About Prader-Willi Syndrome
What is PWS?
Prader-Willi syndrome (PWS) is a rare, complex genetic disorder that affects both males and females from birth and throughout their lives. It causes low muscle tone with consequent motor developmental delays, a mild to moderate learning difficulty, incomplete sexual development, and emotional and social immaturity, which can lead to challenging behaviours. During childhood, an overwhelming and insatiable chronic appetite usually develops which, without rigorous food management and exercise regimes, leads to food seeking, stealing and life threatening obesity. PWS occurs randomly in about 1:22,000 births and it is estimated that there are about 2,000 living with PWS in the UK.
Life as an adult?
As adults, people with PWS have varying abilities in attaining independence, although all will need some form of support or monitoring to help with controlling their food intake, and thus their weight. Despite the fact that many individuals have the intellectual and physical ability to work, they are usually ill-equipped on an emotional and social level to deal with the stresses and demands of the ordinary workplace. However, they can make a positive contribution to society in many ways and may be involved in voluntary work, craft work, or have a part-time job. Many people live with their families, but an increasing number are living in residential homes, or being supported to live in the community.
The Treatment of PWS?
Apart from various hormone treatments and some surgical intervention (eg, to bring down undescended testes), there is currently no "cure" for PWS. There have been many advances in the fields of genetics, and researchers are not far off identifying exactly which genes are involved in PWS. No drug so far has proved to be of lasting help with regard to suppressing appetite. Generally speaking however, many of the adverse effects of the syndrome can be lessened by good dietary management, exercise programmes, good general health care, and by good general management of behaviour and education.