violeta smanagement pw

8/8/2019 VIOLETA Smanagement pw

http://slidepdf.com/reader/full/violeta-smanagement-pw 1/12

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, Zalu



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 decisionabout his/he eating behavior

the common law principle is to act in the ³person¶s best interests´ inthe least restrictive alternative, respecting the individual.

Allowing serious weight gain and the serious health consequences isan 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 fedand are in general unresponsive.

They can also present hypothermia and hypogonadism.

providing an adequate nutrition, dietary fat for braingrowth and development is essential and represents themain 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 observationalskills for observation.



One month to 24 months

normal nutritional guidelines.

programs of physical therapy and

developmental stimulation should beinitiated because development delays

Family counseling should be provided to

emphasize the importance of normal dietand appropriate weight gain during the first2 years of life and neuro-motor stimulation.



Two to five years

During pre-school years, the insatiable appetite becomes apparent inthe majority of children with Prader-Willi and many children becomeobese 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 beaddressed.

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 thefood 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 andrestriction 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 ageappropriate. ( can be mixed with some mainstreamactivities).

Education of extended family, educators, neighbors andcommunity is essential because children with Prader-Willisyndrome can frequently manipulate neighbors, friendsand strangers into providing food. Siblings may requirespecial 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.

R ecommendations 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 intellectualfunction and behavioral needs.

On-site community vocational placements should not include food-related 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 lifefully independent because of the inability to manage money andcontrol food consumption



Adulthood

The transition from childhood to adulthood is difficult.People with PWS now live longer and parents have thesame 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 typeII mellitus, profound hypoventilation and apnea,hypertension, osteoporosis, skin irritations, fractures.

The need for social interactions increase and the desire tolive independently continues

The environment should be prepare to provide it in ethicalmanner



Prader-Willi syndrome requires a

multidisciplinary approach

to delivery of care

P hysicians 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.



Thanks!

violeta smanagement pw

Documents