Prader-Willi
California Foundation Prader-Willi syndrome (PWS) is a rare and very complex non-inherited genetic disorder. Symptoms associated with Prader-Willi syndrome (PWS) are believed to be caused in part by a defect in the hypothalamus, an important supervisory center in the brain that controls metabolism of fats and carbohydrates, the development of muscle tone, the regulation of the sleep-wake cycle, body temperature, blood pressure, heartbeat, the expression of emotions, and many more functions of the body. Symptoms caused by PWS vary throughout the lifetime and vary in severity from person to person. Infants with Prader-Willi syndrome fail to thrive and require varying degrees of assistance in order to survive. Beginning some time in childhood, the brain fails to regulate metabolism and appetite normally. For a person with Prader-Willi syndrome there is a constant preoccupation with food accompanied by an unrelenting, overwhelming, overriding physiological drive to eat. Normal satiety (the feeling of fullness after eating) does not exist. The physiological drive to eat is so powerful that most individuals with Prader-Willi syndrome will go to great lengths to eat large quantities of food. |
||||||||||||||||||||||||||||||||||||||||||||||||||
| Next to hyperphagia
- the insatiable drive for food - probably the most difficult symptom
caused by PWS is the eratic control over emotions and behavior. People
with PWS are often easily frustrated, impulsive, quick to anger, rigid
and concrete thinkers, and highly anxious. Parents and care providers of someone with Prader-Willi syndrome experience some of the highest levels of stress. Throughout their child's lifetime they will require the assistance of a multitude of professionals. There is no cure for Prader-Willi syndrome - yet. No known medication controls or even reduces the hyperphagia symptom, and very few medications successfully manage the behavioral symptoms. No one with Prader-Willi syndrome is able to live independently because without 24/7 supervision for the entirety of their lives, individuals with PWS will die prematurely as a result choking, stomach rupture or tissue necrosis, or from complications caused by morbid obesity. There are, though, treatment and management strategies that can help reduce or manage some of the symptoms caused by PWS and improve the quality of life …and there is hope. Prader-Willi California Foundation (PWCF) was established in 1979 as a non-profit, charitable organization by parents of persons with Prader-Willi syndrome. PWCF is an affiliate member of the national Prader-Willi Syndrome Association (USA). Our founders' aspirations were to improve the educational and residential quality of life of individuals born with the syndrome. Today, PWCF is over 800 members strong. Our mission continues to be to provide to parents and professionals a state network of information, advocacy, and support services to expressly meet the needs of children and adults with Prader-Willi syndrome and their families. Members are parents, friends and relatives of persons with Prader-Willi syndrome, as well as many dedicated professionals and care providers. Together, we are helping individuals born with Prader-Willi syndrome live meaningful and productive lives and pursue their individual hopes and dreams to the full extent of their talents and capabilities. |
||||||||||||||||||||||||||||||||||||||||||||||||||
|
||||||||||||||||||||||||||||||||||||||||||||||||||
| © 1999-2005 Prader-Willi California Foundation |