A Guide to the Essential Features of Residential care and Supported Living for Adults with Prader-Willi Syndrome |
Appendix 1: Research into living situations of adults with PWS
Appendix 2: An example of successful placement in the USA
Appendix 3: Three case histories in the UK
The PWSA (UK) would like to thank the following people for their help in producing this document:
Mike Adams - Community Nurse, Learning Disabilities, South Devon Healthcare
John Booth - Hon Chairman PWSA (UK), Chair Residential Care Working Group, parent of a woman with PWS
Mike Broom - Residential Placements Manager, East Sussex Social Services
James Churchill - Chief Executive, Association for Residential Care (ARC)
Tom Duncan - Director, Rogate Residential Home, East Sussex
Margaret Gellatly - Hon Dietary Advisor to the PWSA (UK)
Duncan Hamilton - Keyworker, Walsingham Homes, Hertfordshire
John Higgins - Development Director, Cottage & Rural Enterprises Ltd (CARE)
Fred Hutton - Director, The Lodge Trust, Rutland
Rosemary Johnson - Welfare Services Co-ordinator PWSA (UK), parent of a young man with PWS
Shona Marshall - Keyworker, The Lodge Trust, Rutland
Vernon Nash - Social worker, Bushey Social Services, Hertfordshire
Val Parkes - Manager, Green Laund, Honormead Schools, Derby
Andrew Regent - Community Nurse, Suffolk County Council/Allington NHS Trust, Community Team for People with Learning Disabilities
Maurice Reynolds - Senior partner, Gretton Homes, Northamptonshire
Myra Rogers - Careworker, St David' s Care in the Community, Pembrokeshire
Sara Sheppard - Care Co-ordinator, Joint Purchasing Consortium for People with Learning Disability, Cambridgeshire County Council/Cambridge and Huntingdon Health Authority
Cathy Spencer - Social Worker, Derbyshire Social Services
Diane Stock - Manager, Nether Priors Residential Home, Essex Social Services
Sam Twigg - Manager, 20 High Street, Weedon, Northamptonshire
Yvonne Wanstall - Head of Care, Woodend, The Lothlorien Community Ltd, Kent
Jackie Waters - Resources and Information Co-ordinator PWSA (UK), parent of a woman with PWS
Laura Willson - parent of a woman with PWS
James - a person with PWS
With grateful thanks to the Beatrice Laing Trust for their generous financial support of this project.
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The Prader-Willi syndrome (PWS) presents many challenges to people with the syndrome and to their parents and carers. Its effects reach into every aspect of daily life, and careful consideration must be given to the way in which people with the syndrome are helped and supported to make choices which are beneficial to both their overall health and their quality of life.
People with PWS have a unique set of needs which are not readily met under existing health and social services arrangements. The syndrome is poorly understood by those who have had little experience of daily living with PWS, and its effects are sometimes fatally underestimated. Hidden risk factors and disabilities lie beyond the veneer of independent living skills and intellectual ability presented by many people with PWS.
However, people with PW S have many skills and qualities which they can use to enrich the community in which they live, providing they have the degree of support, understanding and care they need.
This document sets out to identify those needs, and to suggest ways in which these can best be met within a residential home or a supported living situation. The suggestions are based on practices which have proved to be most helpful in producing a good quality of life for most people with PWS, both in this country and internationally, and are in line, as far as possible, with current social and health services thinking and practice.
The guide can be used to assess the ability of existing residential homes to provide good quality support for an individual with PWS, and will also prove valuable to those with little previous experience of the syndrome who are wishing to provide accommodation and/or support.
The Prader-Willi Syndrome Association (UK) is the only organisation in the United Kingdom and the Republic of Ireland concerned with this relatively rare and complicated syndrome. The broad objectives of the Association are the following :
In addition, the Association runs a number of individual projects. One of these is a working group which is looking at residential facilities and how to stimulate the availability of more appropriate placements for adults with PWS.
The material on which this document is based was originally produced by the PWSA (UK) residential care working group, which needed a specification of the necessary characteristics of a residential care unit able to accommodate and provide care for a small number of adults with PWS.
In November 1996, sixteen professionals with an interest in PWS, from both statutory services and residential care homes, were invited to participate in a two-day workshop with the aim of developing the guidelines for a larger audience. Of those people, one also discussed the draft with a person with PWS, and one invited the comments of a parent of an adult with PWS. Their suggestions have been incorporated within this document.
The outcome of the workshop is that the document has been broadened in context and content, and it is targeted at four main groups of people:
The Association is frequently asked about the desirability of creating residential units exclusively for people with PWS. Proponents argue that there are great benefits in offering specialist services in specialist facilities. Opponents consider it to be a sad and limiting existence to be a member of a community defined by a single medical disorder. The Association takes no position on this issue, considering it to be subordinate to the requirement to match the environment to the individual's preferences and needs. It has evidence of people with PWS living happily in exclusively ""PWS homes". Equally, there are several people in the UK who share their lives successfully in mixed communities, or who live in independent supported living accommodation. It is important to remember that the needs of one individual with PW S may be somewhat different to those of another. However, all share certain characteristics and need some level of support. These vital issues are addressed within this document.
| It must be emphasised that the document
should be seen as a supplement to the Department of Health/Social Services
Inspectorate document:
Guidance on Standards for the Residential Care Needs of People with Learning Disabilities/ Mental Handicap (1992). |
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The Prader-Willi syndrome (PWS) is characterised by extreme floppiness at birth (hypotonia), a characteristic facial appearance, and immature gonadal development (hypogonadism), all apparent at birth. The hypotonia is usually so severe that tube-feeding is necessary after birth. Poor sucking ability and sleepiness mean that babies often show little interest in food, and there is a subsequent failure to thrive in the early months of life.
However, from early childhood (about age 1 - 4 years), interest in food and appetite increases markedly, and over-eating (hyperphagia) can result. This, together with poor muscle tone, can cause severe and life-threatening obesity unless a life-long low calorie (energy-controlled) diet is followed. Later in life, the presence of varying degrees of learning difficulties, delayed or immature secondary sexual development, and immature emotional and social abilities, all become apparent.
The following characteristics, both physical and psychological, may occur in PWS. Not every characteristic is present in every individual, and they can vary in intensity from mild to severe. They may also vary within each individual at different times or in different circumstances.
It has now been established that over half of those with PWS have a deletion in the proximal part of the long arm of chromosome 15 inherited from the father. Most of the remainder have two chromosome 15s inherited from the mother (maternal disomy), instead of one from each parent. A small proportion (about 5%) have other chromosome 15 abnormalities. Some people retain a clinical diagnosis of PWS whilst appearing to have unaffected chromosomes.
People with PWS invariably present with a unique and debilitating problem: that of overeating, which, if left unchecked, can lead to severe obesity and early death. The research of Dr AJ Holland et al (1993) indicates that people with PWS have a specific abnormality of satiety. As they eat, it takes longer for them to lose the sensation of hunger; therefore they continue to eat. It is this excessive food intake, in combination with a general lack of exercise due to poor muscle tone, which leads to increasing weight and obesity Stealing food and a high degree of cunning in obtaining extra food are frequent features in PWS.
Temper outbursts, often referred to as tantrums or rages, are also a recognised personality trait of many people with PWS. The propensity to such outbursts usually continues into adult life. The specific reasons why people with PWS appear more prone to temper outbursts than people with other types of learning disability remain unclear. They may relate to obsessiveness, problems around food, unplanned changes to routine, or no immediately obvious cause. Whilst temper outbursts are relatively mild in some individuals, and are limited to crying and screaming, in others the tempers take a far more aggressive form, and physical violence can occur. Several different management approaches have been identified, although the personality and needs of the person with PWS should always be considered when implementing new management techniques.
People with PWS present with a wide range of abilities. Intelligence levels can vary from an average IQ ( 100) to severe learning difficulties, with the majority of people falling within the range of mild to moderate learning difficulties. Typically, people with PWS have better reading and writing skills than number skills, and have difficulty in understanding abstract concepts. A superficially good grasp of language and vocabulary can mask difficulties with comprehension and social skills. A minority of people with the syndrome have speech and language disorders such as dyspraxia.
Many people with PWS have considerable verbal ability. They may be able to convince others that they are capable of running their own lives, and of exercising freedom of choice responsibly. Often this is not the case, as their families know only too well.
Despite the difficulties involved in having PWS, many individuals have great personal strengths, including determination and perseverance, a good sense of humour, good powers of concentration in certain tasks, and a thoughtfu1, caring attitude towards others. The majority of people with PWS have strengths such as the ability to do complicated jigsaw and wordsearch puzzles. Whilst their overall ability in sports is limited by weak muscle tone, they often excel at swimming. Some individuals inherit family characteristics such as musical ability or artistic talent.
The birth incidence of PWS has been estimated to be between 1 : 10000 and 1:25000. Using an average of about 1:15000, this could mean that there may be about 3000 people with PWS in the United Kingdom, assuming an average life- span. Currently, there are probably less than 3000, because of the early death rate, but the numbers are growing as more people live longer, due to better management and understanding of the syndrome. PWS affects both males and females, and appears to be present in all races.
The PWSA (UK) currently has about 500 family members, plus members in the Republic of Ireland and Europe.
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As with any other group of adults, the desire on the part of the person with PWS to move on from the family home is quite natural, but very careful consideration needs to be given to the type of living arrangements which are appropriate. They have the right to self determination, which involves the responsibility to care for their own health. Unfortunately, the physical and mental makeup of most people with PWS makes obtaining food one of the driving forces in their life. They lack the ability to recognise when they have had enough to eat, and in this respect it is very difficult for them to take responsibility for their own healthy eating, unless ongoing support and supervision is provided. Risk factors resulting directly from the eating disorder include:
Whilst these risks are only too evident to people who have lived on a daily basis with someone with PW S, they are not so obvious to professionals and workers who have only just met the person, or who do not see them regularly, and do not see "beyond the veneer" of the articulation skills and general competence in daily living skills possessed by many people with the syndrome.
Isolated cases of successful independent living in the short term may be held up as examples that "everyone can do it". Similarly, a person's right to independence might be quoted. This raises many ethical and moral questions which are never fully resolved, but it is important to focus on the correctly assessed needs of any given individual, rather than relying on theories or fashionable ideologies.
Dilemmas often arise whereby the individual expresses a wish to live independently, or take control of his or her own finances, but it is recognised that this will lead, or has lead in the past, to life-threatening health problems and/ or challenging behaviour. If residential care appears to be the best option for such a person, it is essential that the care programme is designed to offer creative options for the individual which will preserve his or her integrity and dignity, whilst promoting long term health and safety.
People with PWS can be wrongly placed in residential hostels and homes where there is little understanding of the problems involved, and where philosophies of independence are not managed and balanced with the individual' s abilities and identified risk factors. The outcomes are invariably life threatening to the individual, and cause considerable distress to themselves, staff carers and those other residents with whom they share the home.
Equally however, there are success stories where people are living in homes with a good understanding of PWS and where their needs are met within a framework of care which is acceptable to all involved (see Appendices 2 and 3).
Some people with PWS have problems in accessing services for people with learning difficulties, because they have a relatively high intelligence level. Some features of the Mental Health Act (e.g. sectioning) may not be deemed to be appropriate for people with PWS because they cannot be proved to have a learning difficulty. They are often considered to be a low priority case of need, but this is only because the situation has not reached a crisis level, or because the person is being well-supported by family members. Such circumstances inevitably break down when family carers become older, ill, or overstressed.
Carers' needs must also be considered in determining the need for the person with PWS to be placed in a residential care or supported living arrangement:
Appendix 1 gives further details of research which has been carried out, which supports the claim that people with PWS need some form of supervision in adult life.
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| In residential care situations, all features of the physical environment should conform with the guidelines set out in the DoH/SSI document: Guidance on Standards for the Residential Care Needs of People with Learning Disabilities/ Mental Handicap, 1992, Ch. 7 |
There is a whole range of options when considering the lifestyles of people with PWS. Each of these options has its own advantages .and disadvantages. In considering them, the individual needs of the person with PWS should be paramount. Terrance James ( 1989) identified 17 different models in Canada, of which all had both advantages and disadvantages.
Options currently available in the UK include :
Small group homes specifically for people with PWS have been on offer in the USA, Sweden, Denmark, New Zealand and Australia for several years. Until recently, these have not been an option in the UK, but there is now one such group home (privately owned) in existence, with several others in various parts of the UK still in the planning stages. The advantage of these homes is that the challenges of PWS can be understood and managed within a caring and secure environment. Some would argue that such homes are socially limiting, and that people with PWS do not necessarily benefit from being in close proximity with others who have the same difficulties. However, PWS .specific group homes are the type of residence most often requested by parents and some professionals.
Supported living in the community is a possibility, but should involve an appropriate and worthwhile daytime placement, careful planning, and adequate support systems which are not liable to break down or be unreliable. The support systems should also be able to react. quickly to crisis situations such as large increases in weight or mental health problems.
Whatever type of home or placement is being considered, the following points may need to be considered. Thought should also be given to the future needs of each individual.
As previously indicated, one of the features of people with PWS is the propensity to be grossly overweight, as well as being generally shorter than average. Individual assessment will identify any need for specialist equipment, which may include:
N.B. An exercise equipment room has been excluded in this section, as it is our expectation that community resources would be accessed, depending on individual needs and interests.
Although single room occupancy is envisaged as a pre-requisite for services in general, some people may choose to share and should be afforded this right. However, the following factors may need to be considered :
Unsupervised access to food should be avoided with people with PWS. This will often require the following arrangements :
Staffing accommodation is dependent on the model of care/support being considered. Particularly in residential care situations, 24 hour staffing with sleep-in accommodation or even awake at night should be considered because of the following eventualities:
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In considering any model of care, the following features should be in place for each person with PWS.
In order to meet the above needs of the person with PWS, the support service and/or residential care situation should incorporate the following features:
A longitudinal study should be in place to establish base line indicators of success. Suggested indicators are:
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| The general quality and objectives of the care package should conform with current standards as set out in the DoH/SSI document, Guidance on Standards for the Residential Care Needs of People with Learning Disabilities/Mental Handicap, 1992. It should be noted that individual care plans which set out the objectives and commitments of provider and user of services, and their regular review, are emphasised as being fundamental to a successful care package. |
The high quality of care in a residential home or supported living situation depends crucially on the competence and commitment of the staff and on their good management. In turn, the personal capacity and experience of the manager are what determines the creation of a happy and well-run care package. Appropriate training and sources of information for staff are seen as a vital component. Close supervision and support of staff are also essential factors.
On a day to day basis, people with PW S tend to be quite placid, but can be very stubborn. Many find any unplanned or unanticipated change in the expected routine hard to handle. Additional support at these times is vital. Care staff need training to manage potential outbursts of temper, which may become aggressive towards others. Sufficient support staff should be available to ensure the well-being of other residents whilst the needs of the person with PW S are attended to. There is likely to be a need for pre- and post-incident counselling, and debriefing for keyworkers and other care staff involved in dealing with obsessional outbursts and temper rages of people with PWS.
Appropriate risk assessment should be undertaken around the needs of other individuals, and the staff, as well as those for the individual (as described on page 5).
To ensure a satisfactory life for people with PWS, a high degree of commitment from staff is necessary. Continuity and consistency within the staff group is crucial. The ability and authority of a member of staff to deal with the intense emotional outbursts of some people with PWS has to be highlighted here as an especially demanding feature of the care package. Additionally, people with PWS can be manipulative and may take advantage of new staff, or situations where one member of staff contradicts another. Services should therefore be aware of the possibility of a high turnover of staff, if appropriate supervision and support are not provided.
It is important to involve the person with PWS in any decisions taken and to negotiate an agreed contract for standards of behaviour and care planning. A vital aspect of this contract needs to involve the recognition of the individual's "rights" around the management of their personal monies. Care planning may need to involve decisions around moving on to a different setting. This should be dealt with sensitively, bearing in mind the needs of a person with PWS.
There is reason to expect, as set out in Section 2, that people with PWS need more health care than that provided to the population at large. They may suffer from a range of conditions that require regular review. A high pain threshold may mask serious disorders. Awareness of nutritional and hormonal treatments are also necessary.
It may be necessary to restrict the freedom and independence of people with PWS to come and go as they please. However, this can be overcome by providing a daily structure of training, education and work, with attractive evening and weekend events, and responsibility given to the person when food is not present. Options for personal leisure activities with the appropriate supervision will be required.
The key point is recognition that the normal bodily self-control processes in relation to the volume of food intake are absent or virtually absent in the case of PWS. This is not to say that in the right circumstances the person with PWS will not be able to offer some co-operation in controlling his or her own food intake. It is important to enlist to the maximum the involvement and agreement of the person concerned.
However, co-operation cannot be taken for granted, especially at times when incidents occur which disturb the even tenor of life. The caring situation must therefore control access to food at all times. Proper dietary management and supervised weight control are a necessity. This should become a built-in and therefore a mechanistic part of the environment.
Special consideration needs to be given to the degree to which the person with PW S is involved in cooking and serving meals. Many people with PWS enjoy cooking, but supervision needs to be in place.
| It does require a total commitment by all involved, and an acceptance that food is to be regarded as neither a reward nor a celebration of success. This in turn demands ingenuity in finding appropriate substitutes. |
Whatever living environment is initially chosen by the individual, a need for weight reduction may already exist. It is vital to agree a dietary or healthy eating plan with the individual before the move is made. Energy (calorie) intake will need to be specifically controlled, but in a way that ensures a healthy balanced diet which can allow open access to certain "free" foods. The PWSA (UK) booklets, Prader-Willi syndrome :Food and Health, and Weight Control and Suggestions to Assist with Weight Management give further details about a suitable dietary programme. In a residential home, it would be desirable for all residents to be following a healthy eating management plan with appropriate energy, fibre, vitamins and minerals. (See also Appendix 4 for comments from a person with PW S about food management).
Current best practice puts stress on the recognition of six key values intrinsic to the residential care and supported living environment. These key values are: privacy
The process of consultation with the individual user in obtaining their commitment is an essential feature of the care plan which must recognise and incorporate these values.
In setting up and reviewing the care plans for the individual with PWS, the following specific points need to be borne in mind:
In these circumstances, it is very difficult to devise a pattern of life which provides the normal sources of fulfilment. The weekly schedule should include a daily structure of education, exercise, training and work in a properly supervised setting. Meals should be at regular times, in a family-like atmosphere. Hobbies, games and social activities should be actively encouraged, not just facilitated. Assistance should be given with making and keeping appropriate personal relationships. Religious activity should be supported. In short, great efforts should be made to create full and satisfying lives for people with PWS.
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This document has drawn attention to the unique set of needs for people with PWS, and made specific recommendations to enhance their quality of life. It is hoped that service planners and managers will thus be better informed to research suitable placements for people with the syndrome, and will be in a better position to argue for specifically designed provision.
The PWSA (UK) would be pleased to offer further advice and guidance on the specific needs of people with PWS. Please contact our careline 01332 360 400.
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After a visit to the Pittsburgh Rehab Institute, C was placed in a home with four other PWS individuals. In two years time his weight is now 1701bs (12 stone).
In this home, C does not do his own cooking, but currently is doing his own medication, and he makes his choices as to his social activities. I think this current placement meets C's needs, and he is healthier and happier than ever before.
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A special dietary regime is very necessary. A few stolen biscuits can lead to putting on a few extra pounds. We really have to know what is eaten every day. Bill accepts the need for the diet, but still steals food at the slightest chance. He is very clever at how he gets it and is not able to co-operate with the regime. There is no effect on the other residents from Bill's special diet. One other service user is on a diet at the moment as they need to lose a little weight. Bill has lots of obsessive behaviours - e.g. picking clothes, moving dishcloths, occasional temper tantrums.
With regard to extra care, he has to be supervised in the kitchen area; he has a poor body thermostat, so bathing has to be supervised; and he has always to be accompanied when out. Other residents don't like the noise made while having temper tantrums or when he is attention-seeking. We are not aware that Bill has been affected by any special care needs of the other residents.
With regard to how far Bill is capable of managing his own life, he knows what clothes he likes. He is pretty good at matching outfits, but apart from that he is not at all capable. This view accords with Bill's own image of himself, and he accepts the need to live in a residential home.
With regard to the contribution he makes to the social and working life of the home, he has been very disruptive. He makes little effort to do any work within the home. If he is asked to help, this usually leads to a temper tantrum. Having said this, we feel that at the moment things are changing for the better. He recently started a calorie controlled diet of the same food as the other residents, as suggested when Rosemary Johnson came to speak to us. He has been far more contented and pleasant. Although he appeared to like the Lean Cuisine diet that he was on before he obviously felt he wasn't one of the group. Now he is on the same food but smaller portions he is happier and more of a joy to be with.
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Sue has lived in a residential home for people with learning disabilities for the last three years or so. She is now 31 years old. The home is owned and run as a private charity, and it houses 20 residents, most but not all of whom receive state and/ or local authority funding. Residents are expected to do a normal day's work in the garden, workshop, or laundry facilities provided by the home, and to participate in its social and religious (Christian) activities.
Sue has Prader-Willi syndrome which was diagnosed at the age of five, before overeating and obesity became a serious problem. She has therefore been accustomed for most of her life to restrictions on what she eats. Indeed she usually co-operates in controlling her own intake of food, although this cannot be relied upon. Like other people with PWS, she suffers from sporadic emotional outbursts (temper tantrums) and obsessive behaviour. She is a childlike but sociable person with an IQ in the 60s.
Weight control. Everyone in the home is treated as an individual with individual needs which the management of the home regards itself as contracted to meet, and regards the individuals as similarly contracted to meet certain expectations and standards of behaviour. Thus, for Sue, the control of her weight and the associated dietary regime is an essential condition of the contract, and really rather a matter-of-fact one. Access to food is so controlled that there is minimum temptation. Choice, and positive co-operation, is actively sought at the second level, in the selection of the type of food she eats. Sue is fortunate in that she enjoys salad and green vegetables, and is free to load her plate with these.
These controls are not felt to impinge on the freedoms of other residents, or only marginally through the careful storage of food. There is no sense of spoiling the fun at parties, for example, rather a general recognition of Sue' s need for support in restraining herself at the party buffet. A special fat-free cake is kept in the fridge for her.
Behaviour. The contract extends to behaviour. Good behaviour is expected. No concessions are made to the fact that the basis of Sue' s outbursts may be genetic rather than social in origin. Positive approaches have to be found for dealing with the outbursts when they occur, and Sue
herself is expected to play an important part in dealing with them. There is no judgement for her conduct. The outburst happens but it must not get out of hand. A way has to be found to come out of the situation with self-respect and dignity Often this means taking herself off to her room until she feels better.
Freedom of choice and self-respect. One really hard question is whether Sue, or any of the other residents, accept the need to live in a residential home. Often this question is not about choice but about necessity, since many of these people are unlikely to be capable of living independently, although they may not recognise their limitations. In Sue's case, she could not have been expected to make the choice for herself But great efforts were made to obtain her concurrence in the decision.
Sue now has a busy working life which has helped to build up her self-respect. For example, she derives great satisfaction from having helped, as she puts it, "to install the central heating" in part of the home. Her weight is now totally under control, which also helps with her self image. .
Life in the residential home has not been all fairytale, however. Her worries now relate to the inevitable and usually minor irritations of communal life and the day-to-day challenges these pose to her. It is important that she is personally involved in helping to overcome them and gains further self-respect thereby. Sue' s extreme expectations and dreams remain unrealisable since they relate to motherhood, which to the best of today' s medical knowledge is not possible for women with PWS. She would also love to be involved in the care of young children, but this is only possible through a friendly mother because Sue could not take responsibility for childcare in an institutional setting.
Specialised PW homes. The idea of a residential home solely for people with PWS is a controversial one. Yes, it focuses on the particular problems of PWS, but it categorises and isolates them from the community to an extreme degree. Staff must deal with residents as individuals and not as common problems. The shared obsession with food is likely to be very sad and depressing.
Separation of home and work. It is most desirable, if practicable, to separate the home from the workplace. They can be adjacent physically, but should not overlap in management and operation. Staff in the home should feel they are "making a home". Residents should then feel they are "going out to work".
Outreach services. The principle that the residential establishment is "home" for residents should extend to avoiding any provision of outreach services from the home. The staff may be professionally involved in such services, the work centre (next door) may be an outreach facility, but the home itself should not lose its status as a home. In particular it should not be used for respite care.
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Tracy is 22 years old, and for the past two and a half years has lived in a short-term residential hostel, which is owned by a private organisation concerned with youngsters with speech and language problems. The hostel provides a "stepping stone" between residential school and more independent living. Students attend both college and work experience. Currently, the hostel houses four young men and two young women, including Tracy, and has sleep-in staff. The kitchen is locked during the night.
Tracy has spent much of her life in a special needs residential school, and has a twin brother who does not have PWS. This has made family life very difficult.
When Tracy first entered the hostel, staff became increasingly concerned at her obsessive tendencies and what amounted to almost paranoia that people were talking about her behind her back, making appointments to discuss her with college, or ringing her family without telling her. It became impossible to reason with her, and she had major temper outbursts, needing more than one person to prevent her from harming herself, others or property.
One of the staff at the hostel attended a PWS training day, and listened to a talk given by a psychiatrist who has a particular interest in behavioural problems in PWS. She subsequently asked the PWSA (UK) for contact details for the doctor, who came to see Tracy. He prescribed medication for Tracy which has been largely successful in controlling her behaviour - so much so that the initial dose has been halved.
Weight control. Progress has been made in this area. Initially, staff felt they had to control Tracy' s food intake entirely. She resented this - thus taking every opportunity to "sneak" or buy food when no-one was looking. Staff have now found that by offering Tracy the opportunity to make decisions and choices in her menu, this behaviour has dropped considerably. Each week she constructs her own menu, with guidance from the staff, and has become quite skilled at recognising low calorie foods in shops etc.
She has learned to accept compromise at meal times. For instance, if chicken drumsticks are on the menu, Tracy's portion has the skin taken Off, whilst it is left on for the others. She has only one drumstick, but extra vegetables, and thus her plate looks as full as the others', who have two drumsticks.
Other residents are allowed to have food in their rooms if they wish, and Tracy is allowed to have fruit and diet drinks in her room. Hence, she does not feel unprivileged. The other residents are largely considerate of her needs, although they do have the occasional moan about it. When Tracy is away, or visiting parents, the residents and staff tend to binge a bit! The hostel does not concentrate on "" diet". Weighing takes place once a week, and currently Tracy is doing a sponsored slim for "Children in Need".
Staff have learned to compromise routines around Tracy's needs. For instance, on outings, previously Tracy would be unable to accept that she had to wait half an hour until the minibus stopped, so that she could have her routine mid-afternoon drink. Now someone gets a flask out during the journey, and says, ""Would anyone like a drink?" Problem solved.
Other problems. Tracy's behavioural problems have already been described, and her medication has helped immensely to control what was fast becoming a serious mental health problem. It was found that two tablets made her lethargic, but one tablet leaves her more alert, and has enabled her to have better sleeping patterns. Staff also feel that because she knows the kitchen is locked at night, she does not wake to look for food. Her family is able to accept her better also.
Her temper outbursts upset two residents in particular, who have autism, and are therefore very sensitive to noise. The result was that if Tracy had an outburst (which she did regularly), the other residents' behaviour also seriously deteriorated as a direct result of the noise she made. Medication and better management have substantially reduced the outbursts, and thus the level of stress for staff and other residents.
Sometimes other residents will "wind her up", but often this can be deflected by the use of humour, which Tracy is increasingly able to appreciate. She is largely unaffected by other residents' care needs, and where necessary can compromise, as they will for her.
Freedom and Responsibility. Staff feel that Tracy will never be able to cope on her own, without the continual guidance and support of those who understand her very special needs. According to staff left to herself she would just "buy food and eat it".
She has learned to manage her money better since coming to the hostel, and when aIl the residents go into town, she is allowed half an hour to wander on her own. As receipts are required for all expenditure (so that staff are not wrongly accused of appropriating residents' allowances), she has little opportunity to "cheat".
Tracy's self-image and self-esteem have risen with her ability to control her life (with the guidance mentioned). She is likely to "" comfort eat" if things are going wrong for her, so it is important that she is not ""set up to fail" - she needs to feel that she is succeeding. She is now better at choosing clothes, and although she previously resented anyone going with her to do this, she now prefers to have a companion to give her their opinion. .
She has become far more realistic about her limitations and abilities, and has said that she would like to live somewhere with other people with PWS, because she knows this would help her keep in control of her self. She contributes well to the general wellbeing of the household (although she is not keen on housework), is very sociable, goes to youth club, and has a boyfriend.
Because the hostel is short-term, staff feel Tracy needs to move and ""put down roots". But a suitable placement is impossible to find at present, and therefore Tracy's stay at the hostel is ""open-ended"" until the right place comes up. Staff are reluctant to place her just anywhere, and are prepared to keep her until she can find a place where she will be as well supported as she is now.
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James (20) wanted me to point out one thing about which he feels very strongly. Because he lives in a home with 5 other young men and is the only one with PWS, he initially got very upset because his food was different from theirs.
Now with a lot of juggling and careful planning, we are able to give everyone the same meals - based on James' dietary requirements, and altered subtly for everyone else. This has made him much happier.
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For further details contact:
PWSA (UK),
125a London Road,
Derby DE1 2QQ.
Tel. 01322 365676
Department of Health Local Authority Circular LAC(92)15: Social Care for Adults with Learning Disability (Mental Handicap). Paras 25-27 , October 1992
Department of Health/SSI: Guidance on Standards for the Residential Care Needs of People with Learning Disabilities/Mental Handicap. HMSO 1992
Department of Health/SSI: Homes are for Living In. HMSO 1989
Gellatly, Margaret: Prader-Willi Syndrome: Weight Control and Suggestions
to assist with Weight Management 1991
(free booklet available from the PWSA (UK) office.)
# Gellatly, Margaret: Prader-Willi Syndrome: Food and Health 1996
* Greenswag, LR: Adults with Prader-Willi Syndrome: A Survey of 232 Cases. Developmental Medicine and Child Neurology (1987) 29, 145-152
* Hodapp, Robert M; Dykens, Elisabeth M; and Masino, Linda L: Families of Children with Prader-Willi Syndrome : Stress Support and Relations to Child Characteristics. Abstract, Prader-Willi Syndrome Association (USA) Scientific Conference, 1996
* Holland, AJ; Treasure, J; Coskeran, P; Dallow, J; Milton, N; and Hillhouse, E: Measurement of Excessive Appetite and Metabolic Changes in Prader-Willi Syndrome. International Journal of Obesity (1993) 17 -532 , 527
* James, TN: Residential Options in Western Canada. PWSA of Alberta, 1989
# James, TN and Brown, RI: Prader-Willi Syndrome - Home, School and Community.Chapman and Hall 1992
NHS Executive Health Services Guidelines HSG(92)42: Health Services for People with Learning Disabilities (Mental Handicap). October 1992
# PWSA (UK): Report on a Study Day on the Management of Prader-Willi Syndrome in Older Children and Adults 1990
* PWSA (USA) Newsletter: The Gathered View, Jul-Aug1992
* Thompson, DG and Wett, MA (eds.): Development of a Proper Placement for Individuals with Prader-Willi Syndrome. PWSA (USA) 1993
# Waters, J: A Handbook for Parents and Carers of Adults with PWS (Revised Edition), PWSA (UK) 1996
* Waters, J: PWSA (UK) 1992 Questionnaire and 1994 Residential Care Questionnaire, PWSA News, 43, Dec 1994, and PWSA News, 45, June 1995
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