Developmental
Disability and Ageing

Developmental Disability and Ageing

Edited by
Gregory O’Brien
and Lewis Rosenbloom

2009
Mac Keith Press
Distributed by Wiley-Blackwell

© 2009 Mac Keith Press
6 Market Road, London N7 9PW, UK

Editor: Hilary M. Hart
Managing Director, Mac Keith Press: Caroline Black Project Manager: Sarah Pearsall

The views and opinions expressed herein are those of the authors and do not necessarily represent those of the publisher

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission of the publisher

First published in this edition 2009

British Library Cataloguing-in-Publication data

A catalogue record for this book is available from the British Library

ISBN: 978–1–898683–61–2

Typeset by Keystroke, 28 High Street, Tettenhall, Wolverhampton Printed by The Lavenham Press Ltd, Water Street, Lavenham, Suffolk Mac Keith Press is supported by Scope

To Michael Pountney, who never gave up

Contents

List of Contributors

How to Use this Handbook

1  Overview: Development Disability and Ageing
Gregory O’Brien and Lewis Rosenbloom

2  Dementia in Developmental Disability
Mohammed Nagdee and Gregory O’Brien

3  Ageing in Down Syndrome
Tom Berney

4  Cerebral Palsy and Ageing
Gregory O’Brien, Alf Bass and Lewis Rosenbloom

5  Ageing in Other Syndromes
Marc Woodbury-Smith

6  Drug Treatment for Common Problems among Elderly People with Developmental Disabilities (including Dementia)
Christopher Ince

7  Living with Ageing in Developmental Disability
Claire Middleton and Gregory O’Brien

References

Index

Contributors

Alf Bass, Consultant Orthopaedic Surgeon, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

Tom Berney, Honorary Consultant, Northumberland, Tyne and Wear NHS Trust, UK

Christopher Ince, Consultant in Forensic and Forensic Learning Disability Psychiatry, Northgate Hospital, Morpeth, Northumberland, UK

Claire Middleton, Assistant Psychologist, North Wales NHS Trust, UK

Mohammed Nagdee, Principal Consultant Psychiatrist, Fort England Hospital, Grahamstown, South Africa

Gregory O’Brien, Professor in Developmental Psychiatry, Northgate Hospital, Morpeth, Northumberland, UK

Lewis Rosenbloom, Honorary Consultant Paediatric Neurologist, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

Marc Woodbury-Smith, Assistant Professor, Offord Centre for Child Studies, Hamilton, Ontario, Canada

How to Use this Handbook

This handbook is aimed at clinicians and others who are engaged in caring for ageing adults with developmental disabilities. The text is intended to inform understanding, to promote assessment, to assist in care planning, and especially to improve everyday living for this needy but sadly often neglected group of vulnerable individuals.

The text is not an academic tome. While the statements made are all based on sound evidence, no attempt is made to comprehensively review all the research evidence. The approach taken has been to cite crucial evidence – whether long-established, or new – and to indicate sources of further reading, while focusing on important insights which are likely to be valuable to the clinician interested in the care of the individuals on whose behalf the book has been prepared.

Chapter 1 is a brief general overview of the area. Chapter 2 is a detailed consideration of dementia in the context of developmental disability, including cause, diagnosis, assessment and natural history, with case examples. Chapters 3 and 4 concentrate on two of the most high-profile of all the major groups of developmental disabilities, with their own unique patterns of ageing, Down syndrome and cerebral palsy. Chapter 5 reviews other, less common causal syndromes, and their characteristics with ageing. Chapter 6 is a detailed guide to drug treatment issues in this group. Finally, Chapter 7 considers wider issues of psychosocial intervention and life planning for the ageing individual with developmental disability.

It is likely that the interested clinician will find that the book is merely a pointer to further reading and consideration of the health and social care needs of elderly people with developmental disability. If so, then one of the main aims of the book will have been achieved.

Gregory O’Brien and Lewis Rosenbloom

Chapter 1

Overview: Developmental Disability and Ageing

Gregory O’Brien and Lewis Rosenbloom

Introduction

Children get the service, but adults lead the life.

This sentiment has long been a concern among those who advocate for better care of people with developmental disabilities. To this statement might now be added the concern that:

… the older they get, the worse it can be for them.

Previously, the notion of having a population of older adults with serious developmental disabilities was not widely considered. Most efforts in care, treatment, service development and planning were therefore focused on children. Now, thanks to major recent innovations in health and social care, there is a steadily growing population of elderly adults with developmental disabilities. The health and general care needs of this group, and how best these should be met, are the focus of this text. Beginning with the overview and background review of the present chapter, and moving on to, first, consideration of the major challenges of dementia among this group, followed by detailed exploration of major causes of disability and the special problems older age brings – with chapters on Down syndrome, cerebral palsy and other congenital syndromes of disability – and finally reviews of the special considerations that need to be made in prescribing medication and planning care for the older adult with developmental disability; this brief handbook aims to bring practical insights to physicians, other professionals and carers dedicated to improving the lives of this deserving group.

Life expectancy: general population and developmental disability

People are living longer. This much has been known for a long time, and the trend is set to continue for a long time to come. The old idea of life expectancy being ‘three score years and ten’ seems a distant memory, as normal life expectancy in the general population increases steadily. Figs 1.1 and 1.2 show the projected life expectancy for the UK population: both measures used in official government data are shown here – period expectancy and cohort expectancy. Period life expectancy is the more often quoted figure – this shows that, for 2004, the UK general population life expectancy for men was 84, and for women 87 years. This rising life expectancy is largely due to advances in nutrition, and in medical and care services.

The corresponding rise in the life expectancy of adults with developmental disabilities has already had far-reaching consequences (Janicki et al 1998). In the UK, a recent government White Paper (in the UK, a White Paper is a statement of government policy – the data cited and reviewed therein are the officially accepted figures, on which services are planned) indicates that there are over 200,000 people over the age of 60 years with intellectual disabilities. Fig. 1.3 illustrates the proportion of the general population that this represents (Department of Health 2001). The same White Paper estimates that the population of people with intellectual disability will increase by 1 per cent per year for at least the next 15 years, this increase being mainly attributable to increased longevity among adults with intellectual disabilities. In the USA, the former American Association for Mental Retardation (re-named the ‘American Association for Intellectual Disability’ from 2006) estimates that there are between 600,000 and 1.6 million adults over the age of 60 years with intellectual disabilities and other similarly disabling developmental disabilities. The most recent USA estimate for the average life expectancy of adults with intellectual disability is 66 years and rising (Fisher and Kettl 2005).

These figures need to be placed in context when we move on to consider older adults with developmental disabilities. For, just as these increases in life expectancy among people with significant developmental disabilities are a reflection of improved health and other services, so it follows that it is only through such continued improvements that these increases will continue to be seen. Also, it is well recognized by most people that the concept of average life expectancy in the general population (as outlined in Figs 1.1 and 1.2) is very much an overall arithmetical average, behind which lies great individual variation, due to differing health conditions, nutrition, etc. Such individual variation is much greater among people with developmental disabilities, for a number of notable reasons. First, many of the individual causes of developmental disability feature other health-threatening and compromising conditions, which correspondingly impact upon life expectancy, especially in certain genetic syndromes of disability and other aetiologies. These matters are explored in detail in Chapters 3, 4 and 5, in respect of Down syndrome, cerebral palsy, and other genetic conditions, respectively. Second, there is a strong association between more severe and profound intellectual disability, and a shorter life expectancy. Similarly, the presence of multiple and complex disabilities has the effect of shortening life expectancy. Finally, however, the best available evidence is that, not only can life expectancy increase among people with severe and complex disabilities, but it should do so – because there is a high avoidable mortality among this group. The most recent estimates are that this avoidable mortality is of the order of 10 times. This was highlighted recently in the UK by the Disability Rights Commission, which has carried out a detailed and authoritative inquiry into inequalities in health care among people with disabilities (Disability Rights Commission 2006).

Deteriorating physical health and health care planning

Increased longevity among people with developmental disabilities is increasingly contributing to a population of elderly individuals which suffers from states of greater physical deterioration and dependency. The challenges posed here, in terms of physical debility and locomotor problems, are the subject matter of Chapter 4, which focuses on cerebral palsy, and especially on how physical disability progresses among older adults with cerebral palsy, and how these challenges can be met.

One of the most important principles to bear in mind in planning health care for older adults with developmental disabilities is: get the balance right between expecting deteriorating functioning, and accepting it. Take, for example, the older person with Down syndrome (see Chapter 3). All involved in the care of the individual must be on guard to recognize that premature ageing is common – but not to accept all apparent signs of such as premature ageing, necessarily. Hypothyroidism in particular must be detected, and treated, as must depression and other problems. Further, where dementia is diagnosed, there is immense scope for intervention, through medication (Chapter 6) and careful attention to daily life planning and other interventions (Chapter 7). A major element of this approach lies in educating carers about the features, symptoms and natural histories of the major age-related conditions – especially dementia (Chapters 2 and 7) – so that they can be well-informed regarding both what lies ahead for the people whom they serve, and, equally, what can be done to maximize the person’s health and functioning, especially in the face of organic deterioration (Nochajski 2000). Commenting on this matter, Hogg and Lambe (1998) have emphasized the value and advantages of careful assessment of health problems and disability among older people with developmental disabilities, in order to identify remediable and reversible health problems and also to plan for other problems in which progressive loss of functioning is inevitable.

In addition to the special attention which needs to be paid to the specific health problems of the different causes of developmental disability (see Chapters 3, 4 and 5), it should be remembered that the same common conditions which are responsible for the bulk of morbidity and mortality in later life in the general population are also at least as common among older people with developmental disabilities (Holland 2000). Heart disease, hypertension and other circulatory problems; cancer of the major internal organs; major endocrine problems such as diabetes, with all its complications; rheumatoid and osteo-arthritis and other major skeletal problems: all of these common serious conditions are common among this population, particularly among those who share the same predisposing factors such as diet, smoking and, in certain conditions, family history. However, many of these individuals are far less likely – whether through ability or opportunity – to give voice to their symptoms. For this reason, it is even more important that targeted, pro-active health screening should be carried out among this population, to identify and treat emerging conditions. Such targeted screening needs to be focused also on gender-specific problems, such as breast cancer and testicular cancer, which can be easily missed if regular screening examinations are not carried out (Davidson et al 2003).

Cognitive decline and dementia

One of the greatest challenges facing all who care for people with developmental disabilities is a direct and inevitable consequence of the changes in the age structure of the population: the increasing burden of cognitive decline and dementia in developmental disability. This is the focus of Chapter 2, which reviews clinical presentation, differential diagnosis, investigations and natural history of dementia in developmental disability; treatment and care planning are the focus of Chapters 6 and 7.

The challenge of dementia among people with developmental disability is at its most familiar in the most common cause of intellectual disability, Down syndrome, in which dementia is particularly common, and indeed often occurs early in adult life (see Chapter 3). Carers need to be alert to the changes in self-care and functioning which are typical of dementia as it presents in this population, and to be aware of how this varies with pre-morbid intellectual level, as well as how to adapt towards the needs of this special group the interventions which have been developed for dementia in the general population. Also, there is growing awareness that, as in the general population, normal age-related cognitive decline among older adults with intellectual disabilities must be differentiated from dementia (Holland 2000). In other words, just as in the general population, older people with developmental disability may become mildly forgetful, while not necessarily developing dementia. However, there is as yet little detailed description available of normal age-related cognitive decline in intellectual disability. One key to this distinction is the growing literature on dementia among this population (e.g. Cooper 1997a).

Mental health

Diagnosis of mental health problems among all people with developmental disability poses challenges. All too often, symptoms of depression, anxiety or even of more serious mental illness are ascribed or disregarded as features or ‘understandable’ consequences of having serious disability. This ‘diagnostic overshadowing’ has been shown to be even more likely to occur among elderly adults with intellectual disability. Nevertheless, it is clear that older people with developmental disability are at higher risk than younger adults for mental health problems (Davidson et al 2003). It has also been shown that the same common disorders which are more prevalent among older people in the general population are also more common among older people with developmental disability (Cooper 1997a). These findings have been taken to indicate that many of the same risk factors are likely to operate among the latter population, as in the general population (Holland 2000). For this reason, many of the same mental health interventions which are widely used in the general population are increasingly being used among elderly developmentally disabled people, although these do need to be adapted, especially for those with more severe intellectual disability and/or complex disabilities (see Chapters 6 and 7).

Ageing in carers

‘What will happen to my child when I’m gone?’ The emotive title of this pivotal article (Mengel 1996) gives a powerful insight into the predicament of the carers of ageing people with disabilities. For it has long been recognized that many parents and carers are reluctant to ‘let go’, through concern and conviction that there is no substitute for parental care: and in many cases that conviction is based on experience. Carers in this situation often invest immensely in the care of their charge, through devotion and personal commitment. One dynamic which is often witnessed is that of a parent carer who is – often with good reason – cautious and protective towards their disabled offspring, resulting in a restriction of social opportunity for the disabled adult, a problem which may persist for many years (Ahrendt 2003). Such families can become very precarious, surviving in a homeostasis which can easily be upset by any increase in care burden. Sadly, this increase in care burden often comes from the carers. For, as the carers age, they themselves are more likely to develop their own age-related problems of health and infirmity. These problems, in turn, presenting and most probably subsequently worsening at a time in life when the care of the disabled adult can become more of a challenge, can tip a family into a situation where care of one by the other is no longer feasible, with potentially catastrophic impact on the quality of life of all concerned (Jokinen 2006).

Families in this situation need support and help, but this needs to be delivered in a sensitive and appropriate manner, by professional teams who are familiar with their predicament (Janicki et al 1998). Such careful and timely help and support, offering a gradual introduction into a different pattern of care for the ageing disabled adult, can go a long way to avoid the even greater catastrophe of the need for emergency action, precipitated by the death or sudden deterioration of the carer.

Old age, retirement and life planning

Older people with developmental disabilities will, eventually, seek a change in lifestyle, just as all older people in the general population seek retirement, or some change in employment and/or daytime occupation. In the general population people vary immensely in how they approach this issue, depending on their own abilities, health, inclination, and the need society perceives of any special skill they might possess. Older adults with developmental disabilities similarly differ greatly in their inclinations and desires in the area of retirement, but they typically have less opportunity to make choices on their own behalf in this regard – whether because they are less well-informed, or simply because they have fewer options (Mitchell et al 2006). For many, the overriding need is to maintain some kind of appropriate pattern of daytime occupation or day care, whether in the context of changing physical ability (see Chapter 4), or deteriorating intellectual functioning (see Chapters 2 and 7). The major problem at present is that support, day care and employment services are not sufficiently geared towards the needs of this growing population (Klingbeil et al 2004).

A framework for case management and life planning: the International Classification of Functioning, Disability and Health

The challenges facing the ageing adult with developmental disability are myriad and complex, such that one major problem concerns how to formulate the numerous facets of the person’s changing situation, with evolving health problems, and often increasing infirmity, in the evolving individual social context. Here, the ‘International Classification of Functioning, Disability and Health’ (ICF) (WHO 2001) is particularly applicable. This recently-introduced World Health Organization (WHO) classification schema is complementary to the traditional ICD-10 classification (WHO 1992).

The traditional ICD-10 is based on a medical model, wherein human disorders, diseases and illnesses are classified, according to the affected body systems. In the new classification (ICF), the thrust is to describe the individual’s participation in everyday living. In the traditional ICD-10, an individual with Down syndrome and Alzheimer-type dementia can be classified under these two diagnostic labels, and the degree or severity of associated intellectual disability can also be classified as mild, moderate or severe. This was initially developed in the ‘International Classification of Impairments, Disabilities and Handicaps’ (WHO 1980), which recognized the relationship between the impairment in an organ, the ensuing disability which this causes in terms of function, and the resultant handicap for the individual in life. However, apart from the language employed here – which is now widely regarded as pejorative and stigmatizing – this approach did not go far enough to recognize the complex interaction between individual and society, and the extent to which the same impairments can result in very different patterns of inclusion in everyday living, according to varying social environments. In the ICF there is more systematic recognition of the relationships between:

• Body function: the physiological functioning in a given body system – including psychological functions
• Body structures: the anatomical parts of the body – such as organs, limbs and their components – where functioning occurs
• Activity: the execution of a task or action by an individual
• Participation, which refers to the individual’s involvement in a life situation
• Environmental factors, including the physical, social and attitudinal environment, which impinge on the individual’s actions, and ultimately determine participation and social inclusion for the individual

Concluding comment

This brief introductory overview of aspects of ageing in developmental disability has described the current changes in the index population, and introduced the associated health, mental health and social challenges facing affected individuals and their carers. The situation of the older person with developmental disability shares many of the features of that of all older people – but with additional special challenges. The approaches to understanding and addressing these problems are explored in the following chapters.