Ethical management of patients with Prader-Willi syndrome across life span

Violeta Stan Md. PhD
University of Medicine and Pharmacy ³V Babes´ - Timisoara Emergency Children¶s Hospital ³Louis Turcanu´ - Timisoara
The 2nd Eastern European Conference on Prader Willi Sindrom 29- 30 octombrie 2010, Zal u

To act in the ³person¶s best interests´
‡ It is absolutely critical to have families, health care providers, communities educated about the physiologic, medical, and behavioral characteristics of the adult with PWS in order to avoid abuses. ‡ from an ethical and legal point of view is essential to determine the capacity of a person with Prader Willi Syndrome to make decision about his/he eating behavior ‡ the common law principle is to act in the ³person¶s best interests´ in the least restrictive alternative, respecting the individual. ‡ Allowing serious weight gain and the serious health consequences is an abdication of responsibility from parents and health care providers, not a paternalistic approach.

Management of the disease in the first month of life

Prader-Willi babies are hypotonic, do not wake to be fed and are in general unresponsive. ‡ They can also present hypothermia and hypogonadism. ‡ providing an adequate nutrition, dietary fat for brain growth and development is essential and represents the main concern for the management of the disease in the first month of life. ‡ For parents, it is important to provide counseling to assure that the babies will receive and maintain the proper nutrition and early support in enhancing observational skills for observation. ‡

One month to 24 months
‡ normal nutritional guidelines. ‡ programs of physical therapy and developmental stimulation should be initiated because development delays ‡ Family counseling should be provided to emphasize the importance of normal diet and appropriate weight gain during the first 2 years of life and neuro-motor stimulation.

Two to five years
‡ During pre-school years, the insatiable appetite becomes apparent in the majority of children with Prader-Willi and many children become obese during these years. ‡ low calorie diets are necessary and supplemental vitamins and calcium should be assured. ‡ programs that enhance communication skills, appropriate social interactions and physical and speech therapies many problems can be addressed. ‡ Behavior problems and emotional lability may become a problem parent¶s support group and professional support may be planed to help families ‡ Siblings, extended family members and all caregivers require education and counseling to assure appropriate actions ,including the food restriction necessary for children

Six to eleven years
‡ Weight control at this age is essential in order to avoid uncontrolled eating and obesity. This may require locking food within the home and restriction of excess fluids. ‡ Teachers and other school personnel should be educated to the need for close supervision of Prader-Willi children to insure adherence to a diet. ‡ A regular program of physical activity is also essential to weight maintenance.

Six to eleven years
‡ behavioral and social challenges occur upon entering school so that consultation with behavior specialists, psychologists together with psychotropic medication may be helpful in many patients. ‡ Educational goals require provision of opportunities for success that are developmentally rather than age appropriate. ( can be mixed with some mainstream activities). ‡ Education of extended family, educators, neighbors and community is essential because children with Prader-Willi syndrome can frequently manipulate neighbors, friends and strangers into providing food. Siblings may require special support and counseling to be able to adjust.

Twelve to twenty one years
‡ Adolescence is a time of transition for all children. ‡ Adolescents with PWS become aware of the differences between themselves and their peers. ‡ Recommendations for caloric restrictions should be based on linear growth and in general caloric requirements ‡ Exercise programs should be a part of daily activities, and sometimes one-to one supervision

Twelve to twenty one years
‡ Increased irritability, agitation, loud speech, uncooperativeness, rigidity, and perseveration can be observed during these years so that psychiatric and psychological intervention is often necessary. ‡ education,should be placed in classrooms based on intellectual function and behavioral needs. ‡ On-site community vocational placements should not include foodrelated work ‡ The teaching should focus on training for specific living and social skills ‡ . During this time legal guardianship becomes an important concern. Parents need help to acknowledge that their children cannot live a life fully independent because of the inability to manage money and control food consumption

Adulthood
The transition from childhood to adulthood is difficult. People with PWS now live longer and parents have the same increased responsibility for their children with special needs. ‡ Meaningful work for these people requires special considerations. The major health problem is the obesity, which was associated with serious complications like type II mellitus, profound hypoventilation and apnea, hypertension, osteoporosis, skin irritations, fractures. ‡ The need for social interactions increase and the desire to live independently continues ‡ The environment should be prepare to provide it in ethical manner

Prader-Willi syndrome requires a multidisciplinary approach to delivery of care ‡ Physicians have the due to consider all these aspects of managing individuals with PWS, especially in an age specific manner. ‡ Resource professionals and centers should be able to provide education, counseling and supervision at different levels of intervention ‡ The needs of each age group should be address separately.

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