Although it is rare, Prader-Willi Syndrome (PWS) is the most common genetic cause of potentially life-threatening child obesity. Read: 5 Causes of Obesity The Prader-Willi Syndrome Association explains, “A chronic feeling of hunger that, coupled with a metabolism that utilizes drastically fewer calories than normal, can lead to excessive eating and life threatening obesity.” Read: Three Ways to Know It’s Not Head Hunger
The International Prader-Willi Syndrome Organization (IPWSO) notes that the two “hallmark characteristics” of Prader-Willi Syndrome are an extraordinarily low metabolism and an insatiable hunger. IPWSO quotes Dr. Hans Zellwegger: “PWS children constantly appear plagued by a relentless hunger that dominates their lives. The presence of this insatiable urge to eat, which is beyond the control of the patient, his family, or physician, becomes the primary focus for the child and inhibits all other activities and interest.” Read: Binge Eating Disorder vs Overeating
Parents of children with PWS often have to restrict the child’s access to food by locking kitchen cabinets and refrigerator doors.
Tonya Wilkinson and her family were recent subjects of an ABC 20/20 piece in which Tonya reflects on a day in 14-year-old daughter Hannah’s life and what it’s like to raise a child with Prader-Willi syndrome. Read the story, When Your Daughter Can’t Stop Eating: Raising a Daughter with Prader-Willi Syndrome.
Prader-Willi Syndrome Symptoms
Infants with Prader Willi Syndrome have weak muscle tone, reduced bone mineral density, difficulty feeding, poor growth, and delayed development. At first, they will have trouble growing and gaining weight. Then in early childhood, usually between 1 and 6 years of age, the appetite becomes extreme and leads to chronic consumption and obesity.
Infants with Prader Willi Syndrome may exhibit symptoms at birth such as a small and floppy appearance. Other physical appearances are almond shaped eyes, a narrow bi-frontal skull, short stature, and very small hands and feet. Male newborns may have undescended testicles, and either gender might can have delayed or incomplete sexual development.
Children with Prader Willi Syndrome have some cognitive impairment, learning disabilities, and poor motor skills. Temper tantrums, stubbornness, compulsive behavior and sleep disorders are also common. Most patients with PWS are incapable of having children.
Prader-Willi Syndrome Causes
Discovered in 1956, doctors at first had no idea what was causing the features of Prader-Willi Syndrome. In 1981, it was discovered that many afflicted with PWS had the same gene segment missing from one of their chromosomes. It was found that the missing gene segment on chromosome 15 was responsible for about seventy percent of Prader Willi Syndrome cases. Read: Genetic Causes of Obesity
In another twenty-five percent of cases, the person with Prader Willi Syndrome inherited two copies of chromosome 15 from the mother instead of a single copy from each parent.
All children of all races are susceptible, and the possibility of a child having PWS is about one in fifteen thousand.
Prader-Willi Syndrome Treatment
There is no cure for PWS. Growth hormone has been approved by the Food and Drug Administration to treat Prader Willi Syndrome. The hormone helps to improve strength and agility, height, lean muscle mass, distribution of weight, stamina, and the density of bone mineral. There are concerns that taking growth hormone can lead to sleep apnea, so children receiving growth hormone treatment need annual sleep studies.
The best treatment for children with the syndrome is a multidisciplinary approach that includes growth hormone replacement, physical and occupational therapy, speech and language support, learning disability services, behavior management assistance, family support, and diet and exercise.
Prader-Willi Syndrome Diet
The Prader-Willi Syndrome Association recommends that children with PWS remain on a low-calorie diet. The standard Food Guide Pyramid designed by the United States Department of Agriculture needs modification to address a Prader-Willi diet because the generalized portions are too much for children with the illness.
The recommended 1600 to 2800 calories per day should be decreased to about 800 to 1200. Serving sizes are ½ cup of cooked or raw vegetables, 1 slice of bread, ½ cup of rice, 1 ounce of cereal, ½ can of fruit or ½ cup of juice, 1 cup of skim milk, 1 ounce of cheese, ½ cup of fat-free sugar-free yogurt, 2 ounces lean meat, fish or poultry, 1 egg, and 1 tablespoon of peanut butter.
Fats, oils, and sweets such as butter, sugar, gravy, and fried foods should be limited to one time per month because they are nutrient deficient and add unwanted calories to Prader-Willi diet. Read: Bad Eating Habits That Cause Weight Gain
The Prader-Willi Syndrome Association reports PWS patients may require continued supervision to limit access to food throughout their adult lives. No appetite suppressant has been discovered that will consistently address hunger pangs in people who have Prader-Willi Syndrome.
In good health,
Photo: ABC News