose of teaching are better than 100 amateurish ones performed by volunteer plastic surgeons’. As long as local, trainable physicians are available, the value of an educational ‘mission’ far outweighs the value of a purely procedure-oriented surgical mission. Recognition that education is essential to building or supporting local infrastructure resounds in the medical literature: ‘If you give a man a fish, he feeds his family. If you give him a fishing pole and teach him to fish, he feeds his family for a lifetime.’ Nevertheless, as D’Antonio (1990) states: In practice, this goal is often relegated to a lower priority than a more tangible, measurable goal, such as the number of patients seen or the number of cases managed. This is understandable in that such data is concrete and can be quantified, which is valuable for accountability and fund-raising issues. Obviously, it is more difficult to deal with ambiguous and amorphous goals such as ‘empowering local people’ or ‘training.’ Nevertheless, there should be an increased emphasis on training local health care providers and community leaders in ways and means that they can help to improve the quality of health care for people whether we ever return again or not.

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Besides the benefit of creating sustainable surgical care, training medical personnel will generate positive public relations for surgical ‘missions’ that are often criticized for over-emphasizing numbers of operations per trip.

CONCLUSION In his article, Dupuis (2004) quoted the Chinese proverb, ‘It is better to light a candle than curse the darkness.’ It is laudable to care about people in parts of the world where there is little or no access to surgical care. On a fundamental level, it is important to feel connected to others. It is a privilege to care for others, especially, perhaps, those whom we do not know, will never see again, and from whom we will not receive payment. Even when the highest standards are followed, problems endemic to the surgical and the popular culture contribute to suboptimal interactions between visitors and hosts. Many plastic surgeons in Developing Countries cite the lack of compensation as a factor in their choice not to perform cleft lip/palate repairs. Frequently, however, the host surgeon seems more interested in the latest cosmetic techniques than in learning how to better repair a cleft lip. On the part of the patients, there is often the perception that even when an established cleft team exists in a Developing Country, visiting surgeons from abroad are somehow better. This effect not only is counterproductive to the efforts of the local surgeons but often inaccurate as well. Plastic surgeons have a long history of traveling to Developing Countries to operate on patients in need. They have journeyed as individuals and groups, as small and large organizations, and as sectarian and non-sectarian ‘missions’. Some of these individuals and groups have become well known through the media, while others remain largely anonymous. The highest standards should govern these surgical ‘missionaries’. The teams should be interdisciplinary. All participants must clearly and rationally evaluate the specific requirements of local milieu and the capabilities of both the regional professionals and infrastructure. Each team must have a clear sense of its own core capabilities and resist temptation to stray from its goals. Each ‘mission’ must be tailored to fit the particular location. Equipment should be of the highest possible quality, procedures planned with the utmost care and performed by the most skilled practitioners available. ‘Missionary’ surgery should not be a publicity-generating event or a way to assuage some more or less recognized guilt. As a profession, we must continue to work towards the far end of the spectrum where all patients receive the best possible surgical care, ideally by their fellow countrymen.

NOTES 1. Editorial note: The Smile Train was invited to contribute a chapter to this volume but declined. 2. The editors also draw the reader’s attention to the issue of payment to surgeons by these large organizations for undertaking cleft lip and palate repair. This runs

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the risk of distorting the local surgical environment and rewarding individuals not genuinely interested in these anomalies.

REFERENCES Abelson R & Rosenthal E (1999) ‘Charges of shoddy practices taint gifts of plastic surgery’, New York Times, 24 November. Abenavoli FM (2005) ‘Operation Smile humanitarian missions’, Plast Reconstr Surg 115(1): 356–7. Bearn D, Williams A, Mildinhall S, Murphy T, Sell D, Murray J, Sandy J & Shaw W (2001) ‘Cleft lip and palate care in the UK – the Clinical Standards Advisory Group (CSAG) Study. Part 4: outcome comparisons, training and conclusions’, Cleft Palate Craniofac J 38: 38–43. Bermudez LE (2004) ‘Humanitarian missions in the third world’, Plast Reconstr Surg 114: 1687–9; author reply 1689. D’Antonio LL (1990) ‘Commentary to Ward & James’, Cleft Palate J 27: 15–17. Dupuis CC (2004) ‘Humanitarian missions in the Third World: a polite dissent’, Plast Reconstr Surg 113: 433–5. Fisher JC (1990) ‘Discussion’, Cleft Palate J 27: 16. Laub DR (2000) ‘Discussion – Establishing cleft malformation surgery in developing nations: a model for the new millennium’, Plast Reconstr Surg 106: 890-1. Meo G, Andreone D, De Bonis U, Cometto G, Enrico S, Giustetto G, Kiss A, et al. (2006) ‘Rural surgery in Southern Sudan’, World J Surg 30(4): 495–504. Mulliken JB (2004) ‘The changing faces of children with cleft lip and palate’, New Engl J Med 351: 745–7. Natsume N (1998) ‘Safari surgery’, Plast Reconstr Surg 102: 1304–5. Robinson O (2006) ‘Humanitarian missions in the Third World’, Plast Reconstr Surg 117(3): 1040–1; author reply 1041. The Smile Train, http://www.smiletrain.org (accessed 28 June 2007). Volunteers in Plastic Surgery (2007) http://www.plasticsurgery.org/medical professionals/ service giving opp/Volunteers-in-Plastic-Surgery (accessed 28 June 2007). Ward C & James I (1990) ‘Surgery of 346 patients with unoperated cleft lip and palate in Sri Lanka’, Cleft Lip Palate J 27: 11–15; discussion: 15–17. Wolfberg AJ (2006) ‘Volunteering overseas – lessons from surgical brigades’, New Engl J Med 354: 443–5. Zbar RIS, Rai SM & Dingman DL (2000) ‘Establishing cleft malformation surgery in developing nations: a model for the new millennium’, Plast Reconstr Surg 106: 886–9; Discussion, 890–1.

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So You Want to Help in a Less Developed Country? BRUCE RICHARD

Editorial comment: The author has worked for many years in a Developing Country (Nepal) and also been a member of a large visiting multidisciplinary cleft team (Ethiopa). He gives advice in an informal style, which may be particularly relevant to those who seek an independent path. He also gives a personal view on the benefits and conflicts between different ways of delivering cleft service in Less Developed Countries.

INTRODUCTION You have always promised yourself that one day you will actually go and do one of those Developing Country cleft trips but you’re a little anxious that someone might accuse you of going off on a surgical safari, and you are aware that some of these trips can have damaging effects on a Less Developed Country’s (LDC) cleft surgery infrastructure. I have collected in this chapter some thoughts, attitudes and reflections on various styles of assistance to a Less Developed Country’s cleft service, harvested from a nine-year experience of living in Nepal and setting up a regional cleft service there. You might begin by asking yourself some questions. What is the purpose of the visit and who is going to benefit? Do you have the right skills that are needed? Can you help in a culturally sensitive way? Can you teach rather than demonstrate? Can you leave skills, instead of operative complications, behind? Can you help the national surgeon do a better job with what equipment is to hand rather than importing hightech, expensive, or disposable equipment? Can you or other colleagues visit the same place regularly (annually), rather than helping the national surgeon come to the UK for training and never going home again! What cleft surgery infrastructure already exists, and are you competing or enhancing it by your trip?

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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ISSUES If you are preparing a short-term visit to do cleft surgery in a Less Developed Country, consideration of the following six issues might help you plan: 1. 2. 3. 4. 5. 6.

timing of the trip; visa and work permits; taking care of your health; attitudes and actions; cultural awareness and civility; rest and relaxation.

TIMING OF THE TRIP Your personal time frame for life in your own country is busy, and the itinerary you plan might be tight, but still try and be flexible in planning the visit with your host. They are also constrained by many issues beyond their control, such as national holidays, festival days, local politics, water, electricity or staff shortages, etc. Weather can make a huge difference to patient accessibility to the hospital, i.e. a dry or rainy season, and you need to plan your trip around that, as well as around your home country life! Finalize the date of your trip at least 6–12 months in advance, so that the local surgeon can collect relevant cases for the visit. Usually the local team will not have a method of recalling patients by phone or letter, and can only give them an operating date when they are actually sitting in front of them in the Outpatient Department. Consider how many non-medical folk may come with you on the trip, for example, a spouse, children, friends. There is often nothing really useful that they can do, yet they often want to do something helpful. Make sure that they are not a burden to your host; the host has enough to do without having to organize lots of sightseeing for your relatives, emailing granny or dealing with their gut rot, as well as managing an especially challenging operating list! Sometimes the hassle factor from the national surgeon’s point of view in organizing an international visitor is of borderline benefit relative to the effort required for the opportunity afforded to him. So don’t be miffed if it all seems too hard to organize! VISA AND WORK PERMITS In some countries it will be acceptable to visit as a ‘tourist’ and the local surgeon will give you operating privileges. In other countries this is specifically disapproved of and you will need to get a visiting work permit in addition to a visa. This may not be possible to initiate prior to your visit, and may have to await your arrival in the country. The necessary bureaucracy is often slow and possibly indifferent, so whatever transpires ensure you take a copy of your MD, MB, BS or equivalent graduate qualification, surgical degree and a current professional registration certificate. You will need at least six passport photos and a few dollars to make it all happen. Find out about this well in advance.

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TAKING CARE OF YOUR HEALTH Check that your vaccination status is up to date, and adequate for the country you are visiting. Take special care over your Hepatitis A/B and malaria prophylaxis. Notify and discuss your plans with your personal physician. A very useful UK organization is Interhealth (see Resources on p. 28), a private, excellent and cheap service offering advice on vaccinations, and general health matters, both physical and psychological. It will provide this help both before you leave, while you are there, and debrief you medically and emotionally if necessary, on your return.

ATTITUDES AND ACTIONS Try to be flexible. Do operations with what is locally available rather than taking an entire set of personal instruments with you. This means that the local surgeons also can do the operation themselves after you have gone. Try also to use the local nurses and anaesthetists, rather than coming with a whole team, and showing them how it is done. Do not perform new or difficult operations because you would enjoy the challenge, rather help them to do a possibly more appropriate operation themselves. They may be in awe of you, so be careful on pre-operation ward rounds regarding planning a procedure that is outside of your normal repertoire just because they want you to do it, or because it would be fun. They might be left with the complications. Be careful about ‘taking over’ because of your experience and skill. The idea is to facilitate the local national surgeons to be able to do procedures that they might not attempt without your support, and so enable them to do them again after you have gone. Remember that the local population sometimes considers its own doctors to be corrupt and lazy, and the locals do not allow their own doctors the luxury of having normal complications, etc. For you, however, they will allow a much bigger margin of error, because they have more faith in you, and if the patient dies, it is much less likely for you to be blamed, compared to the national surgeon. This often means that the national surgeon will not undertake risky procedures as failure damages his reputation, but he will encourage you to take the risks. This may help you decide to do a procedure that is not normally done there, but try very hard to let the local surgeon be the actual operator and let the local people see his success. The local, national surgeon will be most deferential and polite to you, the esteemed visitor, so try to identify the real issues, and make sure that your operative suggestions are not being followed just because of you, but because they actually fit the local scene appropriately. The local scene is probably busy and stressed with a normal workload. Be careful your visit does not displace local surgeons from their routine lists by your eight-hour ear reconstruction!! Emergencies probably do not have a separate theatre, so make space for obstetric and other emergencies. If a local system works, however apparently disorganized, perhaps only suggest changes based on your pattern or style of work in your home country as a point for discussion. Explore before your visit what the existing set-up is for elective plastic surgery and whether your visit might destabilize an existing system. A radio announcement

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for free operations can ruin the elective list for another surgeon working elsewhere in the region. You have power by nature of your skill, position and ability to help these doctors possibly visit your own country, so they will be very careful and eager to please you. Beware of the ‘surgical safari’ (Ward, 1994) where you or a more junior colleague use the opportunity to operate on cases such as clefts that you may not be currently managing in your home country’s practice. If anyone is operating on the cleft for the first time, it should be the local surgeon and not a visiting trainee. CULTURAL AWARENESS AND CIVILITY Doctors are still the most senior members of these societies and dress appropriately, so be sensitive to the need to have covered feet, wear shoes not sandals, and wear a tie in cooler weather. Shorts are not the attire of a professional surgeon, unless he is on the beach! Women must keep themselves covered up including their shoulders and upper arms, and in Muslim countries the dress code may be stricter than that. Many Developing Countries have deeply conservative lifestyles and your conduct needs to reflect your respect for that. You may be a guest of the national surgeon, stay with other expatriate personnel or in a hotel. Remember that these people have a fraction of the disposable income that you have, and yet they will try and impress you with their best cuisine, and precious goodies. Be generous in your thanks for their hospitality, and certainly offer to pay for your room and board. Try to eat the local food, rather than taking a suitcase of your local supermarket’s tins with you. They will appreciate it enormously if you eat with them in their humble homes, sitting uncomfortably rather than staying in the local tourist hotels. Remember, PEOPLE and RELATIONSHIPS are often much more important in these cultures than the Protestant work ethic of getting lots of work done. This means less numbers of clefts done per day and more time talking and building a relationship. This is why it is so much better to go back to the same place next year and build on these relationships, rather than collecting T-shirts from each nation in the world! REST AND RELAXATION If you plan sightseeing or travel while there, it is often a good idea to do it immediately after the operating period is finished. Leave time at the end of the sightseeing to do another post-operative ward round or outpatient clinic, to see complications and advise further management. This applies especially after you have performed on a condition or used a procedure that is new to you and the national surgeon.

INTERNATIONAL CLEFT ORGANIZATIONS – THE GOOD AND NOT SO GOOD In December 2005, I went to Ethiopia for the first trip to that nation by an organization called Operation Smile, to treat some patients, but more importantly to begin

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the process of changing the infrastructure so that Ethiopian surgeons could do this work themselves. There were several amazing aspects to this trip. Not least was the size of the team with 49 expatriates to ‘make it all happen’. The team comprised six surgeons and six anaesthetists, numerous operating and post-operative care nurses, intensive care specialists, equipment technicians, photographers, data collecting administrators, a manager and a paediatrician. Unusually, it also included a lawyer and a project administrator to negotiate with government officials, and education experts to help set up teaching modules in the nurse training schools and the university hospital. Additionally, we had students (17 year olds) to give community education and school programmes to help public awareness. We were a diverse group from many nations, comprising a Vietnamese and Ghanaian plastic surgeon, both ladies, as well as American, Canadian, Irish and British (me) plastic surgeons. Other nations included Kenya (five people), Australia, Italy, Argentina, and Ethiopia itself. The youngest was 17 and the oldest (a retired lawyer) was 67. This kind of project needed all sorts of people with a wide variety of skills. In nine days, we saw 419 patients with a cleft lip and/or cleft palate. We successfully operated on 184 of them, ranging in age from 6-month-old babies up to a 60-year-old lady who had never had her cleft lip repaired. We met the president of the country, the leaders of national training programmes, Live Aid donors, ate at a restaurant where Brad Pitt and Angelina Jolie had eaten the night before, and saw a little bit of the rich and ancient culture of Ethiopia. This was a worthwhile trip and Operation Smile has been back each subsequent year and started visits to peripheral centres hundreds of miles from the capital. Organizations such as The Smile Train and Operation Smile have many excellent aspects but also some not so worthy, and I would like to highlight them both for debate and consideration: r Professionalism. One good development is that these organizations have stopped the practice of ‘surgical safaris’ for junior surgeons from Developed Countries to obtain ‘hands on’ experience of cleft surgery by ‘practising on the natives’. This is because each doctor has to be an accredited cleft surgeon in their own country’s health service before they can be accepted as a surgeon on one of these trips. r Safety is also a vital issue for such organizations, with an ethos of ‘no deaths on my watch’. To this end, huge expense is incurred in cargo and equipment, for example, to ensure the best equipment for anaesthesia and monitoring, including pulse oximetry and end tidal CO2 monitoring, and providing the best instruments and suture materials. This is additional to the cost of transporting a large team capable of delivering intensive care for the potential complications of anaesthesia. It may be a First World standard of medicine but is it the local nationals’ plan to carry on like this? This sends a powerful message of ‘You can’t do this after we have left.’ r Education is strongly emphasized, often focusing on infrastructure support for nursing, ITU, the community, and political change to facilitate a cleft service in the future. In concert with this policy I chose to limit myself to 6–7 cleft operations a day (instead of 8–9) to allow time for the national surgeon to do more than just assist, and support him to develop the skills and confidence to do the operation

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himself. Facilitating the national surgeon to learn how to do the operation was encouraged and supported by the surgical team leader. r Epidemiology data collection. This is excellent for world cleft databases. The ‘not so worthy’ is the detrimental side effects of money and power. These organizations are rich, even very rich, and they have enormous power because of this. I think it regrettable that they appear to lack insight into the possible effects of this power, and have a self-righteous smugness in the correctness of their view. Criticism of this is thereby categorized as wrong. This cultural arrogance means that they do not always search out the established infrastructure of the Developing World countries they visit, but rather invent a new one of their own making. Many local nationals will join this bandwagon just because of its success, and because they want to enjoy good food in nice hotels. This can destabilize existing structures and the balance of conditions treated. Where the national infrastructure has taken over cleft cases to co-operate with these organizations, the local surgeon may receive $120–250 per case. I am not against this per se, but it does depend on the integrity of the surgeon to use this money to further cleft surgery availability to the poor and rural patients. The best path of development is for the country to grow its own capacity for funding and doing these surgeries. When I was in Nepal, a patient coming to the government hospital paid $15 for a cleft operation or received it without fee from our hospital’s poor fund, while the visiting ‘big team’ offered a free operation to all. This resulted in one of my cleft lip and palate patients travelling to Kathmandu, in response to a radio announcement offering a free operation, and having a cleft lip repair at age 3. I would have done the lip and palate operation a few weeks later for $15, or free if necessary. The patient came to me again at age 7 with the unrepaired cleft palate. A good speech outcome was now potentially less likely.

THE WAY FORWARD There are several models for delivering and developing cleft services in Developing Countries and all have their strengths and weaknesses. Hopefully this chapter will encourage you to get involved, with reflection and a sense of integrity towards those who you are actually helping and how you go about it. The bottom line: Are you FLEXIBLE, RESOURCEFUL, and CULTURALLY SENSITIVE and do you have TRANSFERABLE SKILLS to pass on?

RESOURCES USEFUL ADDRESSES Department for International Development (DFID) (useful if you want to keep your NHS job but offer your help in an emergency or disaster situation), www.dfid.gov.uk/emergencies Interhealth, 111 Westminster Bridge Road, London SE1 7HR. Tel.: 0207 902 9000, Fax: 0207 928 0927, website: [email protected]

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International Health Exchange has a list of charities, NGOs, etc. concerned with developing world medicine (both emergency and established set-ups) and may be able to help you find a location overseas for a short visit. International Health Exchange, 8–10 Dryden Street, London WC2E 9NA. Tel.: 0207 836 5833, Fax: 0171 379 1239, [email protected] Website: www.ihe.org.uk The Tropical Health and Education Trust: www.thet.org World Service Enquiry offers information on volunteering for development for longer periods: www.wse.org.uk

USEFUL BOOKS British Medical Journal, ABC of Healthy Travel, 5th edn, London: BMJ, £13.95. Available from: BMJ, Tel.: 0207 383 6185, Fax: 0207 383 6662, email: [email protected] Dr Ted Lancaster, Good Health, Good Travel, London: Hodder and Stoughton, £5.99. This is a guide for backpackers, travellers, volunteers and overseas workers. Available from: Interhealth, see above. The Lonely Planet series is good for guides to different countries. People in Aid: Code of Best Practice, February 1997. Available from: International Personnel, BRCS, 9 Grosvenor Crescent, London SW1X 7EJ. Tel.: 0207 235 0895

REFERENCE Ward CM (1994) ‘Teaching, training and travelling’, Br J Plast Surg 47: 280–4.

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Challenges for Cleft Care in the Developing and the Developed World BRIAN SOMMERLAD

Editorial comment: The author is a plastic surgeon with a long record of innovative surgery on cleft lip and palate patients in the UK and in Developing Countries. This chapter addresses the challenges in both the Developing and the Developed World and describes the problems faced by both separately and when working together.

INTRODUCTION The separation of the world into ‘developing’ and ‘developed’ is artificial and rapidly changing. However, the challenges faced by the richer and poorer parts of the world are very different, with each having its own problems and priorities.

THE WORLDWIDE PROBLEMS NUMBERS OF PATIENTS The first obvious difference is in the numbers of patients. There may be 250,000 babies born each year with some sort of facial cleft. Of these, the great majority are born in Developing Countries. This inequality is further compounded by the fact that many are born in rural areas while medical care is concentrated in bigger cities. In many Developing Countries, there are simply too few competent surgeons to cope with the numbers of patients. In the Developed World the opposite may apply. Surgeons in many specialties like doing cleft surgery, and in most Western countries there has been little control over who is allowed to operate on a patient with a cleft. The consequence has been (and continues to be) that there are many teams in the Developed World operating on small numbers of patients. Most surgeons believe that there is a long learning curve in cleft Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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surgery (Sommerlad, 2003) but small volume units simply do not provide surgeons with the opportunity to approach the top of the learning curve. The variety of cleft conditions and operations makes this even more relevant. A cleft team treating 25 new babies per year (regarded in some countries as a reasonable number) would therefore expect to treat two or three babies with bilateral clefts of lip and palate annually, and a surgeon’s lifetime experience would therefore be very limited. It is for this reason that the UK and some other countries have reduced the number of cleft teams – in the UK, from 57 to 9 in England and Wales following the recommendations of the UK government Clinical Standards Advisory Group (1998). ACCESS TO CARE In the Developing World, the discrepancy between the numbers of patients and those available to treat them results in many cleft patients having no access to care and their clefts remaining unrepaired. Others are repaired by surgeons with little or no experience and the outcome may actually be worse than if they had no surgery at all. The problem in the Developed World is that many patients are operated on by low-volume surgeons with limited experience and inadequate teams. PRIORITIES For understandable reasons, Developing Countries have other health issues which receive more attention. They are faced with major life-threatening illnesses such as HIV, TB, malaria and gastro-enteritis. Many Developing Countries also give priority to illness prevention, public health and primary care. Hospital medicine, and especially what might be regarded as ‘quality of life’ surgery, therefore, have to take a back seat. Even in many Developed Countries, cleft surgery may be seen as the rather simple exercise of ‘sewing up’ a lip or palate and the major physical, social and psychological sequelae may not be recognized. SOCIAL AND PSYCHOLOGICAL EFFECTS OF HAVING A CLEFT In Developing Countries, having a cleft may severely limit life prospects. Children with unrepaired or poorly repaired clefts may not go to school and adults may not marry and have families. Local, cultural and religious factors may affect attitudes towards people with clefts and these may differ even within a country. For example, a brief study in Uganda (Chapter 13, Bradbury and Habel) showed that in one part of Uganda a cleft was thought to be the curse of Satan (and many children with clefts were called Ajok for girls or Ojok for boys, meaning ‘of Satan’) while in another part of Uganda it was thought perhaps to be part of God’s plan. In yet another part it was thought to be due to some problem in the womb. Parents’ beliefs may therefore affect their willingness to care for children with clefts and to bring them for surgery.

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In most Developed Countries, there are few patients with unrepaired clefts and having a cleft doesn’t decrease educational prospects, although some studies have shown that fewer develop relationships and marry, and there are significant psychological consequences in a society where so much importance is placed on appearance.

MULTIDISCIPLINARY TEAMS In Developing Countries there may be no tradition of multidisciplinary team working. Surgeons have tended to dominate cleft care and may in fact be somewhat reluctant to share responsibility. In many countries there may be few orthodontists or speech and language therapists, and team management may not be possible, even if desirable. Gradually, in Developed Countries, responsibility has been devolved from surgeons and care is shared by a multidisciplinary team, but this is not always without conflicts and problems – and importantly, in a world where economies in health care are increasingly being sought, multidisciplinary care is expensive.

RECRUITING SURGEONS AND KEEPING THEM INVOLVED IN CLEFT CARE Young surgeons in Developing Countries, faced with the prospect of doing nothing other than cleft surgery because of the numbers of cases, understandably wish to develop other skills. In addition to the workload of cleft patients there are other problems, such as burns contractures, to be faced. In addition, the understandable need to augment income with private practice means that time for cleft surgery may be limited. However, in many Developed Countries, young surgeons (especially plastic surgeons) tend to see cleft care as a rather narrow sub-specialty, with limited opportunities. Here, too, finance is a factor as surgeons may be lured by the rich rewards of private cosmetic surgery.

TRAINING OF SURGEONS Developing Countries obviously have a great potential to train young surgeons, as there are so many patients who require operations. Unfortunately, surgeons from Developed Countries have sometimes taken advantage of this teaching potential for the training of their own residents and registrars on ‘missions’. Some Developed Countries have arguably not yet found the right formula for training in cleft surgery. In the United States, for example, trainees in plastic surgery are all required to have some experience of cleft surgery and yet few will operate on clefts and some should not. The UK, in centralizing cleft care, has taken the position

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that only a small number of surgeons need to experience hands-on cleft surgery, but this poses problems in selection and manpower planning.

MISSIONS – ESPECIALLY ‘PARACHUTE MISSIONS’ Many Developing Countries have been invaded by missions from Developed Countries. Often the surgeons in these missions have been inexperienced surgeons who do not perform cleft surgery in their own countries, sometimes bringing residents to give them an opportunity to learn. The worst examples of these missions bypass local surgeons in organizing their visits. At the end of their 10-day or two-week ‘mission’ they leave nothing behind – except perhaps disasters. Of course, not all missions are as bad as this and ‘mission organizations’ have increasingly recognized the need for liaison and training. However, it is inevitably very difficult for visiting surgeons to adapt in a short period. They cannot understand local problems and they may be unaware of local customs. Nonetheless, at best they can work with local teams and learn from them. There is a great danger, however, that such missions exist primarily for the easing of consciences of relatively wealthy surgeons from Developed Countries and as the means of delivering ‘pseudo-training’ to trainees who would have little opportunity to gain experience in their own countries.

OUTCOMES Patients in Developing Countries are much less likely to return for follow-up and surgeons therefore have less opportunity to assess their own outcomes. Patients also tend to be generally less critical. However, Developed Countries are also facing difficulties in obtaining honest and objective measures of outcome. There is little external audit in the Developed World and patients and parents are poor judges. Studies such as the Eurocleft Study (Shaw et al., 1992) have shown wide variations in outcomes and yet such inter-centre comparisons are rare. The UK CSAG study (1998) showed generally disappointing outcomes across the country but very wide variation between relatively high-volume units. Presumably, however, the parents of children treated in those units were not aware of these differences.

LONG-TERM RESULTS It is recognized that many of the outcomes in cleft care cannot be assessed until maturity. In Developing Countries, long-term follow-up is rare. There have been few objective long-term outcome measures in Developed Countries and unfortunately,

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when a surgeon’s long-term outcomes are known, he or she will be towards the end of their career.

PROTOCOLS AND TECHNIQUES Surgeons in Developing Countries tend to follow what was taught by famous surgeons from Developed Countries. Unfortunately, many of these techniques were unproven and found ultimately to be misguided. The three or four flap push-back palatoplasty of Wardill–Kilner (Wardill, 1937; Kilner, 1937) is an example. This has left a residue of scarred palates, difficult fistulae and retruded maxillae around much of the Developing World. Surgeons in the Developed World may also be slow to move on from what they have been taught.

THE WAY FORWARD 1. Each country must, in time, train enough competent clinicians who will make cleft lip and palate a major part of their practice. 2. The Developed World needs to support evolving cleft care in the developing world – and not with ‘parachute missions’. The aim should be the empowerment of local surgeons. 3. Teams from the Developed World should link with their counterparts in mentoring and twinning arrangements. In future, such links will come increasingly from countries such as India and China as they become increasingly sophisticated in cleft care. 4. Multidisciplinary teams need gradually to be developed, beginning with surgeons, speech and language therapists and orthodontists, but expanding in time to form the more comprehensive teams which provide care in the better Western units. However, equally and perhaps more important are the anaesthetists, paediatricians and nurses, who provide safe care for the children being operated upon. 5. Protocols and techniques need to be evidence-based and appropriate for the situation. Proponents of delayed hard palate closure or nasoalveolar moulding need to reflect whether such techniques, requiring considerable input from a range of clinicians, are appropriate for the Developing World. 6. Politicians and health policy-makers need to be informed of the economic and social benefits of good cleft care. They need to understand that cleft lip and palate treatment is: 1. relatively inexpensive; 2. low tech; 3. concerned with communication – it’s not just cosmetic; 4. important to education and employment AND psychosocial well-being; 5. quality of life surgery.

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CONCLUSION All health-care delivery systems are facing challenges. The problems of cleft care are reflected in these systems with cleft care being a challenge in all of these systems. Surgeons in Developing Countries have a great deal to learn from those working in much more difficult circumstances, with huge workloads but with the opportunity to become world leaders in this field. The priority is good primary surgery, thus minimizing the need for secondary intervention and reducing the burden of care for patients and parents. Although much of the emphasis has been on repair of the cleft lip, in many ways cleft palate repair is more important for communication and longterm outcome and it needs to be given more priority. The aim of all of us should be to improve care worldwide.

REFERENCES Clinical Standards Advisory Group (1998) Report on Cleft Lip and/or Palate, London: The Stationery Office. Kilner TP (1937) ‘Cleft lip and palate repair technique’, in Maingot R (ed.) Postgraduate Surgery, vol. III, London: Medical Publishers. Shaw WC, Dahl E, Asher-McDade C, Brattstrom V, Mars M, McWilliam J, et al. (1992) ‘A six centre international study of treatment outcomes in patients with clefts of the lip and palate: Parts 1–5’, Cleft Palate Craniofac J 29: 393–418. Sommerlad BC (2003) ‘A technique for palate repair’, Plast Reconstr Surg 112: 1542–8. Wardill WEM (1937) ‘Technique of operation for cleft lip and palate’, Br J Surg 25: 97.

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Cleft Lip and Palate Management in the Developing World Primary and secondary surgery and its delivery JAMES LEHMAN

Editorial comment: This chapter is based on the strong personal views of the author and his long-standing experience in South America. Lehman’s views may possibly be regarded as somewhat trenchant but do represent his beliefs and practice. Many might wish to take issue with some of the statements, for example, his views on delayed hard palate closure and that speech is the priority area, together with related comments about the safety of pharyngoplasties and the role of speech prostheses in such an environment. Not everyone in the field has shared his fortunate experience of finding ‘good anesthetic and monitoring equipment’ in the Developing World. While the washing of gloves ‘followed by sterilization’ may be the routine practice in many places, this should not be considered ‘proper techniques’.

INTRODUCTION So you have decided to go on a ‘mission’ outside your home country to perform cleft lip and palate surgery. That decision is fairly easy and you feel good about the prospect of helping disadvantaged children in a Developing Country. The first question you need to ask yourself is: Are you a cleft surgeon? Do you do primary cleft surgery regularly and can you do secondary cleft surgery such as fistula closure, alveolar bone grafts and pharyngoplasty? Even more importantly, do you know how to work with a speech pathologist in a multidisciplinary team setting? If the answers to the above are no, you should rethink your decision or go on a trip where you perform a limited scope of surgery with supervision. Performing this type of surgery outside your own country can be demanding and one should adhere to the standards established by the American Cleft Palate-Craniofacial Association (ACPA). Basically, the ACPA supports the efforts of individuals and

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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organizations to do this humanitarian work but individuals should only provide the type of care for which they have credentials in their home country. The interdisciplinary team approach should be fostered and finally individuals should not attempt to obtain credentials to perform this type of surgery through unstructured surgical experiences outside their home country (Lehman & D’Antonio, 1992). There are two important keys to surgical success: (1) do not leave fistulas; and (2) speech is the number one priority. If you have normal speech, it is easier to get a job even if your lip scar is poor or if you have severe maxillary retrusion. On the other hand, it is difficult to get a good job if your speech is unintelligible no matter how perfect your lip or how beautiful your smile (Neiman & Duncan, 1986)!

CULTURAL ISSUES, CUSTOMS AND FACILITIES Every country has its own unique customs and cultural issues which the visiting team must be sensitive to. You will need to be aware of and respect their values so that misunderstandings can be avoided. For example, in Bangladesh, 260,000 children and adults are waiting for operative correction of their cleft lip and palate. The selection criteria applied may be different than in the western world. Closure of the lip in an 18-year-old girl may be more important than lip closure in a young child because it will increase her chances of getting married (Spauwen & Chandra, 2005).

LOCAL HOSPITAL FACILITIES The facilities in developing countries vary, but most have good anesthesia and monitoring equipment even if the operating room itself is old. Many hospitals reuse their gloves, which is not a problem as long as they are properly sterilized. You must remember you are not at home. Most developing countries resterilize items that we discard and you will not have all the suture choices and special instruments you are used to. If there are special items you feel you will need (instruments, retractors or sutures), take them with you. Take Marcaine and use it on all cleft patients. It will reduce the need for narcotics. Be prepared for surprises. Be adaptive with the equipment. Remember, 90% of the world works on 220 volts. A Swiss Army® knife, superglue, and duct tape are indispensable. The type of hospital where the surgical team will work will influence the volume and scope of surgical procedures that can be performed. In a small hospital (20–30 beds) with only one or two operating rooms and no ancillary services like a blood bank, dedicated recovery room or ICU, the surgery volume will be reduced. The types of cases should be limited to primary lip and palate surgery with no complex surgery. In a large hospital (200 beds) with between four and six operating rooms, a dedicated recovery room and ICU, blood bank and pharmacy, the volume of surgery will be greater and complex secondary procedures can be accomplished.

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PRE-OPERATIVE TEAM ASSESSMENT The pre-operative evaluation should be performed by all the team members on every potential patient and a medical record and photographs taken. The number one priority is to ensure the safety of the patient. The surgeon, pediatrician and anesthesiologist should make the evaluation. The surgeon can decide if the patient’s problem is appropriate, based on the expertise of the team, but the pediatrician and anesthesiologist have the final word regarding medical clearance. Most importantly, a perceptual speech assessment and possibly nasendoscopy should be performed on every patient who presents for correction of secondary speech problems.

TYPE OF SURGERY As indicated previously, the type of surgical facility and its ancillary capabilities will dictate the types of surgical procedures that can be performed. Also the capabilities and training of the team members will influence the scope of procedures that can be performed. In a limited facility, it is probably best to do only primary cases and not to do complex procedures. In a large facility with full service capabilities, primary and secondary surgical procedures can be performed as well as more complex procedures such as orthognathic surgery. Ideally, the goal for patients is to have a well-healed cleft lip with minimal scarring, good nasal correction and a well-repaired and functioning palate without fistula. If we accept the fact that speech is the number one issue, then the priorities will fall into place. Syndromic patients, such as Treacher Collins, with an associated cleft palate can be treated as long as the recovery facilities are adequate. Airway issues seen with Pierre Robin sequence need an ICU for management. Craniofacial patients, such as patients with Crouzon’s syndrome, should be referred to a large medical center in the host country or have special arrangements for them to be treated in the team’s home country. Remember, you do not want to leave a patient with a serious complication that cannot be treated once you return home. In my experience, the pharyngeal flap has a high rate of denasality and is not the best procedure for overseas missions (Pryor et al., 2006). A sphincter pharyngoplasty is a safer approach for velopharyngeal incompetence (VPI) correction with lower risks for denasality (Pryor et al., 2006).

LOCAL SURGEONS The participation of local surgeons with the visiting team is absolutely essential. The visiting team needs to demonstrate how a multidisciplinary team functions and how patients are prioritized. In addition, the local surgeons need to learn how to do the procedures so they can ultimately assume the role of primary surgeon. The goal is to work toward self-sufficiency.

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Teach them a simple basic approach to cleft lip and palate repair that is reproducible. Use the KISS principle! Keep It Simple and Safe. Follow-up care is also an important part of any overseas ‘mission’ and this should be established at a site visit prior to the ‘mission’ (Jacobsen et al., 2005). Suture removal R can be avoided by using Dermabond on lip repairs but some procedures will require suture removal by the local doctor or by the local surgeon you are training. Certain procedures such as Abbe flaps and tongue flaps require a second surgery and should only be done if the local surgeon is capable of performing the second procedure in two to three weeks. ANCILLARY PRE-SURGICAL PROCEDURES When you see the patients at the time of the initial evaluation, most ancillary presurgical procedures are not possible. These include lip taping (Pool, 1995), passive plates, lip adhesions and nasoalveolar molding. In patients with wide unilateral clefts and in bilateral clefts with a protruding premaxilla, lip taping is very helpful in narrowing the cleft gap and in retracting the premaxilla. This is something the local surgeons can do for the patients prior to your arrival. Passive plates and nasoalveolar molding (Grayson et al., 1993) are not practical in the ‘mission’ setting. In special circumstances either a unilateral or bilateral lip adhesion may be appropriate with the plan to complete treatment on the next visit. LIP REPAIR The Millard lip repair (Millard, 1964) is universally accepted as a technique that yields consistently good results because it follows anatomical lines and is relatively easy to reproduce. There have been some modifications (Mohler, 1980; Noordhoff et al., 1995), but basically the key is the initial marking and making the back cut equal to the difference between the two high points of the Cupid’s bow. Muscle dissection with repair and closure of the nasal floor are important complementary parts of the surgery. Doing primary nasal surgery is important as secondary correction will probably not be feasible. Remember, for a lot of these patients this is their only chance. In keeping with this philosophy, we recommend leaving no fistulas. Primary closure of the anterior hard palate in continuity with the nasal floor as performed by the Oslo group (Abyholm et al., 2005) and others using a vomer turnover flap is the best approach (Lehman et al., 1990). This single layer closure of the hard palate simplifies the closure of the remainder of the hard palate and does not interfere significantly with maxillary growth (Lehman et al., 1990; Lehman, 1992). The bilateral cleft lip presents a much more complex situation. Techniques described by Millard and Mulliken both produce reasonably good results (Millard, 1976; Mulliken, 2001). The protruding premaxilla in severe cases presents a difficult situation for the operating surgeon. Pre-surgical taping (Pool, 1995) can reposition the premaxilla over four to six weeks, but this makes surgery at the initial visit impossible. In some situations a lip adhesion may be appropriate. Closure of the anterior hard

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palate and alveolus is not possible at the same time as the lip using bilateral vomer flaps because it may jeopardize the vascular integrity of the premaxilla.

PALATE REPAIR The two-flap palatoplasty initially described by Veau (Veau, 1931) with muscle dissection and posterior repositioning as advocated by Braithwaite (Braithwaite & Maurice, 1968) and Kriens (Kriens, 1969) and refined by Sommerlad (Sommerlad, 2002) is the best approach for overseas ‘missions’. This technique restores normal velopharyngeal anatomy, is reliable and yields excellent surgical and speech outcomes. It is the procedure of choice in my opinion because it is reproducible, safe, and easy to teach. It is also applicable to all types of clefts. The Furlow palate repair (Furlow, 1986; Randall et al., 1986) has received a lot of favorable press but one must remember it is difficult to teach, has a high learning curve, and does not work well with wide clefts. Special care must be taken to address hemostasis of all palate repairs. Bleeding is a serious problem and can be disastrous. Blood bank facilities are usually unavailable at night. If it looks like it’s going to bleed, electrocoagulation of the area is the best rule. Delayed hard palate repair has no place in overseas ‘missions’ (Lehman, 1992; Elander et al., 2005). The claim that delayed hard palate closure results in better facial growth is unsubstantiated (Lehman, 1992; Sommerlad et al., 2005). Palatal surgery undertaken at any time before growth ceases has the potential to cause disturbances in facial growth and dental arches (Mars et al., 2005). This approach violates two basic principles for overseas ‘missions’: (1) it leaves a fistula, which requires a second surgery; (2) it results in poor speech in a high percentage of patients (Lehman, 1992). Secondary velopharyngeal surgery was required in 32% of patients from a recent presentation of the G¨oteborg group (Elander et al., 2005).

LIP AND PALATE TOGETHER When you do a lip repair and you step back and look at the result and take a photo, you have to be pleased, but in the healthy child over six months of age with a complete cleft lip and palate, have you really done them a favor? I think not, because this child will need a second surgery, which he may be unable to get. He may look good, but his poor speech will keep him from getting a job. All healthy children over six months of age who do not live in a situation that is accessible to a second surgery should have the lip and palate repaired in one stage because it is their only chance for good speech. Remember, poor speech may keep the individual from getting a good job reference. This approach is also cost-effective and safe (de May et al., 2005; Gao & Zhang, 2005; Martins et al., 2005). The admonition to do only what you do at home is good advice but one needs to be flexible enough to be creative in special situations. I have personally used this approach and it has been very successful. Adams and associates (Adams et al., 2005)

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have been doing a one-stage repair since 1988 in children over six months and their results compare favorably with that of the Eurocleft results for staged repairs. DO PALATE FIRST AND LIP SECOND In Developing Countries, the nutrition and growth of babies differ greatly from that of Developed Countries. Babies often arrive underweight and anemic and may not be suitable for lip surgery at three months of age. A second surgery for palate repair may not be feasible even at 6–12 months. The usual accepted protocols for cleft management are not suitable. The drop-out rate after lip repair for a second surgery on the palate is high and then the patient is condemned to a life with unacceptable speech. Consideration of these factors prompted Agrawal et al. (2005) to modify their cleft protocol. At six months, the palate is repaired. Three to six months later, the lip and alveolus are repaired. The important point to remember is even if the second surgery is delayed for some reason, the functional outcome is not compromised. Agrawal et al. list the following advantages: (1) no drop-out rate; (2) better followup; (3) anterior fistula rate is low; (4) the repaired palate can be examined at the time of lip repair and fistula can be repaired; and (5) repair of the alveolus is done under direct vision as opposed to repairing the alveolus after lip repair. While I have not used this approach, I totally support it for the situation described. Be flexible and remember that speech is the number one issue. This approach could also be applied on a ‘mission’ where there is not enough operating room time to combine both a lip and palate repair and you want to make sure the family will bring the child back for the second procedure. LIP, PALATE AND PHARYNGOPLASTY In patients over 15 years of age with an unrepaired cleft palate, the chances of a successful speech outcome by repairing the palate alone are very poor. In my opinion, these patients should have the palate repaired along with a pharyngoplasty. This decision should be made in consultation with the speech pathologist but they are almost always in agreement. Saboye et al. (2004) have also published this approach in the Philippines with a very successful outcome for speech. If the lip is also unrepaired, you can do this all together. I have personally used this approach on patients who presented at an older age and who only had one chance to obtain good speech. Your options fall into place when speech is the number one priority.

SECONDARY CLEFT PROBLEMS On every overseas ‘mission’ there are always patients who arrive with complications from previous surgeries. This may be from local surgeons or prior ‘mission’ surgeries. Having the skills to deal with these problems is an important part of the team approach. The following is an approach which has worked.

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VELOPHARYNGEAL INCOMPETENCE (VPI) Correction of velopharyngeal incompetence is the most important aspect of cleft care. A perceptual speech evaluation should be carried out on every child with VPI and, if possible, a nasendoscopy. Following this, a recommendation can be made by the speech pathologist regarding surgery. If surgery is indicated, a pharyngoplasty is preferable because of the lower risk for complications and revisions (Kawamoto, 1995; Pryor et al., 2006).

FISTULAS The incidence of fistulas after palate repair varies but can be as high as 40% (Lehman, 1992, 1995). The occurrence of secondary fistulas represents a failure of surgical repair and is a serious problem in Developing Countries because of the high rate of velopharyngeal incompetence and the lack of resources. The majority of the fistulas are in the hard palate and besides causing VPI, they cause chronic rhinitis and malodor because of the passage of food and liquids into the nose. Closure is important to improve speech and hygiene. In addition, a pharyngoplasty may be a necessary combination for correction of VPI. Failure rates for fistula closure have varied from 12–65% (Lehman, 1992). There are five essential principles to maximize the chances for success: (1) elevation of large palate flaps based on the original incisions; (2) excision of the scarred margins of the fistula; no scar epithelium can be left transversing the fistula site; (3) accurate tension-free closure of the nasal and oral mucosa; (4) use of additional unscarred tissue to close anterior defects or large palate defects (mucobuccal flaps and tongue flaps are most useful); (5) bone graft when indicated but it is not necessary for successful fistula closure. The majority of fistulas seen in Developing Countries need additional tissue for closure and using these principles, one can reduce the recurrence rate to below 10% (Lehman, 1995).

TONGUE FLAP FISTULA CLOSURE A tongue flap for fistula closure is employed when the anterior defect is too large for mucobuccal flaps (Lehman, 1995). In large anterior fistulas the nasal lining can usually be closed utilizing vomerine flaps and then the oral closure is complete with a tongue flap. In Developing Countries, it is important to have good anesthesia and recovery room support before a decision is made to do a tongue flap. In addition, a local surgeon must be able to cut down the flap in two weeks.

SECONDARY RHINOPLASTY Secondary nasal surgery is frequently requested on ‘missions’, but it has a low priority unless there is significant nasal obstruction and the schedule permits it. The approach requires an open technique which most plastic surgeons are capable of performing.

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ALVEOLAR BONE GRAFTS Bone grafting of the cleft maxilla prior to eruption of the permanent cuspid has become an accepted part of the management of patients with clefts involving the maxillary alveolus (Lehman, 1992). The reasons for performing a bone graft are to stabilize the maxillary segments, provide bony support for the teeth adjacent to the cleft, give additional support for the alar base and allow for movement of the cuspid into the lateral incisor position. Typically this procedure is performed at a stage of transitional dentition when the canine root has not fully formed. On overseas ‘missions’, alveolar bone grafting is only possible when there is good dental support to provide preand post-surgical management. To be successful, you need to be working with an established cleft palate team. SCAR REVISION Scar revision has a low priority on most overseas ‘missions’ unless there is a severely deformed lip. Revision can be combined with palate repair. ABBE FLAP Usually this procedure is reserved for severely deformed and deficient cleft lips (Lehman, 1979). Before planning on doing this reconstructive procedure, the surgeon must be in a situation where there is a team or a local surgeon who can perform the secondary cut-down in two weeks. By modifying the original procedure a dynamic Abbe flap can be produced (Lehman, 1979). The plus is that both the cross-lip flap and the cut-down can be performed under local anesthesia. ORTHOGNATHIC SURGERY The most common skeletal deformity in cleft patients is maxillary retrusion (Herber & Lehman, 1993). Correction of this requires a LeFort I osteotomy in the majority of patients. This type of secondary surgery requires a cleft team with established orthodontic services that can prepare the patient pre-operatively and manage the patient post-surgically. Orthognathic surgery also requires experienced anesthesia personnel with recovery room and ICU facilities. The visiting team must bring the rigid fixation equipment because this will not be available in the host country. DISTRACTION Osteotomies followed by distractive osteogenesis have become an integral part of the correction of maxillofacial deformities seen in cleft lip and palate patients (McCarthy et al., 1992). The (KLS-Martin) Rigid External Distraction (RED) appliance allows correction of severe retrusion (>12 mm) (Polley & Figueroa, 1997). As with standard orthognathic procedures, the visiting surgeons must bring the distraction equipment

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and there must be an orthodontist and surgeon who will perform the initial work and the follow-up care. PROSTHODONTIC MANAGEMENT In most overseas ‘missions’, prosthodontic restoration of missing teeth is usually not possible unless there is a dental service and the patient is in a situation where they can return for planning and fabrication. Long-term maintenance is also a problem. Prosthetic speech appliances will be of value for older patients with medical or physical handicaps that prevent a surgical solution. Fabrication requires good interaction between the speech pathologist and the prosthodontist. A prosthesis can assist in speech production as well as obturate fistulae. Again, the issues of long-term management and fabrication make it difficult in the environment of most overseas ‘missions’ to perform this service.

CONCLUSION Working with Rotarian-based ‘missions’ gives one the security of being able to contact individuals in the host country who sincerely support the program. They also organize the local facilities, work on potential patients, and are your local hosts during your stay. They also provide for long-term follow-up. Overseas ‘missions’ provide both direct and indirect contributions to the host country (Salinas, personal communication). The direct contributions include: (1) reduction in the surgical waiting list; (2) help to develop complex procedures such as orthognathic surgery and distraction; (3) donation of orthodontic equipment to get an orthodontic program started; (4) development of non-surgical capabilities such as nasendoscopy. Indirect contributions include: (1) motivation to develop a multidisciplinary team; (2) parents support group; (3) training of young surgeons; and (4) establishment of friendships.

ACKNOWLEDGMENTS This chapter is dedicated to my Chilean friends whom I hope to train out of needing my assistance but not my friendship.

REFERENCES Abyholm F, Ronning E, Kjoll LH, Bollauch FK., Matzen M, Skaare P, et al. (2005) ‘Multidisciplinary management of cleft lip and palate in Oslo, Norway: long-term follow-up of 21 children age 16 born with UCLP’, paper presented at the International Congress of Cleft Lip and Palate, Durban, South Africa.

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Adams TST, Hobby JAE, Phippea G, Richard S & Codier MA (2005) ‘Long-term results of single-stage primary unilateral cleft lip and palate repairs in infancy’, paper presented at the International Congress of Cleft Lip and Palate, Durban, South Africa. Agrawal K, Panda KN & Bolanand S (2005) ‘A modified surgical schedule for cleft lip and palate to suit developing countries’, paper presented at the International Congress of Cleft Lip and Palate, Durban, South Africa. Braithwaite F & Maurice D (1968) ‘The importance of the levator palatini muscle in cleft palate closure’, Br J Plast Surg 21: 60–2. De May A, Swennen G & Malevez C (2005) ‘Long-term results in early simultaneous closure of lip and palate in complete unilateral cleft lip and palate patients’, paper presented at the International Congress on Cleft Lip and Palate, Durban, South Africa. Elander A, Lilja J, Sahlin P & Gudmundsdottin E (2005) ‘Delayed hard palate closure in BCLP patients, surgical procedure and results with G¨oteborg protocol’, paper presented at the International Congress on Cleft Lip and Palate, Durban, South Africa. Furlow LT (1986) ‘Cleft palate repair by double opposing z-plasty’, Plast Reconstr Surg 78: 724–38. Gao J & Zhang L (2005) ‘Applied research in one-time surgeries to repair cleft lip, palate, and alveolus during infancy’, paper presented at International Congress on Cleft Lip and Palate, Durban, South Africa. Grayson BH, Cutting C & Wood R (1993) ‘Preoperative columellar lengthening in bilateral cleft lip and palate’, Plast Reconstr Surg 92: 1422–3. Herber S & Lehman JA (1993) ‘Orthognathic surgery in the cleft lip and palate patients’, Clin Plast Surg 20: 755–68. Jacobsen HC, Hakim SG & Sieg P (2005) ‘Post-op follow-up management in cleft missions’, paper presented at International Congress on Cleft Lip and Palate, Durban, South Africa. Kawamoto HK (1995) ‘Pharyngoplasty revisited and revised’, Oper Tech Plast Reconstr Surg 2(4): 239–44. Kriens O (1969) ‘An anatomical approach to veloplasty’, Plast Reconstr Surg 43: 29–41. Lehman JA (1979) ‘The dynamic Abbe flap’, Ann Plast Surg 3: 401–2. Lehman JA (1992) ‘Cleft palate surgery for the 1990s’, Problems in Plastic and Reconstructive Surgery 2: 76. Lehman JA (1995) ‘Closure of palatal fistulas’, Oper Tech Plast Reconstr Surg 2(4): 255–62. Lehman JA & D’Antonio L (1992) ‘Editorial: Volunteer medical missions’, Cleft Palate J 29: 1. Lehman JA, Douglas BK, Ho WC & Husami TW (1990) ‘One-stage closure of the entire primary palate’, Plastic Reconstr Surg 86: 675–81. Mars M, Batra P & Worrell E (2005) ‘The effects of different timings of palate surgery on the dental arch relationships in Sri Lankan UCLP patients’, paper presented at the International Congress on Cleft Lip and Palate, Durban, South Africa. Martins DM, Martins JL, Lopes LD & Filho NB (2005) ‘Surgical simultaneous repair in unilateral cleft lip, nose and palate in neonatal period: long-term follow-up’, paper presented at International Congress on Cleft Lip and Palate, Durban, South Africa. McCarthy JG, Schreiber J, Karp N, Thorne CH & Grayson BH (1992) ‘Lengthening of the human mandible by gradual distraction’, Plast Reconstr Surg 89: 1–8. Millard DR, Jr (1964) ‘Refinement in rotation-advancement cleft lip technique’, Plast Reconstr Surg 33: 26–38. Millard DR (1976) ‘Bilateral cleft lip repair’, in Cleft Craft, vol. 3, Boston: Little Brown & Co., pp. 51–3. Mohler LR (1980) ‘Unilateral cleft lip repair’, Plast Reconst Surg 80: 511–16.

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Mulliken JB (2001) ‘Primary repair of bilateral cleft lip and nasal deformity’, Plast Reconstr Surg 108: 181–94. Neiman GS & Duncan DS (1986) ‘Perceptions of social and vocational acceptability of adults with facial disfigurement and velopharyngeal insufficiency’, paper presented at ACPA, New York. Noordhoff MS, Chen YR, Chen KT, Hong KF & Lo LJ (1995) ‘The surgical technique for the complete unilateral cleft lip-nasal deformity operative techniques’, Plastic Reconstr Surg 2: 167–74. Polley JW & Figueroa AA (1997) ‘Management of severe maxillary deficiency in childhood and adolescence through distraction osteogenesis with an external adjustable rigid distraction device’, J Craniofac Surg 8: 181–5. Pool R (1995) ‘Tissue mobilization with preoperative lip taping’, Oper Tech Plast Reconstr Surg 2(3): 155–8. Pryor L, LeFaivre JF, Schmidt A, Fox L, Cunningham J, Stoll D, Parker M & Lehman JA (2005) ‘Comparison of speech outcomes and revision rates following pharyngeal flap and pharyngoplasty’, paper presented at ACPA Meeting, Myrtle Beach, SC. Pryor L, Murthy A, Schmidt A, Fox L, Parker M & Lehman JA (2006) ‘Outcomes in pharyngoplasty: a ten year experience’, Cleft Palate J 43: 222–5. Randall P, LaRossa D, Solomon M & Cohen M (1986) ‘Experience with the Furlow double opposing z-plasty for cleft palate repair’, Plast Reconstr Surg 77: 569–76. Saboye J & Chancolle AR (2005) ‘Velopharyngoplasty in an adult patient’, paper presented at the International Congress on Cleft Lip and Palate, Durban, South Africa. Saboye J, Chancolle AR, Tournier JJ & Maurette I (2004) ‘Palatovelopharyngoplasty, an “all in one surgery for adult patients”: our experience in Philippines’, Annales de Chirugie Plastique Esth´etique 49: 261–4. Sommerlad B (2002) ‘A technique for cleft palate repair’, Plast Reconstr Surg 112: 1542–8. Sommerlad B, Mars M, Hay N, Worrell E & Kelkar S (2005) ‘Vomerine flap closure of the hard palate at cleft lip repair. Does it impair maxillary growth?’ paper presented at the International Congress on Cleft Lip and Palate, Durban, South Africa. Spauwen PHM & Chandra PA (2005) ‘Cleft lip and palate treatment in Bangladesh’, paper presented at the International Meeting on Cleft Lip and Palate, Durban, South Africa. Veau V (1931) Division palatine, Paris: Masson.

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Anaesthesia for Cleft Lip and Palate Surgery in the Developing World SARAH HODGES AND ISABEAU WALKER

Editorial comment: This chapter considers the ways anaesthesia may be provided safely using local facilities in a Developing Country. Hodges and Walker have a breadth of experience mainly in rural Africa. They present a comprehensive and flexible approach to anaesthesia, maintaining safety as a core requirement.

INTRODUCTION Surgery for cleft lip and palate in many parts of the world is not available. The incidence is difficult to define, but there is undoubtedly a huge unmet need for surgery in both children and adults. Infants with unrepaired cleft palate have a high mortality due to feeding and respiratory complications. Older children and adults have difficulty integrating into society. Anaesthesia for cleft lip and palate surgery demands good pre-operative assessment and assiduous attention to detail. This is the case in any situation but even more so in Developing Countries where nothing can be taken for granted. Shortages of drugs, equipment and trained personnel make the operating environment hazardous for such specialized surgery. Although conditions in plastic surgical units in some countries are excellent, in many rural hospitals, even the most basic items such as oxygen, intravenous cannulae, drugs and facilities for post-operative care cannot be guaranteed (Hodges et al., 2007).

FACILITIES AND PRIORITIES IN DEVELOPING COUNTRIES STAFFING AVAILABILITY Anaesthesia in Developing Countries is commonly provided by non-medically trained anaesthetists who have undergone a variety of training courses, ranging from up to Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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three years to simply ‘trained on the job’. In many regions in sub-Saharan Africa and elsewhere, there may only be a handful of medically qualified anaesthetists in the country (Hodges et al., 2007). Anaesthesia services are often poorly resourced, being a low priority in areas with significant public health issues such as malnutrition, malaria and HIV/AIDS. Post-operative care on the ward is often largely delivered by parents. There may be few trained nurses, and the children’s wards may be overcrowded and poorly lit at night. Special care needs to be paid to post-operative pain control and instructions regarding observation and charting. Administration of intravenous fluids to a small child in such a setting may be hazardous and impractical. Specialist surgery depends on an integrated team approach, involving different professional skills. This is unlikely to be available in the Developing World. Nursing staff may have no experience of caring for patients after specialized surgery. Protocols used in industrialized countries may not be appropriate and post-operative intensive care or high dependency care is not usually available to manage unexpected complications. Language barriers may further complicate all of these issues. Knowledge of local settings is essential before attempting specialized surgery. The priorities are to undertake surgery on patients who will see benefit from simple interventions, and who will not require sophisticated post-operative care and follow-up, unless concerted efforts are made to provide this (Dupuis, 2004). Nevertheless, many successful teaching programmes have been established, and from small beginnings have led to sustained improvement in local facilities (Ward & James, 1990). ESSENTIAL EQUIPMENT FOR ANAESTHESIA Anaesthetic machines in Developing Countries should be inexpensive, easy to understand and to maintain, robust, able to withstand extreme climatic conditions, serviceable using locally available skills, economical to use, and should not rely on the provision of cylinders or a continuous electricity supply (Eltringham et al., 2003). For these reasons, drawover anaesthesia using ether or halothane is the norm in many rural areas with oxygen supplied by an oxygen concentrator (Dobson, 1993; 1999). Continuous flow anaesthesia may only be possible in larger centres where compressed gases are available (pipeline or cylinder). The Glostavent is an anaesthetic machine that has been specifically designed for use in Developing Countries (Eltringham et al., 2003). It consists of a ventilator, based on the Manley Multivent, drawover apparatus with OMV vaporizer, an air compressor and an oxygen concentrator, with the components mounted on a robust metal frame with two reserve oxygen cylinders. Under normal circumstances, the oxygen concentrator and the air compressor are used as a source of fresh gas – if the electricity supply fails, the reserve oxygen cylinders are turned on to provide back-up oxygen for 20 hours use. The Glostavent has been shown to be cost-effective and to function reliably in adverse conditions. Current versions of the Glostavent ventilator are suitable for use in patients >25 kg; a paediatric version of the Glostavent ventilator is currently under development (Eltringham RJ, personal communication). The standard Glostavent can be used in continuous flow

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mode for children using an anaesthetic T-piece and manual ventilation. An alternative for young infants <20 kg is the drawover apparatus using the Oxford Inflating Bellows to provide the fresh gas flow for the anaesthetic T-piece (Hodges & Hodges, 2000). Anaesthetic agents in common use include ether, halothane, isoflurane and ketamine; the cost of modern anaesthetic agents such as sevoflurane and propofol makes them prohibitive for local economies. Suxamethonium is usually available (often in powdered form for reconstitution); non-depolarizing muscle relaxants are not always available, neither is neostigmine for reversal. Disposable anaesthetic equipment, and paediatric equipment in particular, are in poor supply. It is routine for anaesthetic facemasks, endotracheal tubes and Guedel airways to be washed in dilute bleach solution and reused (Hodges et al., 2007). Equipment for difficult intubation such as stylets or bougies may be purchased or improvised (Bem & Wilson, 1991); sophisticated equipment for fibre-optic intubation may be available, but only in major centres. Pre-cordial stethoscopes and manual sphygmomanometers are used for routine monitoring. Pulse oximeters, capnography, ECG and automated blood pressure monitoring may also be available, but are not the norm. Electricity supplies may be erratic. A back-up generator is required to maintain continuity of oxygen supply from the oxygen concentrator and recharging of batteries for monitoring devices. Induction and recovery from anaesthesia are usually in theatre as a recovery area with separate staff and equipment such as oxygen and suction may not exist (Hodges et al., 2007). PATIENT SELECTION AND PREPARATION Patients should be carefully selected and prepared for surgery. It is useful to have the patients assessed some weeks in advance to allow treatment for concurrent diseases such as malaria or anaemia. Nutritional and iron supplements may be appropriate and will improve the outcomes from surgery. Children should be screened for associated congenital lesions such as airway and cardiac abnormalities. These children should be referred to a regional centre if available, where clinical expertise exists to diagnose the underlying problem and then to manage their surgery. The timing of surgery will vary according to local facilities and the underlying condition of the child. For instance, it may be appropriate to delay surgery in a young infant with Pierre Robin sequence to avoid the requirement for a nasopharyngeal prong which may be difficult to manage safely in a challenging environment. Similarly, a child who is significantly malnourished should have surgery delayed and a supplementary feeding programme instigated. Pre-operative assessment may require the services of a local interpreter. The clinical history often lacks detail, and clinical records may be rudimentary or non-existent. Careful clinical examination, particularly cardiac, respiratory and airway will reveal most problems pertinent to surgery and anaesthesia. Surgery in infants with an acute coryzal illness should be considered carefully as respiratory complications may be difficult to manage post-operatively. Since cleft lip and palate is a non life-threatening malformation, a child can usually be re-scheduled, although this can never be guaranteed in rural environments.

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Clinical investigations will depend on local laboratory facilities, but a full blood count should be routine for palatal surgery or clinically suspected anaemia. In the R absence of a formal haemoglobin assay, a Hemocue is an excellent substitute (Ickeringill, 2001) or the WHO paper colorimeter system can be used. A malarial screen should be performed in any child with a fever in endemic areas. When drawing up the theatre list, ensure adequate time for induction and recovery from anaesthesia in theatre.

LOCAL ANAESTHESIA AND SEDATION Older children and adults undergoing straightforward lip repair may be suitable for local anaesthesia, with the option of judicious intravenous sedation with midazolam or ketamine if required. Careful explanation should be given to the patient as to what to expect. Safe doses of local anaesthetic are determined by the weight of the patient (7mg/kg lignocaine with adrenaline 1:200,000; 3mg/kg lignocaine plain, 3mg/kg bupivacaine). A mixture of lignocaine and bupivacaine may have the advantages of rapid onset and prolonged duration, remembering that local anaesthetic toxicities are additive. All young children and any patient undergoing palatal surgery will require general anaesthesia. CONDUCT OF ANAESTHESIA Patients should be fasted in line with current guidelines. Pre-emptive analgesia in the form of oral paracetamol is useful but logistics may make this difficult – paracetamol suppositories after induction of anaesthesia are an alternative, if available. Parental presence on induction obviates the need for pre-medication in anxious children, but this may not be common in local practice and parents should have an explanation of what to expect. The occasional uncooperative child is most practically managed with oral midazolam or ketamine. The anaesthetist should ensure that all their equipment is fully checked, and that everything that may be required is present in theatre. An anaesthetic assistant should be identified and briefed on the anaesthesia plan. It is sensible to make use of all available team members, including the surgeon on occasion! Induction of anaesthesia may be inhalational or by intravenous or intra-muscular injection. Halothane is the inhalational anaesthetic agent of choice as ether is irritant and provides a very slow induction. However, inhalational induction using the drawover apparatus with halothane is extremely difficult to achieve and this technique is better reserved for use with a continuous flow machine. Intra-muscular ketamine is a useful alternative in small children. The agent for intravenous induction depends on local availability; ketamine or thiopentone is commonly used. Atropine should be used with ketamine to reduce secretions; it is also useful to prevent unexpected bradyarrhythmias (Hodges & Hodges, 2000).

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Tracheal intubation with a preformed south-facing tube is routine following paralysis with suxamethonium and where a difficult intubation is predicted, a bougie or stylet should be available. In an emergency, a coat hanger wire through a nasogastric tube has been described (Bem & Wilson, 1991). Care should be taken that the preformed tracheal tube is not too long, a problem especially in small malnourished children. Anaesthesia is maintained by inhalation or total intravenous anaesthesia with ketamine and with muscle relaxation, if non-depolarizing drugs are available. Intermittent suxamethonium may be used, but care should be taken not to exceed a maximum dose of 8mg/kg (Hodges, 2002). Ether is flammable in air and explosive in oxygen. Diathermy should not be used if ether is used for maintenance of anaesthesia. Atropine and ether may both result in a relative tachycardia – this is to be expected and is normally well tolerated. Treatment by deepening anaesthesia will result in delayed emergence. Respiration should be supported throughout by hand or mechanical ventilation. Ventilation in children <20 kg using the drawover apparatus may be achieved with a combination of the Oxford Inflating Bellows and an anaesthetic T-piece (Hodges & Hodges, 2000). Paediatric ventilators are uncommon. Use of a throat pack is routine but care should be taken to remove it at the end of surgery and this should be communicated clearly, as theatre routines may not be well developed (e.g. written record of pack insertion). The tracheal tube may be compressed when the Boyle Davis gag is inserted and as capnography is rarely available, this is best detected by a change in resistance to hand ventilation or a change in breath sounds audible with the precordial stethoscope. Antibiotic prophylaxis is routine. Intra-operative analgesia is partially provided by infiltration with lignocaine and adrenaline to the surgical field. Care should be taken not to exceed the recommended doses of adrenaline when halothane is being used (7mg/kg lignocaine with 1:200,000 adrenaline). The combination of halothane, hypercarbia, light anaesthesia and exogenous adrenaline is a potent stimulus for cardiac arrhythmias. If arrhythmias are detected by pre-cordial stethoscope, or ECG if in use, the patient should be hyperventilated and halothane concentration reduced. An infra-orbital nerve block should be performed at the end of surgery following lip reconstruction, ideally with a long-acting local anaesthetic such as L-bupivacaine. The use of parenteral morphine (max 0.1mg/kg) or pethidine (max 1mg/kg) intraoperatively is controversial. Useful analgesia may extend into the post-operative period, but there is a risk of post-operative respiratory depression in small infants. This is a particular concern with limited observation on the wards after surgery. Fentanyl may be available as an alternative. When ketamine has been used for induction, the dose of opioids should be reduced as a synergistic action may be anticipated. Paracetamol and NSAIDs (non-steroidal anti-inflammatory drugs) should be given at regular intervals post-operatively. For palatal surgery, a routine dose of dexamethasone, 0.25mg/kg, is advisable to counteract any possible oedema that might develop (dexamethasone is also a useful anti-emetic for the older child).

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The child should be well hydrated using Hartmann’s solution, 0.9% saline or Ringer’s Lactate intra-operatively. As the use of intravenous fluids on the ward may be hazardous, it is important to give maintenance and replace losses from pre-operative fasting. Oral fluids (including breast feeding) should be encouraged following surgery. Blood is rarely required; a healthy child will tolerate a haemoglobin level as low as 6 g/dl before transfusion is indicated. It is important to remember that transfusion safety varies considerably around the world. Surgery usually lasts one or two hours and precautions should be made to maintain body temperature, depending on the ambient theatre temperature. In hot climates, a small child may become pyrexial with the combination of ether and atropine. In cool climates, hypothermia occurs readily and will cause delayed recovery. Good communication with the surgeon should ensure that the volatile agent is reduced appropriately to allow awake extubation after pack removal and suctioning under direct vision. Emergence will be prolonged when using soluble agents such as halothane or ether. Some children may require a nasopharyngeal airway which will be inserted by the surgeon at the end of surgery. A tongue suture may be used as an alternative.

POST-OPERATIVE RECOVERY AND COMPLICATIONS The child should be fully recovered in theatre to allow for continued oxygen therapy, suction and observation by a member of the theatre team. As recovery may be prolonged, it may be useful to run two parallel tables in one theatre to reduce turnover time between cases. Patients can be discharged to the ward when they are fully awake, no longer require oxygen, are not bleeding, and are able to support their own airway without signs of obstruction. Ward staff should be given clear post-operative instructions. The parents should be included in this process as they are usually the main carers. Early feeding is encouraged, but care should be taken that this does not include local solid foods which may be relatively hard. The intravenous line should be protected and can be removed the following day if recovery is uneventful. Oral analgesia should be prescribed regularly, rather than as required. A clear anaesthestic record should be included in the patient notes. Problems on the ward should be anticipated, the main concerns being post-operative bleeding and airway obstruction. The use of opioid analgesia should be limited to the intra-operative period for this reason and post-operative analgesia provided with regular paracetamol and NSAIDs. These may have to be provided if not available locally. Ideally patients should be nursed together in one area of the ward where they are clearly visible to the limited number of nursing staff. There should be clear protocols for when and who to call for help as most post-operative deaths are preventable and may be avoided by good observation and clear communication.

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VISITING TEAMS AND TRAINING Peri-operative complications are not uncommon in any group of young children undergoing surgery on the airway (Murat et al., 2004). In the Developing World, they may comprise the expected – laryngospasm, bronchospasm, difficulties in intubation – and the unexpected, such as failure of the electricity supply and/or the oxygen supply. It is imperative that the anaesthetist is skilled in paediatric airway work and that there is a back-up plan, for instance, a self-inflating bag in the event of oxygen failure. Operation Smile published a report of more than 5000 general anaesthesia cases performed in 1998–99 (Fisher et al., 2001). They confirmed a higher incidence of complications in younger patients; intra-operative complications included respiratory problems (5%), and cardiac arrhythmias (3.3%), including ventricular tachycardia, the latter probably associated with the high use of halothane (86%). Overall mortality during the Operation Smile programme was very low (4 deaths in more than 9000 operations in the same period), but avoidable factors were highlighted: undiagnosed pre-existing conditions, failure of the hospital oxygen supply and absent monitoring. Visiting medical teams undertaking cleft lip and palate surgery in the Developing World must remain sensitive to the local conditions in the hospital where they are working, and be cognizant of long-term objectives. The anaesthetist needs to work as part of a team that is trained and experienced in working in difficult environments. Clinicians should consider previously described conditions on the ground, supplies, integration with local health-care workers and must avoid medical colonialism (Zbar et al., 2000). Visiting teams should not undertake work that they do not usually do at home (Dupuis, 2004). The impact of the visitors on the work of the department (for instance, emergency Caesarean sections) needs to be considered. Nevertheless, visits by specialist medical teams offer great opportunities for training and support of local health-care workers that may have a lasting impact in developing specialist services. Regular visits to the same institution, identifying individuals who are able and willing to train with the visiting team, fostering long-term links and responding sensitively to the needs of the organization are all ways to achieve these aims.

CONCLUSION Anaesthesia for cleft lip and palate surgery in the Developing World presents a challenge to the anaesthetist. With careful patient selection, a skilled team, an awareness of local conditions and attention to detail, remarkable results may be obtained in this highly deserving group of patients. The restoration of dignity and hope from a life blighted by cosmetic and functional disability is possible. The long-term aim should be to work with local teams to foster the development of local specialist services.

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ACKNOWLEDGEMENTS We would like to thank Iain Wilson for his encouragement and helpful comments on this chapter.

REFERENCES Bem MJ & Wilson IH (1991) ‘Coat hanger wire, as an aid to endotracheal intubation’, Top Doctor 21: 122–3. Dobson M (1993) ‘Drawover anaesthesia: practical applications’, Update in Anaesthesia 3: 17–23. Dobson M (1999) ‘Oxygen concentrators for the district hospital’, Update in Anaesthesia 10: 61–3. Dupuis CC (2004) ‘Humanitarian missions in the Third World: a polite dissent’, Plast Reconstr Surg 113: 433–5. Eltringham RJ, Wei FQ & Wilson T (2003) ‘Experience with the Glostavent anaesthetic machine’, Update in Anaesthesia 16: 31–5. Fisher QA, Nichols D, Stewart FC, Finley GA, Magee WP & Nelson K (2001) ‘Assessing pediatric anesthesia practices for volunteer medical services abroad’, Anesthesiology 95: 1315–22. Hodges S (2002) ‘Alternative anaesthetic techniques in less affluent hospitals’, Update in Anaesthesia 14: 29–30. Hodges S & Hodges AM (2000) ‘A protocol for safe anaesthesia for cleft lip and palate surgery in Developing Countries’, Anaesthesia 55: 436–41. Hodges S, Mijumbi C, Okello M, McCormick B, Walker I & Wilson I (2007) ‘Anaesthesia services in Developing Countries: defining the problems’, Anaesthesia 62: 4–11. Ickeringill M (2001) ‘Using the Hemocue’, Update in Anaesthesia 13: 9–10. Murat I, Constant I & Maud’huy H (2004) ‘Perioperative anaesthetic morbidity in children: a database of 24,165 anaesthetics over a 30-month period’, Pediatric Anesthesia 14: 158–66. Ward CM & James I (1990) ‘Surgery of 346 patients with unoperated cleft lip and palate in Sri Lanka’, Cleft Lip Palate J 27: 11–15. Zbar RIS, Rai SM & Dingman DL (2000) ‘Establishing cleft malformation surgery in developing nations: a model for the new millennium’, Plast Reconstr Surg 106: 886–9.

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Approaches to Organization

Editorial comments on Chapters 6–9: The variations in organizational approach and structure presented in these chapters serve to emphasize that there is no ‘right answer’ for the transfer of skills and the delivery of treatment initiated by those in the Developed to those in Developing Countries. Some concentrate on local volunteers, others on ‘missions’. The local conditions in different countries determine that a flexible range of approaches is both important and necessary. The delivery of care by visits to many countries is contrasted with a focus on just one country. Treatment, teaching and research may be accorded different priorities by these teams, as outlined in the different chapters. What is evident from each group is a passion and commitment to improve the lives of others. Unfortunately, the offer to Smile Train to contribute was declined, in part because they are in a period of change in their organization.

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Operation Smile BILL MAGEE

Editorial comment: The forcefulness and long-term commitment of the founder of Operation Smile must be recognized. The combination of a charitable institution with high-powered business structures has been a fruitful source of ideas and innovative processes combined with acceptance of the need to reinvent the organization when required. The author is to be commended for acknowledging the crisis in the 1990s. New rigorous programmes of care and anaesthesia were then introduced. The high school programme reaching out to the younger generation is particularly attractive. The essential difference between emotion and reason is that emotion leads to action while reason leads to conclusions. (Donald B. Calne, Professor of Neurology, University of British Columbia)

INTRODUCTION In November of 1982, my wife Kathy, a trained nurse, and I were given the opportunity by Dr Bill Riley and his team, Philplast, to travel to the Philippines to take care of children with cleft lips and cleft palates. As a young plastic surgeon I never could have anticipated the reality of what I eventually saw. On that trip one of the stops was Naga City, in the Bicol region of Luzon, where approximately 300 patients pushed into a small area to be screened for surgery. Every one of those children had a gaping hole in the lip and roof of his or her mouth and could not speak an intelligible word. We were only able to take care of about 40 children that first year and had to watch as over 250 children were sent away. The Philplast team was not planning to return to Naga City the following year, so after consulting Bill Riley we decided to gather a group of friends to go back and take care of the children that we had turned away. As I reflect on that first trip, the primary reason that I went was to gain greater exposure to taking care of children with clefts. Yet the images of poverty, hopelessness and needs of those families remained very present in my memory. Having had to turn away 250 children was not something that I could forget. The next year, another 300 Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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or more families came to seek care and we were, once again, forced to turn hundreds of children away. On our return to the United States our friends told their friends about the experience and the great need for children to receive surgery, and their friends told their friends. Before we knew it, these activities had become yearly events. In 1987, we were invited to go to Liberia and Kenya. With the help of the Philippine community and many individuals in Virginia, we were able to raise the necessary funds to begin trips to those countries. Today we have teams in 25 countries and have operated on well over 100,000 children in the 25 years of our existence. The growth and development of Operation Smile have been one that has included both successes and failures. Each of these opportunities led us to a greater understanding of the world around us, of the nature of humanitarian care, and of the importance of producing a sustainable model for long-term success. Back in 1982, this was relatively uncharted territory. Today we have a relatively clear and concise idea as to what creates a successful mission and organization. It has been in the wake of failure that we have grown the most, and it is this growth which has enabled us to have the knowledge and experience to achieve even greater success. Failures became valuable learning opportunities that, although painful, were essential for the long-term growth of the organization.

THE GOVERNANCE OF OPERATION SMILE Back in the early 1980s, we began to put together a Board of friends and associates from local contacts in the greater Norfolk, Virginia, area. This was a group who were successful in business and medicine within our community. This allowed us to grow and gave us the governance to meet the necessary requirements of a not-for-profit charitable organization. After four or five years of this type of governing structure, it became obvious that the needs of Operation Smile had grown beyond the fundraising capacity that our immediate area could provide. It was at that time that the Chairman of our Board suggested that all the original board members step down, giving us the ability to organize a Board on a national scale. Today the governance of Operation Smile is similar to any successful non-profit organization structure (Figure 6.1). The Board oversees the hiring of the Chief Executive Officer. The CEO then hires the management of the organization. At the top of our governance structure is the CEO who has reports from the Chief Operating Officer (COO) and the Chief Medical Officer (CMO). The Chief Medical Officer controls the medical education venues, and the quality assurance of missions. The Chief Operating Officer is in charge of the operational side of the organization with reports from warehousing, finance, development, brand marketing and public relations. The organization has grown from one employee first hired in 1985 to over 100 employees presently working worldwide in offices in Rome, London, Dublin, Hong Kong, Brisbane and foundations with boards of directors and legal entities in all of the 25 countries.

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Material Mgmt Director Logistics & Material Mgmt

Figure 6.1. The organization chart of Operation Smile.

Education Director of Education

Central Regional Director

Latin America Resource Development Coordinator

Latin America Regional Director

Asia Regional Director

International Programs

Grants

Advisor

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HR & Administration HR Director

Missions Program Manager of Missions

Donor Relations Director of Donor Relations

Business Operations

Finance Director of Finance

Brand Marketing VP Brand

Office of the Founders

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Development Global Development

Resource Dev. Senior VP Response

CMO

President & Co-Founder

Board of Governors

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CEO & Co Founder

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The Operation Smile program has grown dramatically from a mission-driven organization beginning with an annual trip to the Philippines during the first five years, to one that today has 43 operational sites in 25 countries. Some 60% of the surgery is performed by local doctors on a year-round basis. This is an evolution that took years to develop. It could not have occurred without emphasis on education and resource development within the partner countries and by individuals in those countries.

CHRONOLOGY OF MILESTONES OF THE ORGANIZATION The emotional experience of the first trip to the Philippines in 1982 led to multiple visits to that country which continue to this day. In 1985, our first employee was hired to help us organize the visits. Prior to this, planning was from within my office. In 1987 and 1988, when we took on Liberia and Kenya as mission sites, the organization experienced major growth. This led to the need for more resources and an office of its own. As countries were added year after year, storage facility needs changed, as did the program and resource needs. We shifted locations almost every other year to gain more space and accommodate more employees. By the early 1990s, ten years into our growth, we had hired our first CEO. We were visiting six countries and had moved into a significant office with warehouse space. Our teaching programs at that time consisted mainly of an educational day during the two-week mission experience. The usual mission schedule involved two or three days of screening and then an educational day, followed by one day off and then a five-day surgical week. We felt that this mission schedule could not last for more than two weeks because experienced clinicians were unable to give more of their time. These two weeks had to be packed with energy, enthusiasm and emotional reward for the participants, encouraging their return. The early days involved surgery by ‘the seat of our pants’ with no specific guidelines, other than operating in what was considered to be appropriate in that country. We recognized it was not the same level of care that existed in the United States, however, it was considered to be acceptable at that time in that milieu. In the early 1990s, we met with our first significant setback having hired our first CEO and discovering one morning that the organization, after a little over a year of his leadership, was bankrupt. We had approximately 12 employees and found ourselves on the front page of the local newspaper acknowledging the fact that we had to let every one of our employees go. One of the original business people who had helped us begin the organization arranged financial support and within a week enabled us to hire our people back and to raise money that would not have come in had the crisis not been created. We learnt how important a sound business structure was within the organization. There is a well-known business author named Jim Collins who co-wrote the book Built to Last (Collins & Porras, 1994), which documents successful companies of the twentieth century. One of the basic principles of the book is that all of these successful companies had a ‘B.H.A.G.’. This stood for ‘Big Hairy Audacious Goal’. One of the basic principles of this was that if you do not have a stretch goal for the organization,

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you will not be able to grow because you will have no internal excitement to motivate people to accomplish more than what they would think possible. As we approached the millennium in 2000, a decision was made to organize an event that would stretch the organization. This ‘B.H.A.G.’ was coined ‘The World Journey of Hope’. The value that this ‘B.H.A.G.’ represented was incredible. The beauty of it was that the young people who worked for us, now about 40 in all, really had no idea that it was possible for this ‘World Journey of Hope’ to fail. It was because of their energy and enthusiasm, as well as the leadership of a previous mission coordinator employee who had come back to help us run this project after graduating from the Darden School of Business, that we were able to pull it off. With growth, however, also comes a certain amount of unrest and turmoil within the organization. This was something that we as novices in the business world of humanitarian care did not anticipate. Ten million dollars were raised for this event which took us to 18 countries in nine weeks, during which we operated on 5,300 children. This mission, in and of itself, was tremendously successful and did stretch the organization and show where the weaknesses existed. The goal of this World Journey of Hope was to create sustainability in these countries. By this time in the life of the organization we recognized that in order to really have a sustainable model, it could not be just ‘mission trip’ driven. We would have to encourage the in-country foundations not only to operate on a more year-round basis, but also to begin to develop real business structures of their own. At this time in medical meetings many people were talking about sustainable structures for humanitarian organizations. Education of medical professionals would be the key to sustainability. The mantra was that this could only be attained by training plastic surgeons. What I have found over the years is that this, in fact, was not enough. For Operation Smile to be successful, it could not just be highly skilled medical professionals. It also had to be a good business structure with the ability to raise dollars and manage people. One may have great physicians and health-care professionals, however, without a business structure and a mechanism to raise money in the countries, sustainability is unlikely. An attempt was therefore made to develop legal entities in every single country. These comprised Boards of Directors and management structures. We taught them how to raise money because this was not inherent within the majority of the cultures of the countries where we were operating. We raised a total of ten million dollars for the World Journey of Hope and with that money equipment and supplies were purchased for one operating room for each of the 18 countries. We developed a contract that ensured that one child a day would be operated on while we were gone. A successful, yet controversial development was the decision to lease an L1011 flying hospital which landed in each country. The ‘Flying Hospital’ branded the trip and drew the attention of the political and business leaders in each of the countries. This massive plane had three functioning operating rooms within it (Figure 6.2). As we landed in each of 18 countries during nine weeks, it brought many political and business leaders to our organization to visit the L1011 flying hospital plane. This generated useful publicity for each of the in-country organizations.

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Figure 6.2. Patients and families wait to be screened outside the flying hospital.

As a consequence of the World Journey of Hope in 1999, 60% of all surgery is performed year-round by local volunteer surgical teams funded by the local Boards. We aspire to 90% of surgery by 2010 being provided by these local teams.

PROBLEMS ARISING FROM RAPID EXPANSION The breadth and magnitude of the organization created a tremendous amount of internal controversy. By 1999, we had operated on about 50,000 children and had the unfortunate experience of approximately 18 deaths. One of these in China in August 1998 became the rallying cry for anonymous emails that were critical of the organization, and this story was transmitted worldwide. The New York Times ran a story on the front page two days in a row about the organization in November 1999. To say that this was not painful would be to minimize its impact. Following the article, it was obviously necessary to look at the organization anew from every aspect, including the governance and medical policies and procedures. An outside firm was brought in at the suggestion of the Board to analyze everything. The ultimate result, less than a year later, was a change in leadership at the top of organization and a complete revamping of the policies and procedures to fill in any cracks that were found. The magnitude of this attack stimulated the greater majority of the personnel, who were still loyal to the organization and what it stood for, to ‘bunker down’ and to make sure that any possible frailties in the organization were corrected.

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Although this was an extraordinarily painful time in the life of the organization, it allowed us to do something that we would have never done otherwise. The attack forced us into a whole new way of thinking with the realization that we should not operate on any child, in any country, by any different standards than we would operate on a child in our own country. Most of the deaths were anesthesia-related, some were post-operative, related to respiratory obstruction or dehydration post discharge. The introduction of end-tidal CO2 monitoring, pulse oxymetry and portable Hawk ventilators improved the safety of subsequent anesthesia. More rigorous screening of the credentials of volunteers was instituted. Putting in place Medical Councils, Global Standards of Care and education by the country Boards has ensured an improvement in patient safety. During the next year as this evaluation process unfolded, it ultimately led not only to change in the leadership of the organization (as previously mentioned), but also to the creation of significantly new positions such as Chief Medical Officer and an inhouse Director of Technology. Greater emphasis was placed on the governance of the organization. Quality control protocols, as well as standards of care for equipment and a more thorough evaluation of local sites and practices, were instituted. We reviewed our programs of education and surgical visits.

EDUCATION As long ago as 1987, we began to send surgeons to the United States from our mission countries. These surgeons were brought to Norfolk, Virginia, for a two- to three-week period of intensive training in craniofacial surgery. Equally as important as the training was bringing them together to understand that they were part of a global organization. This has continued to expand so that as of today close to 800 health-care professionals have come to the United States for training. This program coincided with our ‘World Care Program’. As part of that, over 100 children have been brought to the Children’s Hospital in Norfolk, Virginia, where, through the generosity of that hospital and its staff, these children with severe facial deformities such as Crouzon’s Syndrome, hypertelorism and encephaloceles have been given a new lease of life. This program has expanded from being just for surgeons to include anesthesiologists, nurses and dentists. Expanded educational opportunities have been created on the local level within countries, as well as structured educational programs for the different specialties on a larger scale. We have had the privilege of working closely with the American Heart Association, being the only organization permitted by them to give out the American Heart Association accreditation in Pediatric Advanced Life Support (PALS), Advanced Cardiac Life Support (ACLS), and Basic Life Support (BLS). Over 1,600 individuals have been accredited under that program. Links have been established with major universities in the United States and around the world including Harvard, Yale, George Washington University, Duke, University of Southern California, Toronto Hospital for Sick Children, the University of Southern

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Illinois, University of Pennsylvania in Hershey, the University of Rome and many others. Microsurgical education and fellowships have been established in as faraway places as Oman in the Middle East, as well as in Taipei with Dr Fu-Chan Wei. Major educational programs have also included conferences such as those supported by the Annenberg Foundation in Shanghai where two conferences have been held. Each conference had about 400 participants and a faculty of internationally renowned teachers selected by the Chinese. Operation Smile has funded and totally underwritten the expenses for these.

STUDENT PROGRAMS Another unique aspect of Operation Smile has been its high school student program. This now includes over 500 high schools involved on a year-round basis and an annual leadership conference which is in its 15th year. Each summer at a different university, approximately 400 high school students come together for a week of intense leadership training. Out of this group two high school students are picked to go on to each of the mission sites. These students go through extra training during a four-day session to gain greater cultural understanding of the country they are to visit. They also learn to present culturally sensitive educational programs which they deliver to the families who are awaiting surgery. These programs cover topics such as oral rehydration, burn prevention, oral hygiene and nutrition. Student volunteers also visit local high schools and talk about volunteerism and how students in those countries can get involved and help. High school students from 17 different countries participated in the 2007 program which was held at the University of Limerick in Ireland.

LOOKING AHEAD Now in our 25th year, we recognize that it is time to change, once again, some of the direction of the organization. In order to continue to treat more children, we cannot rely solely on a mission model. If we were to continue to rely solely on this model, it would be extremely difficult to grow. We realize that year-round care is an essential ingredient in our organizational growth. Yet we also passionately believe that we must maintain our mission model for the emotional and dynamic components that it adds to the mixture. We also recognize that the continued emphasis on education is critical. We also realize that in order to grow, increased funding is essential and programs on the development side must also change. The infusion of three million dollars from three donors to develop a direct response television program has enabled us to show the need that exists to the public who have responded generously. The use of the internet for fundraising has also shown incredible benefits. Our organization went from a US$8 million organization in 2002 to a US$30 million organization in 2007.

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This has been the result of the growth of the business structure of the organization to meet the medical needs that we saw. The development of IT capabilities was essential to develop not only communication, but also some of the educational products which we now deliver. Programs such as computerized medical records, as well as digital imaging of all of the patients at the time of surgery, immediately after surgery and six months later will enable us to make significant contributions to the value of different procedures at the hands of different surgeons. We will be able to quantify ‘what works best’. Research programs began in the 1990s with Dr Jeff Murray of the University of Iowa and Dr Ron Munger of Utah State, and are now being expanded with the ultimate goal of finding the causes of ‘clefting’.

LONG-TERM GOALS OF THE ORGANIZATION The long-term goals of the organization are similar to what we began with years ago. In the early 1980s, every team that we sent out consisted of all the disciplines of a cleft team to include plastic surgeons, anesthesiologists, nurses in the pre-operative, operative, recovery room, and post-operative phases, as well as dentists, speech pathologists, child life specialists, psychologists, etc. This model continues today. We have also recognized that in order to really be able to catch up with the growth rate of clefts, however, we could not just have a mission model. So as we enter our 25th year we have decided to create another ‘B.H.A.G.’. The World Journey of Hope in 1999 propelled us into local mission care year-round. Today, eight years after its occurrence, we are able to see the fruits of its labor. So, on November 8, 2007 we will begin the ‘World Journey of Smiles’, simultaneously positioning 1,500 volunteers at 43 sites in 25 countries worldwide in one 24-hour period. This World Journey of Smiles will result in operations on approximately 5,000 children, but more importantly it will become the catalyst for the opening of six fixed centers in countries. Centers have already opened in Colombia, Honduras and Vietnam with future centers in Brazil, China and the Philippines in the very near future. This continued process of the development of fixed centers for year-round care in these countries will be one of the mainstays of future development. This will occur concomitantly with a decentralization process where regional centers will provide administrative guidance and structure. The center for the Asian region will be Hanoi in Vietnam, for the Latin American region in Panama and for Africa and the Middle East in Rome. This will allow us to prepare our supplies and equipment and to develop missions out of the local areas efficiently and effectively so that we can take care of more children. This development of centers will in fact increase the need for volunteers to care for the number of children who will access us to address their surgical needs. The international teams will continue in the mission form, but will also be needed to help some of the centers. These centers will have a large component of volunteer caregivers. We anticipate that the centers will allow us to double the number of children that we are treating within the next year.

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It is my strong belief that volunteerism should be the key to this process. An individual financial incentive takes away the emotional rewards that are the main driver of our volunteer base. The fact that our volunteers selflessly give their time and talent remains the only ‘fuel’ necessary to run their individual engines. Our long-term goal with Operation Smile is certainly to make sure that every child that is born with a facial deformity can be treated as early as possible after birth. But Operation Smile has always believed that the value of what we do transcends that of just taking care of a child and their family. The ripple effect of the activity is significant. In order to accomplish these goals, we will continue as an organization to merge the best of the private sector, the corporate sector, the political sector, the ordinary individuals and medical volunteers to utilize each of our talents to the maximum in order to create something that is greater than any one of us could deliver by ourselves. This is the long-term goal of Operation Smile. It is embedded in a strong desire to use our skill sets – ‘Changing Lives – One Smile at a Time’.

REFERENCE Collins J & Porras JI (1994) Built to Last: Successful Habits of Visionary Companies, New York: Harper Business.

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Rotaplast International A study in medical volunteerism ANGELO CAPOZZI

Editorial comment: Rotaplast International’s history, medical framework and particular emphasis on the role of non-medical volunteers from both the donor and host countries are discussed. Ongoing partnership with a genetics research team is highlighted. The success of their model with an increasing emphasis on returning to the same sites marks out an organization in transition. Finally, an audit of the complications of surgery is presented frankly.

INTRODUCTION Rotaplast International Inc., a non-profit organization, seeks to provide long-term solutions to the problem of cleft lip and cleft palate in children around the world by funding and coordinating surgical missions in Developing Countries, training and mentoring local physicians, launching public education projects and advancing research. Through collaborative programs with Developing Countries throughout the world, Rotaplast International’s efforts expand far beyond surgical intervention and help foster international goodwill, peace and understanding. Rotaplast provides free reconstructive surgery to children and untreated adults born with this deformity. We also treat other congenital anomalies and burns where possible. Rotaplast recruits highly skilled and accomplished plastic surgeons, pediatricians, anesthesiologists, nurses, dentists, orthodontists and speech pathologists to provide a multidisciplinary cleft team in our host countries. A partnership with the University of the Pacific Dental School and Genetics Department allows us to fund research and work to provide samples for the further study of causes and prevention of the anomaly.

GOVERNANCE AND MANAGEMENT Rotaplast is governed by a 25-member Board of Directors and managed by an Executive Director with a staff of five professionals. The Officers of the Corporation are Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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the Board Chair, Vice Chair, Medical Chair, Treasurer and the Secretary. The collaborative functions of long-term strategy, policy and protocol as well as the review of day-to-day operations are handled through Board Committees. Every member serves on a committee. Rotaplast is further served by a Board-appointed senior surgeon serving in an advisory and mentoring capacity to Board, staff and volunteers, as the Medical Director. Board meetings are held quarterly and committees meet frequently and as needed.

HISTORY Rotaplast began as a World Community Service Project of the Rotary Club of San Francisco in 1992. Rotaplast was founded by San Francisco plastic surgeon Dr Angelo Capozzi and then club president, attorney Peter Lagarias. When Rotaplast sent its first mission in 1993, it was not done so with the intent of establishing an organization. The enthusiasm and life-changing experiences of the Rotary non-medical volunteers led to greater interest, more invitations, and, as we see today, a self-sustaining organization. In the beginning as it is today, volunteers were willing to promote the project and help raise money for funding. For the first three years Rotaplast sent only one mission a year. In 1996, Rotaplast received a million dollar gift from a private donor enabling expansion of the operation and gradually increased the number of missions to 13–14 a year. In 1996, we also became an independent non-profit California Corporation. In 1997, we established genetic research and education programs aimed at prevention. Rotaplast has treated over 10,000 children in 16 different countries, namely, Argentina, Bolivia, Chile, China, Colombia, El Salvador, Guatemala, India, Mexico, Peru, Ecuador, Ethiopia, the Philippines, Romania, Venezuela, and Vietnam. Rotaplast has completed over 155 missions that have been supported by over 450 Rotary Clubs throughout the United States and Canada. Through further philanthropy, volunteerism and over US$7 million annually in donated services, supplies and equipment, Rotaplast brings the gift of healing and hope to thousands of children in need of cleft lip and palate surgery each year. Rotaplast partners with Rotary Clubs that provide the majority of funding. The organization receives no funding from, nor is it affiliated with, any government or religious organizations. The average cost of a Rotaplast mission is US$44,000. The average cost per child in the 2005–6 year was US$452. In that same year, 40% of the patients received multiple procedures such as lip and palate or lip and nose.

UNIQUE GOALS AND VISION Organizations like Rotaplast exist because of the tremendous need for additional surgical and other medical care for children with cleft lip and palate anomaly. It has

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been estimated that as many as 30% of cleft lip/palate children in South America would not receive any surgery if not for organizations like Rotaplast. There are many reasons for the existing situation. There are a limited number of qualified surgeons interested in this problem and there are very few residency programs. There is a fundamental lack of financial support which leads to lack of instruments, supplies and access to hospitals. There is a lack of education and fundamental knowledge of the condition. Many of the patients are malnourished and live in remote areas without access to health care. Through our education and outreach programs we strive to help make local healthcare providers independent so they can provide free multidisciplinary care year-round with long-term follow-up. Unfortunately this is not easily accomplished and will not happen consistently unless the governments bring children born with congenital anomalies into the medical system and provide an avenue for their care. Until we can change the system, we must continue to encourage host health-care providers to actively participate with the team and to continue to give care when we leave. The host health-care providers eventually should take overall care with reduced participation of the visiting team. In order to do this, you must find a truly interested host healthcare provider who can be trusted and does not have a personal agenda. Rotaplast has successfully made this transition in Chile and feels it will happen in the next few years in several other countries. As long as a site has potential for independence, Rotaplast feels it is justified to return and do the best we can and help as many children as possible. ORGANIZATIONAL GOALS Our long-term goals are: 1. To establish local facilities where patients can receive free multidisciplinary treatment for cleft lip and palate deformity, including dental, orthodontic, hearing and speech therapy, and psychological support. 2. To create educational programs for local surgeons interested in the care of clefts through a Rotaplast fellowship program. 3. To bring about a reduction in the incidence of cleft lip and palate anomalies by genetic counselling, individual dietary education, and supplementation, using our genetics program. 4. To encourage governments to develop a national public health program of grain fortification (with B Vitamins such as folic acid). A broader goal is to collaborate with local surgeons and Rotary Clubs. To accomplish this goal, we recruit a host surgeon who will work with the Rotaplast team and obtain letters of invitation from the local Plastic Surgery Society, the Minister of Health, the host hospital and the host Rotary Club. The medical team is complemented by non-medical volunteers who provide technical and logistical support to the team. They clear the way so the medical team can concentrate on doing surgery.

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DIFFERENCES FROM OTHER ORGANIZATIONS Although there are many other volunteer medical groups that do similar work, Rotaplast is different in two respects: 1. Rotaplast brings a comprehensive cleft lip/palate team to the host country. Rotaplast provides a multidisciplinary team even though at some sites there are no counterparts for dental, orthodontic and speech services. Genetics has also been a part of Rotaplast since 1997. 2. Rotaplast has been and always will be a Rotary-based organization that promotes the highest ideals of Rotary International. Each Rotaplast team has nine or ten Rotary non-medical volunteers, each with a specific job of helping assure the success of the mission.

PROGRAMS RESEARCH Rotaplast has partnered with Dr Marie Tolarova, M.D., Ph.D., D.Sc., Executive Director of Pacific Craniofacial Team and Cleft Prevention Program at the University of the Pacific in San Francisco, to complete the largest study of both cleft and noncleft families ever conducted in Central and South America. She studied blood samples of, and conducted interviews focused on lifestyle, nutrition and medical history, with over 2,000 families with a child with a cleft, and control families. Through her ground-breaking research, a number of factors participating in the aetiology of cleft deformities including genetic predisposition, diet, lifestyle, lack of adequate vitamins especially folic acid, smoking while pregnant, and pollution were discovered and a spectrum of those factors was found to be locally specific. Armed with the locally specific information that can help prevent future cases of this anomaly, Rotaplast provides counseling on lifestyle issues and the importance of multivitamin supplementation to patients and their families at the mission sites. EDUCATION Rotaplast International understands that educating local medical professionals in the sites we visit is the best way to create sustainable programs that treat children born with cleft lip and palate anomalies all year long. Missions not only consist of intensive surgery days, but Rotaplast medical volunteers are asked to give lectures on their expertise to staff and students at the hospital site. This exchange of information is important for establishing long-lasting relationships with our sites and preparing local medical professionals so they can address the epidemic on their own in the future. Rotaplast also has initiated a fellowship program where plastic surgeons from Developing Countries can come to the hospitals in the United States to study special procedures to treat cleft lip and palate cases. As the fellowship program develops

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from its initial stages, it works to provide otherwise unavailable and invaluable training and knowledge that the surgeons can take back and implement in their native countries. PARTNERSHIP WITH ROTARY From its beginning, Rotaplast has had a long-standing partnership with Rotary Clubs and Districts both in the United States and around the world. Working with Rotary Clubs at mission sites, Rotaplast has built a strong logistical capability to support its medical teams, enabling its volunteers to work in the most remote or unstable parts of the world. Whenever possible, these Clubs provide meals, housing and security and act as liaisons with hospitals, governments and local health and humanitarian organizations. In the United States, Rotary Clubs have generously provided partial funding for Rotaplast missions.

MEDICAL MISSIONS SITE SELECTION Medical facilities are evaluated by a medical site team if a new site is being considered, and invitations are solicited for returning sites each year. Team leaders meet with local host health-care providers and host Rotarians in advance of any team arrival. The hospital is required to have an adequate number of operating rooms and anesthesia equipment as well as a properly functioning recovery room. Rotaplast brings its own universally adaptable servo-vaporizers and monitors for the operating rooms and the recovery room. The hospital must have an adequate area for the clinics and enough pediatric beds to accommodate approximately 30 hospitalized patients, a room to secure our supplies and equipment, and a space for the team to eat lunch. A conference room is also desirable. Equipment and supplies must be packed in proper sized boxes determined by the airlines. Each box and its contents must be clearly labeled and an inventory attached. The contents of each box must be listed accurately to ease Customs clearance. Customs requirements will vary with each country. The boxes should be numbered, with durable medical equipment and supplies which must come back with the team separated from disposable supplies and equipment that will be left in the country. PATIENT SELECTION CRITERIA The following are the criteria for selection: 1. Deformity within the scope of services. 2. Patient must be in good health and able to tolerate anesthesia. 3. Receipt of signed informed consent by parent or responsible party.

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4. Ideally, the age should be at least three months for cleft lip patients. ‘The rule of tens’ is often used: ten weeks, ten pounds and ten grams of hemoglobin. Younger patients are evaluated on an individual basis and may not be accepted for surgery. 5. Cleft palate patients should be at least 6–12 months of age and have at least 10 grams of hemoglobin. If patients are seen in this age category and have not had cleft lip repair, they may be done simultaneously if the surgeon so chooses. The general health of the child must be good. Children with upper respiratory illness will not receive surgery. Sometimes if these patients are seen early, they can be treated and placed on the surgery schedule later in the week. This decision is made on a patient-by-patient basis. Children with clefts associated with syndromes must be individually evaluated by the surgeon, anesthesiologist, and geneticist if possible, before being accepted for surgery. Many of these patients have serious medical problems that dictate against surgery. Of course, exceptions can be made depending on the patient. Using two ORs means approximately 60–70 cases can be operated. To facilitate this, we need to see about 120–150 children in the clinic so after 60–70 have been selected, there are enough to fill in the gaps that are created by no shows and illness. CLINIC TRIAGE Patients are placed in Categories 1, 2, or 3 by the evaluating surgeons: 1 = Green Dot: definite surgery 2 = Blue Dot: stand-by surgery 3 = Red Dot: no surgery. Surgeons, pediatricians, and anesthesiologists will evaluate patients in Categories 1 and 2. The final surgical schedule will be decided by the Head Surgeon and Head Nurse, with other medical team input as necessary. SCHEDULE A typical mission lasts about two weeks. Teams arrive at a project site with prepackaged medical kits so they can begin work immediately. The lines are long; the expectations of family members are high and the challenge of selecting who will receive the gift of life changing surgery is difficult. It is not uncommon for Rotaplast medical volunteers to screen hundreds of patients in the first day of the mission. Usually the patients have been pre-screened by local medical professionals. The selected patients are triaged at the opening clinic as to our ability to treat them safely and effectively as explained above. At the end of the screening clinic, the surgery schedule is completed, leaving room for additional cases that may come to the hospital later in the week. Surgery begins the following day and a team will usually run three operating rooms daily for six to eight days. The number of children treated can range from

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80–120, depending on the conditions in which the team is working. Those who cannot be treated due to time restraints are comforted with the knowledge that Rotaplast may return. We try to operate for six to eight days, depending on the travel time and day of arrival of the team. Any problem patient is discussed with the surgeon, anesthesiologist and pediatrician as a group before the patient is placed on or taken off the surgery schedule. Rotaplast always has one extra surgeon and anesthesiologist for the operating rooms available. While this model has proved itself and has served us well, Rotaplast is open to discussing other team models and concepts at the various sites. Rotaplast encourages participation of local surgeons and offers a lecture series on cleft lip and palate when appropriate. The closing clinic is held on the last Saturday of the mission after all surgery has been completed. The goal is to get every patient who has received surgery back for a post-operative evaluation. This goal is seldom achieved. Patients who live a long distance away often are seen mid-week so they can return home. Some just leave the area of their own accord. The highest clinic return we had was in 2003, in Vietnam, when 103 out of 105 patients returned to the closing clinic. The trick was to pay each family a small amount of money if they returned to the clinic. At the clinic we try to work with the local medical professionals to ensure that all follow-up care is provided to patients. Rotaplast attempts to return to each site several times and each team records the names of patients who need additional surgeries and those who, for whatever reason, did not receive needed surgery, so that they can return and be cared for in the future. A comprehensive medical system needs to be developed which identifies and reaches all individuals with untreated cleft lips and/or palates to ensure that they all receive adequate long-term care.

THE MISSION TEAM Rotaplast is an organization built on volunteerism. Each year, hundreds of volunteers donate their valuable time and talents to treat over 1,000 children who would otherwise not receive surgical intervention. Rotaplast endeavors to differentiate itself from other organizations with its roots in volunteerism, not only from its medical professionals but also from Rotarians. Its unique approach to helping children and building relationships with its partners has been extremely successful and provides a spirit that goes far beyond the medical mission. A typical mission team consists of 30–40 members. Approximately two-thirds are made up of medical professionals, and the remaining third are non-medical volunteers. Each team is managed by a Mission Director who works in collaboration with the team’s Medical Director and Head Nurse. THE MEDICAL TEAM Medical volunteers are thoroughly screened and only the most highly qualified medical professionals are accepted for Rotaplast missions. This multidisciplinary team

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includes reconstructive plastic surgeons, pediatricians, PACU and Recovery Room nurses, pediatric anesthesiologists, dentists, orthodontists, and speech pathologists. These highly skilled professionals work closely with each other and the hospital staff and doctors at the mission site. Each position listed below has extensive training materials provided to each team member to articulate expectations, roles and responsibilities on the mission. For the purposes of this chapter, we list the titles of the positions. All members of the medical team are approved in advance based on application and experience by the medical committee of the Rotaplast Board. Airfare is paid for by Rotaplast for all medical personnel. All medical decisions are made by the Medical Director. The Medical Director is an experienced plastic surgeon. The team includes: r Plastic surgeons. Ideally, one more than there are operating rooms (ORs) (i.e., three ORs and four surgeons), including local surgeons who can operate independently. r Pediatricians. Two pediatricians, plus a local pediatrician, if available. r Anesthesiologists. Same number as surgeons, not including local anesthesiologists. r Head Nurse. One Head Nurse with OR experience and organizational skills. r OR nurses. One nurse per OR. r Recovery room nurses. Two or three depending on the number of ORs. r Speech/language pathologist. One. r Orthodontist. One. r Dentist. One. r Geneticist. One. THE NON-MEDICAL TEAM Non-medical volunteers pay for their own transportation to accompany the team. THE MISSION DIRECTOR The Mission Director is a Rotarian, and has the overall responsibility of making sure all logistics are taken care of to insure a smooth and successful mission. The Mission Director and Medical Director oversee the entire mission. The responsibilities of medical and non-medical members of the team are clearly defined as shown below. QUARTERMASTER The quartermaster coordinates the moving of equipment and boxes from the United States to the host country and back again. S/he deals with customs agents, counting of boxes, and unpacking of equipment on arrival and re-packing upon departure. The quartermaster should be in excellent physical health and have the stamina to handle the transport of boxes and medical cargo.

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MEDICAL RECORD KEEPER The medical record keeper must be computer-literate, and be able to work with the Rotaplast digital records system. The responsibility for keeping track of all of the records, including names of all children seen, procedures performed, and final medical values is handled by this individual. S/he must also make sure that all medical record forms are appropriately filled out by the medical team and that they complete the medical summaries after the mission is over. Training in advance of the mission is provided and required. More complete job descriptions for the various volunteers can be found on the Rotaplast website. We try to bring a biomedical engineer, as part of the medical team, to help set up and repair equipment for the team as well as the local hospital. This has been an invaluable service on many trips. The following roles are discussed in Chapter 8 by Jurkovich: r r r r r r r

equipment sterilizer ward coordinator recovery room helper recreation therapist patient transporter photojournalist translator.

LOCAL VOLUNTEERS Rotaplast, through our local Rotary partners, tries to recruit local volunteers who speak good English to act as translators so we can serve our patients better. Local translators offer the safest vehicle to avoid misunderstandings due to the subtleties within a language and culture.

ESTABLISHING CLEFT TEAMS Any group of medical professionals who want to establish a cleft surgical team in a Developing Country must have a long-term goal of educating local health-care professionals who can transition to independence. There is also a certain protocol that should be followed. The cleft team must fulfill the following conditions for each site: 1. 2. 3. 4. 5.

Must have an invitation from the Plastic Surgery Society. Must have an invitation from the Minister of Health. Must have an invitation from the hospital where they wish to work. Must have an invitation from other professional organizations that may be involved. Must recruit medical personnel who are experienced in cleft surgery.

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COMPLICATIONS OF CLEFT LIP AND PALATE REPAIR OCCURRENCE, PREVENTION, AND ROTAPLAST EXPERIENCE Surgical treatment of cleft lip and palate has associated morbidity like any operative intervention. The primary requirement for management is proper patient selection and then adherence to current standards for the delivery of care. All Rotaplast patients are screened by anesthesiologists, pediatricians, and surgeons. A consensus is made of the patient’s fitness to tolerate anesthesia and the operative procedure. Complications can accordingly be divided into those derived from anesthesia and those from surgery. ANESTHESIA COMPLICATIONS Lees and Pigott (1992) report all serious complications were of a respiratory nature and occurred in the first two post-operative days. In their series there was an overall 26.2% complication rate in 164 patients. A study by Takemura et al. (2002) stated that children with complicated clefts had an 8.9% respiratory complication rate as compared to 1.7% who had simple cleft repairs. The Rotaplast experience of respiratory complications is less than 1% in 9,060 operations. There was one pneumothorax secondary to microtia repair using rib cartilage. To prevent these complications, we screen for upper airway disease and exclude active severe disease or treat minor disease with antibiotics and bronchodilators. Postoperative management includes oxygen saturation monitoring, oxygen, inspection of nasopharyngeal and oropharyngeal airways, placement of tongue suture, and close observation as appropriate. All cleft palate patients are kept overnight and a second night if needed. SURGERY COMPLICATIONS The Rotaplast experience of surgical complications is 15% in 9,060 operations. These can essentially be divided into infection and dehiscence (repair breakdown and fistula formation). Amaratunga (1988) reports 21% of 346 cases in Sri Lanka had postoperative fistulae after palate repair. By comparison, Canady et al. (1994) report an overall complication rate of 14% in 260 patients from a Developed Country center. Infection and dehiscence of cleft lip repair are rare at less than 1% due to the good vascular supply of the tissues. Antibiotics are given pre-operatively and repair with proper technique and minimal tension is done to prevent this problem. Palatal fistulae occur because of wide clefts, technical error, and lack of post-operative feeding restrictions. Surgeons are board-certified and have cleft palate experience. Education is given to the nursing staff and patient families to refrain from bottle feeding or any activity that damages the repair. Antibiotics are given pre- and post-operatively. Rotaplast has had one death in 9,060 patients. The fatality occurred in a 1-year-old child with a bilateral cleft lip. The child was discharged from the hospital 24 hours after

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surgery to the resident hotel for patients and families. The mother, against medical advice, elected to take the child home which was an 8-hour bus ride. During the trip the baby was fed by bottle and aspirated. The infant was dead on arrival at the hospital which was a further hour’s ride away. Rotaplast now tries to keep all palate patients in the hospital for 48 hours if possible. INCIDENTS WITH VOLUNTEERS To our knowledge, Rotaplast has had no volunteers return home with infections, parasites or malaria. In May 2006, one of our biomedical engineers died of BSE (bovine spongiform encephalopathy). We are not sure how or where he contracted the disease. We have had one non-medical volunteer hospitalized with a minor heart problem and one non-medical volunteer who had a diabetic crisis and was hospitalized. She did not reveal her entire medical history as required in the application. We have had one nonmedical volunteer hospitalized with a mild ear problem and one for altitude sickness and dehydration while working at 14,000-feet elevation.

CONTACT Contact address: Rotaplast International Inc. 1663 Mission Street, Suite 320, San Francisco, CA 94103 Tel.: 001 415 252-1111 Fax: 001 415 252-1211 Website: www.rotaplast.org

REFERENCES Amaratunga NA (1988) ‘Occurrence of oronasal fistula in operated cleft palate patients’, J Oral Maxillofac Surg 46: 834–8. Canady JW, Glowacki R, Thompson SA & Morris HL (1994) ‘Complication outcomes based on preoperative admission and length of stay for primary palatoplasty and cleft lip/plate revision in children aged 1 to 6 years’, Ann Plast Surg 33: 576–80. Lees VS & Pigott RW (1992) ‘Early postoperative complications in primary cleft lip and palate surgery – how soon may we discharge patients from the hospital?’ Br J Plast Surg 45: 232–4. Takemura H, Yasumoto K, Toi T & Hosoyamada A (2002) ‘Correlation of cleft type with incidence of perioperative respiratory complications in infant with cleft lip and palate’, Paed Anesth 12: 585–8.

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Logistics and Nursing Issues PRISCILLA JURKOVICH

Editorial comment: The author shares her personal experience of surgical ‘missions’ as a volunteer nurse and head nurse with Rotaplast International, Boulder Community Hospital Mante Medical ‘mission’, and Alliance for Smiles. The resources described are those of a Developed World team in optimal circumstances. Her detailed observations on the management, team and local workers’ roles and relationships and cultural sensitivity are pertinent, whatever the size and resources of the team, and complements Chapter 7 by Capozzi.

INTRODUCTION With opportunities to volunteer in international ‘missions’ increasing, medical professionals have a rewarding option to share and understand medical needs and patient care with colleagues throughout the world. A medical ‘mission’ gives exposure to different cultures in a personal way. This chapter is meant as a practical nursing guide to set up and deliver care in sometimes less than optimal conditions.

GOALS OF TYPICAL ‘MISSION’ PROGRAMS The goal of most ‘missions’ is to provide free reconstructive surgeries and treatments to correct life-debilitating deformities or injuries for people who would not otherwise benefit from this type of health care. Each organization usually has its own set of criteria for a person to be eligible for this free service. Education for the local medical personnel may be incorporated, as well as counseling for the affected person’s families regarding ways to prevent cleft palate anomalies through diet, vitamin supplementation, and lifestyle changes, especially alcohol consumption. Collaboration with the local health-care team on follow-up care and development of a comprehensive cleft palate program is a desirable aim.

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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SITE NEEDS ASSESSMENT After the site has been determined by the organization, a medical and nursing needs assessment is done. The needs assessment should take place at least six months in advance of the ‘mission’, comprising a review of the facility, defining host responsibilities, seeking clarification of issues, finalizing planning, and drawing up educational opportunities for the local professionals.

REVIEW OF THE FACILITY The on-site visit will identify the facilities available. The details will include: r Number of operating rooms at the disposal of the team. r Storage, general and lockable secure facilities for equipment, medication and supplies. r Equipment available, and its suitability for the needs of the team: r machines: anesthesia, EKG, pulse oximetry, electrocautery, sterilizer, suction; r electrical voltage used in the country; r suction/vacuum capabilities; r inhalant gases (e.g. oxygen, nitrous oxide, vaporizers); r operating room beds, back tables, Mayo stands, room storage; r area for decontamination and sterilization of instruments; r nitrogen and air equipment if needed; r operating room lighting. r Pre- and post-operative care clinic area: consider the flow of patient check-in, waiting area for pre-operation selection and post-surgery visit. r Pre- and post-anesthesia care unit (PACU): number of beds available, wall oxygen, monitors, suction. r Overnight facilities for post-surgical patients (and sometimes pre-operation patients). r Estimated number of patients and types of surgeries to be performed. r When pre-assessment has taken place: what type of assessment has been done by the local medical facility, and numbers and types of potential surgeries identified. r Local medical and nursing personnel available to assist the ‘mission’ team including local scrub nurses, instrument cleaners, porters, cleaners, PACU support, medical or nursing school involvement, or physicians. r A list of educational requests from, and needs identified by, the medical personnel in the surrounding area, related to all aspects of cleft care. r Availability of narcotics and consumable supplies including anesthetic agents. r A review of financial responsibilities for hospital usage between the hosts and visiting organization. r Laboratory and X-ray availability.

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r Pre-operative assessment of patients including a hematocrit. Some facilities include ascertaining patient HIV status as well. r System for dealing with exposure to blood-borne pathogens in the event that anyone on the team becomes exposed through needlestick injury or contact. r Internet system for communication with team members’ family, and the parent organization. r Facility for copying documents. Through this evaluation the team leaders will determine their requirements for specified items and amounts of durable medical equipment to be brought as well as sterile and non-sterile supplies. The basis of allocation of financial estimates of costs for the host, and visiting volunteers, will be established (see below). Educational and counseling needs of the community will also be determined. COMMON HOST RESPONSIBILITIES REQUIRING CLARIFICATION Most ‘missions’ have budget constraints on the host site as well as on the participating organization. Discussion to clarify the division of the financial responsibilities and specific needs of the participating organization should take place. Some constituent parts of a typical program to consider include: r Drawing up a schedule of events: many ‘missions’ put on events coordinated by the Mission Director that every volunteer is obliged to attend. These generally include meals, parties, educational offerings, special presentations, or meetings. r Meals: decide who will provide or be financially responsible for the team’s meals. Determine where they will be served. Most host supporters will provide the midday meal close to the surgical area to facilitate minimal interruption to the flow of patient care. r Sleeping accommodations: places include host family homes, hotels or special arrangements with local agencies. r Reception or party to celebrate the arrival and completion of the trip: it is useful if the hosts indicate the number of local participants likely to attend. Many end-of-trip parties have a mutual exchange of gifts that may include certificates or items of appreciation for local voluntary workers. Team members should be apprised if a financial contribution is required of them for such tokens. r Local physician involvement for pre-operative assessment and post-operative follow-up to provide for continuation of patient care. r Food and lodging vouchers for families who have to travel long distances. r Local sightseeing: an opportunity to relax and for the host volunteers to show team members the surrounding area. It is important to show appreciation for the time and possible financial support they have given. r Interpreter availability for staff and patients for all aspects of the trip. Many trips have a volunteer interpreter as part of the team. Some countries secure volunteer

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interpreters from a local language school, the host organization or through a general solicitation within the community. r Meeting area (could be required daily): the team needs an area to review activities of the day, problem solve, and develop the team and general communication. This area could be in the same place as the sleeping accommodation when shared by all team members. r Cultural sensitivity issues (e.g. appropriate dress, alcohol usage, religious customs, etc.). Review the cultural habits of the community so the team can be sensitive to the community expectations. r Transportation needs: r airport to lodging at the beginning of the trip; r lodging to and from the hospital daily, at various times; r social team-related events; r lodging to airport on completion of trip; r freight truck for medical equipment and supplies.

PATIENT PROFILE During the needs assessment, the type of patients who will be cared for by the team is discussed. For example, a ‘mission’ with all primary cleft lip and palate surgeries has different needs from a ‘mission’ that includes a Le Fort 1 or a sagittal split with bone implants. The instruments, surgeons, medical personnel, equipment at the hospital, pre- and post-operative care, and host country preparation of the patient are different in those scenarios. In addition, the packing for the trip regarding suture, dressing, and post-operative care instruction must be planned in accordance to the likely patient needs.

EDUCATIONAL OPPORTUNITIES Some sites utilize the expertise of the group to enhance the education of staff at the hospital or medical and nursing schools near the host facility. At this time, the host hospital would give suggestions for their educational advancement. The Medical Director and head nurse’s responsibility would be to assess the education level of the host country nurses and physicians. In addition, it is important to understand the status of the host country nurses and the conditions under which they work. This step is important in the diplomatic role for successful education of the host country medical staff. Preparation of lectures prior to the trip will allow for educational resources, journals, DVDs, and PowerPoint presentations to be collected according to the needs of the local medical staff. Educational responsibility is ongoing during the ‘mission’ with continuous mentoring of the local physicians and nurses who participate in the operating room and post-operative care.

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PREPARATION FOR THE TRIP MEDICAL SUPPLIES After the needs assessment is complete, an inventory is made based on the specific needs of the trip. Requesting medical equipment, medical supplies, or medicine is just the beginning. Many hospitals will donate their serviceable durable medical equipment to ‘missions’ as they update their facility. Agencies such as Project Cure (http://www.projectC.U.R.E.org) have become clearing houses for new and used medical equipment and supplies. Donated equipment such as an anesthesia vaporizer, an electro-cautery machine or a suction machine should be serviced and maintained prior to going to the country. Items such as transformers to the electrical voltage of the country are essential. If headlights are included in the inventory, pack extra batteries and replacement bulbs. Pharmaceutical agencies generally have a foundation that will donate medicines for ‘mission’ trips. Generally, a nominal fee and a medical doctor’s acceptance of the medication are needed. A list detailing the amount of medicines to be used is needed. CUSTOMS Customs formalities at the point of entry to the host country can be a major source of misunderstanding and bureaucratic delay by not observing the regulations governing border Customs clearance of equipment and drugs. The host country representative should visit Customs prior to arrival and details of customary routines should be discussed with the volunteer organization. Inventory lists should be made available to the host country upon request. Personal contacts may be helpful in speeding up Customs clearance. Controlled drugs such as opiates are generally not permitted across borders. If the agency has been given donated controlled drugs or medications not available in the area, the agency or host of the medical ‘mission’ can work with the drug enforcement agency of the country to facilitate a smooth transition across international borders. Each box or item must be packaged and labeled to the host country’s specifications. These specifications may be detailed as to the size of the box, weight (in pounds and/or kilograms), items in the box, etc. INVENTORY An inventory is determined by the site visit assessment and physician preferences. However, a basic inventory that can be used for numerous visits can be systematized into one or more supplies storage boxes (for the author’s list, see http://www.rotaplast.org/). Labeling the items that are in the boxes and keeping an itemized inventory is helpful when determining where the boxes will be stored during the ‘mission’. When packing the items, pack according to usage area. For example,

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pack all the post-anesthesia care unit (PACU) and outpatient clinic needs separately in one or more boxes for each. All the instruments and sterilization materials should be kept together, as should the suture materials, gloves, or similar items. In addition, durable medical equipment such as anesthesia vaporizer, headlights, suction machines, or electro-cautery machines that must return with the medical team should be placed in boxes that will support return travel.

TEAM SELECTION MEDICAL PERSONNEL Organizations generally determine the number of medical personnel needed for the ‘mission’ based on the number of operating rooms, the length of the surgical day, and the number of support staff. r A minimum standard for each operating room is an anesthesia provider, surgeon, scrub and circulating nurse. Some organizations will secure the scrub personnel locally to defray the costs of another team member. r A head nurse, Medical Director and Anesthesia Director are designated staff who coordinate the ‘mission’. r The PACU nurse ratio is usually one Registered Nurse (RN) for every two operating rooms with a minimum of two RNs per trip. r At least one pediatrician who frequently is also designated to manage team illness issues. r An orthodontist to advise the surgeons, and local specialists. r A dentist for pre-operative extractions of badly carious teeth that could otherwise jeopardize palate surgery due to infection. r A speech pathologist can add greatly to the appropriate selection of patients for surgery. r If available, a geneticist can provide a counseling and research role. NON-MEDICAL PERSONNEL Non-medical visitor volunteers usually have some affiliation with the ‘mission’ sponsor. For example, in Rotaplast (http://www.rotaplast.org), the non-medical volunteers may come from the Rotary Club that has given full or partial support of the trip. The roles of the non-medical volunteers are many including Mission Director, quartermaster, ward coordinator, medical record keeper, recreation therapist, patient transporter, translator, or photojournalist. The titles may vary among different organizations, but the tasks are similar. DESCRIPTION OF NON-MEDICAL VOLUNTEER ROLES r A Mission Director supervises the smooth flow of operations. Communication is the primary role of the Mission Director which begins before the ‘mission’ and

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after the team has been selected. S/he shares communication with the local media between the team members and the host. The Mission Director assigns the roles of the non-medical volunteers. r The quartermaster has responsibility for the boxes and equipment. S/he will work with the airline transport and Customs agents and account for each box. In addition, at the ‘mission’ site, the quartermaster will assist the other team members with unpacking upon arrival and re-packing upon departure. r The medical record keeper plays a vital role maintaining the patient records. Most organizations have laptop computers to keep track of all the records. Consequently, use of Word or Excel is a valuable skill for this volunteer to have. The completed procedures, the names of the patients who have been seen in pre-operative, walk-in and post-operative clinic, the surgery schedule, medical summaries and appropriate medical data of the surgeries are part of the medical record keeper’s responsibilities. This important role may require some pre-trip orientation, but will receive guidance from the Mission Director or Medical Director and the head nurse. r The equipment sterilizer volunteer will clean and sterilize the instruments with the guidance of the head nurse. On-the-job training is usually given by the head nurse and pictures of the instruments and the trays are helpful to support this training. The following roles may be combined, based on the number of volunteer staff available for the mission: r The ward coordinator assists with the preparation and transfer of patients from the

r r r r

r

ward to the surgical area. This volunteer assists with the operating room schedule and verifies arrival of the patients and communicates any problems with the head nurse or pediatrician. The recovery room helper assists the nurse in the recovery room by comforting a child or parent, transporting patients or entertaining the patients before surgery and assisting in the patient flow during the pre- and post-operative clinics. The recreation therapist may entertain the patients by playing games, doing magic tricks, puzzles or other activities to decrease the patients’ anxiety. The patient transporter takes patients to the pre-surgery area just prior to the surgery. The photojournalist takes a photo journal of the trip. A daily written update may be sent to the organization to communicate what is occurring on site and include personal accounts by the volunteers. Pictures and verbal accounts of the patients and family are often included. At the end of the trip, the photojournalist will compose a chronological photo journal of the trip. A translator will interpret between the medical staff, patients, families and local hosts whenever necessary.

THE APPLICATION PROCESS AND EARLY ORGANIZATIONAL COMMUNICATION All medical and non-medical volunteers go through an application process. The organization reviews the application for medical and non-medical volunteers. The

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application includes documents such as a copy of the passport, visa (if required for the country), licensure if a medical volunteer, current certificates of satisfactory immunization for Hepatitis A and B, and others as required for the country (e.g. typhoid, cholera, yellow fever) or organization. A letter of recommendation may also be required. Professional indemnity organizations may need to be informed; they generally have a benevolent attitude to humanitarian work. Once the team has been selected and notified by the organization, the Mission Director will start the communication process with a ‘welcome to the team’ letter, and, if reasonably closely domiciled, a pre-trip gathering. Room assignments may also be discussed by the Mission Director at this stage. The organization distributes a list of team members with contact information, travel arrangements, and contact information for family members of the volunteers while they are away. Information may be given on cultural sensitivity issues for the host country including religious customs, appropriate dress or other items specific for the country. The pediatrician or organization may send information such as potential dangers through exposure to disease, and altitude sickness, and suggestions for its prevention as appropriate to the country. Some organizations have name badges or t-shirts that will identify the group members throughout the trip. These should not be worn if a risk assessment highlights security issues for a visible group of foreigners in some unstable areas of the world. Airlines generally permit two checked bags in the hold and a carry-on. It is good practice for the organization to allow the volunteer one checked bag and carry-on and utilize the other checked bag for trip supplies. Team members should be informed by the medical director about the currency of the country and the approximate amount of cash needed for group activities or if they have to pay for their hotel, meals, group party, and entry/exit fees for the country. Suggestions for host gifts may also be included.

ON SITE WITH THE TEAM PRE-OPERATIVE CLINIC, PATIENT FLOWS The pre-operative clinic is best started after the team has had some time to recuperate from the travel. The pre-operative clinic should have a designated waiting area assigned. The medical record keeper starts the chart on each patient with the initial information obtained from the patient or parent during the check-in process. As names may be very similar and confusion could result, a system of numbers and appropriate warning stickers is helpful. Alternatively photo identification is used. Clinically relevant data are sequentially added to as the patient progresses through each station. Each surgeon should have an exam room with the minimum equipment required: a flashlight, tongue blades, and non-sterile protective gloves. Two options for patient assessment in the clinic are either by each individual surgeon, or surgeons assessing as a group: 1. Individual surgeons and anesthesiologists. This system is the fastest if there are a large number of potential patients. In addition, if there is a specialist surgeon in the group (e.g. maxillofacial surgeon or burns specialist), appropriate patients could

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be directed to that surgeon. One disadvantage of this system is that it is difficult to prioritize the patients as the diversity of patients is unknown to the individual surgeon. One advantage for this scheme of patient flow is that the anesthesia providers will have already assessed each patient for anesthesia risks. 2. A group of surgeons. In this process, all the surgeons assess each patient. A slower process, but all the surgeons will have knowledge of the necessity and priority of the individual’s needs. In addition, any surgeon would be likely to agree rapidly to perform the surgery if the surgery schedule is progressing significantly faster or slower than planned in one operating room. Stations for PACU nurse triage, pediatrician, dentist, orthodontist, geneticist, and speech pathologist may also be included, depending on the medical team. A general assessment with initial vital signs to include a temperature is usually done by the PACU nurses, a physical assessment by the pediatrician, and usually a hematocrit if not already obtained. If the patient has been determined as ineligible for surgery, some of these assessment areas would be bypassed as the patient progresses through the clinic. However, a visit to the dentist is advisable even if the patient has not been selected for an operative procedure. The Medical Director, head anesthesiologist, and head nurse determine a system for patient selection (see Chapters 5 and 11 for assessing anesthetic and pediatric risk criteria). Some organizations use a code to identify the patient or chart. It may be set up in three categories as ‘yes’, ‘maybe’ (if we have time in the schedule) or ‘no’ because of safety risks to the patient, not needed or not a priority. In collaboration with the host country volunteers, a system of notification of potential patients should have been determined prior to the clinic so the patient has clear direction on how they will be notified if they have been selected for a procedure. The location, date and time of check-in before the procedure, nothing to eat or drink status, and contact information of the patient are a few items to discuss with the patient. A communication system needs to be in place regarding a scheduled patient with an illness that develops after the patient has been selected for a procedure. In addition, patients not selected for a procedure may choose to wait for an opening if there is a cancellation. Other decision points include a system for ‘walk-ins’ and adding on surgeries. Some considerations of the day-to-day schedule are instrument availability, length of the surgery day, equipment availability, patient availability, skill level of plastic surgeon and the like. For example, the organization may have three lip sets, three palate sets, and one nasal tray. If there are only three operating rooms, the preference would be to alternate procedures (lip, then palate, then lip, and so on) so there are adequate turn-around intervals for the instruments to be re-sterilized and thus avoid surgical delays. In this example, there can only be one nasal procedure at a time. In general, patients with low risk and a straightforward procedure are scheduled for the first case on the first day. As the day progresses and the equipment has been surgically tested, more complicated cases may be added. Other considerations regarding scheduling would be to have patients with less complicated surgery and low risk of bleeding on the final day of surgery.

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The final schedule is reviewed by the medical director, lead anesthesiologist and lead pediatrician. The medical record keeper will provide copies of the daily operating room schedules. Information such as name, birth date or age, hematocrit, procedure, amount of time needed to perform the procedure, the surgeon, type of anesthesia, anesthesiologist, OR nurse and medical record/patient identifier are items generally listed on the schedule. Once the schedule is complete, the patients are notified of the time, date, procedure, and given instructions on nothing to eat or drink prior to surgery and arrival time at the hospital for surgery. Verification of contact information is done through the interpreters at this time.

OPERATING ROOM SET-UP The quartermaster may be directing the placement of the medical supplies during clinic if his/her expertise is not needed during clinic. After clinic is completed, the volunteers will begin to set up the operating room and recovery area. Setting up the operating rooms and recovery areas will have some surprises even with the best needs assessment site visit. There is the confusion of being in a new place with new peers as well as the unfamiliar language and equipment. In addition, biomedical engineers or technicians are usually not available to assist with servicing equipment. Testing each piece of equipment for function will allow for a safe environment for the patient. Once the electro-cautery, suction, EKG, pulse oxymetry machines, oxygen sources, light sources, the sterilizer and other general equipment have been tested, the team will finish preparations for the surgery days. The head nurse will assign the OR nurses to the operating room that they will be working in for the ‘mission’. The philosophy of keeping the OR nurse and surgeon in the same space is followed for the safety and comfort of the patient. Many groups encourage the same individuals to work together to develop a joint routine as well as becoming familiar with the equipment in the operating room to which they have been assigned. The equipment, anesthesia equipment and room basics will become more familiar as each day progresses. An orientation of the decontamination process, instruments and supplies, storage, emergency equipment, PACU, the schedule, OR start times and general information is done by the head nurse with the OR RN, PACU RN and the non-medical volunteers. The Medical Director will discuss with the physicians what the expectations are regarding the organization’s responsibilities on patients’ charts, the schedule, ward ‘rounding’ of patients and their after-care.

PACU SET-UP The PACU RNs will set up their recovery area: r Each bed should have oxygen provided through an adult and a pediatric mask, a nonrebreather mask, nasal cannulas, a yankeur or endotracheal suction of various sizes.

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r Blood pressure monitoring, pulse oxymetry, and EKG capabilities. A variety of blood pressure cuff sizes, and EKG electrodes will also be available. r Emesis management: ‘Chux’ or a linen saver to absorb bodily fluids, an emesis basin and protective gloves. r General supplies in a central area or shelf that has a current drug reference guide and ACLS guidelines, alcohol pads, betadine or chlorahexidine antiseptic wipes, various sizes of syringes and needles, tape, dressing supplies, stethoscope, thermometer, protective gowns, goggles, IV tubing and fluids, and IV start kits. r Portable oxygen supplies to include nebulizer/updraft and humidifier or face tent. r A supply of arm boards may be needed for pediatric cleft care as some surgical teams believe it is necessary to have a way to keep the child’s elbows restrained straight so they cannot reach their mouths. r Bottled water, cups, spoons or straw are also needed to break the NPO status after surgery. Ice packs and ice resources are nice, but may be unrealistic on most ‘mission’ trips. r Blankets to provide warmth, pillows to elevate the head and provide comfort. The PACU RN will review routine orders and post-op care instructions with the surgeons and anesthesiologists. For example, some surgeons will not allow suction on a patient after palate surgery. Copies of these post-operative instructions should be available for the patient or primary care giver. These instructions should be in English and the local language(s) and compiled before the trip. If you need to create the post-op instructions at the location, then find out about printer and computer availability. A general orientation should be completed with nursing students, general hospital staff, medical students or specialized nursing assistants utilized during the ‘mission’. The differences in language, symbols of communication, personal protection from exposure to diseases as well as specifics in their role should all be discussed. The head nurse will have made arrangements with the local nursing personnel regarding scrub nurse coverage for the length of the surgery days. Discussion on the types of suture, the instruments’ names, the procedures, and surgeon’s preferences are among some of the discussion points. Additional issues include a brief review on emergency procedures, location of the defibrillator, arrangements made for complications such as intensive care, bleeding, blood administration, and the like. Each member of the team will learn to adjust from what they have been used to in their home/work environment. Mutual respect and trust are paramount in the development of the team. By the end of the trip, the team members will be comfortable with each other, having developed a trusting relationship and friendship. MEDICATION The head nurse and anesthesiologist will determine with the hospital personnel the control medications that are available in the hospital and how to secure the items for pre-operative, intra-operative and post-operative pain control. Typical control drugs such as midazolam (Versed), morphine, fentanyl, and pethidine/meperthidine (Demerol) may be requested. The common acute life-support drugs such as

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albuterol/salbutamol, antihypertensive drugs, as well as domperidone (Motrin) elixir and tablets, paracetamol/acetominophen (Tylenol) elixir and tablets, antibiotic ointment and tablets are common drugs on ‘missions’. Inventory list them as they are likely to be in inadequate quantities locally for patient use in the hospital, in addition to sending patients home with them.

SURGERY DAYS The surgery days have some similar characteristics and routines. Each person has their assigned role. The schedules are reviewed daily by the Medical Director, lead anesthesiologist and lead pediatrician. The medical record keeper brings the records daily (or they are secured daily on site for patient confidentiality). The recreational therapist, transporter, or patient ward coordinator have communicated with the head nurse regarding the status of the patients on the schedule. Flexibility is the key regarding cancellations, additions, and ‘no show’ patients. Communication regarding transporting a patient to the pre-operative area before surgery is essential. Two-way radio communication is useful, if available. Coordination of instruments, staff rest breaks, evaluation of patients that drop in during the week, and restocking of supplies are just some of the activities that are behind the scenes of the actual surgeries. An ‘On Call Crew’ is established on a daily basis for complications that may require repeat surgical intervention. A calm and relaxed environment should be provided for the patients. Separate areas for pre-operative patients and post-operative patients should be available. It is not in the patients’ best interest to have a pre-operative patient witness a postoperative recovery. Many of the medical ‘mission’ patients and families have fears and unknowns about hospitals and doctors.

PRE-OPERATIVE AREA Prior to surgery, the patient will be unable to eat or drink. The recreation therapist, transporter or other non-medical volunteer will assist in distracting the patient from this restriction. This area should have toys, games, cards, balloons, or other types of entertainment. An outgoing and lively volunteer is helpful in this area! Having an interpreter in the pre-operative area is important to answer questions and give explanations to alleviate any stress and fears. Most of the patients will have been pre-screened and not in need of another set of vital signs. However, a BP and a pulse oxymetry monitor in this area are helpful. Keep any available records and histories of the patient accessible for the anesthesiologist to evaluate. Many of the intravenous fluids are started in the operating room. There may be an occasion that the anesthesiologist would require intravenous fluids in the pre-operative area. Supplies such as IV start kits and IV fluids will be needed.

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PRE- AND POST-ANESTHESIA CARE UNIT (PACU) Appropriate staffing is a minimum of one PACU RN and one non-medical volunteer for every two operating room theatres. Most trips will have two PACU RNs as safe practice. It is unwise to have more than two post-general anesthesia patients for one RN. The OR schedule dictates how the PACU is staffed. If the operating rooms have extended hours, more PACU RNs will be needed. The non-medical volunteer can assist the PACU RNs once the patient has been initially assessed but still needs close supervision. A family member may also be allowed to assist with emotional support of the child during this phase.

POST-OPERATIVE CARE The goal is for safe, thorough, uncomplicated and complete care of the patient. Communication between the physicians and the nurses regarding post-operative care and instruction is needed. During the set-up phase, the PACU team will have organized written instructions for home care and medications and these are now reviewed with patients and families. An interpreter must be available. A dynamic conversation between the RN, the interpreter and family is essential to find out what their resources are and how realistically these instructions can be followed. Most patients are probably going home to very poor and dirty environments. The primary care giver must understand how to care for the patient at home and give a demonstration of their acquisition of the care procedures where applicable. Extra supplies may need to be given to the patient. In addition, a reminder of the date and times of the postoperative review clinic is given for the patient to be assessed in the healing process. On some trips, the pediatricians and local doctors instruct the patient and primary care giver. Most patients will need to be evaluated overnight after surgery. An area for postoperative and overnight care within the hospital should be accessible for patient discharge from the PACU. The hospital local nursing staff and students give emotional support and nursing care to the patient and primary care giver. The pediatrician follows the patient on the unit and is available for any post-operative assessment. The surgeons make rounds on the unit at the beginning and end of the day. On the last surgical day, there is a general thank you celebration with the hospital staff who have assisted during this ‘mission’. Gifts, food and certificates of appreciation are generally given.

REST DAY Most host countries provide a rest day during the surgery days since most ‘missions’ last about two weeks. The plans may be formal or informal and include shopping, visiting sites of interest in the region, sampling local food or just relaxing.

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EDUCATION An educational presentation may be given by some of the medical personnel to the host country medical community. The presentation topic(s) may be at the request of the local medical community. Preparation, content and mode of delivery have been covered earlier in the chapter.

FINAL ACTIVITIES POST-OPERATIVE CLINIC The post-operative clinic is held to assess the progress of healing. The surgeon will review the post-operative care and progress with the patient or primary care giver. Depending on the likelihood of return visits by a surgical or multidisciplinary team, further surgeries or interventions may be discussed. A follow-up appointment is made with the area volunteer physician. FINAL PACKING The quartermaster will organize the packing and delegate activities to other volunteers and some staff not assigned to the post-operative clinic are delegated to assist with packing. Waiting to begin the final packing until after the post-operative clinic has advantages as no further surgeries will be required. Delicate instruments and monitors must be carefully padded when packed for the return trip. An inventory of the boxes for the airlines and Customs is produced by the quartermaster. The head nurse evaluates which supplies are to be donated to the host hospital. FINAL CELEBRATION A final celebration to allow the host country to thank the volunteers involved with the ‘mission’ trip is usually held on the final day before departure home. Allow yourself a rest period after you return home. Celebrate your involvement and how you have blessed the lives of the patients you helped.

CONCLUSION Each country, each setting and each individual have resources to share with others. People want to feel that what they are doing matters to them personally and to the larger world. Exposure to other cultures and settings allows us to become sensitive to our own cultural biases and behaviors. As we aspire to improve patient care, we can expand our roles and responsibilities in this very small world.

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The Sri Lankan Cleft Lip and Palate Project SANATH P. LAMABADUSURIYA AND MICHAEL MARS

Editorial comment: The process of development of a continuous multidisciplinary team approach, incorporating surgery, teaching and research and the creation of sustainable local teams in one country over 25 years are described. Unusually, the co-directors, one Sri Lankan, the other British, are not surgeons. Difficulties encountered within the organization, and local administrative and political problems, are analyzed.

INTRODUCTION The Sri Lankan Cleft Lip and Palate Project started in 1982. It is an example of a partnership between the Developing and the Developed Worlds. Over a period of 25 years it has in some measure fulfilled the long-term goal of establishing trained, sustainable multidisciplinary cleft lip and palate teams in Sri Lanka. This chapter is a critical appraisal of its history, development, achievements, difficulties encountered and shortcomings.

FOUR STAGES OF DEVELOPMENT The Project can be described and discussed in four stages. These reflect its development over 25 years. From the outset it was always concerned with multidisciplinary treatment, teaching and research. For the first five years the surgical teams in the Project performed over 600 operations. There was much teaching but little effective training of local surgeons, for reasons which will be discussed later (Visit 3). The creation of local self-sustaining cleft teams in partnership with the project has taken more than 20 years to establish. This has occurred gradually through direct teaching and training in Sri Lanka and the UK. More interestingly, it has also arisen in an indirect fashion, by a change of attitude in which cleft lip and palate has become recognized as more than a mere cosmetic problem. To some extent this development Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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might be considered a consequence of the physical and cultural impact of the large visiting project teams on the host population of both patients and medical professionals. The Sri Lankan Cleft Lip and Palate Project mirrors and precedes an approach that has been described as a three-stage philosophical model for establishing safe, multidisciplinary cleft teams in Developing Nations (Zbar et al., 2000). Zbar’s model begins with the visiting clinicians undertaking all the investigations and treatment with the hosts as observers in Stage 1 and these roles being reversed by Stage 3. An additional preliminary planning and reconnaissance stage is, we feel, most important and should precede the other three.

STAGE 1, 1982–85 r r r r

Conception. Organizational planning. Reconnaissance visit to Galle, S. Sri Lanka. The establishment of long-term aims.

Unlike most ‘missions’, the Sri Lankan Cleft Lip and Palate Project began with the joint aims of undertaking research into facial growth in cleft lip and palate subjects (especially those who had received no surgery), as well as the provision of surgical support. A set of ideal requirements which were considered to be desirable elements for cooperation and partnership and which might be applicable to any country, was researched. It is important to stress that this background research was undertaken in consultation with Professor Zefebrahim at the Department of International Child Health at the Institute of Child Health in London before any overtures to Sri Lanka were made. It was considered that in that country the elements listed below would ensure that the ideal requirements were likely to be fulfilled (Mars et al., 1990): r It is a relatively small island with a dense population. r There is a good transport infrastructure ensuring that patients could come to a centre taking not more than two days by road or train. r The quality of medical education is high and based on the British model, thereby facilitating integration by the visiting team. r English is the language spoken by all professional staff, and students are taught in English. r There is a very high literacy rate of 85–90% (rare in a Developing Country) and this would facilitate contact with patients. r A local organizer of high calibre (SL), who had undertaken post-graduate studies in London, could be approached to be the Sri Lankan co-director in this project. The second author (MM) wrote to the first (SL) in 1982 suggesting that they might collaborate in a research and surgical programme and an enthusiastic response was received. He confirmed that there were many unoperated patients in Sri Lanka. They

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could act as an important cohort for research and they needed treatment. The project was thus launched. This was a classic example of a situation where the Developing World had plenty of clinical material but very limited facilities to deal with such problems whereas the Developed World had the resources but clinical material was lacking. A reconnaissance visit was planned in 1983 but was deferred until 1984 as the civil war in Sri Lanka broke out at that time. Subsequent visits over the next 24 years were often rearranged at short notice because of flare-ups in the volatile war, which continues to this day. RECONNAISSANCE VISIT TO SRI LANKA, 1984 The first visit took place in July 1984 (Visit 1). A consultant oral and maxillofacial surgeon agreed to accompany the author. The aim of this visit was to undertake reconnaissance and record collection only. All equipment and materials for dental impressions, photographs, and the taking of lateral skull radiographs was carried as hand baggage. This was the first occasion that the two authors had met. SL became the Sri Lankan Co-Director of the Project. In one week, 45 patients of all ages presented for examination. The intention was to record adult patients with unrepaired clefts of the lip and palate. Many younger patients attended, as well as those with histories of lip repair but not palatal repair and some who had received both lip and palate repair at conventional times by Sri Lankan surgeons. Throughout, the authors were assisted by two senior Sri Lankan medical students who acted as interpreters. A local dental practitioner, Dr Nanayakkara, became a helpful friend and in future visits ‘Mister Fix It’, and in 1990 became a co-director. Lateral skull radiographs were taken in a private radiographic clinic so as not to over-burden the state hospital. The cepholastat was brought out from London and attached to the wall using large bolts, which had been brought specially. These first steps in self-sufficiency were to prove an important guiding principle for future visits. Each patient was paid 50 rupees at the end of their investigations, equivalent at that time to about one day’s pay for a labourer. On all future visits patients having records taken were similarly paid to cover their expenses. SL introduced MM to the hospital and university hierarchy, who all seemed keen for the Project to develop. Provisional plans were drawn up for a further visit. The impact of seeing adults with unoperated cleft lip and palate and their gross physical, functional and social disability was so striking for MM that it was uncomfortable being a ‘voyeur’. SL and MM agreed that future visits must and would provide surgery. DEVELOPMENT OF THE PROJECT The broad concept of a research and surgical combined team emerged and the practicalities of team membership, funding, equipment and its transport were immediate concerns. A cause for unease for MM was that the oral and maxillofacial surgeon, who had accompanied the author on the first surgical visit, wished to perform primary surgery in cleft lip and palate, although he had limited experience in this field.

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Professor of Plastic Surgery from Oslo, Frank Abyholm, agreed to head one surgical group, which then included the oral and maxillofacial surgeon. Given the anticipated volume of patients, it was decided to have a second surgical team. This would comprise British plastic surgeons. Each team would work for two weeks consecutively. FUNDING Initial requests for funding were made to the T.C. White Travelling Scholarship of the Royal College of Physicians and Surgeons, Glasgow, the British Council, the Overseas Development Administration (British Foreign Office) and the International Variety Clubs. Later requests were made to CLAPA.UK (the Cleft Lip and Palate Association). Such requests were favourably received. FIRST PLANNING MEETING AND DEVELOPMENT OF THE AIMS OF THE PROJECT The first planning meeting was held in September 1984. Those present were the maxillofacial surgeon, a plastic surgeon, a theatre nurse, a freight organizer and the author (MM). The essential principle of a broad-based multidisciplinary team with four aims was drawn up: 1. 2. 3. 4.

Treatment of patients. Teaching and training of Sri Lankan medical and nursing staff. The collection of data for research. The establishment of self-sustaining Sri Lankan multidisciplinary teams.

It was agreed that the above aims be given equal priority. It was also agreed that the project would be for cleft lip and palate treatment and research exclusively. Team members had expertise in a range of other fields such as burns and cancer surgery but it was felt that provision of a service in these areas would dilute the long-term aims of the project and fail to make the best use of the team overall. EQUIPMENT It was recognized that a large amount of equipment and materials would be required to accompany the teams. The then Head of Freight for the British Armed Services, a contact of MM, joined the first and all future planning meetings as an adviser from 1984 until 1990. The question of the type of equipment which would be required was addressed at this first meeting. It was evident that the teams would have to be almost totally self-sufficient. They could only presume the availability of an operating table, light, oxygen and anaesthetic gases. Everything else would have to be shipped out, including all disposable material, drugs, even nailbrushes and soap. Some materials could have been purchased locally but this would have been time-consuming and

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less reliable. It was imperative that the team should not become a drain on the local hospital resources in Galle, as these had been noted to be very limited. The following principles of equipment provision were agreed: r Self-sufficiency. r The equipment must be transported out by plane with the first team. r The freight manager would handle all negotiations with packers, shippers and airlines and liaise with the team specialists. r All equipment should be basic and of a type for which spare parts and servicing would be readily available in Sri Lanka. TEAM STRUCTURE The initial concept of two surgical teams and one simultaneous multidisciplinary assessment/research team emerged at the first planning meeting. The surgical team would comprise two surgeons, one anaesthetist and one theatre nurse. Subsequently, the team grew to include three anaesthetists, three surgeons, two theatre nurses and one ward nurse. These would work for two weeks each consecutively. The assessment/research team would comprise four orthodontists, two dental nurses, one ENT surgeon, one audiological physician, three speech and language therapists and, from 1990 onwards, a paediatrician and a medical photographer. RECRUITMENT OF PATIENTS SL made announcements of the future arrival of a team from the UK in the national newspapers and on radio and television. Patients requiring cleft lip and palate treatment were asked to write to him. Patients were also referred by medical colleagues and recruited at bus shelters and market places!

STAGE 2, 1985–90 r Three surgical and assessment/research team visits to Galle. r Two additional visits by the assessment/research team without surgical involvement. r The training of speech and language therapy counterparts in Galle. Three surgical and assessment/research visits: November 1985, November 1986 and November 1990 (Visits 2, 4 and 7, see Table 9.1). The following account of the working processes of the first surgical and assessment/research visit (Visit 2 above) describes the general pattern for all three such visits (Visits 2, 4 and 7). Modifications made on the subsequent visits in the light of the experiences of Visit 2 are described and discussed later in the chapter. The first surgical team, with the assessment/research team, left for Sri Lanka in 1985 for a four-week programme of work. The team worked in Galle, which is in

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MANAGEMENT OF CLEFT LIP AND PALATE Table 9.1. Programme of Project visits to Galle Team visits to Galle by the Sri Lankan Cleft Lip and Palate Project.

Visit

Date

Function

1 2 3 4 5

July 1984 Nov/Dec 1985 August 1986 Nov/Dec 1986 1988

6 7

August 1990 Nov/Dec 1990

8–13

1995–2002

Reconnaissance Surgical and Assessment/Research teams Assessment/Research team Surgical and Assessment/Research teams Four months speech and language therapy training of counterparts and treatment for patients who had palatal repair in Visits 2 and 4 Assessment/Research team Surgical and Assessment/Research teams. Speech and language therapy treatment for patients who had palatal repair in Visits 2 and 4 Six further visits for long-term follow-up and research data collection

a quiet provincial region in Southern Sri Lanka. There were none of the normal diversions which might have been expected in the capital Colombo. In this way, all the team were able to devote 100 per cent of their time to the Project work. The multidisciplinary, assessment/research team worked in a large suite of outpatient rooms with water and electrical supplies on the hospital site. The surgeons worked in the General (Teaching) Hospital operating theatres. Immediately on arrival in Galle, 2.5 tonnes of freighted equipment that had arrived with the team in 29 boxes was unpacked. The theatre, pre-operative and assessment/research suites were set up. The assessment/research team worked in the daytime from 8 a.m. until work ceased in the evening, often as late as 8 p.m. The surgical team began work in the theatre at 2 p.m. and finished at 10 p.m., though often continuing beyond midnight. This timetable of operating was designed to cause minimal interference to the daily routine of the local Sri Lankan surgeons. The General Hospital had only two theatres, one of which was always in reserve for emergencies. PRIORITY FOR TREATMENT Before the first surgical visit in November 1985 over 1,200 patients had replied to the press notices. Thirty new patients were invited to attend on each day and about thirty additional patients who had not replied to the newspaper notices attended daily seeking treatment. Surgery could not be offered to all these patients. We therefore drew up a priority system based on the type of deformity and our perception of the degree of disability of the condition. It is interesting to note that a similar process of according priority was later developed by Rotaplast (see Chapter 7, Capozzi). At the start of the working day the names of patients were called and the author (MM) personally examined each one to assess the priority grouping (see Table 9.2).

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THE SRI LANKAN CLEFT LIP AND PALATE PROJECT Table 9.2. Classification of priority groupings for treatment

Priority grouping

Criteria of allocation of patients

Group 1

Patients with unoperated lips at any age over 3 months Patients who had unoperated palatal clefts over six months of age. Priority within this group given to younger patients Patients with previously repaired lip and/or palates by Sri Lankan surgeons, who would benefit from further surgery Patients with medical conditions unrelated to cleft lip and palate

Group 2 Group 3 Group 4

No. of patients requesting treatment 207 319 309 417

It was apparent that not all patients could receive treatment. Those in Priority Group 4 were advised to seek help from local clinicians. Those in Priority Group 3 were discharged. All patients in Priority Group 1 were taken on for investigation and treatment and a proportion of patients in Priority Group 2 were examined and treated. Many in this group were deferred for future visits. The allocation of patients to the operating lists was the responsibility of MM (an orthodontist) supported by a dental nurse, a speech and language therapist and the paediatricians (Ms Christine Godber, Dr Debbie Sell, Dr Alex Habel and Professor Sanath Lamabadusuriya, respectively) working in the management/research team. This worked surprisingly well because only primary surgery was being undertaken (either lip or palate repair), thereby making a simple process of triage possible. Additional anaesthetic safety was built in by the pre-operative paediatric examination prior to final assessment by the anaesthetists and surgeons. INVESTIGATIONS UNDERTAKEN The following records were made of all patients for whom surgery was planned and some others who were placed on a waiting list: r dental study models; r clinical photographs; r cephalograms (lateral skull X-rays taken in a cephalostat for all patients over 6 years of age); r social and psycho-social histories; r audiometry assessments; r speech recordings (audio and video) for children over 5 years of age; r in subsequent visits, paediatric assessments, ENT examinations, and nasendoscopy were included, as well as lateral skull radiographs with patients phonating ‘ee’.

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During Visit 2, 224 subjects were examined and documented in Galle, while 195 operations were performed. TRAINING Every member of the expedition participated in teaching and training usually through formal lectures on a variety of subjects to doctors, medical students and nurses. In addition, clinical tuition was carried out in the operating theatre, on the wards and in the assessment/research areas. Ten senior medical students were assigned to the teams to act as interpreters and they received teaching and training as well. In 1990 (Visit 7), two separate whole-day workshops were conducted for anaesthetists and maxillofacial and dental surgeons who came from all over Sri Lanka. MODIFICATIONS TO ORIGINAL PLANS, WHICH WERE UNDERTAKEN WHILE IN SRI LANKA The original programme devised in London was that all patients presenting with unoperated lips and palates would have both repaired simultaneously. Two factors made this proposal difficult to fulfil, namely the very large numbers of patients presenting and the time required to perform both procedures. At a trial run on the first night, the surgeons treated only three patients using this protocol. It was important that as many patients be helped as possible and that our financial backers, seeing the volume of patients treated and the numbers still in need of surgery, would respond with further funds for more surgical visits in the future. The team agreed that patients would either have their lip or their palate closed but not both simultaneously. Wherever possible, the turnaround time in the operating theatre between one patient starting surgery and another finishing needed to be reduced to the absolute minimum. To achieve this, the anaesthetists ensured that the next patient for surgery was anaesthetized before surgery on the previous patient was completed. A newly scrubbed surgeon was ready to operate on the next case while the previous case was recovering. No patient received pre-medication and all patients sat outside the operating theatre waiting their turn. This process would probably be considered unacceptable in a Developed World situation (ACPA Guidelines, 2000). However, it was introduced at the suggestion of the Sri Lankan Co-Director. He pointed out that these patients had been waiting for many years for this surgery, and a further small wait was tolerable. The Project simply could not spend valuable time transporting patients between the wards and the operating theatre. There were few porters available and patients awaiting surgery tended to ‘wander’ out of the hospital wards thus causing delays. The turnaround time was thereby cut to two minutes, making it possible to undertake eight or nine operations per evening. Despite the provision of just one operating table, the availability of two surgeons and two anaesthetists in 1985 and three of each in 1986 and 1990 ensured optimum use of the facility in the time available.

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VISITS 3 AND 6, JULY 1986, AND AUGUST 1990, ASSESSMENT/RESEARCH TEAM The purpose of these visits was fourfold: 1. To organize the next surgical and assessment/research visits, which had been provisionally planned for November/December 1986 and 1990 (Visits 4 and 7). 2. Provision of speech therapy courses for patients who had received palatal repair in November 1985 and 1986. 3. The documentation of over one hundred adult non-cleft Sri Lankans to act as ‘control’ subjects. 4. Documentation of patients who had received lip surgery in November 1985 and 1986 in preparation for palatal surgery in November 1986 and 1990 (Visits 4 and 7) and the collection of longitudinal data on previously operated patients. ADMINISTRATIVE DIFFICULTIES AND LOCAL AND NATIONAL MEDICAL POLITICS It was possible to achieve the major aims set out in points 2, 3 and 4 above with relative ease. However, attempts to organize the next surgical and assessment/research visit (Visit 4, November 1986) proved to be very difficult. Many local surgeons had presumed that they would, and should, direct the Sri Lankan side of the project. Their perception was that this was a surgical expedition. However, the Sri Lankan CoDirector and the person who was responsible for all local organization up to that point was the Professor of Paediatrics (SL). A senior surgeon from Galle had made efforts to undermine the project just two weeks before the first surgical and assessment/research visit (Visit 2) in November 1985. On a visit to London, he expressed the view to MM that, in his opinion, clefts were merely a cosmetic problem. The Project was perceived as a highly prestigious team visit from a leading UK children’s hospital. Emissaries from Sri Lanka had already visited MM in London in an attempt to bring the project to either Kandy or Colombo (both more established medical teaching hospitals where better facilities were promised), and to abandon Galle, which, at that time was considered to have lesser status. The Co-Directors stood firm in their resolve to work together in Galle, refusing to be intimidated by threats to the future of the Project or coerced by inducements of better facilities elsewhere. Local political difficulties between the academic medical school and the clinical hospital hierarchies resulted in considerable problems when attempting to organize Visit 4, the November 1986 visit. The Sri Lankan Cleft Lip and Palate Project became the battleground for the polarization of both sides. MM met the Director of Galle General (Teaching) Hospital and the Professor of Surgery to discuss plans for the proposed next visit in three months time (Visit 4, November/December 1986). They explained that there were problems of nursing, ward space and theatre availability, which would be difficult to resolve. The offer

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was made to continue to pay nurses from Project funds on a generous overtime rate as on the previous visit. The British surgeons were prepared to work at night, when the theatres would not be in use. The only, but important criticism of the Project’s organization in the previous visit related to the management of post-operative care, once the British team had left. One patient who had received palatal surgery suffered a serious haemorrhage requiring the transfusion of three units of blood. This occurred one week after all the Project team members had left. This problem has been highlighted by several authorities in criticism of ‘surgical safaris’ (Natsume, 1998; Dupuis, 2004; Mulliken, 2004). It was acknowledged that a serious clinical management error had occurred and an apology was made. At the meeting the following measures were put forward to ensure proper aftercare in any future visits. It was proposed that if Visit 4 were to take place, then all palatal surgery, which is prone to post-operative bleeding, especially in adults (Ward & James, 1990), would take place in the first three weeks of the four-week working period. The fourth week would be exclusively given over to lip repair, in which post-operative haemorrhage is extremely rare. In addition, one surgeon and one anaesthetist would remain for an extra two weeks after all surgery had been completed to cover emergencies on any of the patients operated on by the Project. These offers relating to nursing provision, ward availability, theatre use and post-operative care were considered, but were not accepted. The Dean of the Medical School in the meantime had organized a meeting for MM accompanied by SL at the Ministry of Teaching Hospitals in Colombo. He had also arranged a meeting with an elderly, devout Buddhist and wealthy philanthropist, BRD, who had just completed building a hospital in Galle and had presented it as a gift, to the Ayurvedic Ministry. At the meeting with BRD, all the facilities that the Project could possibly require were promised in the new spacious Ayurvedic Hospital. MM and SL attended the meeting at the Ministry of Teaching Hospitals. The officials explained that even before the first visit there had been a great deal of opposition to the Project from some influential sources. The officials pressed hard for the Project to relocate to Colombo and Kandy, where better facilities were assured and support from the local professional staff would be forthcoming. At that time there were three Ministries of Health: the Ministry for Teaching Hospitals was Western oriented, the second was the Ministry of General Hospitals, while the third was for indigenous or Ayurvedic medicine. It was inconceivable and a cause of concern to the Teaching Hospitals officials that a prestigious team of Western clinicians would now operate in the Ayurvedic hospital in Galle. This would cause embarrassment in Sri Lanka. MM and SL explained that given the lack of goodwill from the Director of the Teaching Hospital in Galle, they preferred to relocate. Because of direct pressure from the Ministry of Teaching Hospitals, the Director of Galle General (Teaching) Hospital had to climb down and make facilities available. It was disappointing that the training of local surgeons in Galle was not possible at this stage of the project because of the difficulties discussed above.

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MODIFICATIONS ADOPTED IN VISIT 4, SURGICAL AND ASSESSMENT/RESEARCH TEAM, NOVEMBER/DECEMBER 1986 r Surgery was undertaken from 8 p.m. until 4 a.m. instead of 2 p.m. till 10 p.m. as in 1985, thereby causing no disturbance to the working of local surgeons. The Project paid for theatre cleaning. r Palatal surgery was performed in the first three weeks only of the four-week visit. r A surgeon and an anaesthetist remained on site for two weeks after the last operation to cover emergencies. These measures ensured that there was three weeks cover by the team after the last palatal repair. VISIT 5, FOUR MONTHS SPEECH AND LANGUAGE THERAPY, TRAINING OF COUNTERPARTS AND TREATMENT, JULY–NOVEMBER 1988 This programme is described and discussed in Chapter 16 (Wirt et al., 1990a; 1990b). MODIFICATIONS ADOPTED IN VISIT 7, 1990 A paediatrician, medical photographer and freight manager were included in the team.

STAGE 3, 1990–99 r Research data collection visits in Galle and follow-up of patients operated in Stage 2. r The creation of a university-based speech and language training course in the medical school of Kelaniya, Colombo. VISITS 8 TO 13, 1995–2002. SIX FURTHER VISITS FOR LONG-TERM FOLLOW-UP AND RESEARCH DATA COLLECTION Longitudinal data collection has continued in Galle on over 500 patients operated upon by the Project. Specialists involved include orthodontists, speech and language therapists, paediatricians, audiological physicians and a geneticist. A multidisciplinary team of Sri Lankan surgeons, orthodontists and a speech and language therapist is now established in Galle. The records of all the patients collected during the years of the Project have been handed over to them and the Project team members have started to work alongside this new local team in exchange visits. ASPECTS OF TEAM DYNAMICS Over 60 team members have participated in the project in Galle, several on more than 12 visits. The selection of team members was originally based on the two hospitals in which MM worked, and radiated outwards by personal contacts to include members

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resident throughout Great Britain as well as two Norwegian orthodontists and surgeons. CRITERIA FOR TEAM SELECTION AND DYNAMICS Attendance at the planning meetings, which were held at least fortnightly for four months prior to each visit, was regarded as mandatory and essential to the smooth working of the team when in Sri Lanka. From 1985 to 1990, one nurse, one anaesthetist, and a plastic surgeon were asked to withdraw because of failure to attend planning meetings. Various expatriate Sri Lankans, who lived and worked in Britain, were chosen as team members in the early visits. It was felt that they would be helpful with the language and customs in Sri Lanka. However, the presumed benefits were outweighed by the disadvantages. Several made independent arrangements to visit their families during the visits and another presumed to buy materials and equipment locally without consultation with the Co-Directors. It was at the request of the Sri Lankan Co-Director, SL, that no expatriates were included in later visits. Several clinicians who had no experience at all in cleft lip and palate asked to accompany the teams offering to pay for themselves. These requests were turned down as only those with expertise to offer were invited. No family members/friends were permitted to accompany the team during the working period of the visit unless coincidentally they were active team members. Those who wished to take a holiday after their working commitment had finished could then be joined by their family and friends. The additional problems of the inclusion of such visitors are well expounded by Richard (Chapter 2). DIFFICULTIES WITHIN THE TEAM It is perhaps inevitable that difficulties might arise in expeditions comprising a wide range of professional team members, who were used to a large degree of autonomy as team leaders in their home environment. In hindsight, problems dismissed as being due to fatigue, the heat, tropical diarrhoea and unfamiliarity with the working environment were in large part due to or compounded by two features of the structure within the Sri Lankan Cleft Lip and Palate Project and were responsible for conflict: 1. status incongruency; 2. the conflict between maxillofacial and plastic surgeons in Britain and the demarcation disputes concerning cleft lip and palate patients were exported as part of the ‘baggage’ to Sri Lanka. RESOLUTION OF ISSUES: A VARIETY OF PROBLEMS AND SOLUTIONS Example 1 Pharyngoplasties – to operate or not? An important role of the Co-Directors of the Project was to co-ordinate and motivate the visiting team, and also to act as liaison between them and the team of Sri Lankans.

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In 1990, the plastic surgeons were eager to undertake simultaneous pharyngoplasties and palatoplasties, which the Co-Directors and anaesthetists considered to be ill advised. This type of management decision was seen as an invasion of surgical clinical freedom. Prior to 1990, 410 operations had been performed by the Project without serious mishap. It was important to ensure that no unnecessary risks were taken and that the most good could be provided for the greatest number. Normally, the decision to undertake pharyngoplasties is a surgical one. The fact that there was no readily available blood transfusion service in Galle, very limited monitoring equipment on the wards and only a handful of inexperienced nurses for up to 70 post-operative cases at any one time seemed to be courting disaster. After discussions with all team members, the decision was made that no pharyngoplasties would be performed. There is no doubt that the death of a patient in the care of the team would have been a serious blow and could lead to the possible discontinuation of the Project. The literature at that time had several reports of death and severe obstructive sleep apnoea post pharyngoplasty (Kravath et al., 1980; Shprintzen, 1988). Example 2 Patient triage by non-surgeons and implications for surgical autonomy The structure in which the surgeons worked in Sri Lanka was quite different from that in the UK. In the Project, it was the orthodontist, a speech and language therapist and the paediatrician who selected patients for surgery and assigned them to operating lists. On the whole, this system worked well because only lip or palatal repair was performed and by and large syndromic patients with suspected complex cardiorespiratory problems were not selected (10% of presenting patients were ‘Syndromic’ with conditions such as Pierre Robin Sequence, Stickler, polio, and First Arch Syndromes). Example 3 Surgical craft disputes On all three major surgical visits, there were two surgical teams who each worked for two weeks consecutively. One team was plastic surgery-based, the other was mixed plastic and maxillofacial surgery-based in 1985 and 1986 and exclusively maxillofacial in 1990. The often heated demarcation disputes surrounding the management of cleft lip and palate patients evident in the UK were sometimes exported with the teams to Sri Lanka. These artificial barriers have generally been resolved in recent years, ensuring better patient care with mutual respect between the two disciplines.

STAGE 4, 1999–THE PRESENT r The continuation of research data collection in Galle and follow-up of patients. r Working with the newly created multidisciplinary Sri Lankan team in Galle. r Supporting and training the new teams in Colombo and Peradeniya (Kandy).

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THE FUTURE OF THE SRI LANKAN CLEFT LIP AND PALATE PROJECT IN GALLE A multidisciplinary team of Sri Lankan surgeons, orthodontists and a speech and language therapist is now established in Galle. A further research and data collection visit is planned for 2008. EXTENSION OF THE PROJECT TO PERADENIYA (KANDY) AND COLOMBO, 1999–THE PRESENT There has been a cleft lip and palate surgical service centred on the Dental Hospital in Peradeniya for over 30 years though without a true multidisciplinary team involvement (Chapter 14, Wijekoon). Members of the Project multidisciplinary team have made three visits to Peradeniya since 1999 and treatment, teaching and research have been undertaken. This has become a second centre of collaboration. Members of the Project also support the third centre, the newly created multidisciplinary team in Colombo. They have also given a one-day seminar on speech prostheses in Colombo.

AN APPRAISAL OF ACHIEVEMENT OF THE SRI LANKAN CLEFT LIP AND PALATE PROJECT The aims set out in 1985 were: 1. Treatment. The Project has performed 620 operations in Galle, Southern Sri Lanka. Speech and language therapy treatment has been provided for patients in Galle. 2. Teaching. This has been given to medical students, postgraduate doctors, nurses and speech and language therapists in Sri Lanka. The university-based speech and language therapy training programme (a direct consequence of the Project’s involvement) started in 1997, the details of which are described and discussed in Chapter 16. Two surgeons have been trained in Great Ormond Street Hospital for Children (GOS), each for one year. Four speech and language therapists have been seconded to GOS for four weeks of training. A trainee orthodontist will spend one year with the cleft team at GOS in 2008. The whole team from Peradeniya (Kandy) has spent two weeks visiting GOS. 3. Research and academic achievements. To date, four PhDs (Sell D. 1992, Mars M. 1993, Worrell E. 2005, Liao Y.F. 2005) and 11 MSc degrees (Roberts-Harry D.P. 1998, McCance A.M. 1988, Ng B. 1989, Nayak J.A. 1996, Muthusamy A. 1998, Arshad N. 1998, Sykes D.A. 1998, Birkett L. 1999, Chia Y.S. 1999, Kanjanaplang B. 1999, Aker M. 2001) have been generated by the Project. Twenty-one papers have been published. (Lamabadusuriya et al., 1988; Ward, 1990; Mars & Houston, 1990; Mars et al., 1990; Sell & Grunwell, 1990; Shprintzen, 1990; Ward & James, 1990; Albert et al., 1990; McCance et al., 1990; Wirt et al., 1990a; 1990b; Brown et al., 1992; McCance et al., 1993; Sell & Grunwell, 1994; Sell et al., 1994; Liao & Mars 2005a; 2005b; 2005c; Wickenden et al., 2001; Liao & Mars 2006; Liao et al., 2006).

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Longitudinal analysis has been made possible because the recall rate for bilateral cleft lip and palate patients was 95% and 70% for unilateral cleft lip and palate cases in all follow-up visits. This rate has not been matched in the author’s (MM) hospital in London. 4. The creation of Sri Lankan self-sustaining multidisciplinary teams and the current status of cleft care in Sri Lanka. There are now three multidisciplinary teams in Sri Lanka, in Galle, Colombo, and Peradeniya (Kandy). All have close links with the Project team members in the UK with exchanges of team members between the two countries. There is no longer a reservoir of unoperated cases in that country though there are occasional untreated cases in remote areas.

CONCLUSION The creation, execution and development of the Sri Lankan Cleft Lip and Palate Project have been a life-enhancing, often exhausting, exhilarating and at times exasperating exercise for both Co-Directors over a period of 25 years. In particular, they have striven to sustain a long-term project aimed at the creation of self-sustaining multidisciplinary teams in Sri Lanka. Treatment, teaching and research were and continue to be their aims. If they have succeeded, that is because of the hard work and understanding of the Sri Lankan and UK Project team members as well as the fortitude of Sri Lankan patients. From the outset this was, and remains, a multidisciplinary team project in which research and teaching had equal status to surgery. Insights into facial growth, speech, somatic growth, psychosocial well-being, hearing, project management, the development of local infrastructures, paediatrics, anaesthesia and surgery have all been expanded. In an editorial, Shprintzen (1990) described papers published by this project as valuable lessons for others who also visit the Third World to deliver care, and as excellent descriptions of the mechanics of the expeditions, the surgical decision-making process, speech results after surgery and the establishment of training programmes for local professionals. The focus has been on one country for over 20 years and the authors strongly believe that this is an approach which gives this Project strength and credibility (Mulliken, 2004). It also provides an opportunity to recover from mistakes, refine methods to suit local physical and cultural conditions and in the process develop enduring partnerships and friendships.

REFERENCES Aker M (2001) ‘A cephalometric investigation of facial growth and morphology in unilateral cleft lip and palate subjects: a study of operated and unoperated Sri Lankan subjects with special reference to growth of the posterior part of the maxilla’, MSc, University of London. Albert DM, Garrett J, Specker B & Ho M (1990) ‘The otologic significance of cleft palate in a Sri Lankan population’, Cleft Palate J 27: 155–61.

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American Cleft Palate Association position paper: international treatment programmes. Available at: www.acpa-cpf.org/whoweare/papers.htm (accessed 22 July 2007). Arshad N (1998) ‘Effect of late lip repair on unilateral cleft lip and palate Sri Lankan subjects’, MSc, University of London. Birkett L (1999) ‘A study of speech results and timing of surgery in a Sri Lankan UCLP population’, MSc, University of London. Brown SE, Mars M & Sell DA (1992) ‘The Sri Lankan Cleft Lip and Palate Project: the medical illustrator’s contribution’, J Audiov Media Med 15: 4–7. Chia YS (1999) ‘Facial growth and the timing of surgery in unilateral cleft lip and palate’, MSc project, University of London. Dupuis CC (2004) ‘Humanitarian missions in the Third World: a polite dissent’, Plast Reconstr Surg 113: 433–5. Kanjanaplang B (1999) ‘The timing of palatal repair and its effect on facial growth and morphology in isolated cleft palate: a comparative study of earlier (1.4–4.5 years) versus later surgery (5.6–9.3 years)’, MSc, University of London. Kravath RE, Pollack CP, Borowiecki B & Weitzman ED (1980) ‘Obstructive sleep apnoea and death associated with surgical correction of velopharyngeal incompetence’, Pediatrics 96: 645–8. Lamabadusuriya SP, Mars M & Ward CM (1988) ‘Sri Lankan Cleft Lip and Palate Project: a preliminary report’, J Royal Soc Med 81: 705–9. Liao YF (2005) ‘Timing of hard palate repair and facial growth in patients with unilateral cleft lip and palate’, PhD, University of London. Liao YF, Cole TJ & Mars M (2006) ‘Hard palate repair timing and facial growth in unilateral cleft lip and palate: a longitudinal study’, Cleft Palate Craniofacial J 43: 547–56. Liao YF & Mars M (2005a) ‘Long-term effects of lip repair on dentofacial morphology in patients with unilateral cleft lip and palate’, Cleft Palate Craniofacial J 42: 526–32. Liao YF & Mars M (2005b) ‘Long-term effects of palate repair on craniofacial morphology in patients with unilateral cleft lip and palate’, Cleft Palate Craniofacial J 42: 594–600. Liao YF & Mars M (2005c) ‘Long-term effects of clefts on craniofacial morphology in patients with unilateral cleft lip and palate’, Cleft Palate Craniofacial J 42: 601–9. Liao YF & Mars M (2006) ‘Hard palate repair timing and facial morphology in unilateral cleft lip and palate: before versus after pubertal peak velocity’, Cleft Palate Craniofacial J 43: 259–65. Mars M (1993) ‘The effect of surgery on facial growth and morphology in Sri Lankan UCLP subjects’, PhD thesis, University of London. Mars M & Houston WJ (1990) ‘A preliminary study of facial growth and morphology in unoperated male unilateral cleft lip and palate subjects over 13 years of age’, Cleft Palate J 27: 7–10. Mars M, James DR & Lamabadusuriya SP (1990) ‘The Sri Lankan Cleft Lip and Palate Project: the unoperated cleft lip and palate’, Cleft Palate J 27: 3–6. McCance AM (1988) ‘A study model analysis of Sri Lankan adult unoperated unilateral cleft lip and palate patients’, MSc, University of London. McCance AM, Roberts-Harry D, Sherrif M, Mars M & Houston WJB (1990) ‘A study model analysis of adult unoperated Sri Lankans with unilateral cleft lip and palate’, Cleft Palate J 27: 146–54. McCance AM, Roberts-Harry D, Sherrif M, Mars M & Houston WJB (1993) ‘Sri Lankan cleft lip and palate study model analysis: clefts of the secondary palate’, Cleft Palate Craniofac J 30: 227–30.

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Mulliken JB (2004) ‘The changing faces of children with cleft lip and palate’, New Engl J Med 351: 745–7. Muthusamy A (1998) ‘Effect of late primary lip repair on Sri Lankan subjects’, MSc, University of London. Natsume N (1998) ‘Safari surgery’, Plast Reconstr Surg 102: 1304–5. Nayak JA (1996) ‘A systematic study correlating speech results of Sri Lankan UCLP participants with timing of surgery’, MSc, University of London. Ng BG (1989) ‘Maxillary arch analysis of Sri Lankan unilateral complete cleft lip and palate patients’, MSc, University of London. Roberts-Harry DP (1998) ‘A study model analysis of adult Sri Lankan patients with unoperated clefts of the secondary palate’, MSc, University of London. Sell DA (1992) ‘Speech in Sri Lankan cleft palate subjects with delayed palatoplasty’, unpublished PhD thesis, De Montfort University. Sell D & Grunwell P (1990) ‘Speech results following late palatal surgery in previously unoperated Sri Lankan adolescents with cleft palate’, Cleft Palate J 27: 162–8. Sell D & Grunwell P (1994) ‘Speech studies and the unoperated cleft palate subject’, Eur J Disorders Commun 9: 151–64. Sell D, Grunwell P & Mars M (1994) ‘A methodology for the evaluation of severely disordered cleft palate speech’, Clinical Linguistics and Phonetics 8: 219–33. Shprintzen RJ (1988) ‘Pharyngeal flap surgery and the paediatric upper airway’, Int Anaesthesiology Clinics 26: 79–88. Shprintzen RJ (1990) ‘Editor’s commentary on the papers from the Sri Lankan Cleft Lip and Palate Project’, Cleft Palate J 27: 174–5. Sykes DA (1998) ‘Timing of palatal surgery in unilateral cleft lip and palate patients from Sri Lanka: the effect on craniofacial growth’, MSc, University of London. Ward CM (1990) ‘Surgery of cleft lip and palate in Sri Lanka’, Chirurgie 115: 637–9. Ward CM & James I (1990) ‘Surgery of 346 patients with unoperated cleft lip and palate in Sri Lanka’, Cleft Palate J 27: 11–15. Wickenden M, Hartley S, Kodikara S, Mars M, Sell D, Sirmana T & Wirz S (2001) ‘Collaborative development of a new course and service in Sri Lanka’, Int J Lang Commun Disord 36, Suppl.: 315–20. Wirt A, Wyatt R, Sell D, Grunwell P & Mars M (1990a) ‘Training counterparts in cleft palate speech therapy in the Developing World: a report’, Cleft Palate J 27: 169–73. Wirt A, Wyatt R, Sell D, Grunwell P & Mars M (1990b) ‘Training counterparts in cleft palate speech therapy in the Developing World: an extended report’, Brit J Dis Commun 25: 355–67. Worrell E (2005) ‘The effects of surgery on facial growth in bilateral cleft lip and palate Sri Lankan subjects’, PhD, University of London. Zbar RIS, Rai SM & Dingman DL (2000) ‘Establishing cleft malformation surgery in developing nations: a model for the new millennium’, Plast Reconstr Surg 106: 886–9, discussion, 890–1.

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Facial Growth in Cleft Lip and Palate Subjects MICHAEL MARS

Editorial comment: A unique archive is reviewed to provide an overview of how surgical intervention alters facial growth. It provides a basis for comparison for future studies, using robust methods such as the Goslon Yardstick which is easily understood by all members of the multidisciplinary team.

INTRODUCTION That facial growth in the operated cleft lip and palate subject is often disturbed is well recognized. Maxillary retrusion is a common phenomenon in patients with unilateral cleft lip and palate (UCLP). Much of the growth disturbance of the maxillary skeleton almost certainly results from the surgical repair of the palate. The idea that palate repair is detrimental to maxillary growth originated with a clinical observation of Gillies and Fry (1921) and the experimental and clinical works of Herfert (1958). A popular theory of abnormal maxillary growth was proposed by Ross (1970). He stated that excessive post-operative scar tissue formed by undermining of soft tissues and the creation of denuded palatal bone, adjacent to pterygo-palatine-tuberosity sutures can inhibit forward growth of the maxilla. Longitudinal growth studies have clearly demonstrated that the maxillary retrusion in patients with UCLP is progressive and worsens during the pubertal growth phase (Ross, 1987a, 1987b, 1987c; Enemark et al., 1990; Semb, 1991; Smahel et al., 1993). Only a few studies have evaluated the long-term isolated effects of lip and/or palate repair on craniofacial growth in patients with UCLP (Schweckendiek, 1978; Mars & Houston, 1990; Capelozza Filho et al., 1996; Kapucu et al., 1996; Liao & Mars, 2005a, 2005b). The main problem in evaluating the separate influences of clefting itself and lip and palate repair is that surgery (at least in Developed Countries) is routinely performed long before growth of the facial complex ceases. This may confound the influence of the isolated effects of lip or palate repair on craniofacial growth. This chapter summarizes the findings of studies in UCLP subjects from the Sri Lankan Cleft Lip and Palate Project based on data collected over 20 years involvement Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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(Mars et al., 1990; McCance et al., 1990; Mars, 1993; McCance et al., 1993; Arshad, 1998; Liao & Mars, 2005a, 2005b, 2005c, 2006). These studies employ cephalometrics, reflex microscopic and Goslon Yardstick analyses (Mars et al., 1987). The cephalometric summary is presented. The use of the Goslon Yardstick demonstrates the overall picture and is easily accessible to a multidisciplinary team readership.

BACKGROUND TO THE SRI LANKAN CLEFT LIP AND PALATE PROJECT RESEARCH ARCHIVE The material used in the studies from the Sri Lankan Cleft Lip and Palate Project has been collected over a period of 23 years. More than 500 patients have had longitudinal records taken to include facial growth data from birth to maturity. The overall recall rate for patients exceeded 80% on some 13 return data collection visits for subjects up to 18 years after first examination. The lateral skull radiographs and dental study models have been collected under rigorous controlled conditions to ensure the quality and validity of subsequent analyses. All radiographs were taken in the same cephalostat (head-holding device) with the same film to mid-sagittal plane to anode distances measured each day of each visit. The same magnification for all films and the reliability of longitudinal measurements have thereby been ensured. All impressions were cast in dental stone on the day that they were taken to prevent any distortion. They were all based and orthodontically trimmed after shipment to the UK. The archive comprises over 2,000 lateral skull radiographs and dental study models on cleft lip and palate patients. In addition, the same records have been collected on over 200 normal healthy control subjects. Patients in Sri Lanka presented at all ages from birth to maturity who had no surgery, lip surgery only and in some cases both lip and palate surgery. They had received surgery at varying times. In addition, patients operated on within the Project in 1985, 1986 and 1990 have been followed up to the present day. This has created a rare opportunity to study the long-term effects of clefting itself, the isolated effects of lip and palate repair on facial growth and the influence of the nature and timing of surgical intervention (‘nature’s experiment’). The results of three recent comprehensive cephalometric studies utilizing data from the Sri Lankan Cleft Lip and Palate project archive (Liao & Mars 2005a, 2005b, 2005c) demonstrate the long-term effects of clefting itself and the isolated effects of lip and palate repair. These are presented below. In addition because patients have been followed for almost 20 years it is possible to present longitudinal growth research data (Mars et al., 2005; Liao et al., 2006).

THE UNOPERATED CLEFT Previous studies evaluating cephalograms in unoperated patients with UCLP and comparable normal (non-cleft) controls have found deviations of craniofacial morphology

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(Subtelny, 1955; Ortiz-Monasterio, 1959; Bishara et al., 1976; Mars & Houston, 1990; Capelozza Filho et al., 1996; Motohashi et al., 1994). Unfortunately some findings are still inconclusive because of their methodological limitations (Will, 2000). In the first of this series of three comprehensive cephalometric studies referred to above (Liao & Mars, 2005c), 30 complete UCLP Sri Lankan subjects who had no previous treatments on their clefts and who were aged over 17 years, were compared with 52 control (normal) Sri Lankan subjects also aged over 17 years. This study demonstrated that the intrinsic adverse effects of clefts in UCLP patients were on the vertical development of the basal maxilla, anteriorly and posteriorly, compared to control subjects. However, the overall antero-posterior dimensions and relationships of the maxilla are unaffected by clefting itself. Such patients do not demonstrate maxillary retrusion.

THE LIP REPAIR ONLY GROUP In the second retrospective, cross-sectional study using cephalograms from the growth archive of the Sri Lankan Cleft Lip and Palate Project (Liao & Mars, 2005b), 48 subjects who had lip repair only were compared with 23 totally unoperated subjects all over the age of 17 years. This study demonstrated that the major effect of lip repair was on the antero-posterior and vertical position of the maxillary alveolus and maxillary incisors. Furthermore, there was a differential influence from the tip of the alveolus and the incisal edge to the base of the alveolus and the incisal apex. This resulted in a decreased overjet and increased overbite in the lip repair only group. These findings concur with the reflex microscopic study on dental study models undertaken by Muthusamy in 1998. No adverse effect on the basal maxilla in either the anteroposterior or vertical dimensions was found. In summary, lip repair produces a ‘bone bending’ effect on the anterior maxillary alveolus (alveolar moulding) accompanied by controlled uprighting of maxillary incisors.

THE LIP AND PALATE REPAIR GROUP In the third article in this series, the same 48 subjects who had lip repair only (see above) were compared with 58 UCLP subjects who had lip and palate repair by the Project’s surgeons before the age of 9 years (1985, 1986, 1990). Results of this study demonstrated that palatal repair inhibits forward displacement of the basal maxilla and antero-posterior development of the maxillary dentoalveolus in patients with UCLP. Contrary to expectations, the position of the maxillary incisors is not affected by palate repair, though interestingly this, of course, had been a major effect of lip repair.

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DEMONSTRATION OF THE EFFECT OF NO SURGERY, LIP SURGERY ONLY, AND LIP AND PALATE SURGERY USING THE GOSLON YARDSTICK The Goslon Yardstick has been used for more than 25 years (Mars et al., 1987). The Yardstick is a means of classifying maxillary deformity based on a consensus analysis of study models of patients with repaired and unrepaired UCLP. Groups 4 and 5 (the worst) indicate severe maxillary retrusion which would necessitate midface surgery for correction. Group 1 (the best) demonstrates maxillary protrusion. The correlation of Goslon Yardstick grouping and conventional cephalometric values is high (Mars & Plint, 1985), but unlike cephalometric measurements, the results of the Yardstick can be readily displayed in a bar chart (Figure 10.1). In the Eurocleft study (Shaw et al., 1992; Mars et al., 1992), the Goslon Yardstick was shown to be more sensitive than cephalometrics in distinguishing differences between centres. The first Goslon Yardstick analysis on the Sri Lankan archive material was reported in 1990 (Mars & Houston, 1990). This demonstrated that a very high proportion of patients in the totally unoperated group were from Group 1, and none were in Groups 4 and 5. Patients who have had total lip and palate surgery in infancy appear at the other end of the spectrum with nearly 70% in Groups 4 and 5. It is of interest that patients with operated lips and unoperated palates are evenly distributed between the three acceptable groups (1, 2 and 3). These Goslon results for Sri Lankan patients who had lip and palate repair by Sri Lankan surgeons correlate very closely with those from the UK where Wardill-Kilner palatal repairs had been performed (Mars et al., 1992). In a later study mirroring the cephalometric analyses above (Liao & Mars, 2005a, 2005b, 2005c), the Goslon Yardstick was applied to the same three groups (Mars

Figure 10.1. Goslon Yardstick grouping of unoperated, lip repair and lip and palate repair UCLP subjects of Sri Lankan Cleft Lip and Palate Project patients operated upon in 1985, 1986 and 1990. Source: Mars et al. (2005).

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et al., 2005). This group had surgery performed by the Project surgeons in 1985, 1986 and 1990. It showed that there are statistically significant differences between unoperated and lip repair only, and between lip repair and palate repair patients for arch dimensions and Goslon scores (p <0.05). The Goslon Yardstick groupings for the totally operated group were much improved from the first study and showed 35% in Groups 4 and 5, of which just 5% were in Group 5. The Von-Langenbeck palatal repair had been performed on these patients. These results reflect the influence of the nature of surgery on facial growth outcomes. The following conclusions were made: unoperated patients demonstrated exuberant antero-posterior positioning of the alveolus; lip repair has a moulding effect on the anterior dental alveolus, palate repair results in a significantly greater proportion of severe Class III dental arch relationships. These Goslon Yardstick results concur with the cephalometric measurements in the same group of patients (Liao & Mars, 2005a, 2005b, 2005c).

THE TIMING OF HARD PALATE REPAIR AND FACIAL GROWTH IN UCLP The Sri Lankan Cleft Lip and Palate archive has provided an opportunity to undertake a retrospective longitudinal study addressing the question of the timing of hard palate repair and its effect on facial growth in UCLP (Liao et al., 2006). Some 104 patients with non-syndromic UCLP who had hard palate repair by the age of 13 years were studied in this cephalometric analysis. A total of 290 lateral skull radiographs were analyzed. The timing of hard palate repair had a significant effect on the length and protrusion of the maxillary alveolus and the antero-posterior jaw relation, measured at 20 years. It must be stressed that the maxillary growth improvement for every year of delay in repair, is small (0.4◦ in SNA, 0.4◦ ANB and 0.2 mm in maxillary length). Such small timing effects can also explain in part why previous studies with small samples or differences in the ages at repair failed to detect the growth difference (Robertson & Jolleys, 1974; Hotz et al., 1978; Friede et al., 1999; Swennen et al., 2002). However, Liao and Mars concluded that the timing of hard palate repair significantly affects the growth of the maxilla in patients with UCLP. Late hard palate repair has a smaller adverse effect than does early hard palate repair on the growth of the maxilla. This is attributable to growth being undisturbed before closure of the hard palate.

FACTORS INFLUENCING INTERPRETATION OF RESULTS FROM THE SRI LANKAN CLEFT LIP AND PALATE PROJECT MALNUTRITION AND GROWTH AND DELAYED ONSET OF PUBERTY It should be recognized that the above studies are derived from subjects in the Developing World. Although Sri Lanka is a relatively advanced Developing Country, there is nevertheless significant malnutrition and endemic infections, for example, malaria.

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The failure of infants with clefts to gain weight adequately has been documented by several authors (Avedian & Ruberg, 1980; Ranalli & Mazaheri, 1975). Malnutrition is a well-recognized form of reversible growth hormone resistance, which can be normalized with nutritional supplements. A malnourished mother is likely to give birth to a baby with low birth weight, while children with protein-energy malnutrition do not grow as well as others according to a recent report (Fernando, 1998). This kind of malnutrition is the underlying cause of almost one-third of the deaths among children under 5 years in Sri Lanka. Malnutrition is still a serious problem in Sri Lanka (Rajapaksha & Siriwardena, 2002). Food insecurity is one of the major reasons for malnutrition in that country according to the Dept. of Census and Statistics. Poor financial and physical access to food is responsible for the malnutrition and food insecurity. Drastic price increases of essential food commodities and stagnating or deteriorating incomes create poor financial access to food. The civil war from 1984 to the present day in Sri Lanka has exacerbated the provision of essential food and has created financial problems. A recent survey of 16,000 Sri Lankan children found that only one quarter were properly nourished (Popham, 2002). More than one-third were suffering from third degree malnutrition, the level beyond which children exhibit distended stomachs and skinny frames. Supporting evidence from the National Peace Council indicated that only 4,863 children under 5 years out of a random sample of 16,767 were within normal nutritional limits. 6,371 children had third-degree malnutrition, 3,186 had second-degree malnutrition and 2,347 had first-degree malnutrition (National Peace Council of Sri Lanka, 1998). According to this report diseases such as malaria cause malnutrition first, which is still prevalent in Sri Lanka. Secondary causes of malnutrition are by worm infestations and thirdly by a lack of food. Many of the subjects in this study were social outcasts, who dropped out of school. Females in particular were hidden away in their houses and only one female in the unoperated population was married (see Chapter 13, Bradbury and Habel). Children need a good emotional climate to thrive. The mechanism of the effects of emotional deprivation on growth is not well documented but is linked to reduced growth hormone secretion and its associated growth failure. It has been recognized in the context of growth studies in general and facial growth studies in particular that many patients in the Developing World have delayed onset of puberty (Mars, 1993). Boys may not attain maturity until after 20 years of age and girls until after 18 years. This has important implications for all studies in Developing Countries and failure to address this issue can seriously confound the result of research. The large volume of longitudinal data has enabled this problem to be addressed (Liao & Mars, 2006).

SPEECH IMPLICATIONS While facial growth in the unoperated subject presents without maxillary retrusion – unlike many operated patients, the speech outcomes for the same series of patients demonstrate almost unintelligible speech for the whole sample.

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Research on the Sri Lankan Cleft Lip and Palate archive has demonstrated that surgery when delayed beyond 8 years of age and even earlier results in permanent irremediable speech disorders (Sell & Grunwell, 1990; Sell, 1992) (see Chapter 15, Sell). This study is careful in not recommending the delay of hard palate repair. Previous studies have consistently demonstrated speech impairment associated with delayed hard palate repair (Bardach et al., 1984; Witzel et al., 1984; Noordhoff et al., 1987; Rohrich et al., 1996; Lohmander-Agerskov, 1998).

CONCLUDING COMMENTS This chapter on facial growth has been made possible by the unique archive which has been collected in Sri Lanka from 1984 to the present day.

REFERENCES Arshad N (1998) ‘Effect of late lip repair on unilateral cleft lip and palate Sri Lankan subjects’, MSc, University of London. Avedian LV & Ruberg RL (1980) ‘Impaired weight gain in cleft palate infants’, Cleft Palate J 17: 24–6. Bardach J, Morris HL & Olin WH (1984) ’Late results of primary veloplasty: the Marburg project’, Plast Reconstr Surg 73: 207–15. Bishara SE, Crause JC, Olin WH, Weston D, Van Ness J & Felling C (1976) ‘Facial and dental relationships in individuals with unoperated clefts of the lip and/or palate’, Cleft Palate J 13: 238–52. Capelozza Filho L, Normando AD & da Silva Filho OG (1996) ‘Isolated influences of cleft lip and palate surgery on facial growth: comparison of operated and unoperated male adults with UCLP’, Cleft Palate Craniofac J 33: 51–6. Enemark H, Bolund S & Jorgensen I (1990) ‘Evaluation of unilateral cleft lip and palate treatment: long term results’, Cleft Palate J 27: 354–61. Fernando R (1998) ‘Prevention of non-communicable diseases – challenges in the next century’, Daily News, 28 February, Associated Newspapers of Ceylon. Friede H, Priede D, Moller M, Maulina I, Lilja J & Barkane B (1999) ‘Comparisons of facial growth in patients with unilateral cleft lip and palate treated by different regimens for two-stage palatal repair’, Scand J Plast Reconstr Surg Hand Surg 33: 73–81. Gillies HD & Fry KW (1921) ‘A new principle in the surgical treatment of congenital cleft palate and its mechanical counterpart’, BMJ 1: 335–8. Herfert O (1958) ‘Fundamental investigations into problems related to cleft palate’, Br J Plast Surg 11: 97–105. Hotz MM, Gnoinski WM, Nussbaumer H & Kistler E (1978) ‘Early maxillary orthopedics in CLP cases: guidelines for surgery’, Cleft Palate J 15: 405–11. Kapucu MR, Gursu KG, Enacar A & Aras S (1996) ‘The effect of cleft lip repair on maxillary morphology in patients with unilateral complete cleft lip and palate’, Plast Reconstr Surg 97: 1371–5. Liao YF, Cole TJ & Mars M (2006) ‘Hard palate repair timing and facial growth in unilateral cleft lip and palate: a longitudinal study’, Cleft Palate Craniofacial J 43: 547–56.

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Liao YF & Mars M (2005a) ‘Long-term effects of lip repair on dentofacial morphology in patients with unilateral cleft lip and palate’, Cleft Palate Craniofac J 42: 526–32. Liao YF & Mars M (2005b) ‘Long-term effects of palate repair on craniofacial morphology in patients with unilateral cleft lip and palate’, Cleft Palate Craniofac J 42: 594–600. Liao YF & Mars M (2005c) ‘Long-term effects of clefts on craniofacial morphology in patients with unilateral cleft lip and palate’, Cleft Palate Craniofacial J 42: 601–9. Liao YF & Mars M (2006) ‘Hard palate repair timing and facial morphology in unilateral cleft lip and palate: before versus after pubertal peak velocity’, Cleft Palate Craniofacial J 43: 259–65. Lohmander-Agerskov A (1998) ‘Speech outcome after cleft palate surgery with the G¨oteborg regimen including delayed hard palate closure’, Scand J Plast Reconstr Surg Hand Surg 32: 63–80. Mars M (1993) ‘The effect of surgery on facial growth and morphology in Sri Lankan UCLP subjects’, PhD thesis, University of London. Mars M, Asher-McDade C, Brattstrom V, Dahl E, McWilliam J, Molsted K et al. (1992) ‘The RPS. A six-centre international study of treatment outcome in patients with clefts of the lip and palate: Part 3 dental arch relationships’, Cleft Palate J 29: 405–8. Mars M, Batra P & Worrell E (2005) ‘The separate effects of lip and palate repair on the dental arch relations and dimensions in Sri Lankan UCLP patients’, paper presented at the International Congress of Cleft Lip and Palate, Durban, South Africa. Mars M & Houston WJ (1990) ‘A preliminary study of facial growth and morphology in unoperated male unilateral cleft lip and palate subjects over 13 years of age’, Cleft Palate J 27: 7–10. Mars M, James DR & Lamabadusuriya SP (1990) ‘The Sri Lankan Cleft Lip and Palate Project: the unoperated cleft lip and palate’, Cleft Palate J 27: 3–6. Mars M & Plint DA (1985) ‘Correlation of the Goslon Yardstick rating and cephalometric analysis’, paper presented at the Fifth International Congress on Cleft Palate and Craniofacial Anomalies, Monte Carlo, Monaco. Mars M, Plint DA, Houston WJ, Bergland O & Semb G (1987) ‘The Goslon Yardstick: a new system of assessing dental arch relationships in children with unilateral clefts of the lip and palate’, Cleft Palate J 24: 314–22. McCance AM, Roberts-Harry D, Sherrif M, Mars M & Houston WJB (1990) ‘A study model analysis of adult unoperated Sri Lankans with unilateral cleft lip and palate’, Cleft Palate J 27: 146–54. McCance AM, Roberts-Harry D, Sherrif M, Mars M & Houston WJ (1993) ‘Sri Lankan cleft lip and palate study model analysis: clefts of the secondary palate’, Cleft Palate Craniofacial J 30: 227–30. Motohashi N, Kuroda T, Capelozza Filho L & de Souza Fritas JA (1994) ‘P-A cephalometric analysis of non-operated adult cleft and palate’, Cleft Palate Craniofacial J 31: 193–200. Muthusamy A (1998) ‘The effect of late primary lip repair on the unoperated UCLP Sri Lankan subjects: a longitudinal dental study model analysis of subjects over 10 years’, MSc thesis, University of London. National Peace Council of Sri Lanka, Asian Human Rights Commission (1998) ‘Sri Lanka: health crisis in the Vanni Districts’, 108 (03–04): 1351. Available at: www.ahrchk.net/ hrsolid/mainfile.Php/198vol108no03-04/1351 Noordhoff MS, Kuo J, Wang F, Huang H & Witzel MA (1987) ‘Development of articulation before delayed hard palate closure in children with cleft palate: a cross-sectional study’, Plast Reconstr Surg 80: 518–24.

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Ortiz-Monasterio F (1959) ‘Cephalometric measurements on adult patients with nonoperated cleft palates’, Plast Reconstr Surg 24: 53–6. Popham P (2002) ‘Forgotten victims of war in Sri Lanka’, Tamil Canadian. Available at: com/eelam/hrights/html/article/Su980722073906N155.html Rajapaksha U & Siriwardena J (2002) ‘Sri Lanka: a hard time’, in UN Development Programme (UNDP) Human Development Report, New York: Oxford University Press. Ranalli DN & Mazaheri M (1975) ‘Height–weight growth of cleft children, birth to six years’, Cleft Palate J 12: 400–4. Robertson NRE & Jolleys A (1974) ‘The timing of hard palate repair’, Scand J Plast Reconstr Surg Hand Surg 8: 49–51. Rohrich RJ, Rowsell AR, Johns DF, Drury MA, Grieg G, Watson DJ et al. (1996) ‘Timing of hard palatal closure: a critical long-term analysis’, Plast Reconstr Surg 98: 236–46. Ross RB (1970) ‘The clinical implications of facial growth in cleft lip and palate’, Cleft Palate J 7: 37–47. Ross RB (1987a) ‘Treatment variables affecting growth in unilateral cleft lip and palate. Part 1: treatment affecting growth’, Cleft Palate J 24: 5–23. Ross RB (1987b) ‘Treatment variables affecting growth in unilateral cleft lip and palate. Part 4: repair of the cleft lip’, Cleft Palate J 24: 45–53. Ross RB (1987c) ‘Treatment variables affecting growth in unilateral cleft lip and palate. Part 7: an overview of treatment and facial growth’, Cleft Palate J 24: 71–7. Schweckendiek W (1978) ‘Primary veloplasty: long term results without maxillary deformity. A 25 year report’, Cleft Palate J 15: 268–74. Sell DA (1992) ‘Speech in Sri Lankan cleft palate subjects with delayed palatoplasty’, PhD thesis, De Montfort University. Sell DA & Grunwell P (1990) ‘Speech results following late palatal surgery in previously unoperated Sri Lankan adolescents with cleft palate’, Cleft Palate J 27: 162–8. Semb G (1991) ‘A study of facial growth in patients with unilateral cleft lip and palate treated by the Oslo CLP Team’, Cleft Palate Craniofacial J 28: 1–21. Shaw WC, Asher-McDade C, Brattstrom V, Dahl E, Mars M, McWilliam J et al. (1992) ‘The RPS. A six-centre international study of treatment outcome in patients with clefts of the lip and palate: Part 1 Principles and study design’, Cleft Palate J 29: 393–7. Smahel Z, Betineova L, Mullerova Z & Skvarilova B (1993) ‘Facial growth and development in unilateral cleft lip and palate from palate surgery up to adulthood’, J Craniofac Genet Dev Biol 13: 57–71. Subtelny JD (1955) ‘Width of the nasopharynx and related anatomic structures in normal and unoperated cleft palate children’, Am J Orthod 41: 889–908. Swennen G, Berten JL, Schliephake H, Treutlein C, Dempf R, Malevez C & De May A (2002) ‘Midfacial morphology in children with unilateral cleft lip and palate treated by different surgical protocols’, Int J Oral Maxillofac Surg 31: 13–22. Will LA (2000) ‘Growth and development in patients with untreated clefts’, Cleft Palate Craniofacial J 37: 523–6. Witzel MA, Salyer KE & Ross RB (1984) ‘Delayed hard palate closure: the philosophy revisited’, Cleft Palate J 21: 263–9.

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Paediatric Care in Developing Countries An integrated (holistic) approach ALBERT C. GOLDBERG AND ALEX HABEL

Editorial comment: This chapter is primarily written for visiting paediatricians with limited cleft experience, though the issues discussed also relate to children with clefts seen on a more continuous basis by local medical specialists. The model of care assumes a holistic approach by the paediatrician (Habel et al., 1996) and deals with the pre-, peri-, and post-operative needs of individuals with a cleft in a resource-poor setting. The chapter ends with a brief discussion of some of the common travel medicine problems that devolve to the paediatrician as team physician.

INTRODUCTION: SPECIFIC FACTORS CONTRIBUTING TO INCREASED MORBIDITY AND MORTALITY NUTRITION Breast milk should always be the recommended initial food for all babies, but for many reasons nutrition may fail. Black et al. (2003) highlight the mortality of nonbreastfed infants in the Developing World. Infants aged 0–5 months have a sevenfold and fivefold increase in death from diarrhoea and pneumonia respectively, compared with breast-fed infants. In the Developing World, difficulties in feeding infants with a cleft palate are likely to result in higher mortality than for non-cleft infants. Factors include the lack of appropriate feeding equipment, clean water needed for clean utensils to express breast milk, and the preparation of artificial feeds. In addition, the economic cost of formula feed, and the pressure to dilute reconstituted milk feeds to make them go further, collectively contribute to an increased mortality rate for children with structural feeding difficulties.

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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DISABILITY A survey in Sri Lanka of 25,000 births identified 1.3/1000 clefts, of which 33% were syndromic. By the time children presented for surgery the proportion with syndromes identified in the Sri Lanka Cleft Lip and Palate Project was 9% (Habel, MD thesis in preparation). The marked predominance of cleft lip only in some series, 89% in a rural Africa series (Hodges & Hodges, 2000), may truly represent the survivors and the resultant cleft type that presents for surgery. Factors involved in this ‘nature’s triage’ of infants unlikely to survive include not only those where sophisticated medical resources are lacking, but also cultural or family attitudes to disability. The latter may be no less caring, as highlighted in a study of the care of cerebral palsy children in Mumbai, India (Yousafzai et al., 2003). Parents perceived the cerebral palsied nutritional needs as substantially less than their able-bodied siblings, and thereby contributed to the disabled children’s malnourished state. Some parents observed that the process of eating was difficult and painful, and when asked, rationalize that feeding would improve once the child was stronger, and deferred increasing food intake on this basis. King and Burgess (1993) noted that in some communities a disabled child may be thought of as sick and be fed inadequately because of the fear of making the illness worse.

PREPARATION FOR A VISIT AND PRE-SURGICAL EVALUATION WITH LOCAL HEALTH WORKER INVOLVEMENT Multidisciplinary discussion must take place between the visiting team and local professionals, covering essential issues relevant to the facilities available. These should include the level of surgery, for example, whether pharyngoplasty is an appropriate procedure, the safe level of post-op anaesthetic/paediatric support that can be offered in cases of Pierre Robin sequence, or peri-operative exacerbation of asthma. Will there be facilities for clinical chemistry analyses, blood transfusion, blood gas analysis, or overnight mechanical ventilation? NOTIFICATION OF HEALTH WORKERS AND PROFESSIONALS THROUGH THE HOST ORGANIZATION OF A PENDING MULTIDISCIPLINARY/SURGICAL TEAM VISIT This can provide an opportunity for setting up pre-surgical evaluation (see next section). Linking up with local services can also pay dividends by avoiding some of the more obvious and painful stumbling blocks. Anticipate, through professional contacts, local medico-political sensitivities and possible needs for equipment and particular drugs, e.g. for known local resistance to anti-malarials and antibiotics. Their knowledge and connections can provide access to local specialists, investigations, acute intensive care/support, additional care and follow-up for problems identified at some

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time during the visit. It also provides a forum for exchange of ideas, clinical teaching and reduces barriers to further cooperation. For these reasons, it is undesirable to work in a professional ‘vacuum’, though in some locations this will be inevitable. CALLING FOR SURGERY Identifying cleft individuals for surgery comes through outpatient visits, the local media, advertising, professional contacts, host charities and non-governmental organizations (NGOs). POTENTIAL FOR PRE-SURGICAL WORK-UP Where feasible, potential recipients’ families should be advised to seek a local doctor/personal physician, or be directed to established links with local medical counterparts from previous visits. The families and physicians can be provided with a list of desirable investigations, for example, pre-surgery physical examination for heart murmurs, or treatment of dental disease which would otherwise delay or compromise surgery, dealing with intercurrent infections, testing for anaemia, and, for example, improving asthma control. This should be combined with opportunistic vaccination. Assessing and providing nutritional support will result in a greater number of children being fit for operation. Those who may be sufficiently fit at a subsequent visit, or included towards the end of the presently planned visit as a result of the early treatment intervention, can be identified. In rural areas this could be a counsel of perfection and unachievable.

THE PAEDIATRICIAN’S ROLE One of the major duties of the paediatrician is to ensure that each child selected for surgery is healthy enough to undergo the procedure. Cleft lip and palate surgeries are elective. Avoiding adverse events and catastrophes are, therefore, essential. The responsibility of the paediatrician is to help select, protect, comfort and provide optimal care for each child. It is important for the paediatrician to understand each phase of care from patient selection to hospital discharge, follow-up, anticipatory guidance of dental, orthodontic care, speech therapy, genetic counselling, and psychological support. This section makes few assumptions about previous experience in the field, as many paediatricians may elect to support a surgical team without necessarily having much surgically related experience. When confronted with desperate parents who view the visiting surgical team as a long-awaited dream to miraculously normalize the appearance of their child, it can be exceptionally difficult to make clear, well-thought-out medical decisions regarding eligibility for surgery. The following steps are recommended.

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PRE-OPERATIVE ASSESSMENT In the case of a visiting expatriate team, it is usual for the host institution or organization to arrange a ‘clinic’ where hundreds of families attend with their children, adolescents, young adults and sometimes older, untreated patients. The number of patients to be selected depends on the number of operating rooms allotted by the hospital for the visiting team’s use, and the number of operating days. Typically a team constitutes a group of two or three surgeons, with a similar number of anaesthesiologists/anaesthetists, operating room (OR) and Pre- and Post-Anaesthetic Care Unit (PACU) nurses. In a fortnight with two or three theatres the team will usually operate on 75–150 patients per visit. RISK ASSESSMENT AND PATIENT SELECTION Absolute contraindications are few, but the utilitarian principle of greatest good for the greatest number is practical where operative slots are limited. Our experience of obtaining maximum beneficial change in life experience suggests that preference should be given by age, with the youngest first. Whether girls should receive preference over boys, as advocated by Weatherley-White et al. (2005), especially in cultures that commonly ascribe greater value to boys, could be poorly received by the recipient community. We advocate an even-handed approach. Contraindications to surgery are relative, and related to the medical problems identified in the assessment. Their nature will determine whether the exclusion is utilitarian, or due to limited medical provision. Examples include long-term moderate/severe physical or learning difficulties, or associated malformations causing an anaesthetic hazard, such as Pierre Robin Sequence (PRS), prematurity with chronic lung damage, or complex cardiac malformations not assessable with available resources. Others are short term, such as correction of significant anaemia with a haemoglobin less than 9 g/dl when a palate repair is proposed. The paediatrician shares responsibility with the anaesthetist for the pre-operative evaluation. The objective of the pre-operative assessment is to assess and reduce the risk of anaesthesia-related and post-operative complications. The following is an outline of the aspects to consider and review prior to ‘clearing’ the child for surgery. PAST MEDICAL HISTORY Obtaining an accurate history may be difficult. Parents are often acutely aware that their child’s medical history may prevent them from getting surgery. A translator should be used whenever anything other than the most basic information is required. A history should include family history and extend to examination of other family members, for example, where lip pits are present as in the autosomal dominantly inherited Van der Woude syndrome. Antenatal teratogens include alcohol, steroids and anticonvulsant use and epilepsy in the mother. Brief genetic counselling can be offered and advice on folic acid through diet or tablet supplementation, according

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to the cultural context. Bleeding in boys, especially after previous surgery, shedding of teeth or dental extraction may indicate haemophilia and require further evaluation before offering surgery. The paediatrician must be persistent in pursuing the child’s developmental milestones, speech development and motor development. The possession of significant speech and language delay in the presence of a more global delay inevitably leads to a discussion of risk–benefits of a surgical procedure. This will depend on the number of operative spaces available, whether a lip only, or the potential of more significant risk of a palate procedure with a definite morbidity and potential mortality. These need to be considered in the context of the cultural expectations, educational, social, employment and marital prospects (see Chapter 13, Bradbury and Habel). This can be an opportunity for discussion of the needs of a disabled child, and appropriate advice on handling and stimulation. Thus, even where the team declines surgery, the family will have derived a better understanding of their child’s needs and some benefit from the visit. Although low birth weight is common, also ask for a history of prematurity, as in those less than 34 weeks’ gestation, chronic lung disease and persistent ductus arteriosus may be present. Parents may not reveal that the child is taking anti-seizure medication, in the mistaken belief that this could prejudice selection for surgery, and run the risk of postoperative seizures if the medicines have been abruptly discontinued. Check for ‘A’ history of Asthma, Allergies to drugs, Anaemia, and previous Anaesthestic experiences, including: 1. 2. 3. 4. 5.

Any severe post-operative nausea and vomiting. Unusually long period to awaken, or paralysis. Drug reactions. High fevers (malignant hyperthermia). Bleeding problems.

CURRENT MEDICATIONS ENQUIRY This includes aspirin or aspirin-like medicines, and other over-the-counter medications. Many ‘cold’ medicines contain steroids, such as prednisolone and dexamethasone. It may be necessary to calculate how much steroid, assess whether hydrocortisone cover is advisable for surgery, and confer with the anaesthetist.

POINTS FROM THE EXAMINATION DYSMORPHIC FEATURES, CARDIAC MURMURS, CHEST AND NECK DEFORMITIES MUST BE FURTHER INVESTIGATED It is helpful to be familiar with the syndromes associated with heart murmurs. Cleft palate is likely in the 22q11 deletion syndrome spectrum that includes the Velo-CardioFacial syndrome. A murmur may be due to rheumatic heart disease, which persists

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in many parts of the world, emphasizing the importance of history, examination and appropriate selection of peri-operative antibiotics. A pulse oximeter should be made available from the anaesthetic team to check oxygen saturations in infants with murmurs. It is desirable to obtain a cardiac consultation if a significant murmur is detected, or consider whether surgery is advisable. Children with cleft palate commonly have chronic noisy breathing, which must be differentiated from obstructive symptoms. Care is required in assessing airway obstructive problems such as Pierre Robin Sequence (PRS) with retrognathia, retropositioned tongue and u-shaped palate. Laryngomalacia, in which the aryepiglottic folds may be sucked into the glottis, or have weak cartilaginous tracheal and bronchial rings, and webs or vascular rings (this raises the suspicion of 22q11 deletion syndrome) may present similarly, and as an aid in differentiation usually lack PRS or Stickler Syndrome facial features. Gastro-oesophageal reflux with spillage of acid into the larynx without aspiration may cause mucosal oedema and stridor quite distinct from that of PRS. Characteristic symptomatic features to cause concern include tracheal tug, intercostal indrawing, or snoring with arousals during sleep. These may be position-dependent and resolve with time and can be safe, whereas those PRS with persistent symptoms are best left unoperated. Always be extremely careful in evaluating the PRS airway as difficulties may not be manifest until attempting anaesthetic induction, when commonly a small anteriorly placed epiglottis may be found. The absence of relatively sophisticated investigations such as mini-laryngo-bronchoscopy (MLB) calls for prudence. In addition, facilities for post-operative nasopharyngeal airway lasting more than a few hours requires greater resources than are generally available in the hospitals many teams will be operating in. Weighing up the ability to manage such problems should be an integral part of assessing the local facilities in the pre-visit phase, visiting the hospital, or at a pre-clinic meeting involving the team and local professionals. The previous experience of the anaesthetist, particularly with placing temporary nasopharyngeal tubes, will play an important part in any decision to include such children in the selected group (see Chapter 5, Hodges and Walker). INTERCURRENT PROBLEMS Examples include gross dental caries needing extractions pre-operatively to prevent infection of the operative site, and temperatures due to causes requiring pre-operative treatment such as otitis media and malaria. An upper respiratory infection, acute or recent, may make the airway hyper-responsive under anaesthesia. This can result in intra- and post-operative complications of bronchospasm, atelectasis, laryngospasm, croup, or pneumonia. Differentiate acute from chronic airway obstruction. Distinguishing between chronic PRS-related symptoms, laryngomalacia and an acute process can be difficult (see above). Use clinical judgement and, though not bacteriologically supported, it is a good plan to give an appropriate antibiotic and have the borderline child return for

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re-evaluation in a few days or in the second week of surgery, or else postpone surgery to another visit. CURRENT NUTRITIONAL STATUS Underweight state and stunting have been reported to be more frequent in individuals with clefts than in the local population in the Philippines (Grippaudo & Kennedy,1999) and Sri Lanka (Habel, 2007), although not in Saudi Arabia (Alkofide & Barakati, 2002). Table 11.1 and the accompanying graphs (Figure 11.1a, b, c) of Sri Lankans operated at all ages from infancy to maturity show how growth was affected. The process may stem from stunting in infancy with inadequate ‘catch-up’ growth after repair or psychosocial factors. Protein, calorie, vitamin and mineral (micronutrient) deficiencies resulting from problematic feeding, intercurrent diarrhoea and malaria all play a part. Wound healing problems and dehiscence of the repair may occur with protein and micronutrient deficiency. It can therefore be rewarding to attend to feeding, if necessary with supervised, clean complementary feeds during the team’s stay. The addition of Vitamin A and zinc supplements makes good clinical sense. Quite often an infant has gained sufficient weight in two weeks to benefit from surgery prior to the departure of the team. Despite the concerns about vertical transmission of HIV in breast milk (about 5% in the first six months), the relative risk of death in Sub-Saharan Africa from bottle feeding compared with breastfeeding is six times greater. Where a mother’s supply is plentiful, expressing directly into the baby’s open mouth has been seen to work. The contraindications to introducing formula feeds for infants at risk of vertical HIV transmission inform us of the relative risk of death for infants with cleft palate from gastrointestinal and respiratory disease, and this is best not initiated except under suitable conditions. Appropriate advice from a Developing Country cleft centre can be accessed from Dr Parakrama Wijekoon, Peradeniya Dental Hospital, Peradeniya, Sri Lanka (see Chapter 14). LABORATORY INVESTIGATIONS All children need pre-operative haemoglobin level testing. Depending on the circumstances of pre-visit preparations, some will arrive with lab work from their local doctor’s office. This may be repeated on the night before the surgery or at the pre-operative clinic, if clinically indicated. Some teams bring their own haemoglobin/haematocrit R (e.g. Haemocu ) portable machines that should be set up and checked to be functioning on arrival, with fully charged spare batteries. Cleft lip repairs involve little blood loss. Palate repairs, however, can have significant blood loss and if a child is borderline (Hb < 10 g/dl or haematocrit < 30), take this into consideration before clearing the patient for the procedure.

−0.87 1.13 2.83 to –3.83

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Mean z score SD range

−0.73 1.15 3.38 to –3.89

−1.8 1.05 1.83 to –4.69

−1.9 1.04 1.83 to –4.48 −1.91 1.08 1.89 to –5.09

−2.01 1.1 1.99 to –4.86

British 1990

−0.59 0.84 2.62 to –2.38

−0.8 0.87 3.1 to –3.59

Sri Lanka

−2.69 1.38 0.65 to–6.22

−2.94 1.27 0.57 to –10.48

US CDC 2000

−2.63 1.18 0.69 to –6.14

−3.07 1.32 0.62 to –8.63

British 1990

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Height

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(a) Height comparison of male Sri Lankans, all clefts, versus local and British population 200 180 160

Height (cms)

140 120

Cleft mean UK mean

100

SL mean

80 60 40 20 0

0

5

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Figure 11.1. Graphs showing persistent impaired growth in (a) height, (b) weight and (c) BMI of individuals with clefts in a Developing Country.

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Figure 11.1. (Continued )

GEOGRAPHICALLY LOCALIZED OR GENETIC CONDITIONS FOR INVESTIGATION The principle applied is that conditions should be identified according to the likelihood of causing complications of the procedure, which are predominantly through anaemia and infection. Examples include Africa/India for HbS and Glucose-6-Phosphate Dehydrogenase (G6PD), the Mediterranean countries, the Middle East, the Indian subcontinent and South-East Asia for thalassaemia and G6PD, and in South America infants with acute symptoms of Chagas’ disease. HOSPITAL ISSUES Upon arrival at a new host hospital, the paediatrician must introduce themselves to the host team members. THE HEAD NURSE One should discuss the team’s needs for medications such as morphine, fentanyl, and midazolam for the anaesthetist or for use in the recovery room. From the PostAnaesthetic Care Unit nurse and anaesthetist obtain an estimate of how much of these drugs are likely to be needed. CHIEF PHARMACIST Examine what is on the shelves of the hospital pharmacy. What antibiotics are available for post-operative wound infections? What is the local resistance pattern of antibiotics, especially against Staphylococcus?

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Most pharmacies have ceftriaxone and at a relatively inexpensive price ($2–6 per gram in 2007 dollars). This drug has the advantage of being effective for at least 24 hours, and may be ideal for this post-operative population. Cleft lip post-operative infections do occur and streptococcal or staphylococcal organisms are usually responsible. If the suture line seems red or there are signs of early cellulitis, give ceftriaxone in optimal 24h doses (50 mg/kg, maximum 2000 mg). Follow these patients closely and do not wait for dehiscence to occur. BLOOD TRANSFUSION SERVICE Although rarely administered because of the non-availability of a safe blood supply, some basic aspects need to be considered. Check with the hospital early in your stay. Does the blood transfusion laboratory check for HIV, Hepatitis B or C, syphilis, and malaria? If in South America, one should also include Chagas’, and Leishmaniasis. It is essential to know the time it takes to type and cross-match a patient in the event of an emergency. If the process is likely to take too long, ensure that type O negative blood can be obtained. In very high risk cleft palate patients, it may be prudent to have one unit of blood in the operating room. The cost of blood ordered, not to mention the waste if not returned, should be remembered and avoided. CHIEF INTENSIVIST Make yourself familiar with the location and facilities of the hospital’s ICU. Ask how to best admit a patient to the ICU if there is a need. Ask about night-time cover and leave your room number and telephone number with the night nurse and house officer so they will be able to reach you in the event of urgency or emergency, such as palatal post-operative bleeding. Have a list of the entire team and their hotel room numbers. HOSPITAL STAFF PAEDIATRICIANS These individuals have a wealth of information about practice styles, local diseases and hospital practices, so make every effort to contact them. LIAISON WITHIN THE TEAM 1. Make a ‘code box’ for the recovery room with the help of the PACU nurse and anaesthetist. 2. Have a current ‘needlestick’ protocol. Ask the chief of the laboratory whether they have a rapid Hepatitis B, C or HIV test kit to perform on a patient if their status needs to be known. 3. Remind all non-medical volunteers, if members of the team, to use universal blood precautions against infection by wearing disposable gloves when handling patients. A graphic analogy is that gloves are the condoms of the hands. 4. Hospital dress protocols. The paediatrician must understand his or her environment. Make the entire team aware of the hospital’s protocols and ensure they are followed, even if they appear irrational.

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IMMEDIATE PRE-OPERATIVE CARE The following are reminders about timely management routines. The patients are usually admitted to the hospital the night before the procedure for better control. NIL BY MOUTH (‘NPO’) GUIDELINES Review this with the team anaesthetist, remembering it is the paediatrician who will be ‘writing’ and often enforcing the orders. Be aware that the daily operating room (OR) schedule is subject to last-minute changes that are often not predictable! The older children and teens are usually scheduled in the latter part of the day, however, they are often ‘bumped up’ if a space appears on the list. Generally, the current guidelines permit clear oral liquid intake up to two hours before procedures in infants. Consult with the anaesthetist about breastfeeding mothers and how long prior to surgery the infant should be kept NPO. Toddlers are often kept NPO eight hours prior to surgery, while older children may be kept NPO ‘after midnight’. These guidelines are flexible, therefore communication with the anaesthetist is important. LABEL THE CHILD Use an indelible pen on the arm if there are no hospital armbands available. An identity band (ID) should have been provided at the initial assessment when allocated for a surgical procedure. If not, write the name, age, weight in kilograms and haemoglobin on the forearm. ENDOCARDITIS PROPHYLAXIS FOR ANY CHILD WITH OTHER THAN A FUNCTIONAL HEART MURMUR Current recommendation in other than high risk cases is amoxycillin, or if penicillin allergy is likely, give clindamycin, orally 1 hour before or intravenously (iv) less than 30 minutes before induction. Dose of amoxycillin orally is < 5 years 750 mg, 5–10 years 1.5 G, > 10 years 3 G, and a third that dose if given iv at the time of induction. Clindamycin oral dose < 5 years is 150 mg, 5–10 years 300 mg, and > 10 years 600 mg, and if iv give half the oral dose slowly over at least 10 minutes. PRE-OPERATIVE SEDATION SHOULD BE DISCUSSED WITH THE ANAESTHETIST Relief of anxiety which occurs in young children as well as adolescents is generally easily achieved with a combination of pharmacology and the usual paediatric ‘tricks’ including play therapy, colouring, video games, and Game Boys. Some teams have non-medical volunteers (e.g. Rotaplast) available who can be of great assistance in playing with and distracting the children waiting in a pre-operative area.

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ANXIOLYTICS Valium given orally the night before surgery is often helpful for the anxious adolescent. Midazolam (Versed) is a useful alternative. The oral dose is 0.5 mg/kg to 0.75 mg/kg mixed with something very sweet, such as concentrated sweetened Kool-Aid powder. Maximum dose is 15 mg. Let the anaesthetist know if you plan to use any of these medications before they are given. PERI-OPERATIVE INTRAVENOUS ANTIBIOTICS These are often given in the operating room and continued afterward. Discuss the protocol with the surgeon and anaesthetist. Cefazolin is a favourite (50–100 mg/kg/day, 8 hourly) or ceftriaxone 50 mg/kg/day as a single dose (maximum dose 2000 mg per day). Cefotaxime is 100–200 mg/kg/day, 6–8 hourly. Vancomycin for penicillin allergy or having received more than a single dose of penicillin in the previous month or suspected methicillin drug resistance, or combined with gentamycin (2 mg/kg at induction) for high risk of endocarditis prophylaxis is 20 mg/kg at induction. Vancomycin administration must be given over 100 minutes to avoid hypotension and the ‘Red Man’ syndrome.

POST-OPERATIVE PAIN MANAGEMENT IMMEDIATELY POST-OPERATIVE Intravenous medication for pain relief is mainly given in the Post-Anaesthetic Care Unit (PACU) or recovery area. Post-operative pain management is consequently the paediatrician’s job when s/he is working in the PACU or recovery area. In some teams, the anaesthetist supervises analgesia administration, necessarily from the operating room and without regular bedside observation, and consequently this may be less than optimal. LATER Once the child is transferred to the ward, acetaminophen (paracetamol) with codeine or acetaminophen alone is usually sufficient for pain control. Post-operative throat pain may be severe in the cleft palate patient, especially if he or she refuses to drink. A dry throat makes the pain worse, therefore encouraging the child or adolescent to drink is extremely important. Do not be timid in your ‘encouragement’, be persistent and show the parent how to give about 5 ml of sugar water every 5 minutes. Opiates have a place especially in the older child and adult, but note that increased sleepiness prolonged iv fluid administration in infants in one study, and delayed hospital discharge due to slower return to feeding than with non-opiates. Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibubrufen, alter platelet aggregation and may increase post-operative bleeding (Desikan & Meena, 2004; Marret et al., 2003).

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Their use is controversial and avoided by some North American surgeons although commonly used in the UK after cleft surgery.

THE PAEDIATRICIAN AS TEAM PHYSICIAN TRAVEL HAZARDS Hazards for travellers to the Developing World exist regardless of the purpose of the trip. Of all deaths suffered abroad, injuries comprise 25%, many times commoner than from infectious disease (Hargarten et al., 1991), though up to 64% of travellers report health problems. Avoidance of motorcycles, small vehicles, unscheduled aircraft and swimming in unfamiliar waters is strongly advised. In a recent study of over 17,000 travellers worldwide who became ill after returning home from the Developing World, a surprisingly large proportion, 8%, had gone for missionary or voluntary purposes (Freedman et al., 2006). Travel destinations are associated with the probability of diagnosing certain diseases, so that diagnoses and empiric therapies can be guided by these differences. Systemic febrile illness should make one consider malaria, dengue, and rickettsial tick-borne spotted fever, the latter almost exclusively from Sub-Saharan Africa, or salmonella infection particularly if returning from South Central Asia. Acute diarrhoea from giardiasis or amoebiasis are commoner than campylobacter or shigella, though viral diarrhoea is commonest of all. Strenuous efforts to avoid insect bites should be advised between dusk and dawn and can reduce the likelihood of malaria, dengue, and Leishmaniasis. Non-diarrhoeal gastroenterological problems including worms, gastritis and acute hepatitis occur commonly, with schistosomiasis predominant when returning from Sub-Saharan Africa. VACCINATIONS AND PROPHYLAXIS Advice on vaccinations and prophylaxis against malaria appropriate for the travel destination can be obtained from the national communicable disease centre, logging on to one of the sites listed at the end of this section. Treatment of acute diarrhoea, of 48 hours duration with ciprofloxacin plus metronidazole is generally accepted, whereas ciprofloxacin for prophylaxis is somewhat contentious but may be suitable for short duration trips from the Developed World where every day lost is of such great importance to the beneficiaries. A policy should be discussed in the planning stages of a mission. NEEDLESTICK INJURIES If on-site testing facilities are inadequate and exposure is likely, a decision on urgent repatriation may be appropriate for some cases of Hepatitis B and Human Immunodeficiency Virus (HIV) (see below). If in doubt, US citizens should contact the US National Clinicians’ Post-Exposure Prophylaxis Hotline ([PEPline] 1-888-448-4911) for advice. In other jurisdictions, local specialists may be contacted.

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HEPATITIS B All personnel should be immunized, but in the event of exposure and negative or vaccine non-responder, early treatment within 72 hours, and preferably 24 hours, should be initiated with one dose of Hepatitis B immunoglobulin plus immunization, repeated in one month. HEPATITIS C No routine post-exposure prophylaxis. As the status of the source is unlikely to be known in the field, proceed as follows on return: 1. 2. 3. 4.

Baseline testing for anti-HCV and ALT (alanine aminotransferase). Follow-up testing for HCV bDNA (quantitative) and ALT at four weeks. If HCV bDNA is negative at four weeks, repeat PCR and ALT at 12 weeks. Seroconversion requires referral to a hepatologist for further evaluation and treatment by their personal physician.

HIV 1. Determine the status of the source, commence treatment immediately if HIV antibody positive (where negative and exposure suspected, the risk of being in the ‘window period’ of HIV infection in the absence of symptoms of acute retroviral syndrome is extremely small, but check for symptoms and signs of AIDS). 2. Post-exposure prophylaxis: four-week regimen of two drugs (zidovudine [ZDV] and lamivudine [3TC]; 3TC and stavudine [d4T]; or didanosine [ddI] and d4T for most HIV exposures. Add a third drug for HIV exposures with an increased risk for transmission (e.g. hollow-bore needle). The injured person must consult with HIV/infectious diseases specialists on return to the country of origin. SELF-PROTECTION FOR THE VISITING PROFESSIONAL When considering whether to take part in a multidisciplinary team, the individual should have cause to pause. In some countries, civil authority may be fragile and subject to indiscriminate violent breakdown. Although cleft surgery is life-changing, it is not life-saving and personal risks should be assessed accordingly. Volunteers sometimes take a vacation after a mission, when they may be vulnerable and without the benefit of a local host to guide them. Government business travel websites give helpful advice.

CONCLUSION The needs of children are best addressed by the multidisciplinary team paediatrician who should be prepared for the specific problems cleft individuals present in that locale. In addition to general physical management, developmental and genetic aspects

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of care deserve to be addressed and may be the only such opportunity for that child and their family.

TRAVEL AND TROPICAL MEDICINE RESOURCES INTERNATIONAL GUIDANCE WHO International travel and health: www.who.int/ith/ NATIONAL GUIDANCE Centers for Disease Control and Prevention: Travelers’ Health (US): www.cdc.gov/travel/index.htm CDC: Malaria page: www.cdc.gov/malaria/ National Travel Health Network and Centre (UK): www.nathnac.org Public Health Canada: Travel medicine program: www.phac-aspc.gc.ca/tmp-pmv/ index.html

APPENDIX PAEDIATRICIAN’S LIST Personal: 1. Sample sizes of soap. Supplies for hand washing are inconsistently present. Bring toilet paper and hand sanitizer gel. 2. Penlight, otoscope, stethoscope, extra batteries. 3. Stickers, safe toys, colouring books, crayons, etc. 4. Textbooks: Harriet Lane Handbook or general paediatric text, hardcopy or palm edition, Smith’s Recognizable Patterns of Human Malformation, 6th edn, British Paediatric Formulary for drug dosages. 5. A ‘Code Card’ or reference for emergencies. 6. Your own personal medical kit. 7. Some organizations require personal provision of a white lab coat and name badge (lightweight and short sleeves if going to warm or tropical climate), scrubs (three pairs), boots, cap and mask. In teams. Some organizations recommend the paediatrician to be personally responsible for adding to team supplies to reduce dependence on items that may not be reliably obtainable locally. 1. Antibiotic samples, Tylenol (paracetamol) with codeine and acetaminophen suppositories for the recovery room. Solicited from hospital dispensaries and drug representatives, they can be packed with the paediatric supplies.

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2. Buretrols for use in the under-10 kg group to avoid fluid overload. These are often in short supply.

RESOURCES AVAILABLE FROM THE AUTHORS The Rotaplast pediatrician’s handbook (includes analgesia and fluid management guidelines), and health advice to volunteers. Available at the Rotaplast website: www.rotaplast.org

REFERENCES Alkofide EA & Barakati SF (2002) ‘Growth status of Saudi patients with cleft lip and palate’, Saudi Med J 23: 823–7. Black RE, Morris SS & Bryce J (2003) ‘Where and why are 10 million children dying every year?’ Lancet 361: 2226–34. Desikan SD & Meena NG (2004) ‘Do non-steroidal anti-inflammatory drugs increase the risk of bleeding after tonsillectomy?’ Arch Dis Child 89: 493–4. Freedman DO, Weld LH, Kozarsky PE, Fisk T, Ronins R, von Sonnenburg F et al. (2006) ‘Spectrum of disease and relation to place of exposure among ill returning travelers’, New Eng J Med 354: 119–39. Grippaudo FR & Kennedy DC (1999) ‘General body growth in children with cleft lip and palate in a developing country’, British J Plast Surg 52: 672–7. Habel A (2007) ‘A case for, and the devising of, a local growth chart in a Developing Country using the LMS Chartmake and LMS Growth Comparator’, Arch Dis Child 92(Suppl. 1): A22. Habel A, Mars M & Sell D (1996) ‘Management of cleft lip and palate’, Arch Disease Child 74: 360–6. Hargarten SW, Baker TD & Guptill K (1991) ‘Overseas fatalities of United States citizen travelers: an analysis of deaths related to international travel’, Ann Emerg Med 20: 622–6. Hodges AM & Hodges SC (2000) ‘A rural cleft project in Uganda’, Br J Plast Surg 53: 7–11. King FS & Burgess A (1993) Nutrition for Developing Countries, Oxford: Oxford Medical Publications. Marret E, Flahault A, Samama C-M & Bonnet, F (2003) ‘Effects of postoperative, nonsteroidal, antiinflammatory drugs on bleeding risk after tonsillectomy: meta-analysis of randomized, controlled trials’, Anesthesiology 98: 1497–502. Weatherley-White RCA, Eiserman W, Beddoe M & Vanderberg R (2005) ‘Perceptions, expectations and reactions to cleft lip and palate surgery in Native Populations: a pilot study in rural India’, Cleft Palate Craniofacial J 42: 560–4. Yousafzai AK, Pagedar S, Wirz S & Filteau S (2003) ‘Beliefs about feeding practices and nutrition for children with disabilities among families in Dharavi, Mumbai’, Int J Rehabil Res 26: 33–41.

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Disability, Culture and Cleft Lip and Palate MARY WICKENDEN

Editorial comment: This chapter provides a very interesting, different and thoughtprovoking perspective on cleft lip and palate both in our everyday work and also in our work in a Developing World context. It offers an overview of issues in disability and rehabilitation internationally, and a theoretical context in which to consider possible cultural influences on the development of services for individuals with cleft lip and palate in the Developing World. It presents us with practical suggestions as to how patients might be helped in community and rural settings through the mechanism of community-based rehabilitation, arguing how teams have a responsibility to link up with these workers.

INTRODUCTION This chapter will give an overview of the ways in which services for disabled people are developing, in the light of some radical shifts in philosophy, concepts and language in disability, in the past two decades. One might dispute whether people with cleft lip and palate can or should be regarded as disabled. The organic impairment of the cleft gives an individual a physical difference which may or may not affect their lives in significant ways. A health professional involved in cleft care would perhaps say that the functional outcome depends on the quality of this care, and on whether the individual has other related conditions such as deafness or learning difficulties, and growth, medical or psychological complications. Of course, the availability and success of intervention at the impairment level will be important, and many aspects of this are covered elsewhere in this book. However, this chapter looks more broadly at the way in which the organic impairment is only part of larger processes which influence the quality of life and status of the person. Looking at disabilities, we know that, in general, the range of impairments which occur in populations is universal, with only fairly minor variations in prevalence globally (Scheer & Groce, 1988). So cleft lip and palate, like autism, cerebral palsy or deafness, for instance, occurs at a Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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Figure 12.1. The WHO International Classification of Functioning (ICF).

relatively stable rate across regions, despite increased risks in some settings, linked to factors such as genetics, peri-natal care, nutrition, or hygiene. However, the broader question is whether having an organic impairment such as cleft lip and palate necessarily leads to disability in individual local contexts. Different societies may have different ways of including or excluding people with disabilities from their social structures. Much of the variation that can be seen in quality of life for people with impairments is mediated not just by the availability, prioritization and types of health-related interventions which address the impairment itself, but also by beliefs and practices which are particular to cultural settings. The latest WHO classification of disablement, the International Classification of Functioning (ICF) (WHO, 2001) described in Figure 12.1, emphasizes a complex interaction of factors, encouraging health professionals to look more widely than just physiological and anatomical aspects. A community’s beliefs about the causes and consequences of ‘being different’ have a huge impact on the impaired person’s life opportunities. This chapter explores the concept of disablement and cultural variation and the impact this may have on availability, prioritization and type of provision and uptake of services.

DEFINITIONS OF DISABILITY AND THE WHO VIEW So, what is disability? The word has various and shifting meanings. It is used in EuroAmerican settings in both general and highly specific ways depending on the context. Many health and education professionals and certainly the general public, in western cultures at least, are likely to use words like disability and handicap interchangeably to refer either to the organic impairment or its various effects on the person. The overriding feature usually being described is a ‘difference’ between this person (who

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can’t see, move, hear, think well, etc.) and so-called ‘normal’ people: ‘There are currently some 5.6 billion different people in the world. Some have a difference called disability’ (UN, 2000: 7). It is not clear that there is necessarily an equivalent word for or concept of disability in all languages and cultures (Zola, 1993). Although WHO has established definitions, which have evolved over time (WHO, 1980, 2001), these are not yet accepted or used globally and are still disputed within both academic and disability movement circles, let alone the variation that exists between and within societies (Scheer & Groce, 1988). The term ‘disability’ has only developed a highly specified meaning since the publication of the first WHO definitions in 1980. The initial classification was an attempt to establish the universal application of the following terms: impairment – the organic problem, disability – the effect of this on the person’s ability to perform a range of activities, and handicap – the effect on the individual’s level of social participation. The aim was to specify and clarify aspects of an individual’s difference from the ‘normal’. Subsequently these definitions have been revised thus: r ‘disablement’ as an overarching term; r ‘impairment’ as before, the physiological or anatomical difference; r ‘activity’, the restriction at a functional level; r ‘participation’, the level of social inclusion or exclusion. (WHO, 2001) The new conceptualization also attempts to recognize the importance of environmental, contextual and personal factors on the person and their life. The resulting disablement is now seen as an interaction between the organic impairment, the restriction in activity and the level of participation of the person in a particular context. Thus, it includes the reactions and responses of those around him as well as his own physical and psychological contribution. So, in theory, an individual with a relatively minor organic difference (impairment) may be very ‘disabled’ if the types of facilities and opportunities open to him/her are limited, e.g. a deaf boy who is not provided with hearing aids, or access to sign language or schooling, and a family who believe they cannot take him out to social events. Conversely, someone with a very severe impairment might be relatively enabled if his/her environment is adapted to this and supportive, e.g. a girl with severe cerebral palsy who has appropriate mobility equipment, an adapted home and school, a suitable communication system and a family who include her in all their social activities. Similarly, a boy with an unrepaired bilateral cleft lip and palate and developmental delay, who despite no surgery or speech and language therapy is attending school, plays in the local football team and is regarded as responsible enough to look after his younger siblings may not be seen as disabled. This terminology has caused some confusion even in ‘developed’ or industrialized countries of the North (Bury, 2000) and has been only partially adopted in ‘Southern’ settings. The WHO (2001) revised International Classification of Functioning (ICF),

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as described above, was a response to criticism that earlier definitions overemphasized individual biological/organic aspects. This version attempts to take account of political, socio-economic and environmental factors. This work was a valiant attempt to establish uniform use of the various terms, but in practice only a limited number of professionals use them in this way. Ingstad and Reynolds Whyte emphasize that the ICF definitions do ‘include a consideration of social contexts’ (1995: 5). The years 1983–92 were designated UN International Decade of the Disabled in an effort to spread positive attitudes towards and development of services for disabled people (UN, 1986) and a UN Convention on the rights of persons with disabilities has now been ratified (UN Enable Convention, 2006). However, arguably the concept of disablement as used by UN/WHO is a western construct, which is often inapplicable or irrelevant in many societies, and the model still takes little account of cultural variation. So although the UN boldly declares: ‘Disability is not a tragedy, it is an inconvenience’ (UN, 2000: 2), this may not be universally true. Disability may indeed be seen as a tragedy in some societies, as an inconvenience in others or may not attract a name at all, depending on the nature of the difference and the context.

ANTHROPOLOGICAL VIEWS OF DISABILITY Anthropologists have described the situation of the disabled person as ‘liminal’ (Murphy, 1987). This means that the person is in a transitional and uncertain state. This process is commonly seen in many cultures and contexts where there is a ‘rite of passage’ from one state of being to another (Van Gennep, 1960). Classic examples occurring in different ways across cultures are the transition from childhood to adulthood, and from being single to married. Liminal states are classically described as commonly causing fear and suspicion in other people (Douglas, 1966), and if disabled people are often seen as liminal, perhaps this accounts for the negative attitudes they encounter. They are regarded as ‘the other’ and therefore as unacceptable and sometimes as less than human. However, in most examples of liminality, this is a temporary state, which the individual passes through on the way to a new situation, for instance, achieving adulthood (Nicolaisen, 1995). The disabled person is not so lucky and is arguably stuck in this transitional ‘no-man’s-land’ with its accompanying stigma. As Goffman (1968) suggests, once a person has been noticed as different, the ‘negative attribution’ or stigma sticks and may spread either to other aspects of the person (e.g. deaf person thought of as stupid) or to their whole family (e.g. marriage prospects of siblings are affected). If a person is regarded as less than human because of their ‘abnormality’, it may then be acceptable to treat them in less than human ways. This theoretical explanation is helpful in understanding why disabled people around the world are often mistreated and denied human rights. Children and adults with unusual facial appearance and unintelligible speech, as a consequence of clefting, may well be faced by marginalization, exclusion and ‘inhumane’ treatment because of this liminalizing process. If we accept the western notion of disability, which includes people with a variety of ‘differences’, classified by WHO into five broad types, then their number is large

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and growing. Initial estimates in the 1980s suggested that about 10% of the world’s population is disabled, though this has subsequently been modified downwards to around 5–6% (Helander, 1993). The figures, of course, vary regionally and are also victim to problems of definition and identification. However, such statistics beg the question, who or what exactly is being counted? Nevertheless, the number of disabled people is increasing, despite improved prevention in most regions (e.g. better perinatal care, immunization, treatment of infection, fewer industrial accidents). The main reason for the increase is the demographic changes in the populations of industrial countries which have increasingly elderly populations. The older you are, the more disabled you are likely to be. However, it is interesting that a disability acquired later in life is perhaps viewed differently from that identified early on. An individual who already has a social identity and status who becomes disabled may be less stigmatized than someone with a congenital impairment (Scheer & Groce, 1988) and indeed one’s social position within society will also make a difference. Someone from a high status family may continue to be well regarded, despite their impairment, the reverse also being true (Ingstad, 1995). So a child born with a cleft palate may not have a chance to establish his humanity before discrimination and stigma develop, and his family’s social position will be highly influential in terms of his resulting disability. Historically, there have always been people who were different, and some authors have looked at views of them in diverse times and cultures. Stiker (1982) argues that the way that societies treat disabled people reveals a great deal about them. He describes the way in which ‘difference’ may be expressed in a number of ways, drawing, for instance, on biological, social, ethico-religious or medical language. However, this literature is fairly sparse. If disability is about ‘being different’ from other people, a discussion about what constitutes ‘normality’ is relevant. Historically, the now dominant western positivist habit of measuring and defining people really only emerged in the late nineteenth century. With the rise of empirical science came the rush to design ways of measuring and labelling diverse aspects of ourselves and each other (e.g. Intelligence Quotients, developmental growth charts, blood pressure, cholesterol levels, etc.). Thus once there is a definition of ‘normal’ some individuals will inevitably fall outside the norm. Certain people then attract a different label and become the ‘other’. In cultures where people are ‘measured’ in different ways, categories will be different and the types of people who are labelled may also vary. Thus, for instance, being fair or dark, fat or thin, smooth-skinned or freckled, physically strong, or good or bad at reading will have different cultural values and consequences. The UN have documented their disability-related activities and changes in attitudes and strategies in the past 50 years (UN, 2000) and provide a useful overview of recent changes in legislation at a global level. In ‘the West’ where the UN and the WHO are based geographically and ideologically, despite their global remit, there is a body of rhetoric about different ‘models’ of disability which to some extent stand in opposition to each other. The two most clearly described and differentiated are the so-called ‘medical’ and ‘social’ models of disability and the differing philosophies underlying these have important implications for the provision of services.

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THE MEDICAL AND SOCIAL MODELS OF DISABILITY The medical (or individual) model focuses on the nature of the impairment itself. It is interested in finding causes for, and eliminating or curing, impairments (Gordon, 1988). It is rooted in a scientific paradigm where ‘normal’ can be measured and defined and where the underlying assumption is that everyone ideally wants to be ‘normal’. This model has difficulty with views which accept or celebrate difference. An example of the latter is the deaf community, some of whom reject cochlear implants. They see sign language as part of their culture and thus if everyone could suddenly be made to hear, ‘deaf culture’ would disappear. The medical model offers prevention of the onset or exacerbation of some impairments, the minimizing of some and of course the relief of pain. It aims to improve people’s function and consequently lessen their disability. However, proponents of this model are often fundamentally uncomfortable with people who remain alive but ‘abnormal’. One notoriously difficult concept is that of ‘quality of life’. Health professionals may find themselves in the difficult position of making judgements about who is ‘normal’ enough to live. This comes into play in relation to pre-natal screening and termination for abnormality and of course the availability of pre-natal identification of clefting and related conditions in technologically advanced regions is relevant here. In some parts of the world, neonatal infanticide for children with impairments is probably still practised and in a sense this also arises out of an impairment-rooted view. Some detractors of the medical model of disability describe this approach as a ‘tragedy’ model, because disabled people are seen as deficient and not complete. Traditionally, this view is also linked to ‘charity’, whereby it is society’s duty to help the person to become whole. One of the key criticisms of this is that it is likely to reinforce the idea of the disabled person as being less than human because of their deficit. Thus, power relations, between them and caring professionals, mean that the disabled people themselves have a relatively weak voice. In contrast, the social model sees the disabled person labelled as ‘different’ as a result of society’s inability to accept diversity and lays the blame on health professionals and scientists for promulgating the idea of abnormality. It takes an overtly political stance and sees the solutions to the stigma and isolation often experienced by disabled people as lying within society rather than in changing the individual. This approach fosters independence and autonomy for disabled people. It regards the whole disability issue as one of human rights. Zola (1993), who probably originated the idea that disability was socially constructed, reminds us that we are all on a path towards disablement as we age, and are therefore only ‘temporarily able’. This is a powerful way of reconceptualizing the generally negative way in which disabled people are viewed and tries to make them seem less different: For many disabled people, rejection is catastrophic, it provokes despair and retreat, which in turn widen the gap and produce further rejection and stereotyping . . . How can human behaviour be changed? . . . make it illegal to discriminate against disabled people and prosecute when it happens? . . . or . . . start by disabled people taking the initiative, by refusing to see themselves as victims. (Coleridge, 1993: 37)

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LINKS WITH TYPES OF SERVICE PROVISION These two models, the medical and the social, inevitably lead to different approaches to provision of services for disabled people. Although called ‘rehabilitation’, this is a misleading term, as for many disabled people it is not a matter of rehabilitating in the sense of recovering something lost. For instance, people with a congenital disability such as cerebral palsy or learning difficulties have never acquired some skills, so it is not a matter of relearning them. For the cleft child, he has never had an intact lip or palate, so he does not know what one feels like. The shape of his mouth is normal to him. Additionally for people with degenerative or severe acquired impairments, recovery of function may be unrealistic. Habilitation is perhaps the aim (although another word altogether might be better), i.e. enabling the person and society to adapt to the differences and to have access to the same experiences as everyone else in society. The medical model, with its essentially ‘curative’ agenda, usually aims to ‘fix the problem’ or at least improve aspects of the impairment itself, e.g. help the person walk, talk, see, appear normal, etc. The focus is on the individual rather than on their environment and it is the patient who is expected to do the changing or be changed. Thus, in relation to clefting, direct interventions such as surgery, orthodontics, and impairmentfocused speech and language therapy for articulation disorders will be key. In contrast, the social model accepts the person as different and sees any ‘intervention’ as being about changing the environment to suit the person. Thus, it would prioritize providing, for example, ramps, auditory loop systems, Braille documents, or a modified school curriculum to improve access to the ‘normal’ world. The social model also emphasizes changes in attitudes of those around the different person, so that they are included in whatever anyone else in that context is doing, e.g. going to work, attending the local school and community events. There is a raging debate between these two approaches and certainly strong proponents of the social model argue that disability is a socio-political issue not a medical one. They see the medical model emphasis on individual differences as being oppressive and demeaning (Barnes & Mercer, 1996; Oliver, 1990). The health professional then stands accused of being part of the problem rather than part of the solution: Where there is not . . . an effective cure and the provision of care threatens to become a curtailing burden on their lifestyles, people with abilities [i.e. professionals] have sought to shape the deviant and vulnerable as much as possible to their own image of normality. (Finkelstein, 1999: 22)

However, this is probably at the extreme of a spectrum of views and many disabled people would opt for a combination of both the improvements in the function that medical intervention may offer and the better access, positive attitudes and inclusion in society that the social model promises (ILO et al., 1994). Wyller (1997) has called the combining of the two models the ‘Comprehensive model’ and it seems a sensible middle ground. In Developing Countries, an approach called community-based rehabilitation (CBR) is commonly adopted (Werner, 1987; Helander et al., 1989). This can be based either on a medical or social model

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framework or encompass both and it varies greatly in how it operates. With a medical model philosophy, it may look very much like an extension of health-based services into local centres, perhaps using volunteers or community workers who have a shorter training period than health professionals. A social model programme would concentrate on promoting inclusive education, employment and income-generation schemes, changing attitudes and increasing community participation. However, there is some criticism of this latter approach and it is sometimes accused of being tokenistic in its rhetoric about empowering disabled people (Miles, 1997). The extent to which these programmes use the WHO ICF classification explicitly to guide their philosophy is variable: Another key element in many visions of CBR is the understanding that disability is essentially a human rights issue. Yet CBR programmes based on the WHO model have generally advocated a medical, technocratic approach, and have completely excluded the social and political dimensions of disability. (Lang, 1999: 134)

In fact, it may be that many individuals and families go through a number of stages in the process of living with disability. Initially there may be a period of seeking out impairment focused help (medical model), followed later by a shift of priorities to more concern about the functioning and inclusion of the person in society (social model). Of course, if intervention at the level of the impairment is not available (e.g. no surgery or speech and language therapy), then social model interventions will be important at an early stage.

LOCAL UNDERSTANDINGS OF DISABILITY In many Developing Countries, the western concept of disability has been adopted to a considerable extent by professionals (e.g. promotion of inclusive education which welcomes disabled children into their local mainstream schools), although there is some evidence that it does not necessarily always mesh comfortably with local beliefs and practices (Kalyanpur, 1996; Miles, 1997). Rao (2001) studied perspectives of Bengali families in Calcutta, when talking about their disabled children. The issue of labelling and language arises. She found that mothers used a variety of different words and phrases to describe their children’s differences and some of these did not reflect the language used by professionals. In a remote part of South Asia, the author encountered a child with a bifid uvular whose grandmother described the boy as ‘having two tongues’. However, the family did not identify a problem with his speech (which was mildly hypernasal), and when the possibility of surgery in a far-off city was suggested, they did not think this was a priority at all. So there is always potential for a clash of ideas and priorities between professionals trained in western-style practice and local people. This may lead to misunderstandings on both sides. Again the issue of what is normal, and what any difference is attributed to, recurs. When considering providing rehabilitation services in diverse societies, Devlieger (1995) points out that ‘the notion that cultural beliefs are only barriers to development

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is changing’ and service planners are beginning to realize that ‘traditional beliefs have to be understood before implementing any kind of CBR’ (1995: 94). For instance, in advising about feeding a cleft baby, it may well be possible to incorporate local practices about infant feeding with some ‘imported’ adaptations. Thus, traditional breastfeeding support or locally available utensils may well be useful. With this increasing awareness of the need to work ‘with not against culture’ (Devlieger, 1995: 94), perhaps more culturally sensitive and appropriate services will emerge which take account of local practices rather than working in contrast to them.

IDENTITY AND STATUS ISSUES IN DISABILITY Issues of identity and status loom large in disability and Murphy supports the view that ‘the disabled occupy the same devalued status as ex-convicts, certain ethnic and racial minorities, and the mentally ill, among others’ (1987: 112). Hahn has dissected the roots of negative attitudes to disabled people and suggests: Two critical values in 20th century Western society that especially influence the treatment of disabled people are personal appearance and individual autonomy. The critical importance of these values is revealed by sources that range from the mundane to the philosophical. Persons who fail to meet prescribed standards of physical attractiveness and functional independence not only are assumed biologically inferior, but they are also exposed to a stigma that depicts them as ‘not quite human’. (1988: 41)

Hahn proposes two components of this negative attitude: firstly an aesthetic anxiety, about the look of people, which results in a tendency to subordinate those who are different or strange. Second, there is an existential anxiety which is linked to a perceived threat of loss of function and a ‘there but for the grace of God go I’ concern. He suggests this kind of fear can ‘even outrank the fear of death, which is after all inevitable’ (1988: 43) and that this essentially involves a sense of personal identification with the position of a disabled person. As this makes the non-disabled person uncomfortable, it would explain the feeling of being avoided by others which many disabled writers describe (Murphy, 1987). Since the late 1980s there has been a rise in the formation of politically active disabled people’s movements. These were mainly started in western countries by groups of articulate, educated, disabled people, but have spread world-wide (e.g. Disabled People International). They lobby at local and national levels for an end to discrimination against disabled people and generally for social model approaches to services, although they are not without their critics (Lang, 1998). They can certainly take credit for the increasing legislation to improve access and prevent disability discrimination in many countries (e.g. the UK, the USA, India, Uganda). A recent success is their lobbying of the UN to ratify a Charter for Disabled Persons’ Rights and this is now being signed. Interestingly, however, these groups are dominated by people with physical or visual impairments, while those with learning, communication or behavioural difficulties find it difficult to be heard (e.g. French, 1993; Shakespeare,

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1996, 2006; Finkelstein, 1999). One might hypothesize that even within the body of disabled people there is some hierarchy of status between different impairment groups. Groce (1985) among others (French, 1993), has pointed out that women with disabilities are in a particularly vulnerable position. Women are at increased risk of becoming disabled especially in Developing Countries and, once disabled, are at increased risk of ill treatment and abuse: The greatest problems faced by women with disabilities are their precarious social, financial and legal positions vis-`a-vis all other members of their societies. For women who are disabled, virtually every expected social role is altered significantly. (Groce, 1985)

Thus, in relation to children with cleft lip and palate, it may be that we should pay particular attention to combating stigma and exclusion for girls and women, who are at risk of double discrimination, particularly if they have poor cosmetic appearance and/or unintelligible speech.

ILLNESS AND DISABILITY In some cultures the concepts of illness and disability are not clearly differentiated. So vocabulary relating to ill and disabled people is combined or overlaps. A child or adult with an impairment may be described as ‘not well’, or as ‘a patient’ suggesting illness as well as difference. Interestingly, the western definition of disability can be contrasted with illness, in that it is often a chronic and incurable, relatively stable state, usually without an active disease process at work. But it might be regarded as a kind of twilight zone between illness and health in some situations (Cassell, 2001). However, the situation of someone who is ill is in some ways clearer, as it is resolved either by cure or death, whereas the disabled person remains in an irreparable state of ‘abnormality’. Importantly the disabled person doesn’t necessarily feel ‘unwell’ or different or in need of ‘curing’ and some more radical definitions of disability don’t actually require the person to have a biological difference. In fact, in the light of contemporary western concepts of disability, it seems strange that disabled people were previously cared for in hospitals, as they were generally not ill. People with physical, learning or sensory impairments are a challenge and an embarrassment to doctors and nurses, because they cannot be cured. Thus, the traditional and expected role of the health professional, as a healer, is frustrated. In the case of cleft lip and palate, the picture may seem initially somewhat more straightforward, as here is a fairly easily identifiable feature to be ‘healed’. This is where misunderstandings and disappointments can occur. If a family’s understanding is that once the ‘hole’ or ‘gap’ in the mouth is repaired by the surgeon, everything will be fine, they may be shocked to find that the child still looks different and still can’t ‘talk properly’. Some aspects of the problem we know are more difficult to change. So impaired facial growth, cognitive, psychological, ENT and communication problems may persist. Here then is a case of confusion between illness and disability in action. There may be a need for more ‘social model’ interventions to help the child and family

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adapt to the long-term situation and ensure social inclusion despite the impairment, rather than continuing to search for a ‘cure’. COMMUNITY-BASED SUPPORT The ICF (WHO, 2001) view of disablement encourages a truly holistic view, looking not just at the physical/organic aspects of the cleft lip and palate but at the effect of this on the person and at their function and inclusion in their particular community. Of course, being holistic is the mantra of all health professionals, but in parts of the world where comprehensive specialist multidisciplinary cleft care is limited, careful thought must inform the planning of services. In countries with largely rural and/or very poor populations, living remote from specialist services, the role of local community disability workers can be crucial. These workers are likely to have a relatively short and non-specialized training and to be working with families in their local area with a wide range of different types of difficulties. A CBR project might offer the following for the family of a cleft child: r identification of the impairment; r advice about feeding and nutrition; r emotional support for parents; r links with other families with disabled children; r advice about early development and stimulation; r screening for other related difficulties; r encouragement to accept and include the child into the wider family and community; r hearing screening or information directing the family to another agency for this; r information about/referral to/accompanying to the specialist centre for possible surgery and specialist advice; r help with reading or translating written information; r financial assistance to fund feeding equipment, hearing aids, travel to specialist centre, surgery and follow-up, therapy expenses, etc.; r information for pre-school and school staff to encourage them to include and accept the child; r work with other children about accepting difference and including all children in community activities; r promoting inclusion in education, employment and social opportunities for all; r promotion of alternative and augmentative communication systems such as signing or picture systems for individuals who need them. It can be seen that many of these roles do not address the impairment (i.e. the cleft) directly but focus on aspects of activity and participation in the local context to improve the life of the person. In many countries and regions these may be useful goals alongside specialist surgical, orthodontic and speech/language intervention. However, in some cases they may be the only support available and arguably all the more important, as the child with a late repair or unrepaired cleft is more at risk of stigma and exclusion than one who has managed to access timely surgery and

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specific advice. Furthermore, the implication is that if there is a shortage of specialist professionals, it is incumbent on the few that there are to spend at least some of their time contributing to the training of community-based workers, so that they can support families locally (Hartley & Wirz, 2002; Wickenden et al., 2001a, 2001b).

CONCLUSION Although we know that broadly the whole range of impairments occurs in all societies, the ways in which these are perceived and how the individual is categorized and treated is influenced by culture and context (Scheer & Groce, 1988). The term disability encompasses a range of situations and effects. It is a concept which originated in a particular context, has evolved over time, and has been exported widely but does not necessarily apply in the same way everywhere. The WHO ICF (2001) classification is an attempt to globalize a particular set of terms, and broaden understanding of difference to include social and environmental effects and responses, as well as the organic impairment. Cleft lip and palate and its functional effects may be regarded as a tragedy in some communities and a minor inconvenience in others. The cleft child may be accepted easily into their community or rejected and stigmatized. There has been a gradually widening view of how services can or should be provided and what they should attempt to do. The medical model continues to provide help with the impairment itself, whereas social models of rehabilitation focus on the practicalities of functioning in everyday life and on social inclusion in society. Clearly, there are some fairly universal processes which often marginalize disabled people, particularly those with physical, learning and communication disabilities. A child or adult with a cleft lip and palate is likely to be negatively affected in these ways and may need help focused on many of the different levels of the ICF disablement model. This approach can often be provided by a combination of specialist medical services and community-based disability work, such as that done by CBR programmes. A comprehensive approach is therefore recommended which involves teamwork and communication between different agencies to take into account local beliefs, practices, and needs at the community level. There is a need for training of non-specialist community-based workers to support disabled people by addressing issues of activity and participation, as well as a need to establish and extend specialist multidisciplinary impairment-focused services for cleft lip and palate.

REFERENCES Barnes C & Mercer G (eds) (1996) Exploring the Divide: Illness and Disability, Leeds: The Disability Press. Bury M (2000) ‘A comment on the ICIDH2’, Disability & Society 15: 1073–7. Cassell FJ (2001) ‘The healer’s art: a new approach to the doctor–patient relationship’, in Helman CG (ed.) Culture, Health and Illness, 4th edn, Oxford: Oxford University Press, pp. 47–83.

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Coleridge P (1993) Disability, Liberation and Development, Oxford: Oxfam. Devlieger P (1995) ‘Why disabled? The cultural understanding of physical disability in an African society’, in Ingstad B & Reynolds Whyte S (eds) Disability and Culture, Berkeley, CA: University of California Press. Douglas M (1966) Purity and Danger: An Analysis of Concepts of Pollution and Taboo, London: Routledge. Finkelstein V (1999) ‘A profession allied to the community: the disabled people’s Trade Union’, in Stone E. (ed.) Disability and Development, Leeds: The Disability Press. French S (1993) ‘Disability, impairment or something in between’, in Swain J, Finkelstein V, et al. (eds) Disabling Barriers – Enabling Environments, London: Sage. Goffman E (1968) Stigma: Notes on the Measurement of Spoiled Identity, Harmondsworth: Penguin. Gordon D (1988) ‘Tenacious assumptions in Western medicine’, in Lock M & Gordon D (eds) Biomedicine Examined, London: Kluwer Academic Publishers. Groce NE (1985) Everyone Here Spoke Sign Language, Cambridge, MA: Harvard University Press. Hahn H (1988) ‘The politics of physical differences: disability and discrimination’, J Social Issues 44: 39–47. Hartley S & Wirz S (2002) ‘Development of a communication disability model and its implications for service delivery in low income countries’, Soc Sci Med 54: 1543–57. Helander E (1993) Prejudice and Dignity: An Introduction to Community-based Rehabilitation, United Nations Development Program Report No E93-III-B.3, New York: UNDP. Helander E, Mendis P & Nelson G (1989) Training Disabled People in the Community, Geneva: WHO. ILO/UNESCO/WHO (1994) Joint Declaration Position Paper on CBR, Geneva: ILO. Ingstad B (1995) ‘Mpho ya Modimo – a gift from God: perspectives on attitudes towards disabled persons’, in Ingstad B & Reynolds Whyte S (eds) Disability and Culture, Berkeley, CA: University of California Press. Ingstad B & Reynolds Whyte S (eds) (1995) Disability and Culture, Berkeley, CA: University of California Press. Kalyanpur M (1996) ‘The influence of Western Special Education on community-based services in India’, Disability Soc 11: 249–70. Lang R (1998) ‘A critique of the Disability Movement’, Asia Pacific Disability Rehabilitation J 9: 4–8. Lang R (1999) ‘Empowerment and CBR? Issues raised by the South Indian experience’, in Stone E (ed.) Disability and Development, Leeds: The Disability Press. Miles M (1997) ‘Disabled learners in South Asia: lessons from the past for educational exporters’, Int J Dis Devel Educ 44(2): 97–104. Murphy R (1987) The Body Silent, New York: Henry Holt. Nicolaisen I (1995) ‘Persons and nonpersons, disability and personhood among the Punan Bah of Central Borneo’, in Ingstad B & Reynolds Whyte S (eds) Disability and Culture, Berkeley, CA: University of California Press. Oliver M (1990) The Politics of Disablement, London: Macmillan. Rao S (2001) ‘“A little inconvenience”: perspectives of Bengali families of children with disabilities on labelling and inclusion’, Disability Soc 16: 531–48. Scheer J & Groce NE (1988) ‘Impairment as a human constant: cross-cultural and historical perspectives on variation’, J Social Issues 44: 23–37.

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Shakespeare T (1996) ‘Disability identity and difference’, in Barnes C & Mercer G (eds) Exploring the Divide: Illness and Disability, Leeds: The Disability Press. Shakespeare T (2006) Disability Rights and Wrongs, London: Routledge. Stiker H-J (1982) Corps infirmes et soci´et´es, Paris: Aubier Montaigne. United Nations (1986) Manual on the Equalisation of Opportunities for Disabled Persons, New York: UN. United Nations (2000) The United Nations and Persons with Disabilities: The First 50 Years, New York: UN. United Nations (2006) Enable Convention on the Rights of Persons with Disabilities http://www.un.org/esa/socdev/enable/rights/convtexte.htm (accessed 20/06/07). Van Gennep A (1960) Rites of Passage, London: Routledge and Kegan Paul. Werner D (1987) Disabled Village Children, London: TALC. WHO (1980) International Classification of Impairments, Disabilities and Handicaps, Geneva: World Health Organisation, pp. 27–9. WHO (2001) International Classification of Functioning: Disability and Health, Geneva: World Health Organisation. Available at: http://www/who/int/classifications/icf/site/ icftemplate/cfm Wickenden M, Hartley S & Kodikara S (2001a) ‘Educating for change: the training programme in Sri Lanka for new professionals working with people with communication disabilities’, Asia Pacific Disability J 12: 88–98. Wickenden M, Hartley S, Kodikara S, Mars M, Sell D, Sirimana T, Wirz S (2001b) ‘Where there is no Speech and Language Therapist: collaborative development of a new course and service in Sri Lanka’, in Proceedings of Conference, Birmingham RCSLT IJDC 2001; 36: Supplement 2001, pp. 315–20. Wyller TB (1997) ‘Disability models in geriatrics: comprehensive rather than competing models should be promoted’, Disability Rehab 19: 480–3. Zola I (1993) ‘Self, identity and the naming question: reflections on the language of disability’, Soc Science Med 36:167–73.

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Psychological and Social Aspects of CL/P in the Developing World, Including Implications of Late Surgery or No Surgery EILEEN BRADBURY AND ALEX HABEL

Editorial comment: This chapter looks at cross-cultural responses to visible difference, life experience in the unoperated cleft, and change in life experience after operation. The results of responses to questionnaires in Uganda and Sri Lanka are discussed. These include beliefs on causation, community attitudes to clefting, access to education, bullying, employment, self-worth, and marriage, and are compared with studies from the Developed World. Interventions that may be transferable and adapted for local use are explored. Some observations on the psychological investigative methods and therapeutic approaches conclude the chapter.

INTRODUCTION: CULTURAL RESPONSES TO THE INDIVIDUALS BORN WITH A CLEFT Throughout history, and between cultures, there has been a consistent response to the child born with a visible facial disfigurement, that of stigma. The ancient Greeks described stigma as the process of deliberate disfigurement of individuals who have transgressed, in order that all could see their guilt. It is associated with shame and social rejection. Birth anomalies have carried a particular significance, signalling the ‘wrath of the Gods’. In many societies, a child born with a congenital disfigurement is seen as a sign that the mother (and/or the society in which she lives) is being punished. ‘Birthmark’ translates as ‘mother’s mark’ in many languages. Shaw (1981) described the way in which in certain cultures the child with a cleft is seen as the manifestation of the wrath of God, or as a changeling creature. There is a complex mythology about the interaction between the devil’s familiar (the hare) and the pregnant woman, resulting in the birth of the ‘hybrid’ child with a cleft. Until Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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the twentieth century, butchers in Norway were forbidden from displaying carcasses of hares in their windows in case a pregnant woman saw them (Reed, 1956) and even today in parts of rural Scandinavia, hares’ faces are covered by some butchers (Bradbury, 1996). Local religious beliefs, myths and superstition demonstrably shape attitudes to cleft. Knowledge of cultural and societal attitudes can aid understanding of the belief systems of patients and families, and thereby avoid insult through ignorance, and also promote education. Of 44 Sri Lankan unoperated cleft adults asked, almost half had no prior concept of causation, the remainder in descending order cited karma (fate), ‘happens at birth’, evil in past lives, evil influences, family history, terrorism, or natural phenomena such as new moon, or flood (Habel, 2003). A rural Indian study of 51 Hindu and 1 Moslem families cited ‘God’s will’ or natural causes (84%), sins in past lives (10%) and only one individual considered genetics as causally related (Weatherley-White et al., 2005), although a number had affected relatives. By contrast, in a Venezuelan group of 38 recently operated individuals, 20 patients said they had no idea, one ‘died at birth and after resuscitation had a cleft’, one cited voodoo, six nutritional (folic acid deficiency, chemicals in foods), two each genetic, family history, pregnancy-related illness, and one depression (Habel, 2003). African belief systems emphasize the role of ancestral spirits in causing clefting, a curse by a sangoma (South African traditional healer), or witchcraft (Dagher & Ross, 2004). The local belief system may shape the way sufferers seek help, for instance from traditional (often religious) healers. Ross and co-workers found within South Africa’s diverse ethnic groups the sangomas and Hindu traditional healers favoured collaboration with western professionals (Dagher & Ross, 2004; Parsot & Ross, 2005). It is logical to reach out to involve them in referring for treatment and as community educators, to dispel negative social attitudes, myths and stereotypes in a culturally sensitive way. According to the WHO, 80% of the Developing World primarily or only use traditional healers and may not seek surgery or other treatments unless approved by them. Willingness to collaborate led to setting up workshops and sharing of relevant knowledge and understanding which could serve as a model for cleft care (Liverpool et al., 2004). Guilt or shame on the part of the family with a cleft member may limit social contacts for the individual and family. Some 10% of Indian children were totally isolated, 26% constrained (Weatherley-White et al., 2005).

UGANDA: SOME EXPERIENCES REPORTED BY INDIVIDUALS WITH CLEFTS There are pressing social and economic problems for families in Uganda. Sub-Saharan Africa is the poorest region on earth, and Uganda’s more recent history of civil war, economic hardship because of internal and external factors, and epidemics such as malaria and HIV-AIDS have all made life more difficult for the Ugandan people.

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The average life expectancy has fallen to 40 years. Childhood is short and often ends prematurely. There is considerable competition for scarce educational resources and it is common in Uganda for children with disabilities to be refused education. Like other societies described above, the stigma of a cleft is an additional burden for the families involved. The data reported are from a pilot study carried out in 2002, when a psychologist (EB) accompanied a group of surgeons and anaesthetists to Uganda. An assessment schedule was developed and administered before and after surgery, using the format of a structured interview with questions and prompts, and given generally through an interpreter. The questionnaire was modified over the course of the assessment period and therefore the results are not amenable to statistical analysis, and are reported as preliminary findings. Some 57 complete interviews took place. These were divided into three age groups: 1. under 10 years (N = 36): parents interviewed, with children contributing as appropriate; 2. between 10 and 20 years (N = 16): patients interviewed, with other family members contributing as appropriate; 3. over 20 years (N = 5): patients interviewed. EARLY RESPONSES TO THE BIRTH One of the more striking findings was the similarities between the responses of parents in Uganda and those of parents in urban centres in the UK (Bradbury & Hewison, 1994). They described the following emotional responses: r Sadness, with feelings of disappointment and loss. r Anxiety and fears for the future of their child’s social well-being. r Fears for their child’s educational prospects and their ability to marry and have children. The one significant area of difference related to the child’s physical survival. These parents reported anxieties with regard to feeding their children and giving them sufficient nutrition to survive. The cleft lip in particular was seen as a major impediment to their child’s ability to feed. One mother described how she had been told her daughter would die because she could not feed, but she expressed breast milk into a spoon for months. They also feared that their children would have other physical problems as well as the cleft. Most parents reported mutual support, and denied any mutual blaming, or blaming of the mother by the father or the father’s family. BELIEFS ABOUT CAUSATION The responses to discussion about the cause of the cleft varied dependent on the region of Uganda, and seemed to be influenced by local cultural and religious factors.

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In Northern Uganda, there was a strong belief that the cleft was caused by the curse of Satan. Babies born with a cleft were not given a family name, but were called ajok (female) or ojok (male), meaning ‘of Satan’. In Eastern Uganda, they responded by saying that it was God’s plan, or else that they did not know. In Western Uganda, they tended to the belief that this was caused by an infection of the womb. RESPONSES OF THE COMMUNITY Most of the parents interviewed described negative responses from their community. They described a range of negative responses from mocking to outright rejection. However, they expressed a belief that surgery would be a way back into the community as this would remove the stigma for the child and family and also for the community. Thus these parents found that the community was on the whole supportive of them in seeking surgery. A minority of parents described a positive response from their community, with considerable support both emotionally and practically. Again, the community encouraged them to seek surgery, and offered advice, financial help and practical support to enable this surgery. GENDER ISSUES The parents were asked to discuss the significance of gender – did the gender of the child have an impact on their responses to the cleft? This varied depending on the region. No parent in Eastern Uganda felt that it was significant. This area has been more influenced by the culture and mores of the developed world, with more European involvement in health services. All the parents in Western Uganda believed that a girl with a cleft would be significantly disadvantaged in relation to education, and in particular to marital prospects. However, in reality, many of the older female prospective patients, those in their mid-teens and above, were married and had children, despite their unrepaired cleft. On further investigation, it was discovered that many of these women were second or third wives, and thus did not have the status of a first wife. In Uganda, one-third of households are polygamous (Watson, 1996). They had generally not received any education, but that needs to be seen in the context of a lack of free education in the past. THE COST OF SURGERY TO THE FAMILY Most of the parents were manual labourers who worked as diggers, labourers, or fishermen. They lived on a meagre income and a hand-to-mouth existence. When they were not working, no money was coming into the family. The journey to the hospital was often a long one. For many, it was the first outside their village, and families tended to travel with other village members who acted as a support for them. They generally travelled by bus, often over several days. The cost was high, given their low income. The family was also obliged to make a financial

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contribution to the operation, which although not large, was a significant financial cost to these families on subsistence income. After arrival, they had to find ways of subsisting while at the hospital. Many reported that other members of their village or clan contributed to the financial costs. They also took care of remaining children and elderly family members. THE ROLE OF SURGERY Parents and prospective older patients were asked what they hoped that surgery would achieve for them. They saw the principal benefit as improved social acceptance within their community. They also described their desire to remove the stigma or ‘curse’ which they believed could be achieved by surgery. They viewed surgery as a way of restoring their status within the community as it signalled economic effort and success in achieving this expensive procedure. The surgery itself was seen as very impressive, and a source of wonder to others, and having achieved it, they expected to have their status increased by association. Parents expected that surgery would allow their children to have access to education. Many of the older children had been turned away from school because of their cleft, or had been driven away by the mocking and taunts of other children. Parents and prospective older patients all hoped that surgery would improve their marriage prospects. LIP VERSUS PALATE REPAIR Parents were asked their thoughts about the relative importance of lip as opposed to palate repair. Only one parent saw them as equally important. All the rest saw lip repair as the most important, because of the social impact of the cleft lip. They believed that lip repair would improve speech because it would allow lip closure. POST-OPERATIVE ASSESSMENT Patients who had previously undergone surgical repair were invited back for postoperative assessment through the radio, and were offered a small amount of money to cover travel costs. Some 47 patients were assessed by the psychologist. The majority were adults, though the age ranged from babies to people in their sixties. Given the numbers and the limited time, it was only possible to have brief discussions. The assessment was based on asking specific questions about actual life changes. Those in Northern Uganda nearly all changed their name from ajok/ojok to a family name, generally the paternal name. This process cost money and they had to travel to a town to do this, but they considered it a very important part of the process of removing the stigma, and felt entitled to do so after surgical repair. Other specific outcome indicators included speaking at clan meetings for the first time, getting married and going to school for the first time.

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COMMENTARY Stigma is not only a way of distancing an individual who may breach the accepted norms for that society, but it also renders the individual powerless to change the designation given. One way to change this victim status is by surgery, which can be a way of ‘healing’ the deficit. The community may actively assist in this effort thereby assuaging the influences that may have ‘caused’ the cleft. The giving of money or other forms of assistance enhance the community and spiritual standing of the donor, similar to the Western perception of charitable giving. In Buddhist beliefs, such acts may also improve the donor’s karma for a future life.

SRI LANKA: SOME EXPERIENCES REPORTED BY INDIVIDUALS WITH CLEFTS A social enquiry of the effect of being born with a cleft in Sri Lanka was conducted over 15 years. The following is a synopsis of the salient observations. EDUCATION Educational opportunities were severely limited in unoperated adults. In Sri Lanka, half had never attended school, others often dropped out through bullying. The earlier surgery was performed, the better the educational prospects, so pre-school surgery resulted in 99% attending school, operation during school age resulted in 93% attending (Table 13.1). School entry was postponed in 28% of those operated at school age. Reasons for school non-attendance were shame (being hidden by the family), rejection by teachers (cleft speech perceived as ‘stupid’, appearance as too disfigured to attend school), speech and/or communication problems (see Chapter 15, Sell), bullying, illness, and socio-economic reasons because the children were required to work at home or in the field. BULLYING Regardless of age at operation, significant bullying or verbal teasing, active or past, was reported by 51% of CL/P of school age – 8% of adults with clefts reported it continued in the neighbourhood or at work. This compared with active bullying reported among those without a cleft as 15–20% of children and 1% of adults. UK Table 13.1. Educational prospects according to age at surgery Age at operation

Number

% Schooling

Pre-school School age Adult

141 68 44

Non-cleft population

313

99 93 54 p 0.001 98

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studies comparing children with clefts with the average population have also found that individuals with clefts were at least twice as likely to say they experienced being bullied (Clinical Standards Advisory Group, 1998; Turner et al., 1998), but a more recent study by Lovegrove and Rumsey (2005) found that such children were not actually bullied more, they were in fact more sensitive and felt more vulnerable. Thus perhaps we should not be too disappointed that earlier intervention in Sri Lankan children did not alter their perception of being bullied. SPEECH Speech outcomes were initially not considered by the individuals and their families as important as appearance, but as time passed they described themselves less satisfied with speech than before. Expectations regarding speech had not been achieved in 44% of the individuals who received palate surgery, some 10 to 15 years after surgery. This reflected the lack of speech therapy, velopharyngeal insufficiency causing hypernasality, and diminishing ability over time to correct cleft speech patterns. Research has shown that indeed from 12 years upwards correction of cleft type speech after palate surgery was rarely achieved (see Chapter 15, Sell). The need to inform families and individuals that realistic expectations for speech outcomes are linked to age at surgery is self-evident. SELF-PERCEPTION 1. Appearance. Self-appraisal of appearance was divided into dissatisfaction with lip and/or nose, and dental appearance. The latter was complicated by poor dental hygiene, with gross caries common as a result of the high rice and cane sugar content of the diet. Dental arch deformity due to a lack of alveolar bone grafting was common. Together, they constituted a significant factor in long-term dissatisfaction in 34%, ranging from 56% in BCLP to 2% in CP. In comparison, 18% of the general population sample expressed similar dissatisfaction with facial appearance and dentition. Giving information about the limitations of primary surgery in correcting these deformities could have been helpful. Dental bridges and orthodontic appliances were beyond the financial means of the families in our study, in common with most people in the Developing World. Offering primary dental alveoloplasty at the time of primary repair would be a significant step forward if surgical time and expertise allowed. 2. Coming to terms with disability. When asked, 45% wanted some improvement of speech or appearance, while 26% of those dissatisfied said that they would decline an intervention to improve appearance or speech even if it were offered, suggesting some coming to terms with disability over time. This is similar to reports in the Developed World that both sexes are equally concerned about appearance, and from childhood to late adolescence become less disappointed with their appearance (Broder et al., 1992), although continuing concerns and anxiety about appearance, dentition and speech, and desire for further treatment are common in adults (Ramstad et al., 1995).

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M F

Number of adults at operation

% Employed prior to operation

Number operated and followed up to adulthood

% Employed after surgery

p value

25 19

44 16

37 41

92 53

<0.001 <0.01

Note : Adults at surgery (44) and those operated in childhood followed up to adulthood (78).

EMPLOYMENT Employment prospects in unoperated adults with a cleft were poor. Among 11 men five were employed, and one in six women found employment. Post-operative follow-up from childhood of 78 individuals with clefts into adult life revealed the range of employment reflected the opportunities of the general population of Sri Lanka as a whole, of whom 5% go to university, 80% have manual or semi-skilled jobs, and 15–20% of men are unemployed (Table 13.2). Data for women were not available. Among 40 operated men, 34 (92%) were in employment or training, including two men with repaired bilateral cleft lip and palate who had graduated from university, one as a doctor, and one who became a private secretary to a Member of Parliament. Others were in manual jobs including labouring, farming, semi-skilled garment and gem trades, in common with the majority of the population. Among 37 operated women, 17 (46%) were employed outside the home, usually in the garment industry, probably reflecting the increasing acceptance of women as wage earners. Some, even as single women, worked and lived away from home. PERCEPTION OF SOCIAL EASE AND QUALITY OF LIFE The type of cleft influenced the experience and perceptions of the individual. The most successful group of men socially and in marrying were those with cleft lip, closely followed by unilateral cleft lip and palate. Women with cleft palate were more successful socially and equally successful in marrying. Least successful were those with bilateral cleft lip and palate. Children operated on in early childhood have achieved success in local and national cultural festivals for singing and dancing, and one plays chess for his province. MARRIAGE Marriage in 20 unoperated adults was comparatively unusual, comprising only two men, one woman. After surgery, the rate increased, for both sexes to 20 of 77 (26%) of those eligible. Although this might at first glance appear to lessen the economic burden of an unmarried son or daughter, the downside was that in rural Sri Lankan life a partner might only be found through a marriage broker. A costly dowry was occasionally

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required, causing fresh despair for the family. In addition, in the countryside younger siblings often did not marry until the elder had been found a partner. Interestingly, individuals with clefts tended not to marry each other; indeed a minority of non-cleft partners were disabled or disfigured. On one occasion, caste was an impediment even when a partner had been found. Fortunately for urban families ‘love matches’ in which young couples met at work, or through introductions by friends, were breaking down more traditional arrangements. In northern Europeans, Ramstad et al. (1995) also showed that those with clefts, and especially with bilateral cleft lip and palate, were less likely to marry compared with the average. MENTAL HEALTH Suicidal feelings were sometimes shared with the team and self-harm intent was revealed by four individuals. The rate of completed suicide can only be conjecture, but, based on the Dutch study that found rates 1.5 times the general population in operated clefts for both men and women, it is likely that at least the same or higher would be likely in the unoperated. SURGICAL PRIORITIZATION? Limited resources may cause clinicians to ponder who should have priority. The pre-school child is likely to gain most, with improved nutrition, better expectation of school attendance and adult life. However, even as an adult, the chances of finding a spouse and work are significantly higher than for the unoperated individual with a cleft. Girls are likely to benefit by marriage which would otherwise be denied them, and it has been suggested they receive priority (Weatherley-White et al., 2005). We would counsel an even-handed approach as the local community may be perturbed if boys appear discriminated against, and, in our study, the gender differences for post-surgery education, employment and marriage were not significantly different from their pre-operative proportions, although numbers for some groups were small. Parents in the United States considered speech of secondary importance for girls, though not boys (Broder et al., 1992), although no gender difference was found in Sri Lanka. One of the problems in the Developing World is the likelihood of lack of availability of speech and language therapy or secondary surgery for speech. The proportion of those with good speech and facial appearance is thus likely to be less than in the Developed World, with its greater availability of and access to resources, but, as our study has shown, primary surgery alone is enough to make an enormous difference to people’s life experience.

SUMMARY 1. Education prospects were significantly improved by early surgery. Surgery in the pre-school years was the most beneficial. The longer the delay, the shorter the

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duration of schooling, and half of unoperated adults failed to have any schooling whatsoever. Bullying was perceived as commoner among individuals with clefts. Early surgery did not alter the proportion who reported it. Although social ‘success’ was statistically less than in the non-cleft population, the ability to function socially as reflected in marriage and economic contribution was not significantly different from the general population. Without surgery, the opportunities for employment were significantly less. Marriage in the unoperated was half the proportion in the operated.

COMMON THEMES Before considering ways of assessing the psychological impact of cleft surgery in the Developing World, it is necessary to draw out those psychological, social and cultural issues which are common to those undergoing surgery and their families. Of course there are differences between cultures, but there are also similarities which will form the basis of any further investigations. The following issues arose in both studies, and from the published literature: r r r r r r r r r

parental responses to the birth; responses of the community to the birth; access to education; access to employment; access to marriage status; participation in the local community; self-perception; external evaluation: the impact of bullying; prioritization of surgery: speech or appearance?

ASSESSMENT: HOW TO ASK THE QUESTIONS AND WHAT TO DO WITH THE ANSWERS In order to understand the psychological and social impact of the cleft in the Developing World, and thus make sense of the impact of surgery, it is essential that assessment before and after surgery is carried out in accordance with good practice, both in the formulation of the questions and the manner in which they are asked. FACTORS IN ASSESSMENT r Timing of the assessment. This should be carried out after the decision has been made about whether to proceed with surgery. It should be clear to the individual/family that the assessment has no bearing on the surgery, and that the surgeon will not see the individual responses.

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r Who should be present? Only those immediately involved should be present. Too many family members could make it difficult, for example, for the mother to speak freely. Children should always have a parent present, although adolescents should be assessed alone where possible. r Finding an interpreter. In this sensitive area it is important to ensure that the communication is clear. This requires the assessor to find a suitable interpreter, unless the individual being assessed has an extremely good command of the language used by the assessor. For example, an imperfect grasp of English may cause misunderstandings, especially where the cultural background is not well understood. Where there are several tribal languages, you may need several interpreters. The interpreter should be familiar with the culture of the individual being assessed. r Instructions to the interpreter. The interpreter needs to understand that you require a verbatim interpretation. Many will be inclined to re-interpret what is being said by either side in order to explain further, act as censor or put forward their own version. r The assessment environment. In a busy hospital it can be very difficult to find a quiet place to talk. However, information gained in a corridor with others around and listening will be of limited value for assessment purposes, and violates the privacy of the individual who is giving sensitive information. It is worth ensuring that those with control over space know why this assessment is happening, recognize its importance and find the necessary space. Ensure this is arranged in good time. r Explain the purpose of the assessment. Tell the individual why you are asking these questions and what you are going to do with the results (e.g. ‘we need to understand what it is like for you/your child to have a cleft’). r Gaining consent. Do not proceed with the assessment if the individual is reluctant and does not want to participate. You may wish to obtain written consent. r Eliciting information. The assessors should be calm and relaxed, adopt a relaxed and friendly open posture, and be encouraging of the individual to talk. They should not show any reaction to what is being said, but maintain a friendly and neutral demeanour. r Keeping notes. It will be easiest to have the main questions printed out beforehand, with tick boxes where appropriate and space for comments. FORMULATING THE QUESTIONNAIRE Ensure that you gather relevant demographic data such as: type of cleft, age, gender, employment/school situation, marital situation, family structure, family occupation. It is also useful to ask whether they have consulted anyone else before (such as another doctor or a healer) and what was the outcome (see Dagher & Ross, 2004). You then need to construct your questions, bearing in mind the following broad guidelines: r Keep them relevant (face validity). It should be clear to the individual/family why a question is being asked. For example, ask ‘Has the cleft made it difficult for your child to get to school?’, do not ask ‘What is happening with your child’s education?’. That is too vague. Keep the questions closely tied to the impact of the cleft.

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170 MANAGEMENT OF CLEFT LIP AND PALATE r Make them open-ended. Do not assume anything, especially that you assume the negative effect of the cleft. Do not ask ‘What problems do you have at school because of the cleft?’ Ask ‘Do you have problems at school because of the cleft?’ Maybe school is fine. r Keep it simple. The simpler the question, the less scope there is for misunderstanding. Where appropriate, you can have an in-depth discussion to gain more information, but the further you move from the original question, the harder it will be to quantify the responses and compare results. Always ask the main questions before moving on to discussion. That ensures that you have the basic data. r Questions and prompts. Prepare a list of relevant open-ended questions, with each question followed by prompts. For example: Do you have problems at school because of the cleft? – yes/no/not sure Initial prompts (if yes): what are they? If you are bullied, who bullies, children or teachers or both? Does it happen a little, sometimes, all the time? Second prompt: And how does that make you feel? Third prompt: Does that have an effect on your education? Fourth prompt: What is the effect? With this structure, you will have a simple score (yes/no/not sure) and a list of relevant factors with further information in the form of both quantitative (who bullies) and qualitative (how does that make you feel) data. MAKING SENSE OF THE RESULTS Once you have collected the data, you will need to analyse them in a way which is helpful for your ultimate purpose, that of understanding the psychological and social impact of the cleft, and the psychosocial outcome of surgery to repair the cleft. There may well be other subsidiary questions you would like to investigate, such as whether there are gender differences and whether the psychosocial outcome of surgery is affected by the age at the time of surgery. However, it is likely that your data will be limited because of language constrictions and cultural differences which you do not fully understand. As an outsider, the best advice is to keep the questions simple and do not be over-ambitious in analysing the data. The results will be descriptive rather than predictive. The only way to overcome these limitations is to work with local psychologists/social workers and/or researchers who are familiar with the local language and culture. This is also the most satisfactory way of furthering research in this area of cleft surgery. Further information about assessment tools in this context can be accessed via the website: www.eileenbradbury.co.uk.

PSYCHOLOGICAL INTERVENTION IN BULLYING In the context of the diverse social, cultural and religious communities in which cleft surgery in the Developing World takes place, it would be foolish to be prescriptive

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about psychological intervention. However, as the Sri Lanka study has shown, teasing and bullying can continue even after surgical repair, and those who have had later repair may be entrenched in low self-esteem and social exclusion because of the years they have spent trying to cope with an unrepaired cleft. Support and advice with regard to managing psychological and social difficulties, and developing links with local services, could be an important and integral part of the work of the visiting cleft team. Further information and advice about work in this area can be accessed through the Association for Child and Adolescent Mental Health (www.acamh.org.uk).

REFERENCES Bradbury ET (1996) Counselling People with Disfigurement, Leicester: BPS Books. Bradbury ET & Hewison J (1994) ‘Early parental responses to visible congenital disfigurement’, Child Care Health and Development 20: 251–66. Broder HL, Smith FB & Strauss RP (1992) ‘Habilitation of patients with clefts: parent and child ratings of satisfaction with appearance and speech’, Cleft Palate Craniofacial J 29: 262–7. Clinical Standards Advisory Group (1998) Report on Cleft Lip and/or Palate, London: The Stationery Office. Dagher D & Ross E (2004) ‘Approaches of South African traditional healers regarding the treatment of cleft lip and palate’, Cleft Palate Craniofacial J 41: 461–9. Habel A (2003) ‘Rotaplast mission to Carupano Venezuela’, unpublished report. Habel A, Mars M & Sell D (2002) ‘Change in life experience after primary cleft lip and palate surgery in a developing country’, paper presented at RCPCH Spring Meeting, Archives Dis Child Supplement 1. 86: A34. Liverpool J, Alexander R, Johnson M, Ebba-Ebba K, Francis S & Liverpool C (2004) ‘Western medicine and traditional healers: partners in the fight against HIV/AIDS’, J Natl Med Assoc 96: 822–5. Lovegrove E & Rumsey N (2005) ‘Ignoring it doesn’t make it stop: adolescents, appearance and bullying’, Cleft Palate Craniofacial J 42: 33–44. Parsot S & Ross E (2005) ‘Eastern medicine: beliefs and practices of South African Indian Hindu traditional healers with regard to cleft lip and palate’, paper presented at 10th International Congress on Cleft Palate and Related Craniofacial Anomalies, 4–8 September, Durban, South Africa. Ramstad T, Ottem E & Shaw WC (1995) ‘Psychosocial adjustment in Norwegian adults who had undergone standardised treatment of complete cleft lip and palate. II. Self-reported problems and concerns with appearance’, Scand J Plast Reconstr Surg Hand Surg 29: 329–36. Reed SC (1956) Counselling in Medical Genetics, Philadelphia, PA: Saunders. Shaw WC (1981) ‘Folklore surrounding facial deformity and the origins of facial prejudice’, Br J Plast Surg 34: 237–46. Turner SR, Rumsey N & Sandy JR (1998) ‘Review of psychological aspects of cleft lip and palate’, Eur J Orthodontics 20: 407–15. Watson CE (1996) Children First, Kampala: UNICEF, Government of Uganda. Weatherley-White RCA, Eiserman W, Beddoe M & Vanderberg R (2005) ‘Perceptions, expectations and reactions to cleft lip and palate surgery in native populations: a pilot study in rural India’, Cleft Palate Craniofacial J 42: 560–4.

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The Background, Establishment and Function of a Parents/Patients Support Group in Sri Lanka PARAKRAMA WIJEKOON

Editorial comment: The author presents the creation, aims and working process of a truly rare parents and patients support group in a Developing Country. That two hundred people attended the inaugural meeting was a surprise both to him and his British colleagues, demonstrating their erroneous and misplaced pessimism in the possibility of creating a parent/patient-run support group in a Developing Country. More recently, two further groups have been established, based in Colombo and Galle.

INTRODUCTION Cleft lip and palate is a complex condition, which requires the involvement of many specialists for its management. In a Developed Country, as soon as a cleft is diagnosed, often ante-natally, the cleft team is informed and initial counselling is started. The treatment continues through to adulthood. It has been shown that parent support groups can play a major role in the well-being of these patients. In the United Kingdom, CLAPA (the Cleft Lip and Palate Association: www.clapa.com) is one such parent support group, which plays a major role in the management of cleft lip and palate. Sri Lanka is a small island situated near the southern tip of India. It is approximately 62,500 sq. kilometres in size with a population of about 20 million. The literacy rate is around 85%, which is very high in the region. The prevalence of cleft lip and palate in Sri Lanka is about 1/700, which is similar to European populations. The birth rate is about 1,000 births per day, which gives around 350–400 new cleft cases a year. The health-care system in Sri Lanka is well established and people enjoy a free National Health Service based on the UK model but with considerably reduced resources. The government owns around 95% of the hospitals. Before 1998, cleft lip and palate patients were managed by plastic, maxillofacial and ENT surgeons performing Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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without team involvement. The concept of a team approach was not the practice before 1998. There was only one speech and language therapist for the whole country! In 1999, a Cleft Lip and Palate Project in Peradeniya, Kandy (see Chapter 9, Lamabadusuriya and Mars) was started with the main objective of improving the quality of cleft care. As there were inadequate funds for this project, CLAPA UK came forward to help us. The team concept was introduced by the Great Ormond Street Hospital (GOSH) multidisciplinary team. They played a major part in the creation and training of an equivalent team in Peradeniya, Sri Lanka. In this project the cleft team at Peradeniya was given short-term training on three visits in Peradeniya, Kandy, and in the United Kingdom in this project. I was selected for a one-year comprehensive training in the management of cleft lip and palate. This included primary surgery, alveolar bone grafting, osteotomy and speech therapy within a multidisciplinary team approach. Importantly, I was introduced to the workings of CLAPA UK. The visiting team was instrumental in establishing a long-felt need for a speech and language therapy training programme in Sri Lanka. Dr Michael Mars and Dr Debbie Sell with a group from GOSH and the Institute of Child Health were the pillars behind this work. They were involved in organizing both the course and the financial support for the infrastructure. This training programme was started as a diploma course attached to the University of Kaleniya Colombo, and it has now been upgraded to a degree programme (see Chapter 16, Sell). In addition, the speech and language therapist at Peradeniya was given a one-month training programme in the Department of Speech and Language Therapy at Great Ormond Street Hospital, London. During my stay in the UK, I was impressed by the role played by CLAPA. This included mutual support between patients, parents and professionals, fund-raising methods, utilization of the media and the Internet. Increase in public awareness was enhanced by social events. I came to know that CLAPA had started as a small association in 1979 and now it has many branch associations throughout the UK. I thought that a similar association in Sri Lanka was needed. As a result of several discussions with Dr Michael Mars (founder of CLAPA UK), I was determined to start a similar parent support group in Sri Lanka. On returning to Sri Lanka in 2001, I discussed with parents and patients the need for and benefits of such associations. There was tremendous interest and enthusiasm. The first Cleft Lip and Palate Parent Association in Sri Lanka was formed in 2003 at Peradeniya. Over two hundred parents came with their children from all over the country to attend this inaugural meeting (Figure 14.1). ‘Sanyatha’ is the name of the association which means ‘joined strongly together to achieve a worthy purpose’. We have a unique logo, which was designed by one of our parents (Figure 14.2). The central flower symbolizes the child and the torn petal the cleft. Two large hands protecting the flower symbolize the parents. The concept is a cleft child is well protected by the parents. Our motto is ‘Uththanena Nappa Madana’, a quote from Lord Bhuddha’s teaching, which means ‘Be courageous and stand on your own feet without delay.’

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Figure 14.1. Parents at the inaugural meeting of the Sanyatha group.

Figure 14.2. The Sanyatha logo.

AIMS OF THE ASSOCIATION The main objectives of the association are: 1. To provide financial, moral and psychological support for patients and their parents. 2. To increase awareness of cleft lip and palate and possible treatment modalities among the patients, their parents and the public. 3. To organize social activities for cleft patients and their families, and raise funds for patient care. We were able to organize parent-to-parent support within the membership. When a new patient with a cleft is brought to our clinic, we arrange for a Sanyatha member who lives close to that family to meet them. We find that this is a very effective way of educating the family of the new patient about the condition and its management. We have observed a marked improvement in parents’ attitudes and understanding in the subsequent visits. It is generally considered that literature on cleft lip and palate either in printed or in electronic form is very important for patients, their parents and health-care workers.

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In a Developed Country like the UK, there are vast amounts of literature available relating to cleft care. The situation was quite different in Sri Lanka. Prior to the setting up of Sanyatha, no printed literature was available on cleft care. Access to electronic forms of information via the Internet is limited. The majority of people speak Sinhalese, many use Tamil and very few use English. Considering all the above factors, we were able to produce a comprehensive booklet on cleft lip and palate in all three languages. This was the first booklet on clefting in our country. The booklet is distributed free of charge upon registration in our clinic and copies are given to all the general, base and peripheral hospitals. To increase awareness, our team members have been on television as well as a radio programme to spread the news, and we have received a large response from the public. Our website, Cleft Care, was launched in 2004 and since then, has been visited by more than ten thousand visitors locally as well as internationally. It allows free download of our cleft booklet in all three languages. Sanyatha will develop its organization to maintain the aims listed above. In addition, it has plans to organize variety entertainment shows and cleft camps for the patients. In 2005, CLAPA UK celebrated its Silver Jubilee. Coinciding with that, Sanyatha was recognized as CLAPA’s first overseas branch. This development is an unusual example of how a structure created in the Developed World has been exported and functions well in the Developing World.

ACKNOWLEDGEMENTS We would like to acknowledge the considerable financial contribution of The Smile Train to both the establishment of Sanyatha and the support to our hospital.

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Speech in the Unoperated or Late Operated Cleft Lip and Palate Patient DEBBIE SELL

Editorial comment: This chapter provides an overview of speech outcomes in the late operated patient. The author points out some of the problems in studies in the literature, and illustrates some of the methodological approaches required in these types of investigations using the Sri Lankan Cleft Lip and Palate Project as an example. The chapter will provide evidence to inform decisions about palatal surgery particularly where resources are limited.

INTRODUCTION For more than half a century, it has been common practice for surgical teams from the Developed World to undertake visits to Developing Countries in order to perform surgery on unoperated patients (Law & Fulton 1959; Keunen 1966; Boo-Chai 1971; Lamabadusuriya et al., 1988; Mars et al., 1990; Yeow et al., 2002; Reeve et al., 2004). Teams sponsored by charity organizations are commonly involved in such projects. Interplast, Rotaplast, Operation Smile, The Smile Train, Transforming Faces are all examples of the better known ones. This approach is especially important given that 80% of the world’s population have no access to elective surgical care (Mahler, 1981). Large groups of unoperated patients, of all ages, with different extents of cleft, varying levels of education and social inclusion or exclusion, are still found in the Developing World. Nagarajan (2006) estimates that in India alone there are 35,000 children born with CLP every year, with an estimated one million individuals who are unoperated. Moreover, there are many more needing revisionary surgery and rehabilitation.

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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HISTORY OF THE INVOLVEMENT OF THE SPEECH AND LANGUAGE THERAPY PROFESSION IN DEVELOPING WORLD VISITS Typically in the earliest of these surgical visits, there were no speech and language therapists in the team. The focus was on ensuring the largest number of operations, in Dupuis’s words ‘the body count’ (Dupuis, 2004), and the transfer of surgical skills (Natsume, 1998). The International Task Force on Volunteer Missions (Yeow et al., 2002) reported that only 3 of the 120 institutions regularly visited included a speech and language therapist. Two quotes are rather telling, written a decade apart. D’Antonio, in 1990, wrote: Few efforts have included attention to associated rehabilitation areas such as otologic care and speech treatment. Most of us reading these comments would not think of providing such unidimensional services for our cleft patients at home. Why are we allowing ourselves to accept this level of care abroad?

Ten years on, the literature did not reflect much change from this position. Zbar et al. (2000) wrote, ‘Successful cleft management requires a multidisciplinary, long-term team approach. To send a cleft surgeon to a remote region of the world without consideration of a genetic, dental, speech or hearing evaluation of the patient population is perhaps irresponsible.’ Fortunately, in recent times, the need to provide long-term care in the form of a sustainable local multidisciplinary team has been more widely recognized and changes to the composition of the visiting teams have been implemented (Mars et al., 1990; Ruiz-Razura et al., 2000; Zbar et al., 2000; Hodges & Goodacre 2002; Yeow et al., 2002; Abenavoli 2005; Interplast; Operation Smile).

NEED FOR SPEECH AND LANGUAGE THERAPY FOR SERVICE DELIVERY, TRAINING AND RESEARCH There are many reasons why speech and language therapy should be involved. First, speech and language therapy coverage is very limited in the Developing World where approximately 80% of the world’s population live (Ruiz-Razura et al., 2000; Yeow et al., 2002; Bleile et al., 2006; Prathanee et al., 2006). Prathanee et al. (2006) list a lack of services in Thailand (40 speech and language therapists for a population of 63 million), and in other developing areas of Asia, including Vietnam, Indonesia, India, Laos People’s Democratic Republic, Burma and China. Yeow et al. (2002) attributed this to socio-economic factors that dictate spending on acute health problems and disease prevention. Indeed, interestingly, Ruis-Razura et al. (2000) attributed omission of speech and language therapy in their overseas visits to the fact that there were no such available follow-up services. It could be argued that this is the very reason to include the

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profession in the overseas team. Another very good reason for inclusion is in order to build the evidence base. Although it is well recognized that the primary reason for palate repair is to facilitate normal speech development (McWilliams et al., 1990), there have been very few studies on the effects of late palate repair on speech, after speech has developed. Indeed, Canady (2001) states that there are surprisingly few outcome studies of any kind in populations receiving medical services provided by voluntary charitable organizations abroad. The International Task Force on Volunteer Missions concluded that research was a lower priority in these types of ‘missions’, prioritizing initially service provision, training and development of the host nation’s cleft programme (Yeow et al., 2002). However, the position paper of the American Cleft Palate-Craniofacial Association (www.acpa-cpf.org/whoweare/papers.htm) stated that, where possible, quality assessments of care outcomes should be undertaken. A UK report has also stated that much more rigorous research and evaluation of what is needed in the Developing World are required (Crisp, 2007). In a study in Amazonian Brazil, Reeve et al. (2004) found that improvement in the ability to speak was the single most important anticipated outcome for patients and their families, with the assumption that surgery would make an immediate and dramatic positive difference. Reeve et al. state: Many children did not really understand that surgery alone would not make a great deal of difference in their lives. This was particularly true among older patients coming in for surgery for cleft palate. They desperately wanted surgery and believed that their lives would improve significantly immediately after surgery.

Knowledge of outcomes of late surgical repair, undertaken after speech has developed, is therefore vital in order to ensure the best use of visiting medical teams and local resources in the Developing World, and for informed counselling of patients regarding realistic expectations of surgery in terms of speech benefits (see Chapter 13, Bradbury and Habel; Reeve et al., 2004). Finally, speech and language therapy has an important educational role, and arguably a requirement to address communication needs more broadly than purely at the impairment level, a theme which will be developed in this and the next chapter.

EVIDENCE BASE FOR SPEECH OUTCOMES Those who have visited the Developing World will have heard the typical severely disordered speech associated with the unrepaired palate which is now more rarely heard in developed regions. Hypernasality, restricted sound systems often characterized by nasal consonants and oral consonants requiring relatively little intra-oral pressure such as /l/w/y/, together with the prevalent use of glottal stops, are very common. For example, in this author’s experience, individuals speaking Tamil, Sinhalese, Hindi, Male (all Indo-Aryan languages) demonstrated these speech characteristics, irrespective of the language’s unique sound system (Sell, 1992; Langthorne, 2006).

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There is considerable evidence that there is a high prevalence of speech disorders and structural disorders in patients with cleft palate in a Developing World context. Sell and Grunwell (1994) undertook a critical review of studies of patients who had established their speech in the presence of an unoperated cleft palate. Early reports of unoperated patients were largely anecdotal. They were, in the main, descriptive impressions by professionals who were not speech and language therapists with no attempts to systematically evaluate speech (Davis, 1951; Law & Fulton, 1959; Innis, 1962; Pitanguy & Franco, 1967; Boo-Chai, 1971; Jackson et al., 1983; Ehmann & Schonenberger, 1991). Some of these studies tended to report rather positive findings. For example, Law and Fulton (1959) stated that adults with unrepaired cleft lip and palate had ‘near normal voice quality’. Ehmann and Schonenberger (1991) concluded that despite a lack of speech therapy, the ‘nasal speech had definitely improved in their adult group’. All these types of studies, however, are considered as the lowest level of scientific evidence (Reilly et al., 2004). Landis and Cuc (1975) reported on the pre-operative speech characteristics of 54 Vietnamese patients with varying types of unoperated palatal clefts but there was no opportunity to study speech post-operatively. They concluded that cleft type and pre-operative speech patterns may be of value in planning surgery. An early important contribution regarding older patients came from Ortiz-Monasterio and colleagues, who made comments on speech production in the unoperated patient, the efficacy of pharyngeal flaps, and on speech therapy following surgery. They reported that the progress of adults who have palate repair in adulthood is disappointing (Ortiz-Monasterio et al., 1959; 1966; 1974). Ortiz-Monasterio et al. considered age to be an independent variable in their study with an average age of 27.5 years, and the youngest age was 12 years. No age range was given. Sell and Grunwell (1994) concluded that most of the above studies were characterized by flawed methodologies with regard to contemporary expectations, conflicting findings and usually with no speech and language therapist involvement (Sell, 1992, 2005; Sell et al., 1994; Wyatt et al., 1996). Speech studies under the auspices of the Sri Lankan Cleft Lip and Palate Project addressed some of these issues. First, speech and language therapists led the speech research. The cohort was studied according to age and cleft type, and exclusion criteria were made explicit. Speech and language therapists designed the speech sample based on a study of the sound system of Sinhala (the majority language in Sri Lanka), and standardized audio and video recordings were systematically gathered on a longitudinal basis, pre-operatively, post-operatively, post-therapy, and then following a long period of no intervention. A perceptual rating scale was specifically devised to evaluate speech by trained non-Sinhalese-speaking speech and language therapists (Sell et al., 1994). Nasendoscopy, audio/video recordings and lateral still X-rays of patients phonating ‘ee’ were also undertaken in order to evaluate structure. All methods were tested for their inter- and intra-rater reliability with satisfactory results. Using such methods, longitudinal studies reporting the speech results and outcomes of velopharyngeal function in adults, adolescents and children following surgery have been undertaken and are summarized below (Sell & Grunwell, 1990; Ward & James, 1990; Sell, 1992; Sell et al., 1994, Nayak, 1996; Birkett, 1999).

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CROSS-LINGUISTIC ISSUES Before summarizing this evidence, it is appropriate to comment on the issues regarding assessment, where the speech and language therapist involved does not speak the language of the patient. There is evidence that it is possible to make judgements about speech in individuals speaking Indo-Aryan languages with cleft lip/palate in a Developing World environment (Sell, 1992; Langthorne, 2006). The speech and language therapist needs to study the sound system of the language, and together with a native speaker, design an appropriate speech sample with which he/she becomes very familiar (Hutters & Henningsson, 2004; European Collaboration on Craniofacial Anomalies: Eurocran: http://www.eurocran.org). The reader is referred particularly to recent work from an international group in which guidelines for developing a speech sample are explicitly given (Henningsson et al., in press). It is, however, important to appreciate that the assessment is based on a ‘controlled’ speech sample, meaning a sample which is restricted to rote speech, single word repetition and repeated sentences, usually consisting of high pressure consonants which are vulnerable to cleft palate. Natural conversational speech cannot be evaluated by a non-native speaker.

SPEECH OUTCOMES IN OLDER PATIENTS AND CHILDREN FROM THE SRI LANKAN CLEFT LIP AND PALATE PROJECT OLDER PATIENTS One of the aims of the Sri Lankan Cleft Lip and Palate Project (see Chapter 9) was to examine speech outcome following late palate closure. The initial group studied consisted of 67 Sinhalese-speaking non-syndromic patients with cleft palate with or without cleft lip who were all over the age of 11 years, when they had palate repair by the visiting British team. Surgery took place in one of two team visits, either in 1985 or 1986. There were 24 adults and 31 adolescents in the study group. The adult group had an age range of 20–52 years with a median age of 25 years. The adolescent group was defined as those patients aged 11–19 years with a mean age of 13, and a median age of 14 years. Twelve patients were excluded: those with severe hearing loss, incomplete speech records, those who had overlying speech disorders or who were Tamil-speaking. Subsequent study by Mars (1993) has shown, however, that the use of the term adolescence is rather misleading since many rural Sri Lankans do not strictly become ‘adolescent’ in the sense of growth and bodily changes until 17 or 18 years of age. The distribution of cleft type is shown in Table 15.1. Major clefts consisted of bilateral (BCLP), unilateral cleft lip and palate (UCLP) and complete clefts of the hard and soft palate (H&S), whereas the lesser cleft group consisted of clefts of the posterior third of the hard palate and the soft palate, or the soft palate only. These were only found in the adolescent group.

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MANAGEMENT OF CLEFT LIP AND PALATE Table 15.1. Patient sample according to age and cleft type

Adults Adolescents

Median age (years)

Range (years)

BCLP∗

UCLP∗

H&S∗

LESSER†

Total

25 14

20–52 11–19

4 (5%) 4 (5%)

15 (17%) 12 (14%)

5 (6%) 5 (6%)

0 10 (11%)

24 31

Note : ∗ Major cleft groups: bilateral cleft lip and palate (BCLP), unilateral cleft lip and palate (UCLP), complete clefts of the hard and soft palate (H&S). † Lesser cleft groups: clefts of the posterior third of the hard palate and the soft palate or soft palate only.

All the patients had audio/video speech recordings of a controlled Sinhalese speech sample, taken pre-operatively, and at either 8 or 20 months post-operatively (depending on the year of surgery). At this stage, residential intensive speech therapy courses were carried out by the visiting British therapists working with local counterparts. Audio/video speech recordings were taken immediately post-therapy, and finally after a lengthy period of no intervention, either at four or five years post-operatively. Nasendoscopy recordings and lateral still X-rays of patients phonating ‘ee’ were also undertaken at four or five years post-operatively. Despite the known disadvantages of lateral cephalograms as a way of assessing velopharyngeal function, they are usually available in this environment, in contrast to videofluoroscopy. They are not dependent on overseas visitors, and provide some information about structure in the complete absence of other investigations. ASSESSMENTS Based on studies of the sound system of Sinhala, a perceptual rating scale was devised for use by trained non-Sinhalese-speaking speech therapists (Sell et al., 1994). A hypernasality rating scale and two separate scales of place and manner were separately examined for their intra- and inter-assessor reliability with satisfactory results. By combining place and manner scores, 17 different speech patterns were identified, which on the basis of phonetic criteria, were then grouped into a hierarchy of six speech categories: Category A representing the poorest articulatory patterns, and Category F representing near normal and normal articulatory patterns. The evaluation of post-operative velopharyngeal function was also considered an important outcome. Satisfactory inter- and intra-assessor reliability was found in the judgement of degree of closure for nasendoscopy and a lateral cephalogram phonating ‘ee’. SPEECH RESULTS Pre-operatively, 81% of all the patients were distributed in the three worst of the possible six rating scale categories, confirming the glottal/pharyngeal quality of speech which had developed in the presence of an unoperated palate. This was also found

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post-operatively, when no therapy had been provided, providing evidence that surgery alone does not change articulation. Following a brief course of therapy, and at the subsequent four- or five-year follow-up, while there was some improvement, 60% remained in the worst three categories. If we study the impact of cleft type on speech, it was found that at both the preoperative and post-operative stages, only the lesser cleft group was found in the highest two rating scale categories, with few major clefts. All of this group were in the adolescent age group. In contrast, the majority of the adult group, all of whom presented with major clefts, made no or minimal changes with therapy. The outcome of adults with lesser clefts is unknown, as no patients presented with this for surgery. SPEECH THERAPY INTERVENTION Experience from the Sri Lankan study suggests that it is easier to make therapeutic changes to speech where there is some pre-operative occurrence of oral place and weak manner types of plosion, frication and affrication. In addition, a speech pattern which is characterized predominantly by nasal consonants and those oral consonants requiring relatively little intra-oral pressure such as /l/w/y/ showed potential for change if velopharyngeal closure or near closure was achieved at surgery. In the main, however, the benefits of therapy were largely not maintained, or observed to carry over into conversational speech, and therefore there was little functional benefit to speech for many of these adolescents and adults with major clefts. POST-OPERATIVE VELOPHARYNGEAL FUNCTION The overall incidence of velopharyngeal insufficiency was very high in comparison with regimes of surgical timing common in the Developed World. No closure was found in 77% of the nasendoscopy examinations and 78% of the lateral cephalograms of phonating ‘ee’. This was found in both age groups, with the exception of adolescents with lesser clefts, who were almost completely characterized by borderline or adequate closure. The adult group had the highest level of velopharyngeal inadequacy (81%), of whom 31% were grossly inadequate. The adolescents had a similar incidence of inadequacy to the adults (86%) but only 7% were grossly inadequate. This difference may be due to the difficulties of surgery in adulthood described by Ward and James (1990) and discussed below, and/or the natural involution of adenoidal tissue, and the changes in velopharyngeal architecture that take place during growth. This latter factor may be particularly relevant given that the median age of the adolescent group was 14 years and Mars (1993) has reported that many of these patients were still pre-pubertal. In summary, palate repair alone did not result in velopharyngeal closure for the majority of patients over the age of 11 years at palatal surgery. Furthermore there was a high occurrence of fistulae in the adults (N = 9; 37%), the majority of which were large or medium-sized, and occurred almost exclusively in the BCLP and UCLP groups, which together with the outcomes of velopharyngeal function are suggestive

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of severe structural problems post-surgery. Based on the perceptual scale of nasality, 82% of the total cohort was hypernasal. CHILDREN It is a commonly held view that it is beneficial to repair cleft palate in early childhood. Within the Sri Lankan material a similar methodology was adopted to study outcomes in children as for the older cohort, with listener reliability studies undertaken. First, Nayak (1996) compared two groups of Sinhalese-speaking non-syndromic children (under the age of ten years) with UCLP. One group (N = 15) had palate repair between ages 1.04–3.02 years compared with a second group (N = 11) who were aged between 5.01–7.02 years. All surgery was undertaken by the visiting British surgical team. The early surgery group had better outcomes compared with the late surgery group. Some 73% of the early surgery group had oral tone in contrast to only 36% of the late surgery group; and 60% of the early surgery group had normal speech, compared with 18% of the late surgery group. Second, Birkett (1999) subsequently studied a larger cohort of 77 non-syndromic Sinhalese-speaking patients with UCLP, who were under 8 years of age at palate repair; the mean age at surgery being 3.11 years. All the children received a von Langenbeck surgical repair by the visiting British surgical team, and were over the age of 10 years at data collection. In contrast to the adults and adolescents, and Nayak’s early surgery group, 44% had oral tone post-operatively; 52% had a non-oral glottal speech pattern. Somewhat surprisingly, one-third of patients who were over 24 months of age at palate repair achieved normal or near normal speech despite no speech and language therapy, audiological management or secondary velopharyngeal surgery. Saboye et al. (2004) interestingly reported a 50% VPI rate in children who were aged 6–12 years at surgery which is similar to Birkett’s results.

OVERALL EVIDENCE FOR SPEECH OUTCOMES Overall, Sell (1992) found that the nature of the pre-operative speech pattern, age, cleft type, years of school attendance were all associated with outcome. The results of palate repair in children with UCLP suggest that surgery is worthwhile in this age group. This is in contrast to the older patients in the Sri Lankan cohort. Taken together with the findings of Ortiz-Monasterio et al. (1959, 1966, 1974; Ortiz-Monasterio & Serrano, 1971) surgical repair in adulthood appears to be of little benefit to speech. Ortiz-Monasterio et al. reported that ‘speech results are consistently poor in adult cleft palates’ (1959, 1966), even when speech therapy was given, and in a later paper (1974) they stated that there was ‘no improvement in adults’. These were probably sound conclusions although based on observation rather than rigorous scientific evidence. In addition, it is quite possible that it is success with adults with lesser clefts (i.e. clefts of the posterior third of the hard palate and soft palate or soft palate only) that has led to comments of a high level of speech performance in the literature (Fletcher, 1985).

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Ortiz-Monasterio et al. (1966) and Ward and James (1990) suggested that surgical repair of adults and older patients was more difficult than that of younger children and infants, because of increased width of the cleft and the vertically displaced palatal segments, caused by the continuous upward pressure of the tongue into the unrepaired palate (Ortiz-Monasterio et al., 1966), a situation which is exacerbated further if there is lip involvement. This is also associated with under-development of the soft palate muscles due to fibrosis and shortening of the levator palatini muscle. Ward and James (1990) reported marked mucoperiosteal fibrosis, which not only hampered the dissection of the oral and nasal layers, but also caused excessive bleeding. All these factors made it difficult to achieve a complete tension-free closure of the nasal and oral layers of the hard palate, with separate dissection and union of the levator palatini muscle sling, leading no doubt to the fistulae and VPI reported. This supports the hypothesis proposed by McWilliams et al. (1990) that the physiological potential of the muscles comprising the velopharyngeal valving mechanism is irretrievably lost by the delay in moving them into a normal relationship. Other factors which may influence outcome, such as the decreased facility for learning and adaptation with advancing age, is probably also relevant. Fletcher (1985) proposed the developmental hypothesis as an explanation of the benefits of early palate repair for speech. He suggested that early surgery results in less permanent maladaptations in speech physiology, and that articulation movements and posture are more malleable at earlier ages. Indeed, most physiological functions are best learned at an early age and at the optimal time, otherwise ineffective co-ordination and abnormal compensation habits result. Kaplan (1981) hypothesizes that it is probable that palatal speech functions have an optimal time for normal development, and ‘uncorrectable’ abnormal patterns occur if the palate is repaired at a later age. Current literature suggests that articulation patterns and ‘palatal speech functions’ cannot easily be changed in adulthood, and the Sri Lankan data lends support to the hypotheses proposed by both Fletcher and Kaplan. This short summary would certainly confirm that palate repair in childhood is beneficial. This might possibly extend to include those patients in the age bracket of 10–13 years, particularly if factors such as the nature of the pre-operative speech pattern, school attendance and the availability of post-operative speech therapy are also taken into account (Sell, 1992). Adolescents with lesser clefts also benefit from late surgery. The use of resources for surgery on unoperated palates in adulthood is in some doubt. It is, however, important to appreciate that perhaps there are other culturally related important positive outcomes which are unknown to overseas visitors which may have an important influence. As Reeve et al. (2004) state, ‘Patients exist in a socio-cultural matrix in which the meaning of the condition they have and their futures are determined by a host of factors, including cultural, such as beliefs and practices.’ For example, it is possible that repair of the palate carries some status within the patient’s local community, perhaps improving employment opportunities. Alternatively, palate repair may have the positive benefit of encouraging others in the local area to seek treatment. Therefore, it is important to be sensitive to local cultures when using the evidence base.

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PHARYNGOPLASTIES There has been much debate about how to manage VPI in a Developing World context. It is the case that undoubtedly many of these patients have disappointing outcomes in terms of structure, and ideally require secondary velopharyngeal surgery. Even with a pharyngeal flap, the results are disappointing (Ortiz-Monasterio et al., 1974). Sell and Grunwell’s (1994) analysis of this paper suggested that in none of the 140 patients (which incidentally represented 64% of the cohort) was the pharyngeal flap effective in eliminating nasality. Skoog (1965) also presented results of pharyngeal flaps in adults but unfortunately a critical review by Sell and Grunwell (1994) concluded that methodological flaws, such as the mixture of unoperated and operated subjects and a framework using ‘intelligibility’ as the outcome measure, meant it was impossible to extrapolate any useful information. In contrast, in their work in the Philippines, Saboye et al. (2004) have advocated an orticochea pharyngoplasty combined with palate repair in patients older than 12 years of age, and have since operated on 34 patients according to this method. Of the 24 patients seen for follow-up, 14 (60%) were described as ‘phonation 1’ by the team’s speech and language therapist. It is assumed this is the Borel-Maisonny 4-point rating scale, but this is not stated in the article. Phonation 1 is described as ‘normal velopharyngeal competence’. However, it is unclear how the authors have visualized the velopharynx to arrive at this conclusion. No mention is made of perceptual analysis of the speech parameters of nasality, nasal airflow errors, and articulation which are widely accepted as indicators of speech outcome. Furthermore, there is no information on reliability studies. Saboye et al. (2004) reported that the best results were in the adolescent group, and where the cleft type was cleft palate only. This team is probably one of few teams supportive of pharyngoplasties in a Developing World context, bearing in mind recent increased recognition of associated airway problems. Whether or not pharyngoplasty is an appropriate secondary procedure, in terms of safety with the increased risks of bleeding and sleep apnoea, needs to be considered within the context of the local facilities, and also the priorities of the team with its available resources and expertise. Furthermore, the very frequent need for postoperative speech therapy for disordered articulation, and indeed typical absence of this provision, are also very relevant in a decision about pharyngoplasty.

LEARNING POINTS In conclusion, there are several learning points that arise from this chapter. NATURE OF SURGERY Palate repair alone is often associated with postoperative VPI for patients older than 11 years at surgery, except for patients with clefts of the posterior third of the hard and soft palate only, or soft palate only.

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AGE The commonly held view that palatal closure over the age of 10 years is of minimal benefit is largely confirmed. Surgery alone in patients older than 11 years usually does not lead to improved speech, except in the lesser cleft group. When post-operative speech therapy is available, however, some of the younger adolescents (i.e. 10–13year-olds) with major clefts may benefit. Adults unfortunately rarely benefit. With regard to children, however, the Sri Lankan data has confirmed that surgical repair can be of benefit for some children even with no supporting speech therapy, audiological management or secondary velopharyngeal surgery, confirming that this should be viewed as a priority group in the Developing World. CLEFT TYPE Speech outcome is significantly better for the lesser cleft group following surgery alone or when supported by therapy. SPEECH THERAPY Speech therapy should be provided for patients in order to maximize the benefits of surgery. Progress even then will be limited by persistent structural problems in many patients with disappointing results, and patients should be counselled to this effect (see Bradbury and Habel, Chapter 13). Reeve et al. (2004) stated, ‘Improved speech is viewed as essential for bettering the patient’s ability to perform in school and to obtain gainful employment.’ Reeve also documented that there was an appreciation of the importance of speech therapy post-operatively. It is interesting, however, that Murthy and Raman (2005) found that only 10% of patients (N = 20/200) who had palatal surgery after the age of 10 years complied with therapy. The complex issues over the delivery of speech and language therapy in this environment will be discussed in Chapter 16. In summary, however, the difficulties of accessing this provision underline the importance of not only the need for primary repair at a young age but also that the quality of the palate repair is paramount. It is incumbent on visiting surgical colleagues that surgical techniques in cleft palate should be easily transferable, effective, reliable and robust.

REFERENCES Abenavoli F (2005) ‘Operation Smile humanitarian missions’, Plast Reconstr Surg 115: 356–7. American Cleft Palate Association (2007) Position paper – international treatment programmes. Available at: www.acpa-cpf.org/whoweare/papers.htm (accessed 22nd July 2007). Birkett L (1999) ‘A study of speech results and timing of surgery in a Sri Lankan UCLP population’, MSc project, University of London. Bleile K, Ireland L & Kiel T (2006) ‘The professions around the world: new web based directory goes global’, The ASHA Leader 8–9: 26–7.

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Boo-Chai K (1971) ‘The unoperated adult bilateral cleft of the lip and palate’, Brit J Plast Surg 24: 250–7. Canady JW (2001) ‘Smiles, kudos and comments’, Anaesthesiology 95: 1311–12. Crisp N (2007) Global Health Partnerships: The UK Contribution to Health in Developing Countries, London: The Stationery Office. D’Antonio LL (1990) ‘Commentary to Ward & James’, Cleft Pal J 27: 15–17. Davis AD (1951) ‘Unoperated bilateral complete cleft lip and palate in the adult’, Plast Reconstr Surg 7: 482–91. Dupuis CC (2004) ‘Humanitarian missions in the Third World: a polite dissent’, Plast Reconstr Surg 113: 433–5. Ehmann G & Schonenberger AM (1991) ‘Late results of one-stage primary surgery in 118 adult Africans with clefts of lip, alveolus, and palate using the wave-line procedure and bridge-flap technique’, in Pfeifer G (ed.) Craniofacial Abnormalities and Clefts of the Lip, Alveolus and Palate, Stuttgart: Thieme Publishers, pp. 468–71. Fletcher SG (1985) ‘Speech production and oral motor skill in an adult with an unrepaired palatal cleft’, Journal of Speech and Hearing Disorders 50: 254–61. Henningsson G, Kuehn DP, Sell D, Sweeney T, Trost-Cardamone JT & Whitehill TL (in press) ‘Universal parameters for reporting speech outcomes in individuals with cleft palate’, Cleft Pal Craniofacial J. Hodges A & Goodacre T (2002) ‘The cutting edge’, Tropical Doctor 32: 86–7. Hutters B & Henningsson G (2004) ‘Speech outcome following treatment in crosslinguistic cleft palate studies: methodological implications’, Cleft Pal Craniofacial J 41: 544–9. Innis CO (1962) ‘Some preliminary observations on unrepaired hare lips and cleft palates in adult members of the Dusan Tribes of N. Borneo’, Br J Plast Surg 15: 173–81. Jackson I, McLellan G & Scheker LR (1983) ‘Primary veloplasty or primary palatoplasty: some preliminary findings’, Plast Reconstr Surg 72: 153–7. Kaplan EN (1981) ‘Cleft palate repair at three months?’ Ann Plast Surg 7: 179–90. Keunen HF (1966) ‘Plastic surgical safari with A.M.R.F. in E. Africa’, Plast Reconstr Surg 37: 72–80. Lamabadusuriya SP, Mars M & Ward CM (1988) ‘Sri Lankan Cleft Lip and Palate Project: a preliminary report’, Journal of the Royal Society of Medicine 81: 705–9. Landis P & Cuc T (1975) ‘Articulation patterns and speech intelligibility of 54 Vietnamese children with unoperated oral clefts: clinical observations and impressions’, Cleft Palate J 12: 234–43. Langthorne S (2006) ‘The impact on speech outcomes and velopharyngeal function of very delayed hard palate closure in Hindi-speaking children’, BSc project, University of London. Law FE & Fulton JT (1959) ‘Unoperated oral clefts at maturation’, Annals of the Journal of Public Health 49: 1517–24. Mahler H (1981) ‘The meaning of health for all by the year 2000’, World Health Forum 2: 5–22. Mars M (1993) ‘The effects of surgery on facial growth in UCLP’, PhD, University of London. Mars M, James DR & Lamabadusuriya SP (1990) ‘The Sri Lankan Cleft Lip and Palate Project: the unoperated cleft lip and palate’, Cleft Palate J 27: 3–6. McWilliams BJ, Morris HL & Shelton RL (1990) Cleft Palate Speech, 2nd edn, Philadelphia, PA: BC Decker.

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Murthy J & Raman S (2005) ‘Speech outcome in late cleft palate repair – an analysis’, paper presented at the 10th International Congress on Cleft Palate and Related Craniofacial Anomalies, Durban, South Africa. Nagarajan R (2006) ‘Speech-language pathology in cleft care: emergence of the sub-specialty in India’, paper presented at international conference on treatment of speech disorders in individuals with cleft lip and palate, Loma Linda University, California and via videoconference, Sri Ramachandra Medical College and Research Institute Deemed University Chennai, India. Natsume N (1998) ‘Safari surgery’, Plast Reconstr Surg 102: 1304–5. Nayak JA (1996) ‘Systematic study correlating speech results of Sri Lankan UCLP participants with timing of surgery’, MSc project, University of London. Ortiz-Monasterio F, Olmeda A, Trigos I, Yudovich M, Velazquez M & Fuente-del-Campo A (1974) ‘Final results from the delayed treatment of patients with clefts of the lip and palate’, Scand J Plast Surg 8: 109–15. Ortiz-Monasterio F, Rebeil AS, Valderrama M & Cruz R (1959) ‘Cephalometric measurements on adult patients with nonoperated cleft palates’, Plast Reconstr Surg 4: 53–61. Ortiz-Monasterio F & Serrano RA (1971) ‘Cultural aspects of cleft lip and palate treatment’, in Grabb WC, Bzoch KR & Rosenstein S (eds) Cleft Lip and Palate, Boston: Little, Brown and Company, pp. 130–41. Ortiz-Monasterio F, Serrano RA, Barrera GP, Rodriguez-Hoffman H & Vinageras E (1966) ‘A study of untreated adult cleft palate patients’, Plast Reconstr Surg 38: 36–41. Pitanguy I & Franco T (1967) ‘Nonoperated oral facial fissures in adults’, Plast Reconstr Surg 39: 569–77. Prathanee B, Dechongkit S & Manochiopinig S (2006) ‘Development of community-based speech therapy model: for children with cleft lip/palate in Northeast Thailand’, Journal of the Medical Association of Thailand 89: 500–7. Reeve M, Groce N, Persing J & Magge S (2004) ‘An international surgical exchange program for children with cleft lip/cleft palate in Manaus, Brazil: patient and family expectations of outcome’, Journal of Craniofacial Surgery 15: 170–4. Reilly S, Douglas J & Oates J (2004) Evidence-based Practice in Speech Pathology, London: Whurr. Ruiz-Razura A, Cronin E & Navarro C (2000) ‘Creating long-term benefits in cleft lip and palate volunteer missions’, Plast Reconstr Surg 105: 195–201. Saboye J, Chancholle A-R, Tournier J-J & Maurette I (2004) ‘Palatovelopharyngoplasty, an “all in one surgery for adult patients”: our experience in Philippines’, Annales de Chirugie Plastique Esthetique 49: 261–4. Sell D (1992) ‘Speech in Sri Lankan cleft palate subjects with delayed palatoplasty’, unpublished PhD thesis, De Montfort University, Leicester. Sell D (2005) ‘Issues in perceptual speech analysis in cleft palate and related disorders: a review’, International Journal of Language and Communication Disorders 40: 103–21. Sell D & Grunwell P (1990) ‘Preliminary speech results in a subgroup of unoperated Sri Lankan cleft lip and palate adolescents following late palatal surgery’, Cleft Palate J 27: 162–8. Sell D & Grunwell P (1993) ‘Speech in late operated cleft palate subjects’, in Grunwell P (ed.) Analysing Cleft Palate Speech, London: Whurr. Sell D & Grunwell P (1994) ‘Speech studies and the unoperated cleft palate subject’, European Journal of Disorders of Communication 9: 151–64.

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Sell D, Grunwell P & Mars M (1994) ‘A methodology for the evaluation of severely disordered cleft palate speech’, Clinical Linguistics and Phonetics 8: 219–33. Skoog T (1965) ‘The pharyngeal flap operation in cleft palate’, Brit J Plast Surg 18: 265–82. Ward CM & James I (1990) ‘Surgery of 346 patients with unoperated cleft lip and palate in Sri Lanka’, Cleft Palate J 27: 11–14. Wyatt R, Sell D, Russell J, Harding A, Harland K & Albery E (1996) ‘Cleft palate speech dissected: a review of current knowledge and analysis’, Br J Plast Surg 49: 143–9. Yeow V, Lee-Seng-Teik T, Lambrecht TJ, Barnett J, Gorney M, Hardjowasito W et al. (2002) ‘International task force on volunteer cleft missions’, Journal of Craniofacial Surgery 13: 18–25. Zbar RIS, Rai SM & Dingman DL (2000) ‘Establishing cleft malformation surgery in developing nations: a model for the new millennium’, Plast Reconstr Surg 106: 886–9; discussion, 890–1.

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Speech Therapy Delivery and Cleft Lip and Palate in the Developing World DEBBIE SELL

Editorial comment: The aim of this chapter is to explore and critique the different models of speech therapy delivery in the Developing World context. It illustrates the potential contribution and importance of a broader role than just focusing on delivering services at the speech impairment level.

INTRODUCTION Chapter 15 highlights the importance of the availability of speech and language therapy (SLT) in the Developing World. Many authors recognize the need for speech therapy following surgery, not least because many patients are well past the age when speech is acquired at the time surgery is performed (Wilcox, 1994; Sell, 1992; Sell & Grunwell, 1990; Prathanee et al., 2006). It is recognized that there is a high level of unmet need. Access is complicated as populations are often rural (Prathanee et al., 2006; Schwarz & Bhai-Khadka, 2004). Transport links are rudimentary and families find it difficult to travel, often losing wages for days not worked. Any services which do exist tend to cluster in the hospitals of large urban centres and be accessed by the urban community only. The distribution of SLT services worldwide is without doubt concentrated in the Developed World (Wirz & Hartley, 1999). Even in this context, services are often restricted. Realistically, speech and language therapy provision in the Developing World is unlikely to change in the near future, particularly with regard to the specialist speech therapy required in the treatment of speech in cleft palate. Even when speech therapy is provided, our experience suggests that it would need to be prolonged and intensive in the older patient or patients who have late repairs, and in many cases speech therapy will be ineffectual, given the major structural anomalies that are frequently present (Sell, 1992). Nagarajan (2006a) emphasizes that speech therapy services for

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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r Short time frame of surgical visits, with little emphasis on interdisciplinary team working.

r Lack of vision as to how speech and language therapy services can be factored into volunteer cleft programmes.

r Profession does not exist locally. r Very few professionals. r Services only based in urban tertiary type settings. r Presence of many different languages and dialects. r Family and patients have misconceptions about causes and availability of treatment.

r Poor families based in rural settings. Source : D’Antonio & Nagarajan (2003).

patients with cleft palate require a different time commitment and different model of service delivery than surgical services, and particularly in the area of rehabilitation. In Table 16.1, D’Antonio and Nagarajan (2003) have summarized the many barriers to the development of SLT services and have been pioneers in alternative models of service delivery. In 1990, D’Antonio stated ‘Many of our old models are no longer possible even in the countries of origin’ where the demands are high and resources are limited. There are, however, broader issues than speech at the impairment level, which impact on the way that speech presents as it does. Families often have minimal knowledge of the cleft condition (Schwarz & Bhai-Khadka, 2004; Prathanee et al., 2006), including the need for early surgery, the pathway of care, coupled with limited written material and usually no parent support group (see Chapter 12). Wickenden et al. (2001a) summed up the situation rather well: ‘Families are likely to expect a cure, or attempted cure through medical intervention, relatively little individual attention, not much information or choice about the management of the problem and a one-off visit, not an ongoing service.’ The latter comment is particularly pertinent given the typical ongoing nature of care for individuals offered in developed settings and particularly in speech therapy. In under-resourced regions there is often little coordinated follow-up with regards to hearing, ENT problems, and orthodontic care, or such services just do not exist. There are often no possibilities for pharyngoplasties/revisionary surgery. Furthermore, local professionals may find detailed consideration of speech/language and communication and the types of therapy offered an enigma. Many may have limited experience of normal speech outcomes found in the Developed World, so their level of expectation of speech outcomes is may be set too low. Alternatively, such individuals may have spent time in a sophisticated service in the Developed World and expect non-specialist individuals to deliver a similar service in this difficult environment. They may have been exposed to technology and believe that this may provide the necessary outcome measurements, often with little training or understanding of the limitations of such systems. However, local professionals are very aware that

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families are concerned about speech disorders and their functional and social impact (see Chapter 13, Bradbury and Habel; Reeve et al., 2004). The way in which speech services for individuals with this condition has been delivered in the Developing World has varied considerably. There are different models of speech and language therapy provision ranging from fully trained indigenous SLTs, visiting therapists from the Developed World undertaking treatment, to counterparts or assistants, community-based rehabilitation (CBR) workers, through to training packages for parents, families and friends. The aim of this chapter is to explore and critique the different models of delivery in the context of the knowledge gained from the previous chapter.

WHERE THERE IS NO LOCAL SLT, WHAT CAN OTHERS DO? Before describing models of delivery, perhaps it is helpful to offer a framework in which to consider these: that of the WHO International Classification of Functioning, Disability and Health (WHO, 2001). The reader is referred to Chapter 12 in this text where Wickenden develops this issue in some detail. Speech and language therapists involved in the care of these patients in the West largely focus on the speech impairment with the main aim of improving or curing abnormal speech. This is usually referred to as the medical or impairment-based model. Perhaps this is not surprising given the way in which the profession’s roots developed from a medical context of cleft management. The impairment-based model is usually urban-based, relies heavily on professional expertise, and is limited in coverage or the population it reaches (Helander, 1993; O’Toole & McConkey, 1995). It is often the approach expected by doctors, parents and patients. An alternative approach is the social model in which outcomes are focused on the real-life consequences of a speech impairment and how that might be helped, including activity, how restricted a patient is at a functional level, and the impact on participation, i.e. the level of social inclusion or exclusion. This approach tries to capture the importance of environmental, contextual and personal factors, i.e. reactions and responses of those around him, and, as Duchan (2004) states, emphasizes the impact of clients’ communicative differences rather than deficits. Threats (2006) describes these as outcomes which capture how patients are functioning in their communicative lives. This approach emphasizes the capacity of the community to accept and adapt to the person with disabilities and it is the therapist’s role to facilitate the community’s adaptation (Wickenden et al., 2001a). The social model encompasses the transfer of skills and knowledge, professionals acting in a supportive and advisory role, and requires extensive support from family, patients, teachers as ‘experts’, and CBR workers. Wickenden (see Chapter 12) states that this model is important where no surgery or speech and language therapy are available. As we review the different models of intervention below, it may be helpful to reflect on how these sit within this conceptual framework of disability.

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COUNTERPARTS OR ASSISTANTS TRAINING This impairment-based model has been advocated, often where there is a strong commitment to cleft care, and involves the training of other professionals in the principles of therapy. For example, in China, where speech and language therapists are especially scarce (WHO Report, 2002), 200 oral maxillofacial surgeons and nurses were trained to deliver speech and language therapy services for children with cleft lip and palate (D’Antonio, 2003). Landis and Cuc (1972) first reported an approach in which a counterpart in Vietnam was trained in the principles of articulation therapy. Based on their work, a major aim of the intensive speech therapy programme in the Sri Lankan Cleft Lip and Palate Project was to train counterparts to continue this work once the Western SLTs had left (Wirt et al., 1990a, 1990b). Five local individuals received training for four months in working on articulation skills in patients with cleft palates. A critical evaluation of this programme showed, however, that there were several problems. Overseas therapists needed to be involved in the selection process of the counterparts. There were sustainability issues after the Western therapists had departed. Issues arose around continuing education and supervision. There were practical considerations, such as where the counterparts would work, exacerbated if the counterparts’ superiors had not been involved in the programme. Furthermore, it was not clear how this activity would fit into the counterparts’ usual jobs, and how and by whom they would be paid was problematic. The training programme needed to be recognized within the existing health/education structure but this was difficult to negotiate within very hierarchical systems. Finally, with regard to transferring clinical skills, it was possible to teach a new learner the mechanical technique of how to remediate an articulation error, i.e. step 1, step 2, step 3. It was much more difficult to teach a counterpart how to respond sensitively to the progress and motivation of a patient, i.e. moving on to step 2 more quickly/slowly, moving sideways because step 1 was not working, determining why someone wasn’t progressing and what to do about it. These areas develop from experience and ongoing supervision and through learning a much broader range of problem-solving skills than it is possible to teach in a very short training course, which was related to one condition – cleft palate. Therefore, this approach is not without its difficulties. Notwithstanding this, where there is no provision at all and unlikely to be one for the foreseeable future, providing interested and keen local individuals with knowledge and skills may be appropriate. Perhaps being aware of the pitfalls described above, it may be possible to minimize their impact.

COMMUNITY-BASED REHABILITATION (CBR) WORKERS Community-based rehabilitation (CBR) involves community responsibility for a broad range of types of impairments, including communication disorders, and emphasizes rehabilitation through social integration (Mendis, 1994). This needs to be viewed as an extension of and complementary to those services provided by the tertiary

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setting, and is implemented through the combined efforts of disabled people themselves, their families and communities, and the appropriate health, education, vocational and social services. Many Developing Countries have invested in developing locally appropriate models of community-based rehabilitation for health care and rehabilitation (Nagarajan, 2006b). In fact, it was in 1990 that D’Antonio first referred in the cleft literature to the use of CBR workers in Nepal. In 2003, D’Antonio and Nagarajan described a consensus workshop that explored the application of the CBR model to address the need for SLT services for children with cleft palate when western-style SLT services are limited or not available. Importantly, Nagarajan (2006b) suggested that the community-based model should not be restricted to local workers but should be integrated into visiting international cleft programmes. In India, speech pathology training programmes based on a western model have been in existence since 1967, although many graduates leave the country for overseas work, for which, arguably, their training has been designed. Nagarajan (2006a) has implemented an initiative which has provided additional training of speech pathologists in the cleft area. This workforce has had responsibility for training the CBR workforce. Nagarajan (2006b) reported pilot data which illustrated that rural community-based rehabilitation workers in Southern India can be trained to provide accurate identification and screening of communication impairment in children with cleft palate. The next and telling stage of this work is to evaluate whether CBR workers can be trained to deliver an early intervention programme (work in progress, Nagarajan & Sherer), and perhaps ultimately whether it is possible for CBR workers to deliver an effective impairment-based articulation programme. Wickenden (Chapter 12) describes the possible role of the CBR worker with individuals with cleft lip and/or palate which could usefully incorporate some of the preventative advice speech and language therapists provide, and it is possible that Nagarajan and colleagues will refine this role in due course. A similar approach has been advocated in North-east Thailand in the absence of an SLT workforce. Prathanee et al. (2006) used participatory workshops in order to develop a community-based speech therapy model. They aimed to promote the development of health care within the local community, to share knowledge and skills, and generally develop and strengthen the community’s capacity to care for itself. This was achieved by combining CBR primary health care and medical approaches in order to develop a CBR model which focused on speech and the cleft palate condition. The extent to which this programme will be able to implement the approaches developed in India, where there is, in contrast to Thailand, a speech and language therapy workforce to support it will be very important to evaluate.

INDIGENOUS SPEECH AND LANGUAGE THERAPISTS In most Developing World countries, the profession of speech and language therapy does not exist or there are only a few therapists. Yeow et al. (2002) made the erroneous assumption that speech and language therapists are available in all the areas where

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visiting cleft overseas programmes exist. They advocated training of this local workforce either locally or as a clinical attachment in the visiting team’s home centre. As stated, in most places there are no speech and language therapists. Indeed, conversely, as Nagarajan points out, the Sri Lankan Cleft Lip and Palate Project became the catalyst for the development of the profession in that country (Wickenden et al., 2001b). Up until the mid-1990s there was only one speech and language therapist for a population of 19 million in Sri Lanka. Initially a diploma course was developed (Wickenden et al., 2003), which has since been converted to a degree course. The diploma ensured a critical mass of the workforce has been established in Sri Lanka, and prevented the typical exodus overseas, where a degree was required for employment. In a needs assessment before the start of the diploma programme, Kodikara (1999) found that both Sri Lankan community workers and parents wanted information about communication disorders. Like many other Developing Countries, 88% of the population live in rural settings. The course was therefore designed to provide a comprehensive model of service, encompassing both social and impairment-based approaches to rehabilitation, while taking into account language aspects and the cultural, economic and demographic context. This approach, in the context of a small professional workforce, meant that the curriculum needed to incorporate modules on transfer of knowledge (i.e. training), community and social approaches to disability and cultural awareness. A community placement with CBR was included in the programme where students would learn about working in remote and rural settings. Multidisciplinary working was relatively under-developed and cleft lip and palate provided the ideal opportunity to develop this. Ten years on from the start of the project, the programme is self-sufficient and largely run by Sri Lankans at the University of Keleniya, Colombo. There are now more than 60 trained speech pathologists in Sri Lanka, mainly based in cities with very large caseloads. They are, however, based not only in the government hospitals but also in schools. The therapists provide consultancy, support and training to teachers, parents, CBR workers, allied health professionals and nurses. Within the arena of cleft lip and palate, therapists work in the tertiary setting of the multidisciplinary cleft lip and palate teams, and prioritize young patients and those assessed as having the potential to change speech while encouraging the development of patient information in the local community and preventative work. The therapists have been trained to evaluate their populations, providing process and outcome data. For example, Dassanayake et al. (2000) undertook an audit of a consecutive series of the first 200 patients referred over their initial four months of working after qualifying from the Speech and Language Therapy Training Course at the University of Keleniya. They divided their group into two: good intelligibility (group 1) and poor intelligibility (group 2). Two-thirds fell into the latter group, one-third into group 1. Structural problems were prevalent with 85% hypernasal in group 2 and 55% hypernasal in group 1. Three-quarters of group 2 had major consonant production errors. These data provide further evidence of the very special challenges of managing speech in this context often without the possibilities of secondary surgery and sophisticated team management that are common in Developed Countries.

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PARENTS AND FAMILIES Another impairment approach which is attracting interest is the training of parents and other lay people to deliver articulation training to children who have established their speech with an unrepaired palate, using an approach called ‘Corrective Babbling’ (www.ref-earthspeak.org). Training of the parent and child is delivered over five days in the speech camp concept. The approach involves working through all the vowels and consonants of a language, regardless of the nature of the speech disorder. Of particular significance is that it is restricted to patients who are judged as having adequate hearing and velopharyngeal closure, the latter rarely found in this context in the author’s experience. The authors state that most parents/children successfully complete the programme in four to nine months. They are aiming to replicate the programme in different languages and for this treatment to be self-sustaining. Jobe et al. (2005) reported on 154 subjects aged 4–33 years and stated that 60% eliminated glottalization and grimacing; hypernasality/nasal emission was minimal or none in 70% and intelligibility of spontaneous speech improved by 10–25%. To date, however, there have been no peer-reviewed published outcome studies, and the scientific community awaits these with interest. Given the frequent finding of structural impairment in the Developing World, this type of approach may be suitable for limited numbers of patients, particularly children and young adolescents (see Chapter 15, Sell). Certainly if it is possible to use trained family and friends, then this would seem a very sensible approach given the lack of availability of speech and language services. However, issues of sustainability for this method for subsequent patients need further clarification. It would also be interesting to have data on the number of patients excluded from the programme because they presented with velopharyngeal insufficiency.

CONCLUSION Marshall (2003) argues that development work of any sort needs to fit within existing structures, exploiting and adapting available resources, and stresses the importance of setting up systems which increase the chances of services being sustainable once outside support is withdrawn. It is immediately obvious that the counterpart training programme in Sri Lanka failed probably because it did not meet these requirements, in contrast to the subsequent programme to develop a speech and language therapy profession with a broader range of skills and a clear professional status and identity within the local health system. Authorities seem to be converging on the view that there is a responsibility for all professionals to engage CBR workers, local health-care providers and community leaders in endeavours to help patients with this condition (D’Antonio, 1990; Wickenden et al., 2001a, 2001b; Hartley & Wirz, 2002; Reeve et al., 2004; Nagarajaran, 2006a). Nagarajaran (2006a) extends this concept to overseas

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programmes. Sell (2007) also concludes that surgical expeditions have a responsibility to encourage the development of patient information in partnership with the local community. This chapter has tried to illustrate that speech and language therapy can be facilitated using both the social model and the medical impairment-based model. For example, it can be seen that adopting this combined approach to training speech and language therapists provides a greater opportunity to reach individuals with cleft lip and palate in rural and very under-resourced settings. CBR workers can provide information, improve awareness, have a preventative role and improve inclusion and acceptance within the local community, focusing mainly on activity and participation issues. Notwithstanding the social model approach described above, it is recognized that tertiary speech pathology services focusing on the impairment itself are required. Clinical expertise is required for the treatment of patients. In addition, it is vital that there are more outcome studies and the different methods of delivery of SLT are evaluated. Tertiary speech pathology services need to be developed hand in hand with community services. It is however an undeniable and probably impossible challenge for specialized interventions to be delivered in the absence of specialized professionals. If, however, families can be encouraged by the CBR community to bring their children for surgery in infancy, then there is a greater possibility that they will not require impairment based services, and hopefully the problem of the older unoperated patient may start to be eradicated.

REFERENCES D’Antonio LL (1990) ‘Commentary to Ward & James’, Cleft Palate J 27: 16. D’Antonio LL (2003) ‘Speech training symposium held in China’, The Smile Train, Fast Track 3: 7. D’Antonio LL & Nagarajan R (2003) ‘Use of a consensus building approach to plan speech services for children with cleft palate in India’, Folia Phoniatrica at Logopaedica 55: 306–14. Dassanayake N, Rathnayake S & Walker V (2000) ‘An audit of outcome in 200 patients with cleft lip and/or palate’, paper presented at the Sri Lankan annual meeting of the Dental Association, Sri Lanka. Duchan JF (2004) ‘Where is the person in the ICF?’ Advances in Speech-Language Pathology 6: 67–70. Hartley S & Wirz S (2002) ‘Development of a communication disability model and its implication on service delivery in low-income countries’, Soc Sci Med 10: 1543–7. Helander E (1993) Prejudice and Dignity: An Introduction to Community-based Rehabilitation, Geneva: United Nations Development Programme. Jobe A, Lundberg M, Davison P, Jobe R & Reddy M (2005) ‘Corrective babbling: an experimental approach to the problem of providing speech therapy to surgically corrected subjects with habituated cleft palate speech patterns in developing nations’, paper presented at 10th International Congress on Cleft Palate and related craniofacial anomalies, Durban, South Africa.

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Kodikara S (1999) ‘Needs for services for children with communication disorders in Sri Lanka’, MSc project, CICH, Institute of Child Health, UCL, London. Landis P & Cuc T (1972) ‘Articulation patterns and speech intelligibility of 54 Vietnamese children with unoperated oral clefts: clinical observations and impressions’, Cleft Palate J 12: 234–43. Marshall J (2003) ‘International and cross-cultural issues: six key challenges for our professions’, Folia Phoniatrica at Logopaedica 55: 329–36. Mendis P (1994) ‘Disability prevention and rehabilitation’, in Lankinen KS, Bergstrum S, Makela PH & Peltomaa M (eds) Health and Disease in Developing Countries, London: Macmillan, pp. 531–42. Nagarajan R (2006a) ‘Speech-language pathology in cleft care: emergence of the sub-specialty in India’, paper presented at international conference on treatment of speech disorders in individuals with cleft lip and palate, Loma Linda University, California, and via videoconference Sri Ramachandra Medical College and Research Institute Deemed University Chennai, India. Nagarajan R (2006b) ‘Development of community based speech services for individuals with CLP in India: lessons for around the world’, paper presented at international conference on treatment of speech disorders in individuals with cleft lip and palate, Loma Linda University, California, and via videoconference Sri Ramachandra Medical College and Research Institute Deemed University Chennai, India. O’Toole B & McConkey R (1995) ‘Towards the new millennium’, in O’Toole B & McConkey R (eds) Innovations in Developing Countries for People with Disabilities, Chorley: Lisieux Hall Publications. Prathanee B, Dechongkit S & Manochiopinig S (2006) ‘Development of community-based speech therapy model: for children with cleft lip/palate in Northeast Thailand’, J Med Assoc Thai 89: 500–7. Reeve M, Groce N, Persing J & Magge S (2004) ‘An international surgical exchange program for children with cleft lip/cleft palate in Manaus, Brazil: patient and family expectations of outcome’, J Craniofacial Surg 15: 170–4. Schwarz R & Bhai-Khadka S (2004) ‘Reasons for late presentation of cleft deformity in Nepal’, Cleft Palate Craniofacial J 41: 199–201. Sell D (1992) ‘Speech in Sri Lankan cleft palate subjects with delayed palatoplasty’, unpublished PhD thesis, De Montfort University, Leicester. Sell D (2007) ‘A critical look at speech outcome measures and speech therapy delivery in this context’, invited presentation in mini-symposium on outcome measures and related topics in a perspective of cleft work overseas, Craniofacial Society of Great Britain and Ireland, Dublin. Sell D & Grunwell P (1990) ‘Preliminary speech results in a subgroup of unoperated Sri Lankan cleft lip and palate adolescents following late palatal surgery’, Cleft Palate J 27: 162–8. Threats T (2006) ‘Towards an international framework for communication disorders: use of the ICF’, J Commun Dis 39: 251–65. WHO (2001) ICIDH-2, Geneva: World Health Organisation. WHO (2002) The World Health Report 2002: Reducing Risks, Promoting Healthy Life, Geneva: World Health Organisation. Wickenden M, Hartley S, Kariyakaranawa K & Kodikara S (2003) ‘Teaching speech and language therapists in Sri Lanka: issues in curriculum, culture and language’, Folia Phoniatr Logop 55: 314–21.

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Wickenden M, Hartley S & Kodikara S (2001a) ‘Educating for change: the training programme in Sri Lanka for new professionals working with people with communication disabilities’, Asia Pac Dis Rehab J 12: 88–99. Wickenden M, Hartley S, Kodikara S, Mars M, Sell D, Sirimanna T & Wirz S (2001b) ‘Collaborative development of a new course and service in Sri Lanka’, Int J Lang Commun Disord 36: 315–20. Wilcox D (1994) ‘Cleft palate rehabilitation: interim strategies in Indonesia’, Cleft Palate Craniofacial J 31: 316–20. Wirt A, Wyatt R, Sell D, Grunwell P & Mars M (1990a) ‘Training counterparts in cleft palate speech therapy in the developing world: a report’, Cleft Palate J 27: 169–73. Wirt A, Wyatt R, Sell D, Grunwell P & Mars M (1990b) ‘Training counterparts in cleft palate speech therapy in the developing world: an extended report’, Europ J Dis Commun 25: 355–67. Wirz S & Hartley S (1999) ‘Challenges for universities of the north interested in community based rehabilitation’, in Stone E (ed.) Disability and Development: Learning from Action and Research on Disability in the Majority World, Leeds: The Disability Press, pp. 89–106. Yeow V, Lee-Seng-Teik T, Lambrecht-Thomas J, Barnett J, Gorney M, Hardjowasito W et al. (2002) ‘International task force on volunteer cleft missions’, J Craniofacial Surg 13: 18–25.

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ENT and Audiology Care for Cleft Palate Patients in the Developing World TONY SIRIMANNA

Editorial comment: This chapter provides an overview of the common ENT and audiological problems associated with cleft palate in the Developed World, contrasted with the types of problems seen in the Developing World. It is not surprising that audiological services are particularly difficult to deliver in a Developing World context, given the triple problem of a lack of highly trained specialist personnel, specialist testing environments and equipment, coupled with the high expense of each of these. Suggestions are given as to how to manage hearing loss in the absence of specialist care.

INTRODUCTION Ear Nose and Throat Surgery (ENT) is a well-established speciality in some Developing Countries although it is not uncommon to see this service provided by single-handed specialists. In those Developing Countries where there are not enough ENT surgeons, general surgeons tend to provide some ENT care. With the shortage of ENT surgeons, the super-specialization that is normally seen in most ENT units in the Developed World is not found in the Developing World. Further, because of the fewer number of ENT surgeons in some Developing Countries, it is possible to find one specialist covering a population of over a million. Most specialists in Developing Countries tend to have postgraduate qualifications from countries such as the United Kingdom, France or the USA, although a few countries have now developed their own training programme, e.g. Sri Lanka, Ghana. In the Developing World, ENT teams are not always supported by audiology services (O’Driscoll, 2005) and often when there are Audiology Departments, these are located in major hospitals. It is not unusual for an ENT Department to function without any audiology support in peripheral hospitals. Further, in some Developing Countries

Management of Cleft Lip and Palate in the Developing World. Edited by Michael Mars, Debbie Sell and C 2008 John Wiley & Sons, Ltd Alex Habel.

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trained audiologists are either unavailable, such as in Zimbabwe (Chidziva, 2003), or limited to one or two per hospital. Examples of this come from Kenya (Macharia, 2003) where there are only 25 qualified ENT surgeons, five audiologists and four speech and language therapists for the whole country, based mostly in and around Nairobi. In Malawi, there is only one ENT specialist for a population of 12 million in spite of the fact that up to 6.7% of the population have chronic otitis media (Van Hasselt, 2003). Another huge challenge for audiology is lack of resources. Soundproof rooms in which hearing tests are carried out are very expensive to build and hence beyond the budgets of most hospitals. In the Developing World, audiological equipment is often limited. Although almost all the audiology departments have equipment for pure tone audiometry, not all units have facilities for impedance measurements (tympanometry). Equipment for objective testing of hearing such as auditory brainstem responses and otoacoustic emissions, that are standard procedures in the Developed World, are not usually available mostly because of the high cost, and when available, there is often no mechanism for regular maintenance. Often calibration facilities for audiological equipment are not available locally and therefore the accuracy of audiometric measurements cannot be guaranteed. Most importantly, however, there are usually very few audiologists who have had training in the use of equipment and therefore objective tests of hearing are rarely used in many Developing Countries. Not surprisingly, perhaps, teamwork is not often found in the Developing World especially in the way it is practised in the Developed World. Where established, multidisciplinary teams are usually led by doctors with the rest of the team following the lead and not functioning as equal partners. However, there are a few genuine examples of teamwork in some countries, e.g. Sri Lanka, Pakistan, Nigeria and the concept is gradually being accepted.

ENT AND AUDIOLOGICAL PROBLEMS IN PATIENTS WITH CLEFT PALATE ENT PROBLEMS In non-syndromic patients with cleft palate, the main ENT problem is otitis media with effusion (OME) (synonyms: middle ear effusions, ‘glue ear’, non-suppurative otitis media) (Dhillon, 1988; Albert et al., 1990; Handzic-Cuk et al., 2001), and this is mostly temporary and treatable. However, there are a few patients, mainly syndromic, who have permanent sensorineural or mixed (conductive and sensorineural) hearing loss (Webster & Eldis, 1978; Reyes et al., 1999). These syndromic patients may also have other ENT problems such as microtia, meatal atresia and choanal atresia. It should be noted that non-syndromic children with cleft lip only do not run any additional risk of having OME compared to non-cleft children (Sheahan et al., 2003). However, syndromic children with cleft lip may have a hearing loss related to the particular syndrome.

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In the Developed World, OME in children without cleft palate is a common problem with an incidence as high as 40–60% in the 1–6 year age group (Lous et al., 2005). In the majority of these, OME resolves without any treatment within a few months and only a small percentage require surgical intervention (Cassellbrant et al., 1985; Zielhuis et al., 1989). Surgery is mainly limited to inserting middle ear ventilation tubes (grommets) with recurrence of OME requiring re-insertion of grommets often accompanied by surgical removal of adenoids (Mills, 1996; Maw, 1983). Medical treatment of OME, including antibiotics, antihistamines, decongestants and autoinflation, is usually not successful. In children with cleft palate, the incidence of OME can be as high as 90% (Dhillon, 1988). Further, in this group of children, OME is much less likely to resolve spontaneously compared to patients without cleft palate, and palate repair does not necessarily lead to its resolution (Dhillon, 1988). However, in some of these patients middle ear function improves spontaneously as they get older (Timmermans et al., 2006), while others may develop chronic suppurative otitis media or cholesteatoma (Sheahan et al., 2002, 2003, 2004). Zanzi et al. (2002) conducted a small study in which children who had cleft palate repair at the age of 6 months were subsequently reviewed at 3.6 years of age. They showed that early repair of the palate is likely to result in better hearing and speech. This has not, however, been supported by most other studies exploring the otological and audiological outcome following cleft palate repair by various surgical techniques (Guneren et al., 2000; Sheahan et al., 2004). Further, Nunn et al. (1995) found that very early closure of cleft palate did not alter the subsequent need for grommets. The function of the Eustachian tube (ET) is to normalize the pressure within the middle ear cavity. When there is dysfunction of the ET, the pressure drops leading to accumulation of an effusion within the middle ear. ET dysfunction could be due to inflammation of the tubal mucosal lining from recurrent upper respiratory tract infections or upper airway allergy, congenital narrowing of the Eustachian tube due to developmental reasons (especially in syndromic patients) or dysfunction due to poor muscle control. The levator palatine muscles which usually help to ‘open’ the ET often do not function well in patients with cleft palate, leading to a sustained drop in the middle ear pressure resulting in OME (Arnold et al., 2005). Therefore, patients with cleft palate show a significantly higher incidence of OME with very little possibility of spontaneous resolution (Tuncbilek et al., 2003). Hence proactive management of OME is required in cleft palate patients in order to maintain normal hearing (Paradise et al., 1969) and follow-up at agreed regular intervals is important. Early detection and management of OME are essential in order to avoid complications such as chronic middle ear disease including cholesteatoma (Goudy et al., 2006). In the Developing World, OME is not a common ENT problem as shown in a study in Jamaica by Lyn et al. (1998), and in Nigeria by Ogisi (1988). In fact, acute ear infections (acute otitis media – AOM), chronic discharging ears with perforations (chronic suppurative otitis media – CSOM), and mastoid disease such as cholesteatoma, are more frequent. However, with improvements in health care in the Developing World

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and the use of antibiotics to treat acute infections in the early stages, there has been a reduction of chronic middle ear disease during the last two decades or so, but interestingly, this has also been associated with an increase in the incidence of OME (Sirimanna T, unpublished observation; O Sei Lah V, personal communication). AUDIOLOGICAL ISSUES The hearing loss found in non-syndromic patients, including those with submucous cleft palate, is usually conductive and of mild to moderate degree of severity. However, in syndromic patients with cleft palate, there may be either a permanent conductive hearing loss due to external meatal atresia or fixation of middle ear ossicles, a sensorineural hearing loss due to cochlear involvement, or a mixed hearing loss due to a combination of the above. These could be of varying degrees of severity. For example, Reyes et al. (1999) showed that in a cohort of 166 patients with 22q11DS, 44% had hearing loss and 11% of these had a sensori-neural hearing loss. In another study of 98 patients, with cleft palate with or without cleft lip, Webster and Eldis (1978) found an 8% incidence of sensori-neural hearing loss. There is also evidence of auditory processing difficulties in children with cleft palate. Ceponiene et al. (2000) investigated pre-attentive auditory discrimination in patients with cleft palate using the mismatch negativity component of the auditory evoked response potentials and found smaller responses from babies with cleft palate compared to normal healthy babies suggesting poor discrimination abilities.

CHALLENGES FOR ENT/AUDIOLOGICAL MANAGEMENT IN THE DEVELOPING WORLD It is a well-recognized fact that the early diagnosis of hearing loss and appropriate management leads to a better outcome for the child (Yoshinaga-Itano et al., 1998). Most audiology services in the Developed World run universal newborn hearing screening programmes (NHSP) while others continue with targeted newborn hearing screening. Some Developing Countries also have established targeted hearing screening at birth while a few are in the process of developing universal NSHP, e.g. Nigeria (Olusanya et al., 2007), or Albania (Hatzopolous et al., 2007). Therefore, in those countries where there are universal or targeted NHSP, babies born with a cleft palate have a hearing assessment soon after birth, leading to early identification and appropriate management. In the Developing World hearing loss (deafness) is not often considered a priority compared to more pressing conditions such as malaria, typhoid, malnutrition, HIV/AIDS, or mental health (WHO, 2004–2006). As previously stated, specialist audiology services are frequently unavailable (O’Driscoll, 2005). Where there are no newborn hearing screening programmes, patients usually present late with significant otological disease and hearing loss that could well have been prevented if detected early.

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ACCESS TO SERVICES IN THE DEVELOPING WORLD The Developing World has a comparatively small number of physicians per head of population. A significant proportion of people live in rural areas with poor transport links and long travel distances to centres where the medical facilities are available. This has led to the emergence of ‘local doctors’ such as witch doctors in Africa and devil dancers in Sri Lanka (Obeysekera, 1969) and other alternative methods of treating patients with a particular condition or ailment. These alternative approaches to managing medical problems are another reason for late presentation of otherwise treatable conditions, usually presenting with complications that lead to inevitable deficits and poor outcomes. Some countries in the Developing World, with the help of the World Health Organisation (WHO) (Prevention of Deafness and Hearing Impairment: www.who.int) and other organizations such as Hearing International (www.hearinginter.com) and Commonwealth Society for the Deaf (HARK project) (Ogilvy & Michelson, 2003), have established screening, health education programmes and satellite clinics to address this issue (Olusanya et al., 2004). In certain cultures in the Developing World, having a child with a deformity or a disability including deafness may be considered as a ‘curse’ and often the mother is blamed for this (see Chapter 13, Bradbury and Habel). It is possible for these children not to be accepted into a local school because of lack of facilities in catering for the child’s needs. Furthermore, because the closest school for the deaf is often a significant distance away, these children are usually kept at home leading to illiteracy and poor speech and language development. The reader is also referred to Wickenden (see Chapter 12) for information on community-based rehabilitation which clearly has relevance to individuals with hearing loss. Establishing a culture of teamwork in its truest sense, involving ENT, audiology, teachers and where possible, speech and language therapists, gives an opportunity to address some of these issues. It raises the importance of a multidisciplinary holistic approach to care, putting together vital specialities that are involved in the joint management of the cleft child to achieve the maximum benefit from interventions.

ENT AND AUDIOLOGICAL ASSESSMENT AND MANAGEMENT OF THE CHILD WITH CLEFT PALATE Early identification of hearing loss and any treatable conditions such as OME is vital in optimizing the speech, language and cognitive development of the child, and more so than when speech and language development is put at risk by the condition of cleft palate. This is arguably even more important given the often delayed surgery in the Developing World, the development of speech with an unrepaired palate and associated severe speech disorders (see Chapter 15, Sell). Shaw et al. (2003) in a 10-year retrospective study of 109 consecutive patients with cleft palate found that those receiving grommets did have a better speech and language outcome. In addition, there is considerable evidence to support the view that

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early intervention for significant hearing loss gives rise to better speech and language outcomes (Ramakalawan & Davis. 1992; Robinshaw, 1995; Yoshinaga-Itano et al., 1998). It is therefore extremely important that newborn babies with cleft palate are referred for an audiological and ENT assessment as early as possible. A careful history to include the pregnancy, birth and the immediate postnatal period should be taken in order to detect any factors that may have affected hearing. This should include history of ototoxic medication and hearing loss in both sides of the family. Full clinical examination of the child is vital to detect any congenital developmental abnormalities not only associated with the head and neck, but also involving the limbs, cardiovascular and renal systems, and the skin. In particular abnormalities of the pinna, ear canal, eyes and face including ear pits and branchial sinuses should be excluded. Deep external meatal atresia can be overlooked unless examined properly using a magnifying otoscope or an operating microscope.

AUDIOLOGICAL ASSESSMENT Audiological assessment in the newborn is carried out mainly by using objective measures, although objective tests of hearing can be used on a child of any age. These include tympanometry to help diagnose middle ear effusions (OME), transient evoked or distortion product otoacoustic emissions (TEOAE or DPOAE) to record the outer hair cell activity when the middle ear is clear of OME, and Auditory Brainstem Evoked Response Audiometry to determine the threshold of hearing under natural sleep. TYMPANOMETRY (ACOUSTIC IMPEDANCE TEST) Tympanometry (acoustic impedance test) is a simple test that involves placing a probe in the ear canal and gives a measure of middle ear compliance and pressure. A high frequency probe tone is used in babies up to about 4 months of age, compared to a 256 Hz probe tone used in older children. A flat graph (Figure 17.1) indicates presence of an effusion in the middle ear or OME. OTOACOUSTIC EMISSIONS (OAES) Otoacoustic emissions (OAEs) are tiny sounds generated by the outer hair cells, when external sounds reach the inner ear. A proportion of these generated sounds leak back through the middle ear and are recorded by a probe placed in the ear canal and displayed on a screen. The cochlea is fully developed by about 32 weeks of gestation and the presence of transient evoked otoacoustic emissions (TEOAEs) suggests intact outer hair cells. Over 99% of these babies with TEOAEs have normal hearing although a small number, especially those who have had perinatal problems such as hypoxia and jaundice (less than 1%), may still have a sensori-neural hearing loss, e.g. auditory neuropathy. In these babies, a full audiological assessment is essential to detect the degree and nature of hearing loss.

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TEST 2 Ytn 1000 Hz R

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–200 0 +200 600/200 daPa/s EARCANAL VOLUME: 0.7 daPa mnho TYMP 1: TYMP 2: TYMP 3:

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Figure 17.1. Tympanometry test result showing an effusion in the middle ear.

BRAINSTEM EVOKED RESPONSE AUDIOMETRY (BERA) Brainstem Evoked Response Audiometry (BERA, synonym: Auditory Brainstem Responses – ABR) requires placing a few electrodes on defined locations on the scalp/forehead/mastoid region to record the electrical response generated within the auditory neural pathways in the brainstem as a response to a sound that could be a click or a tone. The methodology is described as a protocol at the UK National Newborn Hearing Screening Programme site (http://hearing.screening.nhs.uk). In a term baby, on average, it takes about 4–6 weeks for the auditory pathways to mature (myelinate) up to the brainstem. However, in some babies auditory pathways are fairly well myelinated up to the brainstem at term, although in others the process may go on for up to a year from birth (Moore et al., 1995). Therefore increased BERA threshold during the first few weeks of life does not always indicate a permanent hearing loss and hence it is important to repeat this test when the baby is 4–6 weeks old. The aim should be to complete the hearing assessment before the child is about 3–4 months of age. Apart from leading to early amplification for significant permanent hearing loss, this provides an opportunity for the team to consider inserting grommets during early surgical procedures for cleft lip and/or palate. Andrews et al. (2004) suggested testing babies around the age of 3 months and inserting grommets approximately at the age of 6 months under the same anaesthetic for cleft palate surgery, when there is a hearing loss of more than 55dB in the better ear on ABR (BERA) testing. Reassessment of hearing following grommet insertion is essential in these children in order to ensure that there is no residual permanent hearing loss due to fixation of the middle ear ossicles or to inner ear causes.

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Children aged 7 months and over can be tested using behavioural hearing tests. These include distraction tests (DT), visual reinforcement audiometry and various speech based hearing tests. The DT is based on the principle that a child is able to locate a sound presented in a horizontal plane (by turning towards it). Sounds used for this test have reasonable frequency specificity and therefore the presence of a response indicates the child’s hearing at a frequency range. By changing the intensity of the sound the threshold of hearing can be determined. As the sounds are presented in a sound field and heard in both ears, it is the better ear hearing thresholds that are recorded by this test, although experienced testers are able to determine whether there is an asymmetry of hearing between the two ears. VISUAL REINFORCEMENT AUDIOMETRY (VRA) Visual reinforcement audiometry (VRA) works along the same principles but has a better control of stimuli as sounds are presented using an audiometer through a speaker, insert earphones and bone conduction transducer. The child is rewarded for making a timely response to a sound using a visual reward. When carried out using insert earphones, ear-specific hearing thresholds can usually be obtained. Both the DT and VRA can be carried out on children from the age of 6 months to 24 months. Usually 6-month-old babies need slightly louder sounds to initiate a response while older children may inhibit the turning response, especially if the stimulus is repeated. Children over the age of 3 years should be able to carry out play audiometry. This is where the child performs a task, e.g. putting a man in a boat, peg on a board or brick in a basket, as a response to a frequency-specific sound presented in a sound field or through headphones. Those children who are developmentally older for DT or VRA but not ready for play audiometry form a group who are not easy to test. A number of speech-based tests are used in this age group where the child is asked to perform a task according to the instructions given by the tester, e.g. ‘Give this to daddy.’ Using a choice, for example, between ‘daddy’, ‘mummy’ and ‘teddy’ some indication of the child’s hearing levels can be determined. These tests have been developed in non-Englishspeaking countries using phonetically similar words (e.g. in Sri Lanka). Those with moderate, moderate to severe and severe hearing loss (WHO classification of hearing loss) are most likely to benefit from amplification. Although detailed discussion of hearing aid selection and fitting is beyond the scope of this chapter, it is important to use accepted processes of providing amplification including hearing aid fitting formulae ensuring that the child has good aided hearing (Sirimanna, 2002). The aim is for the child to have access to normal conversational speech with hearing aids. Most deaf people in the Developed World, if not all, use digital hearing aids for providing amplification. These aids have an additional advantage of having various noise cancellation and speech enhancement systems over the analogue hearing aids. The price of digital hearing aids has come down significantly and it is possible that soon these will be affordable to families in the Developing World. However, these

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comments are in the context of an appreciation that most Developing Countries do not provide a comprehensive hearing aid service. Even when hearing aids are provided, a significant proportion of parents cannot afford the batteries, cost of repairs and replacement. Although some authors have preferred amplification rather than surgical intervention as the first line management of significant hearing loss in cleft patients (Maheshwar et al., 2002), the above factors may make this unrealistic in the Developing World. There has been some move towards developing solar-powered hearing aids (Parving & Christensen, 2004) and solar energy rechargeable batteries for hearing aids (IMPACT – www.impact.org.uk). These solar-powered hearing aids are still at a very early stage and bulky, and certainly not very attractive to wear, especially to teenagers. In addition to resolving the issue with hearing aid batteries, there is a pressing need to develop cheaper, good quality, cosmetically acceptable, and cheaper to maintain, hearing aids for the Developing World. In October 2006, the World Health Organisation (WHO) and Worldwide Hearing (WWHearing) agreed to work together to enable provision of affordable hearing aids in the Developing World (WHO, 2004) to address some of these issues. In the absence of sophisticated equipment, soundproof rooms for testing, access to ENT and audiology services and hearing aids, managing hearing loss in cleft palate children becomes a challenge and will depend on parental and relatives’ observation of the child’s responsiveness to sound or lack of speech development. Children are normally well over 12 months of age when such concerns are raised. Alternatively it may be possible to develop a reasonably sound-treated room using imagination and adaptations. It is helpful to find a very quiet room in the hospital or elsewhere, with double doors if possible. Ideally the windows need to be adapted to have rubber seals around their edges. The judicious use of curtains, carpets and a ceiling covered with cloth to absorb sound will also contribute to making a more appropriate testing environment. Children who are developmentally 7 months of age or over can then be tested using behavioural tests such as the distraction test (see above) or performance tests. Physicians can be trained to perform pneumatic otoscopy that will help to determine the lack of movement of the eardrum seen in middle ear effusions. If grommets are not available, myringotomies alone will provide a temporary period of hearing improvement. Older children with hearing loss, in the absence of a conventional hearing aid, can be managed with preferential seating (nearer the teacher) or using amplified voice through a simple amplifier and a set of headphones. However, these are far from ideal measures and therefore every country should aim to develop good audiological and ENT care for children with cleft palate.

HEARING IN THE SCHOOL SETTING Hearing is a vital sense that helps children to develop oral language and optimize their educational potential. Those children with milder hearing losses usually do not require amplification. They may have difficulties with hearing in the classroom

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setting or when there is background noise. Such difficulties should ideally be identified early so that appropriate measures such as preferential seating and FM (Frequency Modulated) amplification systems can be used to help them. Further, most children with hearing aids require additional support at school by trained teachers and/or speech and language therapists. In a significant number of Developing Countries these professionals are either not available or scarce. Most children with hearing aids in the Developing World are sent to specialist schools for the deaf where there is little integration with normally hearing children and therefore may not develop speech and language even if this is potentially possible.

CONCLUSION ‘Glue ear’ (otitis media with effusion or OME) is the commonest otological problem in patients with cleft palate but a small number of, especially syndromic, cleft palate patients have sensory neural or mixed hearing loss as well. Early detection of this hearing loss, together with appropriate management coordinated by working as a multidisciplinary team, is essential in order to maximize educational potential and speech and language development, and minimize chronic middle ear disease with its long-term consequences. In the Developing World, ENT and audiology services are well developed in some countries and non-existent in others. In most Developing Countries these are only available in major teaching hospitals. Grommets for surgical management of ‘glue ear’ and hearing aids for hearing losses that cannot be managed by other means are not readily available in the Developing World. Lack of skilled professionals, financial/resource limitations and different health priorities all contribute to this situation. All these factors pose a number of challenges related to the ENT and audiological care of children with cleft palate. It is therefore important for Developing Countries, where there are sub-optimal ENT and audiology services, to establish appropriately trained multidisciplinary teams in a few specific locations to provide a holistic service for cleft palate patients. This should be supplemented by training health-care workers to identify ENT and audiology problems associated with cleft palate, and refer these patients to such centres. Establishing satellite clinics based on a ‘hub and spoke’ model, perhaps with mobile audiology facilities such as those used by HARK in Africa (Ogilvy & Michelson, 2003), can provide a viable solution in those areas where the transport links for the patients are not good. Cheap hearing aids, possibly solar-powered or run on batteries recharged using solar energy, would be a cheaper practical solution to those needing amplification. Developing easily administered screening tests of hearing for each country is an essential part of this process and will help to minimize the costs and improve the yield, related to early identification of cleft palate children who require intervention.

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Index

22q11 deletion syndrome 131, 132 Abbe flaps 40, 44 access to care 32 acetaminophen see paracetamol acoustic impedance test 208 activity, definition 147 acute otitis media (AOM) 205 aesthetic anxiety 153 AIDS see HIV/AIDS albuterol 92 alveolar bone grafts 44 American Cleft Palate-Craniofacial Association (ACPA) 12, 37, 181 American Heart Association 65 amoebiasis 140 amoxycillin 138 anaemia 51, 131, 136 anaesthesia 49–55 complications 55 conduct 52–4 essential equipment 50–1 intra-operative 53 local 52 patient selection and preparation 51–2 post-operative recovery and complications 54 sedation 52 tracheal intubation 53 ventilation in children 53 visiting teams and training 55 anaesthetist 44, 50, 55, 132, 137, 138 anesthesiologist 65, 69, 74, 76, 88, 89, 91, 92 Annenberg Foundation, Shanghai 66 antibiotics 136–7, 142 peri-operative intravenous 139 anxiolytics 139 application process 87–8 aspirin 131 assessment of patients 88–9, 168–9 asthma 128, 131 atelectasis 132

atropine 52, 53 attitudes towards work 25–6 audiology in the Developing World 203–12 access to services 207 assessment 208 cleft palate, assessment and management 207–8 lack of resources 204 management challenges 206 Auditory Brainstem Responses (ABR) 209–10 belief system 159–60 beliefs about causation 161–2 blood transfusion service 137 Brainstem Evoked Response Audiometry (BERA) 209–10 breast feeding 54, 133, 153 breast milk 127, 133, 161 bronchospasm 132 bullying 164–5, 168 psychological intervention 170–1 bupivacaine 52, 53 buretrols 143 campylobacter 140 cardiac murmurs 131–2 care post-operative 50, 93 pre-operative 138–9 causation, beliefs about 161–2 cefazolin 139 ceftriaxone 137, 139 cephalometric assessment 116, 117, 118, 119 cerebral palsy 128, 151 Chagas’ disease 136, 137 Chang Gung Memorial Hospital 18–19 Charter for Disabled Persons’ Rights 153 Chief Intensivist, role of 137 Chief Pharmacist, role of 136–7 choanal atresia 204 cholera 88 cholesteatoma 205 chronic discharging ears with perforations 205

215

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216 chronic suppurative otitis media (CSOM) 205 ciprofloxacin 140 civility 26 Cleft Care website 176 Cleft Lip and Palate Association (CLAPA UK) 173, 174, 176 Cleft Lip and Palate Parent Association (Sri Lanka) 174–6 clindamycin 138 codeine 139, 142 Comitato Collaborazione Medica (CCM) 16 Commonwealth Society for the Deaf (HARK project) 207, 212 community-based rehabilitation (CBR) 151–2, 155, 156, 195, 196–7 contraindications to surgery 130 cost of surgery to family 162–3 croup 132 Crouzon’s syndrome 39, 65 cultural awareness 26 cultural issues 38 cultural responses to cleft 32, 159–60 customs, local 38 customs formalities 85 deaths 78–9 due to surgery 64, 65 infant 1, 2, 127 delayed onset of puberty 120 dengue 140 dental caries 132, 165 Dermabond® 40 Developing Countries, exporting plastic surgical care to 7–21 challenging the principles 16–17 common principles 13 creation of long-term self-sufficient care 13–14 destination 8 development of medical infrastructure 19 education of local medical professional 19 evolving principles 12–13 example of surgical mission 18–19 existing local infrastructure 13 models of delivery 8–9 organizations 10–12 resource allocation 17 standards of care 14–16 teaching 18 dexamethasone 53, 131 diarrhoea 140

INDEX didanosine 141 disability 128, 145–6 anthropological views 148–9 community-based rehabilitation (CBR) 151–2 community-based support 155–6 definitions 146–8 identity and status in 153–4 illness and 154–5 incidence 149 local understandings of 152–3 medical and social models of 149, 150, 151, 152, 154 service provision and 151 Disabled People International 153 disabled people’s movements 153 disablement, WHO definition 146, 147 distortion product otoacoustic emissions (DPOAE) 208 distraction 44–5 distraction test (DT) 210 domperidone 92 dress 26, 88, 137 education and training access to 32, 33, 161, 164, 167–8 of local medical professional community 19–20, 27–8, 84, 94 of surgeons 33–4 visiting teams and 55 employment 166, 168 encephaloceles 65 endocarditis 138, 139 ENT in the Developing World 203–12 access to services 207 in cleft palate 204–6, 207–8 management challenges 206 problems 204–6 epidemiology, data collection 28 equipment availability 89 equipment sterilizer volunteer 87 ether 51, 53 Eurocleft Study 34 existential anxiety 153 facial growth 115–21 facilities 38, 49–50 review of 82–3 fentanyl 53, 91, 136 final celebration 94 First Arch Syndrome 107 fistulas 39, 41, 43 follow-up 34

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217

INDEX formula feed 127, 133 Furlow palate repair 41 gastritis 140 gastro-enteritis 32 gender issues 162, 167 genetic counselling 130 gentamycin 139 giardiasis 140 Glostavent 50 Glucose-6-Phosphate Dehydrogenase (G6PD) 136 glue ear see otitis media with effusion (OME) goals of mission programs 81 Goslon Yardstick 116, 118–19 grommets 205, 212 growth impairment 134, 135 guilt 160 Haemocu® 133 haemophilia 131 Haiti, plastic surgical care in 9–10 halothane 51, 52, 53, 55 HARK project 207, 212 Head Nurse, 76, 89, 90, 91, 136 hearing aids 211 hearing in school setting 211–12 Hearing International 207 hearing loss 206 Hemocue® 52 Hepatitis A 25 vaccination 88 Hepatitis B 25, 137, 140, 141 vaccination 88 Hepatitis C 137, 141 HIV/AIDS 32, 50, 133, 137, 140, 141, 160 hospital dress protocols 137 host responsibilities 83–4 hydrocortisone 131 hypertelorism 65 ibubrufen 139 identification of patients for surgery 129 impairment, definition 147 infant mortality 1, 2, 127 Interhealth 25 International Plastic Reconstructive and Aesthetics Society 12 Interplast 10, 179, 180 inventory 85–6 isoflurane 51 ketamine 51, 52, 53

labelling, child 138 laboratory investigations 133 lamivudine 141 language barriers 50 laryngomalacia 132 laryngospasm 132 learning difficulties 151 LeFort I osteotomy 44 Leishmaniasis 137, 140 lignocaine 52 liminal states 148 lip adhesion 40 lip repair 40–1 lip taping 40 lip/palate repair 41–2 local infrastructure 13 local surgeons 39–40 local volunteers 77 long-term results 34–5 malaria 32, 51, 119, 132, 137, 140, 160 prophylaxis 25, 140 malnourishment 51 malnutrition 119–20 Marcaine 38 marriage prospects 32, 33, 148, 162, 166–7, 168 maxillary retrusion 115 Goslon Yardstick 116, 118–19 of lip and palate repair group 117 of lip repair group only 117 timing of hard palate repair and 119 unoperated cleft and 116–17 McKay Nursing School 19 meatal atresia 204 Medical Director 86, 87, 89, 90, 92 medical history 130–1 medical model of disability 149, 150, 151, 152, 195 medical record keeper 77, 87, 88, 90, 92 medical supplies 85 medication 91–2, 131 mental health 167 Mercy Ships 2 methicillin 139 metronidazole 140 microtia 204 midazolam 52, 91, 136, 139 middle ear effusions see otitis media with effusion (OME) Millard lip repair 40 mini-laryngo-bronchoscopy (MLB) 132

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218 mission 2, 13, 14, 19, 34, 37, 41, 42, 43, 45, 81 Mission Director 76, 86–7, 88 money 28 morphine 53, 91, 136 Motrin 92 multidisciplinary teams 33, 35 nasoalveolar molding 40 needlestick injuries 140 needlestick protocol 137 neostigmine 51 newborn hearing screening programme (NHSP) 206, 209 nil by mouth (NPO) guidelines 138 non-governmental organizations (NGOs) 3 non-suppurative otitis media see otitis media with effusion (OME) Noordhoff, Samuel 18–19 notification of impending visit 128–9 NSAIDs 53, 54, 139 nutrition 42, 127, 128, 133 onset of puberty, delayed 120 operating room set-up 90 Operation Smile 9, 10–11, 26–7, 55, 59–68, 179, 180 chronology of milestones 62–4 deaths 11, 64–5 education 65–6 Flying Hospital 63–4 fundraising 63, 66 future 66–7 governance 60–2 IT 67 long-term goals 67–8 problems and re-evaluation 64–5 student programs 66 volunteerism 67–8 World Care Program 65 World Journey of Hope 63–4, 67 World Journey of Smiles 67 orthognathic surgery 44 otitis media 132 acute (AOM) 205 chronic suppurative (CSOM) 205 with effusion (OME) 204–5, 206, 207, 208, 212 otoacoustic emissions (OAEs) 208 distortion product (DPOAE) 208 transient evoked (TEOAE) 208 outcomes 34 Oxford Inflating Bellows 51

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INDEX packing 94 PACU 93, 139 nurses 89, 90–1 set-up 90–1 paediatric care 127–43 paediatrician 13, 101 hospital staff 136, 137 list of 142–3 role of 129 as team physician 140–1 pain management, post-operative 139–40 palate repair 41 paracetamol 52, 53, 54, 139, 142 parachute missions 34, 35 Parents/Patients Support Group in Sri Lanka 173–6 participation, definition 147 Partners in Health 9 passive plates 40 patient assessment 51, 88–9 patient profile 84 patient selection 73–4, 89, 130 patient transporter 87 pediatrician 69, 76, 86 penicillin 138 peri-operative intravenous antibiotics 139 persistent ductus arteriosus 131 pethidine 53, 91 pharmacies 136–7 pharyngoplasty 39, 42, 106–7, 188 Philplast 59 photojournalist 87 Pierre Robin sequence (PRS) 39, 51, 107, 128, 130, 132 play audiometry 210 pneumonia 1, 132 polio 107 Post-Anaesthetic Care Unit see PACU post-operative assessment 163 post-operative care 50, 93 post-operative clinic 94 post-operative pain management 139–40 post-operative recovery and complications 54 power 28 prednisolone 131 pre-operative area 92 pre-operative assessment 51, 130 pre-operative care 138–9 pre-operative clinic 88–90 pre-operative sedation 138 pre-operative team assessment 39 preparation for visit 85–6, 128–9

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219

INDEX pre-surgical evaluation 128 pre-surgical work-up 129 priorities 32 professionalism 27 Project Cure 85 prophylaxis 140 prosthodontic management 45 protocols 35 psychological assessment of cleft surgery 168–70 psychological effects of cleft 32–3 quality of life 150, 166 quartermaster 76, 87, 90 questionnaire 161, 169–70 recovery room helper 87 recreation therapist 87 recruitment of surgeons 33 ‘Red Man’ syndrome 139 reflex microscopic analysis 116 rehabilitation 151 relaxation 26 religious effects of cleft 32 rest 26 rest day 93 rhinoplasty, secondary 43 Rigid External Distraction (RED) appliance 44 risk assessment 130 Rotaplast International 69–79, 86, 100, 138, 179 anesthesia 78 clinic triage 74 complications of repair 78–9 cost per child 70 cost per mission 70 differences from other organizations 72 education 72–3 establishing cleft teams 77 fatality 78–9 goals and vision 70–1 governance and management 69–70 history 70 incidents with volunteers 79 medical missions 73–5 medical team 75–6 mission team 75–7 non-medical team 76 occurrence and prevention of complications 78 organizational goals 71 partnership with Rotary Clubs 73 patient selection criteria 73–4 programs 72–3 research 72

schedule 74–5 site selection 73 surgery complications 78–9 Rotary Club 73, 86 safety 27 salbutamol 92 salmonella 140 Sanyatha 174, 175–6 scar revision 44 schistosomiasis 140 secondary cleft problems 42 self-perception 165 self-protection 141 shame 160 shigella 140 site needs assessment 82–4 Smile Train, The 9, 11–12, 20, 27, 179 Smile Train Express 11 Smile Train Medical Exchange Program 11 social ease 166 social effects of cleft 32–3 social model of disability 149, 150, 151, 152, 154–5, 195 speech and language therapy (SLT) 180, 187, 193–200 CBR workers 196–7, 199 counterpart/assistants training 196, 199 history of involvement in Developing World 180 indigenous therapists 197–8 intervention 185 need for 180–1, 189 parents and families 199 speech outcomes 165, 179–89 age 189 assessments 184 cleft type 189 cross-linguistic issues 183 evidence base for 181–2 in children 186, 187 in older patients 183–4, 186–7, 189 nature of surgery 188 pharyngoplasties 188 post-operative velopharyngeal function 185–6 results 184–5 Sri Lanka, experiences of individuals with clefts in 164–7 bullying 164–5 education 164 employment 166

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220 Sri Lanka, experiences of individuals with clefts in (Continued ) gender issues 167 marriage 166–7 mental health 167 quality of life 166 self-perception 165 social ease 166 speech outcomes 165 surgical prioritization 167 Sri Lankan Cleft Lip and Palate Project 95–109 aims 108–9 appraisal 108–9 development 95–6 Stage 1 (1982–85) 96–9 aims 98 equipment 98–9 first planning meeting 98 funding 98 project development 97–8 reconnaissance visit (1984) 97 recruitment of patients 99 team structure 99 Stage 2 (1985–90) 99–105 administrative difficulties and local/national medical politics 103–4 investigations 101–2 modifications to original plans 102 priority for treatment 100–1 surgical and assessment/research team visits 99–100, 103, 105 training 102 Stage 3 (1990–99) 105–7 difficulties within team 106 long-term follow-up and research data collection 105 patient triage 107 pharynogoplasties 106–7 surgical craft disputes 107 team dynamics 105–6 team selection 106 Stage 4 (1999–the present) 107–8 extension of project to Peradeniya (Kandy) and Colombo 108 training of speech therapists 197–8 Sri Lankan Cleft Lip and Palate Project facial growth study 115–21 background 116 Goslon Yardstick 116, 118–19 malnutrition, growth and delayed onset of puberty 119–20 Research Archive 116, 121

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INDEX results 116–17, 119 speech implications 120–1 timing of hard palate repair 119 staffing availability 49–50 stavudine 141 Stickler Syndrome 107, 132 stigma 148, 149, 154, 159, 164 in Uganda 161, 162, 163 suicide 167 surgeon 8, 10, 11, 15, 17, 19, 25, 31, 33–4, 39–40, 71, 72, 76, 88, 89, 100, 107 surgery days 92 surgical prioritization 167 surgical safari 3, 23, 27 suture removal 40 suxamethonium 51, 53 Swiss Army® knife 38 syphilis 137 team, liaison within 137 team selection 86–8 medical personnel 86 non-medical personnel 86 non-medical volunteers 86–7 techniques 35 thalassaemia 136 thiopentone 52 tick-borne spotted fever 140 timing of trip 24 tongue flap fistula closure 43 training see education and training Transforming Faces 179 transient evoked otoacoustic emissions (TEOAE) 208 translator 77, 87 travel hazards 140 Treacher Collins syndrome 39 tuberculosis 32 two-flap palatoplasty 41 Tylenol 92, 142 tympanometry 208, 209 type of surgery 39 typhoid 88 Uganda, experiences of individuals with clefts in 160–1 beliefs about causation 161–2 cost of surgery to family 162–3 early responses to birth 161 gender issues 162 lip vs palate repair 163 post-operative assessment 163

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221

INDEX responses of the community 162 role of surgery 163 UN Enable Convention 148 UN International Decade of the Disabled 148 vaccinations 25, 140 Valium 139 vancomycin 139 Van der Woude syndrome 130 Velo-Cardio-Facial syndrome 131 velopharyngeal incompetence (VPI) 39, 43, 188 velopharyngeal surgery 41 Versed 91 visa 24 visual reinforcement audiometry (VRA) 210

Vitamin A 133 Volunteers in Plastic Surgery (VIPS) 12 ward coordinator 87 Wardill-Kilner palatal repairs 35, 118 work permits 24 World Health Organization (WHO) 211 International Classification of Functioning (ICF) 146, 147–8, 152, 155, 156 Worldwide Hearing (WWH) 211 yellow fever 88 ziduvudine 141 zinc supplements 133