The purpose of this article is to present a review of the literature on metabolic and exercise responses of children with a diagnosis of Prader-Willi syndrome (PWS) and to suggest considerations when designing a program of physical therapy for these children. Prader-Willi syndrome is a complex genetic disorder that results in impairments in multiple systems including musculoskeletal disorders; sleep disturbances; hormonal deficiencies; autonomic nervous system alterations; metabolic disorders; and cognitive and behavioral dysfunctions. Children with PWS are at risk for scoliosis and childhood-onset osteoporosis. The hypotonic characteristic of the disorder could interfere with the development of postural control and respiration. The accompanying obesity can increase the energy cost of gait and daily activities and lower energy reserve for educational, social, and recreational activities. Sleep disorders and impaired parasympathetic functions can influence behavior and interfere with growth hormone release, which in turn can affect growth and development. As young adults, they are at risk for cardiovascular disease, diabetes, and hypertension.
Prader-Willi syndrome requires life-long management by a team of informed professionals. Suggestions are made for strength training in combination with aerobic conditioning to increase muscle strength and lean body mass, improve vital capacity, and minimize childhood-onset osteoporosis. Improving the exercise responses of children with PWS can enhance their functional abilities and quality of life.
© 2000 Lippincott Williams & Wilkins, Inc.