Television Shows Highlight Rare Prader Willi Syndrome
The Starkweather family of Tulsa, Oklahoma was the recipient of an “Extreme Home Makeover” last night from Ty Pennington and ABC. Toen and Amy are parents to a nine-year-old son who suffers from Prader-Willi syndrome. Extreme Makeover Home Edition is just one of two popular television shows in recent months to spotlight the rare genetic condition.
Mystery Diagnosis on the Discovery Health Channel also aired an episode on March 22nd about Conor Heybach and his parents Sue and John, a part of the USA’s Prader-Willi Syndrome Association advisory and governing boards. The family lived without a diagnosis for Conor’s condition for 16 years.
PWS is a rare disease that results from an abnormality on the 15th chromosome. It occurs in about one in 12,000 to 15,000 children and affects males, females, and all races equally. Although feeding problems and poor weight gain are often seen in infancy, rapid weight gain usually takes place between the ages of 1 and 6.
The condition causes a chronic feeling of hunger coupled with a slowed metabolism that can lead to excessive overeating and life-threatening obesity. It is also characterized by low muscle tone, short stature (if not treated with growth hormone), and incomplete sexual development. Children also are often affected with learning issues and social and motor deficits.
The food compulsion, or hyperphagia, makes constant supervision necessary. Amy Starkweather must lock the cupboards and refrigerator and cannot leave Ethan alone for risk of eating himself to death. She also has the unimaginable horror of hearing her child cry with hunger, but knowing she cannot feed him.
To date, no medication or surgical intervention is available that would help eliminate the need for strict diet and supervision around food. Psychotropic medications may be used, such as the SSRI’s fluoxetine and sertoline, may help with obsessive-compulsive symptoms.
Ethan Starkweather has so far exceeded his own doctors expectations, and also has a strong determination to help other kids with Prader-Willi Syndrome.