About-IPWSO » What is Prader-Willi Syndrome?
Prader-Willi Syndrome is a genetic disorder caused by a small deletion in the 15th chromosome. There seems to be no apparent reason why this should happen, and to date it appears to be quite random, in other words, it is not necessarily inherited, it is not the father's, nor the mother's fault, it is the developing embryo that fails to pick up a very small piece of genetic material from the father, or, instead, picked up two pieces of the same genetic material from the mother. Scientists have dedicated many years of research to find out the reasons why this should happen. To date, we still do not have a reason why.
This missing piece of genetic material is responsible for a disturbance in a large range of behaviours and characteristics, the main two being that the person will always have extreme difficulty in managing his or her diet - they will continue to eat without feeling full. The "on/off" switch that controls the appetite, does not work. Therefore, food becomes dominant and paramount in their lives. This will always have to be managed by parents and care-givers, and meals will need to be correctly balanced in accordance with the person's needs. Many will remain on a restricted intake of calories all their lives. Without this restriction, weight gain can be rapid and disastrous.
The second important issue with PWS is the characteristic emotional behaviours. In other words, there will be times when seemingly irrational behaviours will take over, arguing becomes out of hand and temper can escalate. Therefore, knowledge of the syndrome and how it affects a child or adult, is paramount to the way we learn to manage the environment, behaviour, and care.
This website has many pages dedicated to understanding the syndrome. We hope that if you have any questions, you will contact us directly.
Prader-Willi Syndrome Information Sheet: this pdf was put together by Monash University (Australia) as a medical reference for professionals. You may find this useful to download and have ready to give any professionals who will be caring for your child.