Management of Prader Willi Syndrome

By | May 20, 2012


Care providers of a PWS patient must understand that the intervention means the management of clinical symptoms. Because PWS undergoes different developmental changes they must understand its impact on both patient and their families. Creation of a management strategy should be drown on the condition of disease. Professional experiences and from the knowledge of parents, even if the over weight is avoided, interdisciplinary interventions are essential. Genetic counseling, prevention of obesity, behavior management, environmental controls, special education and vocational services, and psychotherapeutic and guidance for future must be emphasized.


Nutritional intervention requires being well planned and committed; it requires considerable time because it is very difficult to discourage food-forging habits. A broad knowledge of accurate baseline data, education of care providers, environmental control, and frequent monitoring of growth curves and calories intake are essential. Early diagnosis of a PWS could be helpful to avoid initial weight gain.


Individual with PWS needs special education on account of their cognitive limitation. However, the behavioral changes intensify with age, Zellweger and Schneider (1968) suggested that these changes occur between the ages of 3-5. Affability replaced by stubbornness and hyperactivity, erratic and unpredictable behavior increases; depression and in rare cases psychotic episode may occur (Hall & Smith, 1972). Efforts to control insatiability results in to foraging, hoarding etc. Adults frequently appear to develop personality problems. Sleeping disorder has been noticed in 90% of the PWS patients. Nocturnal sleep disorder suggests decrease in oxygen saturation, which may lead to sleep apnea. Obesity and small upper air way may also responsible for the sleep apnea in some cases.


Greenswag indicated emotional and psychosocial adaptation in adolescence and adulthood complicates case management. Adult PWS individuals lack social experience, which leads to limited interpersonal skills and antisocial behaviour. Sexually transmitted disease, and a desire to live independently increase during the age of 13-19. After schooling parents or care providers face restricted life and erratic behavior, hence future planning is an important concern