What is Prader-Willi Syndrome?
Prader-Willi syndrome (PWS) is a complex genetic disorder caused by the absence of active genetic material on chromosome 15. PWS occurs in approximately one in every twenty-thousand births and equally affects males and females of all races and ethnic groups. The disorder develops in two stages: infants have muscle weakness, feeding difficulties and poor growth. During childhood, this is replaced by an insatiable appetite, slow metabolism, behavioral problems, and learning difficulties. Severe, life-threatening obesity results if food intake is not strictly controlled. The symptoms of PWS are thought to be caused by dysfunction of the hypothalamus in the brain, an area that regulates appetite, metabolism, pain, temperature, hormone levels and behavior/mood. PWS is a common genetic cause of morbid childhood obesity. Research into the cause and treatment of obesity in PWS may provide important insight into obesity in the general population, generating new opportunities to alleviate childhood obesity, which has reached epidemic proportions.
How does Prader-Willi Syndrome Impact Lives?
PWS is a life-long condition; currently there is no cure. The impact on families is significant as behaviors related to eating and diet, routine, and structure of the home must be quickly and radically altered to accommodate the needs of the individual with PWS. The hunger, metabolic, and behavioral characteristics of the syndrome force individuals with PWS today to require constant and lifelong supervision in a controlled environment. Families must be constant educators since everyone who interacts with individuals with PWS must be aware and supportive of their limitations, restrictions, and needs. Due to these restrictions, independence for individuals with PWS is a distant dream today, but one that we expect to realize with your support.
View our video that was produced and played at the PWS Derby 2011!