Prader-Willi Syndrome Teen’s encounter with hunger


Hannah Wilkinson’s, a 14-year-old girl from Phoenix in Arizona, encounter with intense hunger is life threatening.

Hannah’s mother, Tonya Wilkinson, says that even if the teen has just eaten, she still feels hungry.

The lives of Wilkinsons revolve around the teen’s hunger. At their home, the kitchen door is locked down, their refrigerator is padlocked, food is removed from the cabinets and the pantry door is constantly locked.

Tonya said Hannah was once caught eating their dog’s food prompting them to lock up the dog food too.

Doctors have diagnosed that Hannah was born with a very rare condition named Prader-Willi syndrome. A chromosomal flaw causes the syndrome, which afflicts one in 15,000 people and can cause muscles weakness, slow metabolism and learning issues.

The condition creates a ravenous appetite and that is what had pushed Hannah to weigh 350 pounds.

Tonya said a part of human brain called hypothalamus controls human hunger. In adults and children suffering with Prader-Willi Syndrome, this part of the human brain is shut off. With the result, people afflicted with the syndrome don’t know that they are not hungry.

For Tonya, this condition forces her to be on constant vigilance. She recalled that once when she was cleaning the kitchen post dinner, Hannah shoved a massive portion of pot roast into her mouth.

Tonya said when she turned around from her work; she found that Hannah was choking. People with this syndrome generally don’t chew but swallow the food. Most of the deaths among people with Prader-Willi are due to choking.

Similar to Hannah, a 12-year-old girl from Cibolo in Texas named Alexis Shapiro is afflicted with the intense desire to eat. In 2011, Alexis had undergone a surgery for the removal of a rare brain tumor. Soon after that Alexis developed something called hypothalamic obesity. Alexis developed the syndrome after the surgery, unlike Hannah.

Alexis’s father Ian Shapiro said soon after she woke up after the surgery, she began asking for food.

Alexis gained 3.62 kg even before she got released from the hospital and the situation got worse when the family reached home. She was 23.58 kg at the time of the surgery. But post surgery, she touched 45.35 kg.

Alexis’s mother Jenny Shapiro said the girl wears something twice, but the she keeps on outgrowing it.

Apparently, Alexis’s surgery altered a part of her brain, which constantly regulates appetite. This development in her is similar to the symptoms in Hannah. Alexis finally weighed 92.07 kg.

Obesity experts, including Dr. Robert Lustig, are studying cases similar to Hannah and Alexis to get a better understanding on weight gain. The experts opine that the key lies with the hypothalamus. When damaged, hypothalamus releases insulin that causes an insatiable appetite in the patients.

The key to the solution in fighting obesity lies in controlling that insulin.

However, Dr Lustig’s study is still in an experimental phase. But Alexis’s parents, in their desperation to halt the kid’s abnormal weight gain, turned to surgery.

Following the surgery, Alexis’s appetite has come back to normal and she lost 22.67 kg and is more active.

In Hannah’s case and others born Prader-Willi, surgery may not be an option as they never suppress their appetites.

According to Tonya, Hannah’s only hope would lie in getting admitted to a specialized facility that monitors 24/7 and plans meal timings. However, that hope seems to be a remote task as Tonya cannot get insurance cover for such a facility.


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