Constant hunger plagues those with Prader-Willi Syndrome

Can you imagine eating and eating, but never feeling full?  The part of our brain that controls hunger and fullness does not work like it is supposed to in people with Prader-Willi Syndrome (PWS).  One Lake Charles family is doing everything they can to keep two-year-old Ryan Orgeron from overeating his way to dangerous obesity.

It is an extreme measure of love and protection:  locking the refrigerator to keep Ryan out.  "The refrigerator and cabinets and food are like a loaded gun for them," said Ryan's mother, Jill McKinney.

Ryan's parents have had to change the way they live to let him live the healthiest way possible with PWS, the most common genetic cause behind life-threatening childhood obesity.  "They can eat so much that they can rupture their stomach and die, so there is no way he will ever be able to live independently," said Jill.

PWS affects one in every 15,000 births because of a defect on chromosome 15.  It causes mental delays, weak muscles, slow metabolism and constant hunger.

There are two stages of the syndrome: inability to feed during infancy, followed by an insatiable appetite.  "So the irony of it is they go from not wanting to eat at all to non-stop hunger," said Jill.

Ryan is not yet feeling the emptiness of hunger.  He is on human growth hormone to help with his development and on a special low calorie diet.  But as he gets older, the hunger will grow and his father, Ronnie Orgeron, is already drawing up plans for a protected pantry.  "I'm gonna make a pantry and lock the door," he said, "so it's going to be all foods locked up."

At home, Ryan's eating is very controlled.  Meal time is structured, with the family eating together at a table and not eating forbidden foods in front of Ryan.  Then, there are the locks on food storage spaces.  "We will have to graduate to a pad lock on the fridge when he's older," said Jill.

There are fears down the road that environments like a school cafeteria could get out of control.  "He's gonna want to eat everything," said Ronnie, "he's going to want to take food off of other kids' plates and that's my biggest concern."

There is no cure for PWS, just careful management.  An obesity drug being tested in clinical trials is showing positive signs.  "They're feeling full, they're losing weight," said Jill, "and that just started."

Until a new treatment method comes along, Ryan's family plans to keep doing what they know to be the best medicine: love, limits and laughter.  "He has taught me that no matter how hard it can get, he wakes up with a smile," said Jill.

"We go through life like, 'oh this and oh that,' and look at the negative," said Ronnie, "but look at him. It's a picture with the negatives he's got, and he's the happiest kid."

The only way to diagnose Prader-Willi Syndrome is through a blood test scanning genetic defects. The PWS region of chromosome 15 is one of the most complex pieces of the entire genetic make-up.

The Foundation for Prader-Willi Research is hosting a fundraising walk on Saturday, May 17 in Houma.  Click here for more information.

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