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Throughout, emphasis is placed on the fact that individuals with PWS share common traits, but they can also be very different from one another. Care has been taken to ensure that the needs of people with PWS within their adult status are addressed, but this can be difficult to reconcile with their daily lives because so many people with the syndrome require a high level of support and even control to ensure that they lead a healthy and fulfilling existence. As a parent of an 18 year old girl with the syndrome myself, I am only too aware of this very difficult dilemma. I have tried to remain unbiased, but inevitably subjective opinions may occur throughout.
| JACKIE WATERS, |
Some names have been altered to protect identities.
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It is estimated that about 1 in 15,000 people have the syndrome. It occurs in both sexes and in all races.
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Before the mid 1980s, diagnosis of PWS relied mainly on those features which could be identified in a clinical examination. These features are: Central hypotonia. Floppiness at birth, with poor muscle tone in later life. This problem lies not within the muscles, which are normal, but within the brain. Muscle tone can improve with regular exercise, or with growth hormone injections.
Hypogonadism. Incomplete development of both external and internal sexual organs.
Obesity. Caused by excessive appetite (hyperphagia) and, because of decreased energy expenditure due to poor muscle tone, a need for fewer calories than normal to maintain body weight. Early feeding difficulties result in a baby which "fails to thrive". the excessive appetite begins around the ages of 2 to 4 years.
Characteristic Facial and Bodily Appearance. Features include almond-shaped eyes, small mouth with downturned corners, and small hands and feet - particularly noticeable in adults. The hands are narrow, with a straight ulna border (outside edge of the palm, on the side of the little finger). Most people with PWS are shorter than average, especially if they have not had growth hormone treatment. Men with PWS average about 5' 2" (155cm) and women average about 4' 10" (145cm).
Developmental Delay and Learning Difficulties. Learning difficulties range from very mild to severe, with the majority of people falling within the mild to moderate range. Skills in reading and writing are usually better than those in mathematics.
Although doctors are now beginning to diagnose PWS earlier and more frequently, there are still many adults with the syndrome who were not diagnosed until their teens or much later.
| "How helpful it would have been (to
have a diagnosis) during the difficulties of the first 13 years - schooling
problems could have been avoided, social workers' help obtained, the PWSA(UK)
would have been available to us, etc." - the mother of Jennifer (20) |
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In recent years, geneticists have made great strides in identifying the chromosomal abnormalities which cause PWS. The clinical diagnosis can usually be confirmed by a chromosome test, which involves taking a small sample of blood from the person with PWS, and if possible, from the parents. Three types of abnormality have now been identified, any of which can result in the majority of the features of PWS. No blame for these abnormalities is attached to either the father or the mother of the person with PWS. These abnormalities are:
Some forms of brain damage can also cause PWS-like symptoms, most notably in terms of eating and behavioural problems.
It appears that the genetic abnormality on chromosome 15 affects the hypothalamus, which is situated deep within the brain. The hypothalamus is the "control centre" of the body, and amongst other things, it is known to control the endocrine system, the ovaries and testes, appetite, and sleep - disturbance of all of which figure prominently in PWS. Research is continuing into how these systems operate, and gradually knowledge is increasing.
Currently no overall cure has been found for PWS. Some aspects of the syndrome can be corrected by surgery, drugs and hormonal treatment, and doctors are now researching the cause and treatment for the overeating associated with the syndrome. In the meantime, much can be accomplished with good dietary and behavioural management.
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As more has become known about PWS, other characteristics have been identified which appear in many, but not all, people with the syndrome. It is unlikely that any one individual would exhibit all these additional features. These are:
| "Robert is nearly 18 years old, has the classic features
of PWS, including the appetite drive. He is fortunately free from many other
symptoms such as speech impediment and skinpicking and his I.Q. level is
quite reasonable... there have been bad times: shoplifting, money taken,
screaming in the house, excessive weight gain. There have been good times
when he has fitted in, when he has tried hard."
"Andrew (21) is very overweight, approx. 5' I" (155 centimetres) tall, waist size 52" (133 centimetres). He is loving and caring. Temper tantrums are a thing of the past." "Katie (21) has a bright lively personality and is well liked by everyone who meets her. She is 5' 1" (155 centimetres) tall and at the moment weighs 10 st. 10 lbs. (68 kilograms) - the heaviest she has ever been." "Liz (24) is under 5 (152 centimetres) tall and very obese. She has an I.Q. of 93 and is very articulate. She suffers from mild asthma, for which she uses an inhaler when necessary. She also has diabetes which is controlled by tablets." |
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Sexual development is immature in both men and women. In males, undescended testes are very common. They may be brought down by surgery, or sometimes descend spontaneously. The penis may be small or normal in size. Puberty is usually delayed, and with scanty growth of facial and body hair. Testosterone replacement may be considered, but this should be on an individual basis. Those individuals who receive testosterone should be carefully monitored, as there is anecdotal evidence that there is an increase in aggressive behaviour.
In females, the immature sexual development is less visually obvious. Breast development may not be totally complete. Periods (menstruation) may be very delayed - sometimes as late as 25 to 35 years of age, or they may not happen at all. They are often very irregular. Cyclical oestrogen in the form of oral contraceptive pills may be prescribed for women who are not having periods and wish to have them. Additional advantages of taking oestrogen are that it provides some cardiovascular protection, and also protects the bones against osteoporosis.
A few people with the syndrome may begin puberty very early, but they also do not develop completely.
There are no definite reports of either men or women with PWS having a child.
| "Michael (27) developed the usual adolescent symptoms in his late teens but these were made much worse when the consultant decided he needed injections of testosterone. He only had one and within a couple of days was having nightmares, delusions and becoming very aggressive. We since found out (after changing consultants) that he was making enough of his own hormones and we were advised that he should have been started off with a low dose tablet first to see what the side effects would be" |
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Some typical psychological characteristics such as temper tantrums or rages, obsessive behaviour, and skinpicking have been observed in people with PWS. These are discussed in the chapter on Psychological Issues and Behaviour Management.
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People with PWS are living longer than previously, as understanding about better management grows. There have been several recorded cases of people in their 60s with PWS, and the oldest recorded person died at the age of 71 (1).
A survey of people aged between 30 and 55 years which was carried out in the USA, found that abnormally high blood pressure (hypertension) and diabetes were the major health problems of adulthood, together with obesity, osteoporosis, restrictive lung disease, and leg problems (oedema, cellulitis). Psychiatric and behavioural problems were the most common cause of hospitalisation. Strokes, heart diseases and cancer were not common (2).
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Yet even though many people with PWS have a level of intellectual ability which would ordinarily enable them to live fairly independent lives, there is still a continuing debate which engages both parents and professionals, and on which they sometimes take opposite sides. It is simply stated, but it involves many complex issues:
Should a person with PWS be allowed to eat as much as they wish, and by so doing greatly decrease their quality of life and risk the very real chance of what is sometimes a very unpleasant and distressing death before they are 25 years old? Other questions follow.
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Research has shown that nearly all individuals with PWS appear to feel hungry more frequently, and will eat considerably more than other individuals when given free access to food, in an attempt to satiate the feeling of hunger (1). As shown in the section on the Characteristics of Prader-Willi syndrome, this need to eat results from an organic dysfunction in the brain; it is not pure greed.
The results of unchecked eating can be disastrous. Most people with PWS are shorter than average, yet they can reach weights of over 30 stones (191 kg). In the early 1980s, a man in his 20s died weighing 46 stones (292 kg).
Anecdotal evidence from parents shows that although the person with PWS may display detailed knowledge about their dietary requirements, and will refuse fattening food in supervised surroundings, the temptation to take food when unsupervised is usually very great. One parent recounted how her daughter refused roast potatoes at a family gathering, insisting she should only have the boiled variety. The daughter was later discovered eating the leftover roast potatoes from the rubbish bin.
On the other hand, further anecdotal evidence from parents suggests that there is a minority of people with PWS who are less likely to take food, even when unsupervised. People may also go through phases, sometimes being very self controlled around food, and then losing control. This is one of the reasons why it is difficult to lay down any firm rules about dietary control for people with PWS. The section on Dietary Management includes ideas which take account of all levels of ability to control appetite.
There is definitely a case to be argued that most people with PWS have a disability which makes the presence of food in an unsupervised environment potentially life-threatening to them. However, it may be more difficult to argue that an individual does not recognise the consequences of overeating, and understanding of this may vary widely between individuals.
There are no easy answers to the ethical question of whether a person with PWS should be allowed to eat as much as they like. What is important is that the person with PWS is fully informed about the options available to them, and that they have some understanding of the likely outcomes. It is far more likely that they will co-operate with a care regime which will help them live a healthy life, if they feel that the decision has been made by themselves.
Those with a more severe learning difficulty who find it difficult to understand the implications of their eating behaviour are ultimately dependent on their parents or carers to make the decisions for them or with them.
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In the UK there are no laws which relate specifically to people with Prader-Willi syndrome. However there are some legal actions which may be taken to protect the person either from themselves or from other people. In very rare instances, action may need to be taken to protect other people from the person with PWS. If legal action is being considered, it is important to discuss all the issues with everyone involved before proceeding, including if possible the person with PWS.
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Ordinarily, the law acknowledges that certain types of contract with regard to the sale of goods and services which are necessary for everyday living should not be withdrawn from people with learning difficulties. These rights are encapsulated in the Disability Discrimination Act and they include buying food.
However, if the person enters into a contract which is injurious to them (i.e. will substantially damage their health), the vendor can be taken to court. If the vendor alleges that they did not know and had no reason to know about the person's disability, the contract may be upheld.
It is therefore important that local traders are informed about PWS if the person regularly shops with them. It would be difficult to prove that someone buying a couple of snack bars was injuring their health, but an individual trying to buy two dozen bars might be considered to be doing so.
| "Andrew (21) is very good at reading and writing. Money Understanding is still very poor. He has pocket money and keeps a record of his spending in a cash book so that I can see where it's gone and help him understand what he is spending and how much change he should get. We have been doing this for about 18 months but progress is very slow." |
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If a person opens a bank, building society or post office account, they are entering into a contract. If they understand the significance of the transaction, i.e. that money is paid in, and then money is drawn out, and if the person is able to sign their own name, they should be able to open the account.
However, if the person is deemed to be vulnerable as a result of being able to do this, there are two options which can be taken :
Unless you can be very sure that the person with PWS will not be spending their money on food, it might be wise to take up one of these options when an account is first opened. A person who already has sole access to their bank account is unlikely to give up this right without a fight.
| "Esther (18) signs her own allowance books and goes with me to the Post Office to collect the money. While we are still in the Post Office, she hands over most of the money to me for her keep, clothes and bills. She keeps £22, which disappears very quickly on presents, magazines, stationary, craft items, etc. Sometimes she manages to save a little in her Building Society." |
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Appointees are delegated by the Department of Social Security to receive and apply for State benefits on behalf of a disabled person who cannot manage their financial affairs. When a person is living at home, this is usually a parent or other relative. In the case of someone with PWS, a very detailed case may need to be prepared in order to persuade the DSS and social workers that appointeeship is appropriate.
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Trustees are usually appointed to look after a person's affairs after their parents have died, especially if they inherit money or property from their parents' estate. Trustees can be any adult who does not have a mental disability, but it is advisable to have two or more people as Trustees, and to include a professional advisor such as a lawyer, as well as relations. The advice of a solicitor who specialises in trusteeships for people with learning disabilities should be sought when making a will.
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| This section on the Mental Health Act 1983 is contributed by Dr Tony Holland, Senior Lecturer in Learning Disabilities, Department of Psychiatry, University of Cambridge. |
In some cases the behaviour of the person with PWS may become so difficult, and/or be such a danger to themselves or to other people that there may be provision under the Mental Health Act to help that person.
The Mental Health Act (MHA) became law in England and Wales in 1983, superseding the 1959 MHA. Scotland and Northern Ireland have their own mental health legislation. There are a total of ten parts to the MHA which are concerned with different matters relating to people who may have or definitely have a mental disorder. These include the detention in hospital for the assessment and treatment of a mental disorder, guardianship orders in community settings, criminal proceedings when the person concerned may have a mental disorder, consent to treatment for a mental disorder, rights of appeal to Mental Health Review Tribunals (MHRT), the management of property and affairs of people with a mental disorder and other matters including, for example, the right to after-care when discharged from hospital.
In understanding the use of the MHA it is important to appreciate, firstly, the broader legal framework within which we make decisions about our own lives and specifically about whether or not to accept any form of treatment, and, secondly, what is meant by the term "mental disorder".
Consenting to Treatment by Adults.
In the UK adults (18 years or older) can choose to consent or not to treatment recommended by a doctor or other health professionals. As the law stands at present, if an adult is unable to consent, either because they are unable to understand the nature of the decision or unable to communicate their decision (e.g. because of a severe learning disability or advanced dementia) then medical treatment can only proceed if it is considered to be a "necessity" or an "emergency". No-one else can legally consent on their behalf. Where a person does not have the "capacity" to consent and a decision relating to medical treatment has to be made, but is neither a "necessity" nor an "emergency" then the Courts must be involved. Under these circumstances the Official Solicitor acts for the person concerned and seeks independent expert advice.
Recent Court cases have highlighted the difficulties created by this lack of framework with regard to decision making on behalf of an adult who cannot make an informed decision themselves. The Law Commission has proposed a new Act of Parliament (The Mental Disability Act) which if passed would provide such a framework. The proposal recognises that the "capacity" to make decisions is not an all or nothing phenomenon. Some decisions are clearly more complex than others, and therefore someone may have the capacity to make some decisions but not others. The above situation applies at present to treatment of physical disorders. If a person is thought to have a mental disorder then a person may consent or not to accept treatment for that disorder. If the person does not wish to consent there are powers under the MHA whereby a person can be legally detained in hospital against their wishes in order to receive treatment for their mental disorder. The circumstances where this may apply are outlined below.
What is meant by "Mental Disorder" in the MHA.
In the case of people suffering from a mental disorder (e.g. a mental illness) it was recognised that the mental disorder itself may seriously affect the decision-making capacity of that person and therefore under specific conditions it may be appropriate to admit that person to hospital without their consent for treatment of their mental disorder. For example, a person with profound depression who felt that his or her life was hopeless and that they did not deserve to live should be prevented from harming themselves and treated for their depression in the knowledge that when well they would no longer feel that way. The MHA enables such compulsory treatment to take place.
The MHA defines "mental disorder" as '". . . mental illness, arrested or incomplete development of mind, psychopathic disorder and any other disorder or disability of mind . . ." Arrested or incomplete development of mind is further defined under the categories of "mental impairment" and "severe mental impairment". These are essentially synonymous to "'mental handicap" or "learning disabilities" but with the addition of a clause regarding the person's behaviour. To meet criteria for '"mental impairment or severe mental impairment" as described in the Act there has to be evidence of mental handicap/learning disabilities and this has to be "associated with abnormally aggressive or seriously irresponsible conduct . . ." When compulsory assessment and treatment of a person with a mental disorder is concerned, very specific criteria have to be met. These are outlined below.
Assessment and Treatment under the MHA.
Crucial to understanding the role of the MHA is the appreciation that it only applies to people suffering from or suspected as suffering from a mental disorder and that it only applies to the treatment of mental disorders. It cannot be used to force someone to accept treatment for a physical disorder. Furthermore, "'mental disorder" is defined broadly for some parts of the MHA and much more strictly in other parts. In deciding whether the use of the MHA is appropriate or not the following need to be considered. This is a brief summary and does not deal with the full complexity of using the MHA.
If appropriate criteria are met as described above, a person may be detained in hospital under what is referred to as a "'Section" of the MHA. The two most commonly used civil Sections are Sections 2 and 3. If detention in hospital is being considered when the person has committed an offence, different sections, but with broadly similar powers as civil Sections, can be ordered by the Court (e.g. Section 35, 37 or 38). For civil sections to take place, usually a social worker makes the application after consultation with the nearest relative; and two doctors, one a psychiatrist, and the other a doctor who knows the person (e.g. GP), have to recommend that such a course of action is appropriate.
The criteria required to be detained under a Section 2 are less stringent as this is primarily for assessment and only lasts 28 days. A Section 3 lasts up to six months but can be removed earlier by the psychiatrist in charge of the person's care, or it can be renewed for a further six months. During this period the person has to stay in hospital. The nearest relative can also ask for the person's discharge and there is a right of appeal to an independent Mental Health Review Tribunal with both Sections 2 and 3. Under these Sections there is the power to treat a person's mental disorder without their consent. In practice this primarily applies to the giving of medication for the treatment of a mental illness. Second psychiatric opinions are required for such treatment to continue after three months without the person's consent. Although leave of absence from hospital can be granted there are no powers under the MHA to insist that the person continues to take medication after they are discharged from their Section and from hospital. This may change in the future.
Guardianship Orders.
As described above, the MHA has very limited powers with regard to the treatment of mental disorders outside of hospital. However, Guardianship Orders provide a framework for supervision and support in community settings. The MHA lays down specific criteria which need to be met if such an Order is to be used. The local Social Services Department apply for and act as the Guardian, with the agreement of the nearest relative. The Guardian has the powers to insist that the person lives in a designated place and that they allow access to named people such as the social worker and treating psychiatrist. In the context of an offence the Courts can also place a person on a Guardianship Order providing appropriate conditions are met and recommendations are made.
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Mr GS is aged 19 years. He has PWS and has recently left the local special needs school and is now attending classes in the local college. He has chosen to take literacy and numeracy classes and life skills which include cookery, budgeting and shopping. At home his parents and younger sister have closely monitored his food intake for fear that he will eat excessively and become seriously overweight. At night food cupboards are locked. A full needs led assessment is being undertaken by the local Social Services Department and he has stated to the social worker that he would like to live independently.
Some months after starting college his parents become aware that his weight has increased from 11 stone (70kg) to over 13 stone (83kg). His behaviour has also changed. He has become moody, irritable and at times tearful. He insists that he is leaving home to manage his own life. There are increasing temper outbursts. A small flat is found for him and he leaves his parents to live by himself with some support from the local Social Services Department. They would like to offer more but there are limited resources and Mr GS does not wish for further help. He is noticed to be putting on weight at an alarming rate, he has been in trouble shoplifting for food and his appearance and physical health have deteriorated.
At a case conference his family express serious concern about their son's behaviour and his now significant obesity. Mr GS himself refused to attend and has told his GP and social worker that he can manage on his own. The question is raised as to whether the use of the MHA is possible and whether this would be an appropriate course of action.
In these circumstances it has been recognised by all concerned that the ideal solution would be to work with Mr GS at his home, to identify the reasons for his change in mood and behaviour and to help him modify his diet and loose weight. However, this has proved impossible and all agree that this approach is unlikely to be effective. It is also accepted that "something needs to be done" but everyone is nervous and uncertain about using compulsory powers.
Given the above circumstances it is appropriate that assessment and treatment under the powers of the MHA should at least be considered. Other than Mr GS himself, the key people are his parents (one being designated as his "nearest relative"), an approved social worker, his GP , and a local psychiatrist (probably in the learning disability service). Given uncertainties about the nature of and reasons for some of his present difficulties the most appropriate "Section" to consider would be Section 2. This is for assessment and lasts 28 days.
The first priority at this stage would be to ensure that a detailed picture exists of Mr GS, including his past history and his present difficulties. Every attempt should be made to see Mr GS in order to assess his present mental state and to discuss options with him. On the basis of previous information and the recent assessment it should be possible to decide whether Mr GS meets the criteria for having a "mental disorder" necessary for Section 2. Given that he has PWS he is likely to have had some early developmental delay and to have had learning disabilities in association with mental impairment. If this is the case, he would meet the criteria for " arrested and incomplete development of mind", the phrase used in the MHA. Given the history of his other problems, depression may be suspected. He can therefore be said to have a "mental disorder" and if it is "of a nature or degree which warrants the detention of the patient in a hospital for assessment . . ." and if it is necessary "in the interests of his own health or safety or with a view to the protection of other persons" then a Section 2 could be used in order to detain Mr GS in hospital without his consent.
During the 28 days that this Section lasts, assessments should be undertaken to determine the reasons for the mental health problems and to consider the future course of action. If continuing treatment in hospital is considered appropriate, then a Section 3 may need to be used. The conditions for this are more stringent as the person has to meet criteria for a specific "mental disorder" (e.g. mental illness, mental impairment, severe mental impairment or psychopathic disorder) as well as other conditions.
A further question which needs to be considered is whether the obesity which occurs is due to a mental or physical disorder. If the former then the MHA can apply, if the latter it cannot. My own view is that the obesity is directly related to having PWS and is due to the inability to feel satiated following food intake. I would consider that this is a mental problem in the same way as it is for people suffering from anorexia nervosa, but in an opposite way.
If all these criteria are met then further detention in hospital under a Section 3 may be possible. The purpose of this would be to ensure weight reduction, treat any additional mental health problem (e.g. depression) and to try to develop a strategy for longer term support with Mr GS's agreement.
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The police should not interview a person who has learning difficulties except in the presence of an "appropriate adult". This can be a relative, a social worker, carer, or anyone else not employed by the police.
A particular problem arises with regard to those who are both superficially very articulate, and also intellectually able. It may be useful to provide such individuals with a card they can use in an emergency stating that they have Prader-Willi syndrome, and giving a contact name and telephone number. You may also want to give the phone number of the PWSA (UK) who can confirm that this is a bona fide case. This is obviously very important if the person is arrested.
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Voting. People with disabilities of any kind have the right to vote if they can distinguish between the candidates and can answer the question :"Are you the person whose name appears on the Register of Electors as . . . ?" People who are subject to Guardianship or compulsory Admission Order under the Mental Health act cannot vote.
Driving Licences. People with learning difficulties and physical disabilities can hold a driving licence unless they cause a danger to the public. A few people with PWS in the UK have had driving lessons, but have given up when they found it too difficult. A few can drive tractors and farm vehicles on private land. As far as we are aware, no-one with PWS holds a full driving licence in the UK, although at least one case is known in the USA. In a talk to members of the PWSA (UK), Louise Greenswag from the USA mentioned a young man who passed his test and drove around quite well until he saw someone else crossing an intersection when the lights were against them. The man with PWS followed the perpetrator and rammed his own car into the other person's as a punishment for disobeying the rules. This aptly demonstrates the difficulties involved when a person with PWS is intellectually and physically able to do something, but is emotionally unequal to dealing with any unforeseen circumstances which may arise.
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If you need a solicitor to help with any legal issues, it is important to ensure that he or she has experience of the law relating to learning disabilities. Your local Mencap group may know of such a solicitor in your area.
The PWSA (UK) is always willing to offer help and advice in any issue concerning PWS, especially if the knowledge of an "expert witness" is required. Contact the Secretary/Welfare Officer for more details.
(1) Characteristics of the eating disorder in Prader-Willi syndrome: implications for treatment, Holland AJ, Treasure J, Coskeran P , Dallow J: (Journal of Intellectual Disability Research, (1995) 39, Part 5, pp373-381).
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As discussed in the previous chapter, limiting food intake to maintain the best level of health can be a great challenge for both people with PWS and their parents and carers.
However, it must be borne in mind that the consequences of severe obesity can place many limits on a person's quality of life, and their health will almost certainly suffer. The following are some of the most common outcomes of severe obesity in PWS:
Currently, the only effective long-term prevention or treatment of obesity in PWS is a low-calorie diet, coupled with regular exercise.
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Most parents would agree that their son or daughter's quality of life and happiness does not begin and end with food, and that support should be offered to help them realise this. However, there may be a few parents who feel that their son or daughter will only be happy with unlimited access to food, or who, having tried everything within their power, just feel like giving up.
No one can judge what is right for your son or daughter, but there are perhaps a few considerations you should think about before taking the step of withdrawing any sort of control. Does the person with PWS really want to become so overweight that they cannot move, and so overweight that they will die within a few years? Have you discussed this with them? What are the opinions of other family members, care staff and social workers? Is coping with the physical needs of an extremely overweight person, who will become less and less able to perform any actions for themselves, any easier than trying to support and help them with their diet and behaviour? Only you and your son or daughter can decide, but perhaps you need to think of the implications first, and to consider implementing some of the alternatives given below, before you give up entirely.
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If a person has Prader-Willi syndrome, keeping weight to a reasonable level is doubly difficult. They have the same nutritional requirements as everyone else, but because of poor muscle tone and short stature, people with PWS require fewer calories than most to maintain stable weight levels - yet they almost always feel hungry. Most parents and professionals would agree that it helps the person adjust to the problems imposed by the syndrome if the person has been used to living on a restricted calorie intake since childhood. However, with many adults still being diagnosed in their teens or later, they may need a great deal of support to adjust to a lower calorie intake if they have not previously been used to this.
If you are in doubt about how to ensure that the person is getting all the nutrients they need, or are unsure about how many calories they require, it is a good idea to get a referral from your GP or consultant to a registered dietitian. The PWSA (UK) booklet Prader-Willi Syndrome: Food and Health gives more information about the dietary and nutritional needs of people with PWS.
Not everyone with the syndrome has the same problems around food. However, most have food as one of their main interests in life - so much so, that some people seem to be continually asking when the next meal is due, what it is, what everyone else is having, and so on. Some would never steal food from shops or other people; some unfortunately will. Some are capable of preparing their own meals with little or no supervision; for others, time spent in the kitchen is time to eat as much as they possibly can, and even to secrete food on their person for later. Some people will eat items such as pet food, frozen food, garden produce (including potentially poisonous berries) and food from garbage bins, whilst others will not. So far, no-one has been able to account for the reasons for these differences in eating behaviour, or to say whether it is due to different levels of damage to the hypothalamus, or to different social, cultural and family influences. What is certain is that one person with PWS will require a different level of help and support to another, although all will require some support.
| "Andrew (21) is supervised quite well at the centre but takes every opportunity that occurs for food stealing or scavenging." |
The issue of self-control becomes all-important as the person becomes an adult. If control over food at home is impossible for the person with PWS, control in many other environments, such as shopping and going out independently, is also very limited. This lack of personal control in so many areas of life may be one of the contributory factors to the behavioural problems which are discussed in the following chapter. It is therefore important to give back some control to the person in other ways, for instance, in agreeing a contract about food and diet, or consulting them about the day's menu. Some people become very upset by the word "diet", so you may need to use another term such as "healthy" or "sensible" eating. Responsibility and control can be provided in other ways, such as asking the person to do their own laundry and keeping their room tidy.
Other ideas for supporting the dietary needs of people with PWS are given below, and range from almost total segregation from food to greater degrees of self-control.
| "Through vigilance and constant diet, Martin (28) is quite slim. He is 4'11" (150cms.) and weighs just under 8st. (50kgs.)." |
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Environmental control basically means the removal of accessibility and thus temptation. If food is not available, the person is less likely to seek it. Environmental controls can take several forms, ranging from major to minor, and they will be needed for those people with PWS whose self-control around food is minimal. Here are some suggestions:
These can be ordinary locks and keys, padlocks, or more sophisticated electronic locks, where a code is punched in by those who have legitimate access to the kitchen or fridge. Keep tools stored safely away - these may be used to force locks. You could consider giving back control to the person with PWS by providing them with their own locked table-top fridge (with their own code) stocked with small amounts of, for example, diet drinks, fruit, or even the day's meals (if you can be sure they will not be consumed all in one go). Even more control can be given by suggesting that the person buys their fridge and lock with their own money.
Do not put sauce bottles, bread, etc on the table before the meal commences. Clear everything from the table immediately after a meal and put leftovers straight into the rubbish bin or into the fridge for later (both of which may need to be locked). Though expensive, it may be worth buying a waste disposal unit to deter those who forage through rubbish bins. Make sure that family members do not leave sweets, crisps etc in coat pockets or unlocked drawers. It is important to try to teach the person about the significance of private property. If everyone in the family agrees, there should be some cupboards or rooms in the house which belong to each family member, and no one else should be allowed to go into them without their permission. This has to apply to the person with PWS as well, who should also have their own private space.
This is a difficult contract to make unless the person is never allowed out on their own. For those who do go out independently, the risk is always there. A sudden increase in weight may indicate illicit visits to food shops, but it may also mean that the person has been obtaining food by some other means, such as stealing from shops, foraging in bins, taking milk from doorsteps, or asking strangers for food or money. You can minimise some of the risk by alerting local shopkeepers and neighbours, but you may also need to agree with the person that going out independently is a privilege which they can have as long as they keep to the contract, and which will be withdrawn if they do not. Regular weighing is the best way of monitoring whether the person is getting outside access to food. It is important not to make the person feel as if they are failing if the contract is not kept - emphasise what they can do whilst suggesting that next time someone goes with them. It is also a good idea to point out a few of your own shortcomings at the same time.
If a person is very uncooperative about food, will not agree to any form of contract, and is severely overweight, you may have to consider taking complete control yourself. This is the sensible option in the face of overwhelming difficulties, but you should be aware that, as the person with PWS is an adult, you do not have an automatic legal right to control their food intake. In most cases, common sense will prevail, but you may have to resort to some of the legal options outlined in the previous section on Ethical Issues.
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There are many ways in which you can help the person reduce calories by adapting your methods of preparing, cooking and serving meals. These should not impinge to any great effect on the family's daily lifestyle. Here are some of the most commonly used:
There are many other suggestions in the PWSA (UK) leaflet, Weight Control and Suggestions to Assist with Weight Management.
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There are some kinds of eating behaviour which the person can learn to see as socially acceptable, or which will gain them favour with other people. The social environment can also be altered to be more supportive. Educationally, most people with the syndrome benefit from knowing about the effect PWS has on them, and the need to limit their food intake for the sake of their health.
People can therefore be educated about what is a reasonable portion of food to have on their plate, not to eat whilst they are talking, and to wait until others are served before commencing the meal. Reward or praise the person when they do this correctly.
Keeping meals to set times can also be helpful, as the person will learn not to expect food at other times. This, of course, is not always possible, except in the most well regulated of households, so the person also has to learn that sometimes meals will not be at the normal time. If possible, warn the person in advance that the meal will be late, even if it is only by half an hour.
Others in the family can be supportive by not eating in front of the person with PWS, joining in with the "healthy eating" programme, and remembering not to leave leftovers lying around.
Many people can also be taught to recognise foods which are high in calories and those which are lower in calories, and your son or daughter may enjoy shopping with you for the healthier options. Some people will be able to read food labels for themselves. It may be advisable to ask the person to help you make the shopping list before you set out - this will minimise arguments in the shop. Always praise the good choices, and suggest alternatives when an inappropriate choice is made. Make sure that YOUR facts are correct before passing on your knowledge to the person with PWS: the PWSA (UK) dietary management leaflets provide useful information about the calorie content of most food and drinks.
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Some people with the syndrome appear to benefit from having greater personal control over what they eat, especially in terms of improved behaviour. However, this is not a step to be taken lightly, as it can quickly get out of hand, and in any case, the person is unlikely to remain slim if allowed totally unsupervised access to food. Some other factors you may wish to consider before taking such a step are:
Here then is one step-by-step suggestion for helping the person take more control. Take one step at a time until you are sure it is working, go back a step if not. You may wish to change the order of the steps to suit the particular needs of your son or daughter. for example, you could make meal preparation the first step before going onto shopping.
Go shopping with the person for vegetable items for a meal. Offer choices between two items at a time. If you go first thing in the morning, the person is likely to be less tired, and the shops will be quieter. Do this a few times to see how they manage the decision-making process.
Go shopping as in Step 1, but let the person have their own basket and pay for the items they choose. Do this several times to make sure additional items are not being added to the basket.
Let the person come into the kitchen with you to watch you prepare the meal for which they have chosen the items. Explain what you are doing and why as you go along. If the kitchen is normally out of bounds, go back to the contract and make sure they agree not to take food whilst they are there. Stay on this step for a while to ensure that the person can cope with being in a kitchen environment. If chaos ensues, go back to Step 2, and perhaps try again in a few months time.
The person prepares their own simple meal supervised by you. Make sure they know how to use the correct utensils for each separate task. If this is successful, choose items and prepare a meal for the whole family.
Gradually extend the choosing, shopping and cooking to other items and more complicated meals.
Your son or daughter decides what they are going to do if their weight goes up: cut down on calories and/or take more exercise; or hand back more control to you.
If appropriate, withdraw some of the supervision in shopping, cooking and preparing meals.
Stick to the contract, and go back a step or two if it is not working out. Regular weighing is a vital part of the process, otherwise you will not be able to judge what is the right amount of control for your son or daughter's weight management programme.
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Eating out is one of the pleasures of life for most people, with many outings and special occasions organised around a meal in a restaurant, or a party. There is no reason why the person with PWS should be left out of the celebrations, but some advance planning and agreement may be required.
These are a few of the options you could consider.
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There are now many diet drinks on the market, which are generally considered to be safe as long as they are not consumed in large quantities. Cola, while OK in moderation, has a decaying effect on teeth, which are often very vulnerable to decay in PWS. Therefore, if possible, a variety of other drinks should be offered.
Use skimmed milk for the person with PWS's tea and coffee, and sweetener rather than sugar. Some people may enjoy herb, fruit or lemon teas. If possible, use different types of sweetener in different drinks, so as to avoid a very high concentration of any one type of sweetener in the total intake.
Anecdotal evidence from parents suggests that alcohol has very similar effects on people with PWS as it does on the rest of the population, although a person with PWS may become intoxicated more quickly than most people, and having drunk smaller quantities of alcohol.
Going to the pub can be a very helpful socialising experience for adults with PWS, but if possible someone should go with them who understands their dietary needs. Alcoholic drinks have a high calorie content, but there are now some drinks on the market which are lower in calories. If a visit to the pub is planned, try to make up for the extra calories by going without a dessert at mealtime. Low calorie lemonade, tonic water, etc, can be used as mixers to keep overall alcohol and calorie levels lower.
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Regular exercise is very important for people with PWS and whenever possible it should be incorporated into their daily programme. It is important for several reasons:
Martin (28) with one of the England swimmers after doing a sponsored swim to send the England team to the Commonwealth Games. |
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Exercise can be taken in regular sessions with the aid of videos, exercise bikes and step machines (while watching favourite TV programmes or listening to music); by going to classes, or even by hiring a personal trainer using the person's Disability Living Allowance this is especially good for those who thrive on one-to-one attention. It can also be taken in less formal ways: housework, gardening, cycling, walking, riding, dancing, door to door collections, delivering newspapers, etc.
| "One of the successes that we had with Jane was buying a small dog. Jane (20) could take it walks on her own and it wouldn't say if it had been via the sweet shop. Jane likes walking and, if she is in a good mood and has a target, will walk quite long distances. Whilst at college she did a 10-mile sponsored walk" |
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It may be necessary to support the person with PWS to lose several kilograms in weight. This will involve a decrease in daily calorie intake with, if possible, an increase in the level of physical activity. You may need to get advice about calorie levels and calorie counting from your GP or dietitian. If they have little or no knowledge about PWS, send them appropriate leaflets on PWS in advance of the appointment. The leaflet, Notes for Dietitians, obtainable from the PWSA (UK), is especially aimed at dietitians, and the booklet Prader-Willi Syndrome: Food and Health also offers some useful suggestions.
Keep to realistic expectations: 0.5-1 kg (2-4lbs) per month is quite adequate. Some people find a sponsored slim a very attractive way to lose weight, whilst others may get some benefit from going to a Weight-watchers Club, or similar (check with the organisers first before going ahead). It obviously helps if someone else in the family or a friend is also attempting to lose weight and can sympathise. Some may find working towards fitting into a fashionable outfit is a sufficient target, whilst others may need the extra support of colourful charts, and tokens towards rewards such as CDs or outings.
Another idea is to start a "video diary" - using a camcorder to take a few minutes of tape each week, so that the person can see their weight loss from week to week, and talk about their difficulties and successes in losing weight. A successful video diary would be a very valuable resource for the PWSA (UK) and extremely useful for encouraging others.
If a video is not available, take a series of photographs, perhaps with the tape measure showing the person's measurements.
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Miracle diets.
Generally speaking, commercial "miracle diets" may work in the short term, but their effects do not last. Some diets are dangerously short of essential nutrients - consult a registered dietitian or your GP before embarking on any of the latest fad diets.
Appetite suppressants.
Up to now, conventional appetite suppressants have had mixed results on people with PWS, often having unwanted side effects on the nervous system, and in a few cases triggering episodes of mental illness. They do not always succeed in reducing appetite and they are rarely effective in the long term. Effects in normal people only last about 6 weeks.
Surgery.
Gastric by-pass operations can be performed, but usually as a last resort. There is a risk for very obese people under anaesthetics, and side-effects of gastric by-pass such as continual diarrhoea and unwanted folds of skin can be very unpleasant.
Wiring of the jaws is generally considered inadvisable. One parent recounted that her daughter still managed to eat a lot of food even though her jaw had been wired.
Hospitalisation.
Hospitalisation may be the only alternative in cases of severe obesity. A person may need to be admitted to hospital to reduce weight under supervision (see the article on the Mental Health Act in the previous section). This will only work if the person does not gain access to food (e.g. from other patients) and nursing staff are aware of the person's cleverness in obtaining food from unusual sources.
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In recent years a growing body of knowledge from both parents and professionals has identified several behavioural problems which occur frequently in people with PWS.
These common behaviours associated with PWS are:
| As with every other symptom of the syndrome, the degree to which these behaviours occur varies widely from one individual to another, and some may not occur at all |
The typical personality of an adult with PWS has been described as " . . . extraordinarily stubborn, clever, manipulative, moody, and prone to temper outbursts . . . perseveration and egocentrism are often accompanied by self-stimulating behaviour such as skin-picking. Dishonesty in the form of lying is also common along with poor interpersonal skills, obsessions, and occasionally frank psychoses. Hoarding, pilfering of food and non-food items, and verbal aggressiveness are reported; social skills appear to decline resulting in deteriorating peer relationships." (1).
Some parents may immediately recognise their son or daughter in this description; others may recognise some of the traits, but feel that they are either very mild in their own son or daughter, or balanced by more positive aspects of the person's character, such as kindness, friendliness, willingness to help others, and so on.
| "Esther (18) copes well with minor upsets to her routine
very well and usually sees the funny side of things. Although she has the
occasional outburst, she is generally a very caring and thoughtful person,
with a good deal of common sense."
"Moira (20) is caring, shrewd, determined, sensitive, an accomplished liar, thief of food (or money for food) can be spiteful, loyal and patient." "Janet (38) is still demanding and can be stubborn with a characteristically obsessive nature. She needs a firm hand and regular routine but she is also friendly, sociable and enthusiastic and likes to be busy." |
One of the most frequently asked questions is : "Are behavioural problems due to Prader-Willi syndrome, or is it something else, or both?" Generally speaking, the answer is "Both, although in different ratios according to the circumstances". Whilst people with PWS have some traits in common, they are still individuals with different lifestyles and histories, which also need to considered when assessing behavioural problems.
These, then, are some of the factors which need to be taken into account when supporting the person with PWS to change their behaviour for the better:
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Each of these factors will now be examined in more detail, to attempt to identify how they may cause behavioural problems, and what can be done to manage their effects.
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Learning Disabilities. In the UK, learning disability (previously known as "mental handicap"') is defined as " a state of arrested or incomplete development of mind that includes significant impairment of intelligence and social functioning" (Mental Health Act 1983).
All people with PWS have some sort of learning disability, learning problem or cognitive disability, although it may not be immediately noticeable. A minority of people have a tested intellectual level (IQ) approaching the average for the general population, which is 100. In such cases, learning difficulties become evident when the person has the factual knowledge, but does not have the emotional maturity to use it appropriately in social or work situations.
In fact, most people with PWS have an IQ of around 60-70, so that their learning difficulties are classed as "mild" or "borderline". A minority of people with PWS have more severe disabilities, with an IQ of below 50 which places them in the "moderate" to "severe" range (2). Whatever their tested IQ level may be, people with PWS tend to perform at a lower level in their day to day life because of their emotional immaturity.
Generally speaking, people with PWS are better at reading and writing than they are at mathematics and more abstract concepts. They often excel at jigsaw puzzles and "Wordsearch" puzzles. Short term memory is often a weakness, whereas long term memory can be good. Information is remembered more readily if it is visually presented than if aurally presented. In other words, don't just tell the person to do something, show them as well, and if necessary use pictures and words to remind them.
There are often difficulties in sequential processing, which means that the person finds it difficult to follow more than one instruction at a time, and quickly becomes confused by complicated requests. They tend to see situations in terms of "black and white", and most feel happier when they can operate within an established routine. There may be problems with learning to tell the time and the concept of future time, so that if the person is told something will happen "next week" they have little idea of when that actually is. For some people with PWS, these difficulties are so great that they become anxious about what they should be doing and when, and will continually seek reassurance about when something will happen.
| "Richard (28) has no concept of time whatsoever. It's a constant battle to get him ready in time for the minibus which calls to take him to the local education training centre. We insist that he doesn't get breakfast until he is washed and dressed and made his bed - needless to say there are some mornings when he goes without breakfast (not many though)." |
The difficulties in problem solving also mean that once the person has found a way of doing something, they are unable to envisage any other way of carrying out a task without considerable internal anxiety or confusion. This results in a stubborn refusal to change, and because they lack the skills to argue reasonably, they resort to screaming and shouting. It can also mean that if a certain behaviour (e.g. screaming) has always resulted in getting what they want in the past (e.g. food), they will continue to do this.
| "Jane (20) easily falls into a routine (and thrives on routine) a change of plan has to be carefully managed. She has good days and bad days - woe betide you if it's a bad day; the only good thing is that tomorrow will likely be a good day. Jane has a good sense of humour and an outgoing person can get a lot out of her." |
The repetition of questions and topics of conversation can be extremely wearing to the listener, and sometimes no amount of answering can prevent the person from asking or telling the same thing again and again. One American researcher, Barbara Whitman, says that this is a function of a nervous system whose on/off switch gets stuck in the "on" position (3). Thus what the person is saying is not the real issue, what is important is to get the person to "switch off".
Some researchers have postulated that the IQ level of people with PWS decreases as obesity and/or age increases, but this has yet to be completely proven.
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Many people with PWS have poor muscle tone even as adults, and find it difficult to move quickly, run, c1imb or jump. They also tend to tire very easily. This can be a very frustrating limitation for them, and they may need a good deal of support and encouragement, as well as patience, when carrying out activities which require some exertion.
Some individuals have further disabilities such as severe speech problems, curvature of the spine, foot and leg abnormalities, etc. All these add to their sense of frustration at not being able to do the things others do naturally. Some people may have very low self esteem as a consequence of their perception of themselves as less than adequate, and will need support and encouragement to recognise the things which they are good at, and where they can be Successful.
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People with PWS do not reach full sexual maturity, although some may receive hormone treatment or surgery to help with this. There is also considerable immaturity in relationships and in understanding about what is appropriate behaviour when relating to others. Again, a basic knowledge of the mechanisms of sexual activity is not supported by an understanding of when and how this is used, and a considerable amount of support and education needs to be used to help the person learn the subtleties of adult relationships. (See also the section on Social Life and Relationships).
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The area of the brain which appears to be most strongly affected by PWS is the hypothalamus (see also the section on the Characteristics of PWS). This is the "control centre" of many of the body's hormonal functions and also of emotional stability. This appears to be immature in PWS, with adults displaying a level of control over their emotions which would normally be associated with that of a young child. Children usually learn to control their emotions as they get older, but people with PWS find this a difficult process, and will need to learn "cues" to realise when their emotions are getting out of control.
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This also seems to be related to the impaired functioning (dysfunction) of the hypothalamus. Many people with the syndrome feel hungry most of the time. To understand this, it is useful to think about what most people do when they feel hungry. They may feel irritable and unable to concentrate; they may keep checking their watch to see if it's time for a meal, and if no food is forthcoming, have a snack to stave off the hunger pangs. This is what people with PWS are doing, but for them the hunger pangs return far more quickly. Once again, the person has to learn how to control this and to realise when they have had enough to eat. They will need much help, support, and supervision to achieve this. (See section on dietary management).
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The hypothalamus also regulates sleeping patterns. Very young babies with PWS sleep most of the time, and this need for sleep continues to a greater or lesser degree throughout the person's life. Parents often say that their sons or daughters will sleep immediately after a temper outburst, then act as if nothing has happened on awakening.
People who have sleep apnoea experience breaks in their nightly sleeping pattern, but some people with PWS who do not have sleep apnoea have also been found to have fragmented sleeping patterns (4). This inevitably impacts on the person's ability to cope with stressful situations during the day.
Again it is helpful to think about how most people feel when they have not had enough sleep. irritable, sluggish, prone to make mistakes and liable to drop off into short dozes. This may happen to people with PWS far more frequently than most people.
A few people with the syndrome have a reversed sleeping pattern, whereby they fall asleep during the day and are very restless at night.
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People with PWS appear to be more liable to have an obsessive personality than members of the normal population. Symptoms may include hoarding, arranging things in a certain order, continual washing, and doing certain things at certain times. Researchers have found that there is a relationship in PWS between obsessional tendencies and family stress (5). Again, the obsession may be a way of coping with a situation which is otherwise beyond the person's ability to manage rationally.
| "We are non-smokers but Leslie (27) has started smoking at 20 and the habit is as obsessional as food and drink" |
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Skin picking occurs when the person continually picks at a spot or sore, or scratches the skin surface until a wound is established. This activity is likely to be more distressing to the observer than to the person actually doing it. It is thought that this behaviour releases pain relieving
substances akin to morphine (endorphins} in the brain, and is actually pleasurable to the person, whose high pain threshold masks the initial discomfort of scratching at the skin's surface. Although skin-picking is commonly confined to the arms and legs, it can occur anywhere on the body, including the rectum. Skin-picking can be distinctly antisocial in its manifestations, and also carries the risk of infection and blood poisoning, so the person needs to be helped to control this behaviour.
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A survey of parents from the USA, Australia and New Zealand found that conflict between parents contributed to severe behavioural problems (6). Any disagreement and lack of a common goal will confuse the person with PWS, especially when one parent or family member is saying the person can do one thing, whilst another relation is saying he cannot: the person needs to know where he or she stands.
Like any other person, an individual with PWS will also learn certain behaviours from other family members. For instance, a family which often quarrels is likely to produce a child who is verbally aggressive.
A chaotic and noisy environment can be extremely confusing to someone with PWS. The presence of food is always an additional distraction. On the other hand, a very quiet, unstimulating environment will usually send the person to sleep: sleeping whilst travelling is very common in people with PWS, along with sleeping in front of the television. A good balance has to be struck between too much stimulation and too little.
An environment which is too hot or too cold may make the person lethargic or irritable.
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Some life events, such as births, deaths, marriages, divorces, accidents, moving house, illness and so on, can be extremely stressful for all concerned. The person with PWS may find this equally distressing, but be unable to share his or her feelings with others, or to express them appropriately. This may result in an increase in difficult behaviour.
Equally, it is sometimes possible to trace difficult behaviour to a stressful event of which you have no personal knowledge, such as bullying or abuse by others. Look for other signs of serious stress such as bedwetting, increased skinpicking, and depression. If these are present, try to discover if there is something bothering the person which they need to talk about.
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Lack of understanding from others.
We all complain from time to time that nobody understands us. People with PWS perhaps have more right than some to say this. PWS is a difficult condition to imagine, and outsiders may underestimate the serious of its consequences. It is important that those who have daily contact with the person understand about PWS, and also that they maintain the same level of support for the person as they get in their own family.
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All the above factors have some relevance for how you can help the person change their behaviour. Understanding what is causing the problem is the first step towards changing it.
Here are some suggestions for behaviour management strategies, based on our understanding of how the person with PWS is operating. They may not all work for everyone and they may not all work all of the time, but they should provide some guidelines towards supporting the person with PWS to change their behaviour for the better. They are not in any order of priority.
| "We are trying to educate Martin (28) to take the newspapers he collects to the Recycle Bin at Tesco's. I think we are winning." |
| "We have had the same Social Worker for four years and continuity helps . . . . but nothing helps him so much as love and kindness, firmness where necessary and providing security (removal of temptation) in as much of his life as possible." |
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Just like everyone else in the population, people with PWS can experience mental health problems (mental illness}. This may take the form of depression or severe compulsive disorders, and occasionally it may result in psychotic or schizophrenic episodes, where the person hears voices, thinks people are talking about them, and loses touch with reality (8, 9). These may happen spontaneously, or be triggered by stressful life events or by adverse effects of other drugs which act on the nervous system. The help and advice of a qualified psychiatrist should always be sought in such cases: it is often possible to help the person with a course of drug treatment. Our knowledge of PWS is insufficient at present to say whether mental health problems are greater in people with PWS than in the normal population, but there are indications that this is the case.
| "Leslie (27) has episodes of psychotic behaviour. This makes her very unhappy, noisy and uncooperative. Because of this behaviour our social services have withdrawn day care at a local resource centre." |
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Generally speaking, drugs would only be used to control behaviour when management strategies are manifestly failing, and where there is an underlying mental health problem. Some drugs have unwanted side effects on people with PWS, either exacerbating the behaviours they were supposed to control, or rendering the person almost incapable of doing anything. American researchers have found that the most effective drugs are Haloperidol (Haldol), Thioridazine (Melleril), and Fluoxetine (Prozac) (7), although their effectiveness will vary from one individual to another. Haloperidol and Thioridazine are used to stabilise mood and behaviour, especially in those who are manic or paranoid. Then the medication is shifted to Fluoxetine, which appears to reduce irritability and makes the person more mellow. Fluoxetine may also be used for those who are depressed. The researchers do warn, however, that the dosages need to be specific for each individual person, and carefully monitored over time by qualified professionals. The effects of taking these drugs over a very long period have yet to be monitored. This research is still in its infancy, and new drugs may be invented which could prove to be of further help.
| "Last year Janet (38) worried us all with signs of delusions
and imaginary illnesses but she received psychiatric help and mild medication
has put her back on an even keel."
Michael (27) is now on Prozac and is much better as regards depression although it still flares up from time to time but nowhere as frequent as before." |
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(1 ) Psychological and Behavioral Management, Barbara Y Whitman and Louise R Greenswag, in:Management of Prader-Willi Syndrome (Second Edition) : Louise R Greenswag and Randell C Alexander (7995) p 126.
(2) Learning Disabilities: The Fundamental Facts, The Mental Health Foundation (1993) p6.
(3) Psychological and Behavioral Management (op cit) p 134.
(4) Sleep Disorders in Prader-Willi Syndrome: Management Considerations, Gila Hertz and Mary Cataletto, in : Management of Prader-Willi Syndrome (Second Edition) : Louise R Greenswag and Randell C Alexander (1995) p330.
(5) Phenomenology of Obsessive-Compulsive Disorder in Persons with Prader-Willi Syndrome, Elizabeth M Dykens, James F Leckman and Suzanne B Cassidy (1995), in: The Gathered View, October 1995, Vol. XX, No 5, p5.
(6) The prevalence and type of behaviour problems and the contribution of extended family health histories and current family stress to such problems in persons with Prader-Willi syndrome: A cross-cultural study, Barbara Y Whitman (1994) in : The Gathered View, August 1995, Vol. XX, No 4, p5.
(7) Psychological and Behavioral Management (op cit.) p 139.
(8) Prader-Willi Syndrome and Psychoses, David J Clarke: British Journal of Psychiatry (1993), 163, pp680-684.
(9) Prader-Willi syndrome and psychotic symptoms: report of a further case, David J Clarke: Irish Journal of Psychological Medicine 1995 March, 12 (1): pp27-9.
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