Prader Willi Syndrome

WHAT IS PRADER-WILLI SYNDROME?

The Prader-Willi Syndrome (PWS) presents many challeneges to people with the syndrome and to their parents and carers. Its effects reach into every aspesct of daily life, and careful consideration must be given to the way in which people with the syndrome are helped and supported to make choices which are beneficial to both their overall health and their quality of life.

People with PWS have a unique set of needs which are not readily met under existing health and social services arrangements. The syndrome is poorly understood by those who have had little experience of daily living with PWS, and its effects are sometimes fatally underestimated. Hidden risk factors and disabilities lie beyond the veneer of independnet living skills and intellectual ability presented by many people with PWS.

However, people with PWS have many skills and qualities which they can use to enrich the community in which they live, providing they have the degree of support, understanding and care they need.

STATISTICS

The latest UK estimates of birth incidence, taken form an epidemiological study of PWS in the Oxford and East Anglia Health region are 1:22,000, with a population prevalence of 1:52,000. This means that they may be as few as 1,150 people with PWS in the UK. (Other estimates world-wide range from 1:10,000 to 1:25,000 for birth incidence).

The same UK study found that mortality rates for people with PWS across teh range was 3% compared with 1% per year in the overall population (Whittington et al, 2001). However, with better weight and health management, due to increased knowledge about PWS, prevalence rates are likely to rise. There are also likely to be many undiagnosed cases of PWS, particularly in adults. The oldest living person known to the Association is 72 years old.