Understanding Rett Syndrome

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2 Understanding Rett Syndrome

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4 Understanding Rett Syndrome A Practical Guide for Parents, Teachers, and Therapists Barbro Lindberg Stockholm Institute of Education Department of Educational Research Foreword by Prof. Dr. Andreas Rett, Vienna Published under the editorial supervision of Ludwig Boltzmann Institute for Research on Brain Damage in Children, Vienna, Austria

5 Library of Congress Cataloging in Publication is available via the Library of Congress Marc Database under the LC Control Number Library and Archives Canada Cataloguing in Publication Lindberg, Barbro Understanding Rett syndrome : a practical guide for parents, teachers, and therapists / Barbro Lindberg ; foreword by Andreas Rett. -- 2nd rev. ed. Includes bibliographical references and index. ISBN X 1. Rett syndrome. 2. Rett syndrome--patients--care. 3. Rett syndrome-- Patients--Education. I. Title. RJ506.R47L C by Hogrefe & Huber Publishers PUBLISHING OFFICES USA: Hogrefe & Huber Publishers, 875 Massachusetts Avenue, 7th Floor, Cambridge, MA Phone (866) , Fax (617) ; info@hhpub.com EUROPE: Hogrefe & Huber Publishers, Rohnsweg 25, Göttingen, Germany Phone , Fax , hh@hhpub.com SALES & DISTRIBUTION USA: Hogrefe & Huber Publishers, Customer Services Department, 30 Amberwood Parkway, Ashland, OH Phone (800) , Fax (419) , custserv@hhpub.com EUROPE: Hogrefe & Huber Publishers, Rohnsweg 25, Göttingen, Germany Phone , Fax , hh@hhpub.com OTHER OFFICES CANADA: Hogrefe & Huber Publishers, 1543 Bayview Avenue, Toronto, Ontario M4G 3B5 SWITZERLAND: Hogrefe & Huber Publishers, Länggass-Strasse 76, CH-3000 Bern 9 Hogrefe & Huber Publishers Incorporated and registered in the State of Washington, USA, and in Göttingen, Lower Saxony, Germany No part of this book may be reproduced, stored in a retrieval system or transmitted, in any form or by any means, electronic, mechanical, photocopying, microfilming, recording or otherwise, without written permission from the publisher. Photography by Åsa Eklund Cover photography by Göran Dellskog Printed and bound in the USA ISBN X

6 With my thanks to Ulrika and to all the other girls.

7 Acknowledgements This study was supported by grants from: The Allmänna Barnhus Foundation, The Claes Groschinsky Memorial Foundation, The Folke Bernadotte Foundation, The Frimurare Barnhus Foundation, The Norrbacka-Eugenia Foundation, The Sven Jerring Foundation, and The Sävstaholm Foundation.

8 Table of Contents Foreword ix Preface xi Preface to the Second Edition xiii 1 Introduction 1 Background My Own Background and Personal Inducements Aim Sample Methods A General Outline of the Development and Progress in Rett Syndrome A Brief Presentation of the Individuals in the Study An Account of Typical Symptoms and Behaviors Deficiencies in Perception and Sensory Integration Stereotypic Behaviors Dyspraxia Want of Success Severe Motor Disabilities Difficulties in Coordination Mental Retardation Communication Disorders Emotional Channels Large Fluctuations in Behavior Emotional Reactions Insecure Identity

9 viii Table of Contents 5 Guidelines for Treatment and Teaching Two Principal Teaching Groups Multihandicaps Understanding the Environment Using the Body Interacting and Communicating with the Environment Being Active Expanding One s World Postscript Appendices Questions Forming a Basis for Interviews with Parents Questions Forming a Basis for Interviews with Parents/ Care Givers Questions Forming a Basis for Interviews with School Personnel References Index

10 Foreword Rett Syndrome: A Survey of Psychological and Educational Experiences in Sweden In this volume, with great devotion and impact, Barbro Lindberg presents the clinical picture and treatment modalities of Rett syndrome. The disorder was first described more than 30 years ago. However, only through the incredible activities undertaken by Kathy Hunter in the United States has it received such unusually widespread international attention. Described here is a clinical syndrome that has now been diag nosed in more than 31 countries, and the extent to which the picture is everywhere consistent, is nothing less than remarkable. This is a disorder found exclusively in girls, presenting a particular characterization of neurological symptoms. Today research is being conducted worldwide in search of its cause. Barbro Lindberg s special involvement has been in the day-to-day lives of these girls and women. Having studied the roles of their parents and guardians, she presents her data and discussion with painstaking exactness and detail. This monograph affords a very empathetic view of the situation these parents are in and will certainly be a help in enabling coping with such a difficult chronic situation that concerns not just the mother, but the entire family. I hope this book will be widely disseminated. The reader should note that it offers valuable help with regard to the social, physical, and ethical aspects of dealing with a most difficult congenital disorder. Summer 1991 Andreas Rett, M. D.

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12 Preface Thís report was written to be the book I would have liked to have had when a girl with Rett syndrome was placed in my class at a time when I had no knowledge or experience of the disorder. This student of mine, and my feeling of inadequacy for her, incited me to find out more about the syndrome. I realized that I had to really understand her acting in order to understand how to act myself. This book is primarily intended for people close to those with Rett syndrome; parents and professionals who are trying to make everyday life easier and better for them. It is based on a study of Swedish females, but the conclusions are general and valid to all those with classical Rett syndrome, and so I hope that this English version will find many new readers, and that it will provide an answer to some of the questions you may have. I want to thank Professor Andreas Rett for his generosity and interest in the translation of the book, Dr. Mary Murphy, members of the IRSA, erstellt and many good von friends ciando who helped me with the translation, corrected me, and made the language more enjoyable. And my special thanks to Kathy Hunter, for all her support and understanding. Summer 1991 Barbro Lindberg

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14 Preface to the Second Edition This is a revised version of the book Understanding Rett Syndrome (1991). The book is based on a survey of girls with Rett syndrome, accomplished in Twenty years have passed, and due both to some new medical findings and to a better understanding in schools of the needs of students with severe functional disorders, I have made some changes and amendments to the book. The book is still based on the original survey, but through the years I have gained more and deeper knowledge. I have followed the medical research and developments. I still work with profoundly disabled students, and along the way I have met with new techniques and new pedagogical concepts. I have also made many visits to other schools and preschools, and met new girls, parents, and the people around them. And I am still in touch with Ulrika, my former student. These experiences have all contributed to this new version of the book. I have made some additions to the current text, but the major changes are an extension of the parts concerning intellectual ability and learning, pedagogical method and communication. My aim has been to provide a better theoretical background to the advice and hints I suggest for coping with problems of everyday life. Note that, even though I have had the possibility to partake of findings also from other countries, this book is based only on experiences from girls living in Sweden not due to any value judgments, but simply because I did not consider myself able to give a representative and comprehensive picture of anything else than the terms and conditions of Swedish girls. I wish to give my thanks to the Folke Bernadotte Foundation, the Claes Groschinsky Memorial Foundation, the Norrbacka-Eugenia Foundation

15 xiv Understanding Rett Syndrome and the Sävstaholm Foundation for giving me the opportunity to realize this revision of my book. Thanks also to the staff of Rett Center, for so many fruitful discussions, and to Professor Bengt Hagberg, for so much help in many ways. Thanks to my husband for his never-ending support! And many thanks to Inger for checking my English! Thanks to all parents and personnel for so willingly sharing their findings and sensible reflections. And finally many, many thanks to girls with Rett syndrome for being there and reminding us that there are more than one gauge to choose from when we are trying to measure the values of life! Visby in April 2006 Barbro Lindberg