by Ruth Adams

Created on: June 22, 2008

Recently there was a death in a family that lives near me. The woman who died was only 40 years old and she suffered from Prader-Willi syndrome. Prader-Willi syndrome is not well known at all. I only found out about it when I started working in a rehabilitation hostel nearly six years ago. It is unfortunate that the woman who died was not a resident of the hostel where I work. If she had been, she would surely have had a longer life.

The residents of our hostel are teenagers and young adults. About 15 of them have the syndrome. It is an absolutely horrible condition. People who have Prader-Willi syndrome are obsessed by hunger all the time. They are so hungry that some of them are liable to eat from the rubbish bin, to eat cigarettes and to eat themselves to death. For that reason, the kitchen in the hostel is kept barred and locked. Meals are carefully planned and the residents' intake of food, especially carbohydrates and fats, is well monitored. The residents are weighed twice every day. If they have managed to steal food from somewhere (which is very common) they are given only vegetables to eat for the next meal, or number of meals. This is partly to teach them not to steal food and partly to re-balance the body. They also have a mandatory exercise program.

In addition to being obsessed with food; victims of Prader-Willi syndrome typically have problems in auditory processing. This means that someone with the syndrome can often feel inappropriately threatened or rejected, because they have been unable to process the subtle clues in a person's speech or tone of voice. They depend very much on visual information to supplement what they hear.

It is common for people with Prader-Willi syndrome to also have learning difficulties; and some of the residents do. Others manifest a high intellectual ability but this is undermined when they are in a negative emotional state. For example, if someone is being reprimanded because they stole food or did not take enough exercise, or even if they just feel misunderstood about something; they can easily lose their temper and become totally hysterical and out of control. This causes them to lose their reasoning, even though they know and understand about their condition and its implications. Every week, one or more people in the hostel gets hysterical about something.

The residents of the hostel are either finishing school (in Special Ed.) or in sheltered employment. In addition they do recreational activities which vary. Sometimes they go swimming; sometimes they do art, go on trips or take dogs for walks. My job is to provide activities which improve thinking and communication and which help them to address their feelings.

Prader-Willi syndrome affects between 1 in 12,000 and 1 in 15,000 people. It is caused by a deficiency in the paternal chromosome. Babies who have the syndrome are often lethargic, have problems in breathing; and have hypotonia weak and floppy muscles. Because of the hypotonia they have difficulties in eating; but between the ages of 2 and 4 they are likely to start over-eating. Their acquisition of speech may be delayed and they will have poor physical co-ordination. The syndrome is diagnosed through genetic testing and if an infant has hypotonia, it is recommended that they are tested as soon as possible, so as to enable early intervention.

Learn more about this author, Ruth Adams.
Click here to send this author comments or questions.