The boy whose brain won’t tell him he is full

2013-10-28 00:00

IMAGINE that your desire to get food is so strong that you would do anything to get it, even crawl out of your bedroom window in the middle of the night to walk to a shop several kilometres away. One of the many symptoms of the rare illness Prader-Willi Syndrome (PWS) is excessive hunger.

PWS is a genetic disorder that is caused by an abnormality on the 15th chromosome during conception and affects an area of the brain called the hypothalamus.

The PWS Association in South Africa is looking to bring awareness about the syndrome to the public, with May 2014 as International PWS Awareness month. The driving force behind this campaign, vice chairperson of the PWS Association, Janet Legemaate, hopes through awareness a cure will be found.

Legemaate, who lives in Gillitts, in the Highway area, said that simple everyday things such as going out shopping, to lunch or a braai with friends can easily become an explosive situation, all due to the driving desire of an individual with PWS to want to eat because they never have a feeling of satiety (feeling full).

“This is just the tip of the iceberg when it comes to dealing with the syndrome because it is such a multifaceted syndrome, affecting various areas of the individual. More importantly, few medical practitioners have much knowledge of the syndrome or have dealt with individuals with the illness.”

Other symptoms include low muscle tone, slow metabolism, high pain threshold and the most well-known symptom of PWS — the lack of feedback from stomach to the brain.

“My seven-year-old son Luke was born as a limp, non-responsive baby with incredible feeding problems. He missed many milestones and it was even suggested that he was brain damaged. At the age of two years and eight months Luke was conclusively diagnosed with PWS, and based on the recommendation I started him on growth hormone therapy immediately,” said Legemaate.

“Even though he is now seven years old, we still do a variety of therapies on a weekly basis.”

According to Legemaate, the syndrome is hardly ever recognised and many children with this illness are very often misdiagnosed. “Prior to his diagnosis, we took Luke as a two-year-old to a braai where he sat with an adult friend of the family at the food table. When we took him home a little later, it felt like Luke had shoved a beach ball under his shirt, as his stomach was so distended,” she said.

Lesley Jones, Luke’s school teacher, described him as a delightful and enthusiastic little boy.

“He interacts extremely well with everyone and is enthusiastic in class,” said Jones.

Legemaate’s day-to-day struggles do not end there, as her elder son, Matthew, was born with a congenital heart defect.

His condition has caused damage to several organs and he is currently on the transplant waiting list for a heart and double lung transplant.

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