The quotes in boxes are all taken from responses received from a questionnaire to brothers and sisters, which was circulated with the March 1996 PWSA(UK) newsletter. |
People with Prader-Willi Syndrome (PWS) are special, but so are you.
"Brothers and sisters are often forgotten, and they need support too, just like the parents." |
We think that only about 1 in 15000 people are born with PWS, and this means in the average family of "2.4 children" there are only about 1 in 10000 people who have a brother or sister with PWS (including your other brothers and sisters, if you have any). It is unlikely, but not impossible, that there is anyone you know living near you who also has a brother or sister with PWS, so it may be hard for your friends and neighbours to understand what it is like to have someone with PWS in the family.
However, there are many more people with all sorts of disabilities, and it is quite likely that someone you know has a brother or sister with a different disability who will share some of your concerns and feelings.
Another word for "brother or sister" is "sibling".
The first thing to say is that no-one is to blame. PWS just happens - it is a fluke of nature. And in the vast majority of cases it does not happen more than once in a family. There are about 5% of families who may have hereditary cases of PWS, but nowadays geneticists can test you to tell you whether or not you are one of these "at risk" families.
If you want to know more about the genetics of the syndrome, you can get a free leaflet from the PWSA (UK) entitled: The Genetics of Prader-Willi Syndrome and the Possibilities of Recurrence in the Family.
A syndrome is a collection of symptoms or signs, which when they occur together, show that a person has a particular disease or condition. There are thousands of different syndromes known to medical science, some of which you are born with, and others which develop later in life.
The symptoms that your brother or sister has are called Prader-Willi Syndrome. There is a simple reason why these symptoms have this very strange name. If a scientist discovers a new law, a new disease, a new invention or a new syndrome, it is very often called after him or her. For instance, vacuum cleaners are often called "hoovers" - after William Henry Hoover, who made the first machine.
The set of symptoms your sibling has were first "described" (published in medical journals) by Dr A Prader and Dr H Willi, who come from Switzerland, and the syndrome was then named after these two doctors. This was in 1956, but we are certain that the syndrome existed before then, even if it did not have a "'name".
If you need to know how to pronounce Prader-Willi, it is Prahder (to rhyme with "larder), and Willi (as in the boy's name, to rhyme with " Billy"). |
PWS is something you are born with. If you are older than your brother or sister with PWS, you may remember how difficult it was for your parents to feed him or her, and perhaps how it took your sibling much longer than other children to learn to walk, talk, and do other things. This is because people with PWS have poor muscle tone - their muscles are not as strong as yours are. No one knows why this is, but muscle strength can be improved with lots of exercise.
Scientists think that the part of the brain that is affected in PWS is the hypothalamus. This is the "control centre" of the body, and regulates things like: appetite, growth, sexual development, and emotional stability. All of these things do not work properly in PWS.
The message that tells you that you have had enough to eat does not "switch on" for people with PWS, and so they go on feeling hungry for much longer than most people, even when they have already had a lot to eat. Sometimes they feel so hungry they will steal food from anywhere.
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Most people with PWS are shorter than average, and they will almost certainly be shorter than other members of your family. This, together with the weak muscles and the need to eat, means that it is VERY difficult for your brother or sister to keep their weight down, and they need all the help and support they can get. Before much was known about PWS, many people died early because they got very fat, but with better management, they are living well into their 40s and 50s and beyond.
If you have a brother with PWS, you may have noticed that he has a smaller penis than other boys of his age. This is because the hormones which help sexual development do not work properly in PWS. In fact, as far as we know, no-one with PWS, male or female, can have children, although they can have relationships with other people if they wish. Your brother or sister may find it difficult to come to terms with the fact that they are unlikely to have children, so you need to be very careful when talking about this. They may be too young to know at the moment anyway, so it is best to leave it to your parents to discuss this with them.
People with PWS can become easily confused, anxious and upset. They find it very difficult to change direction when they are set on a particular path of thought or action, and they may exhibit their confusion, frustration and anger by having "temper tantrums". These are very similar to the type of tantrums that toddlers and young children have - your brother or sister may find it very difficult to grow out of them.
Holly who has PWS (left, 3 years) with sister Cloe(20 months). |
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Many people with PWS have learning difficulties, which means they may need to be in a special class or a special school, and may not be able to read, write, or do maths as well as other children. Some have more problems than others.
Your brother or sister also has many shared characteristics with you and the rest of your family. Although people with PWS are similar to one another, they are also as individual as the rest of us, so don't blame everything they do wrong on PWS!
Here are some things which some brothers and sisters have said are good about living with a sibling with PWS:
And here are some of the things that are not so good:
None of us are perfect, and we can't be expected to be in a good mood all of the time, but there are some things which might help you to cope when the going gets tough.
"It makes me very tired when I come home from school and she is sometimes crying loudly. I get really annoyed with her." |
The order in which you were born can affect the way you interact with your brother or sister, and may be the cause of some difficulties.
If you are the eldest in the family, or if your brother or sister with PWS is the youngest, there is a likelihood they will be treated as the baby of the family, and given less responsibility as a result. They may even get away with things which their older brothers or sisters cannot. You may tend to make allowances for them, which you might not otherwise have done.
If you are younger than your brother or sister with PWS, you may be allowed to do things which your older brother or sister cannot (e.g. go out on your own, go to the shops), and as time goes by, you are likely to go on and get a job, go to college etc - i.e. you will overtake them. For some people with PWS this can be very difficult to come to terms with, and can be the cause of arguments and difficult behaviour.
Our whole society revolves around food. You only have to switch on the TV, open a magazine or go in a shop, and it is not too long before you are confronted with images of food. How many times have you seen an advert on TV, smelled cooking as you walk past a restaurant, and thought, "I really fancy eating that!" This is what life is like for people with PWS all the time, because they are hungry most of the time.
We also tend to eat when we are bored, or feeling a bit down. It is no different for someone with PWS - if there's nothing to do, the first thing they will think of is food. It is not easy for you, as the sibling of someone with PWS, to control your eating all the time, but it can be a great help if you do not talk about food or eat sweets in front of the person. Your mum or dad can help by giving you sweets or other food treats when your brother or sister is not around.
If you find out that your brother or sister has been taking food without telling your parents, what should you do? Perhaps the best course is to discuss this with other family members first, to decide on a course of action. It is not nice to "tell" on your brother or sister, but they need to be aware of what the consequences will be if they are found out.
On the other hand, it can be very depressing for the person with PW S to be followed around all the time "'just in case".
"(One of the worst things is) . . . . the concentration on diet. . . . . If you are not careful, you begin to end up with an obsession with food yourself!" |
There are many ways that a person with PWS can be helped to manage their weight, and the free booklet, Weight Control and Suggestions to help - with Weight Management, might give you more guidance.
These can be embarrassing, very noisy, and very stressful, especially if
they happen in public. It can be useful to remember that your sibling with
PWS does
have very real difficulties in controlling their emotions, especially anger,
and things can quickly get "out of control". There are some things you can
do to stop them happening in the first place, although sometimes they will
happen no matter what you try to do. But try these first:
If you are lucky, your brother or sister will not have this trait, but the majority of people with PWS do - and it can be very wearing, having to repeat yourself over and over again. Here are a couple of things you can do to help them "switch off".
"We can't go anywhere on the spur of the moment. She goes on and on about things. She's stubborn." |
All brothers and sisters argue from time to time - it would be strange if they didn't. In some cases it may be only occasionally, and in' others much more frequently, but there seems to be a difference when it comes to arguing with your brother or sister with PWS. For one thing, they just won't listen to reason - or so it seems! But did you know that researchers have found that people with PWS actually have a great deal of difficulty in understanding things which are just said to them: they need showing as well. So if you know you are really in the right, try to prove your point by showing the person what you mean, rather than just yelling at them.
"It gets annoying because of her behaviour, and I have to watch what I eat in front of her. She always wants to do what I do, and arguments begin." |
If your sibling is a persistent arguer - and there are some! - you may find that your only course of action is to walk away, after warning them that this is what you intend to do in so many minutes time.
People are more likely to be understanding of your sibling if they know something about them. Showing them a PWS leaflet or this Web Page may help.
Being out with your brother or sister can sometimes be very embarrassing. Nowadays, people are getting more used to seeing people with disabilities out and about, so hopefully they will be a little more understanding , The thing about embarrassment is that only you can do anything about it. Try not to let it get to you. If someone is staring at you or making remarks, it is their problem not yours - they are the ones who should be embarrassed!
Every family will have different viewpoints on how much relatives should be involved in caring for a disabled member of the family, either in the present, or in the future when your parents are no longer able to care. Much will depend on your religion, culture, or family situation.
It is important that you and your family discuss what your responsibilities might be, now and in the future, so that you can iron out any difficulties before they come to crisis point. Although many people are reluctant to involve Social Services, they can be helpful in providing practical support to families with a disabled member.
"My father has died and my mother is caring for my brother on her own. My sister lives away so I have a feeling of responsibility for my brother's upbringing and care. I worry what will happen to him if my mother becomes ill." |
Life with a brother or sister can be very stressful, and everyone has different ways of coping: going out for a walk, staying overnight at a friend's house, listening to music or watching TV in your bedroom (if you have one to yourself) etc.
If you need to talk to your parents about something important, don't try to do it when your brother or sister is around. If that is difficult, let your parents know you want to discuss something with them, so that you can arrange a time when your brother or sister is either out of the house or in bed.
If your sister or brother never goes away from home, either to stay with relatives or for respite care with your local Social Services, ask your mum or dad if they can arrange for some time for you to be on your own with them.
"Make sure the County Council (Social Services) take responsibility for keeping them housed. Then you will be able to visit a happy person, instead of looking after an unhappy one." |
Yes, life with a brother or sister with PWS is not fun all the way; but with better understanding and management things can get better. Here are some of the positive comments made by people who have brothers or sisters with PW S:
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If you would like to speak to a female with a PWS sister or a male with a PWS brother about the difficulties you are facing, e-mail the Sibling Telephone Support line with your telephone number and a short message and we will e-mail you back a telephone contact number.
This is web site for brothers & sisters of disabled people (of all ages and disabilities) and you can subscribe to a e-mail list which will put you on a distribution list that is mainly American but covers includes people from other countries including the UK. You can send your own messages to it for distribution. In other words, you can sit at your computer and swap moans and groans and questions and answers with people all over the world!
This organisation can give you information on whether there are sibling groups
in your local area. They also publish a free eight page leaflet Siblings
and Special needs which is available from:
Contact a Family, 170 Tottenham Court Rd, London W1P 0HA.
PWSA (UK) Leaflets available from:
PWSA (UK), 125a London Road, Derby DE1 2QQ (please send SAE when requesting
leaflets)
Brothers, Sisters and Learning Disability - A Guide for Parents
Available from: BILD Wolverhampton Rd, Kidderminster, DY10 3PP
21 page booklet provides information and advice for families who have a child
with a learning disability. It looks at the impact of a child with a learning
disability on other children in the family and considers what parents can
do to encourage acceptance and understanding.
Hello, Sister! Hello, Brother - Living with a brother or sister with
disabilities.
Free leaflet available from: NCH Action for Children, 85 Highbury Park, London
N5 1 UD
Mencap Sibs.
Video and workbooks for hire, plus publications list from:
Mencap, 123
Golden Lane, London EC1Y 0RT
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