Prader-Willi Syndrome

-Genetic condition
-Disease characteristics
-Developmental delays
-Poor muscle tone
-Short stature
-Small hands and feet
-Incomplete sexual development
-Unique facial features
-*** Insatiable appetite, leading to obesity
-***In infancy, hypotonia leads to poor feeding, failure to thrive
-Developmental delays
-Learning disabilities
-Weight gain
After initial FTT, weight is gained excessively from ages 1-4. Uncontrolled appetite, overeating,
low BMR, decreased activity all leads to obesity.
Decreased LBM, increased body fat
TEE may be 53% of that of normal obese adolescent
-Anthropometrics-Should be taken frequently and charted on CDC growth charts
-Biochemical-Blood glucose- to monitor for diabetes, insulin resistance
-Diet Hx-Toddler years- careful assessment of portion sizes, frequency of feeding, types of food
-Energy needs lower due to low lean muscle mass, slow development of motor skills and
activity
-Ages 5-12, child hungry all the time, difficult behavior, tantrums
-Information should be obtained from parent about environmental controls (locking fridge,
pantry, kitchen, etc)
Nutrition Recommendations-50 CHO, 25 Protein, 25 Fat
Intervention-

1-4 years- structured meal patterns so pattern of grazing does not happen. Small servings of
meat, F&V, grains, dairy, limit sweets. Early good meal practices is important in controlling issues
later. Wt, Ht, intake monitored closely, physical activity must be encouraged
School age-Collaboration with the school food service program. E needs calculated per
centimeter of Ht. Energy need usually 50-75% of unaffected children. Environmental controls.
Growth hormone therapy may not increase feeling of satiety. Appetite suppressants dont usually
work.

EAL- Guidelines of Pediatric weight management (no recommendations specific to

prader-willi)
Family Diet Behaviors and Increased risk of pediatric obesity: consumption of food away
from home, breakfast skipping, increased portion sizes, parent restricting highly palatable foods.
Lack of parental support or over-possessiveness, lack of parental concern over childs weight
status.
Foods associated with an increase in the risk of overweight: increased dietary fat intake,
increased calorically sweetened beverages
Decrease risk of pediatric obesity: Regular Physical activity, sports participation. Family
cohesion, expressiveness, democratic style, parental support, cognitive stimulation at home.
Increased risk of ped obesity: Excessive TV watching, video games
Nutrition assessment: reported total energy intake and fruit juice intake
Increased risk overweight: inadequate dairy and calcium intake, include in nutrition
assessment
EAL guidelines for Nutrition Intervention:
Behavior therapy
Multi-disciplinary team
Decrease sedentary behaviors
Weight stabilization may be associated with improvements in BMI and other measures
of adiposity

From: http://consultantdietitian.com/prader-willi-menus-are-now-available/
Prader Willi Syndrome individuals must control their diet and should be closely supervised at all times. PWS individuals
should follow a strict low calorie diet plan in order to control their weight. Typically an adult PWS individual can only eat
about 1,000-1,200 per day because they have low caloric needs. PWS individuals only need about 60% of a typical
persons diet. PWS individuals have low muscle tone and are often inactive which contributes to their low dietary intake
need. Individuals with PWS should supplement their diet with a multi vitamin supplement to ensure they are meeting their
recommended daily allowances. Mega doses of vitamins are not recommended because some PWS individuals have had
adverse reactions with higher doses. PWS individuals should ask a doctor or registered dietitian what is the right vitamin
supplement for them.
Individuals with Prader Willi Syndrome must closely monitor their portions in order to control their weight. PWS
individuals who do not control their diets can become morbidly obese putting them at risk for diabetes, hypertension, heart
disease, cancer and other obesity related diseases. The PWS syndrome diet is designed a little different than the typical

American diet. PWS syndrome still have the same main food groups-grains, vegetables, fruit, meat and milk but PWS
syndromes are offered more vegetable than grains than the typical American diet. The reasoning behind offering more
vegetable is to offer more volume and fiber in the diet. Individuals with PWS often have trouble with constipation.