Prader-Willi Homes of Oconomowoc. Prader-Willi syndrome (PWS) is a genetic disorder involving a defect on the 15th chromosome and subsequent dysfunction of the brain structure (hypothalamus) responsible for hunger. PWS initially presents as a failure to thrive at birth, and then evolves into an insatiable appetite with onset usually between the ages of 2 and 4. If access to food is not adequately supervised, this inability to feel satiated can lead to potentially life-threatening medical conditions associated with obesity.
In addition to the intense drive to eat, individuals with PWS usually have metabolic dysfunction, low muscle tone (hypotonia), incomplete sexual development, cognitive and learning disabilities, neurological manifestations and significant emotional and behavioral challenges.
ndividuals diagnosed with Prader-Willi syndrome do much better in a highly structured living environment. Carefully controlled diets, structured access to food, and regular exercise are central to managing weight gain. Occupational therapy may be helpful in promoting motor development and improving hypotonia. Early and ongoing speech therapy can benefit individuals with speech and language problems.