Denyse couldn’t sleep. There was food in the fridge.
It haunted her, like bad dreams.
Denyse pleaded. Only a German Shepherd chained to the fridge would bring her peace, she said. The food was too close.
Denyse is one in 15,000. She was born with an empty stomach and no way to fill it. She has Prader-Willi Syndrome, a rare genetic disorder characterized by a constant sense of hunger caused by never feeling full.
While the condition is rare, patients are being treated at a special program in Gainesville: Greg, who ate bags of corn meal and jiffy mix; David, who drank an entire bottle of cooking oil; and Adele Elchert, who ate the lunch tables at Coral Springs High clean, treasuring her peers’ discards.
“Imagine the hungriest you’ve ever been,” a Prader-Willi patient said. “That’s how I feel all the time.”
Like Greg, David and Denyse, Elchert will never know the satisfaction that accompanies a full stomach.
You’d never guess Elchert once weighed 318 pounds. Her 4-foot-11-inch, 130-pound frame slowly moves toward the wooden table. She takes out her lunch: A container of sliced zucchini and yellow squash, a yogurt cup, a plum, green beans, a hot dog, a baked potato, a slice of cheese and turkey and a chai tea bag with two Sweet ‘n’ Low.
Her brunette hair is wiry and short, cut a couple of inches below her silver-rimmed, oval glasses. Her two large front teeth peek out as she chews.
This lunch will cost her. She only has 300 calories left to spend of her 800 calories per day — about half of what would be recommended for a woman her size.
Prader-Willi patients could essentially eat themselves to death, making calorie restriction vital, said Steve Drago, CEO of the Arc of Alachua, a nonprofit organization that treats Prader-Willi patients. Drago has worked with Prader-Willi patients for more than 20 years.
When Elchert, now 38, arrived at the Arc almost 10 years ago, her body was giving up, she said. At 318 pounds, she had an enlarged heart.
The Arc put her on a restricted calorie diet, requiring her to record everything she ate, including the food’s calorie counts. If she exercised, she was allowed 200-300 more calories. It took her two years to lose the weight, Drago said. No medication—only the restricted-calorie diet and exercise.
“My life was on the line,” Elchert said.
Elchert, 38, started life in Columbus, Ohio. As an infant, she refused to eat. She grew up in Coral Springs and moved to Gainesville to seek treatment at the Arc.
Prader-Willi babies are born with poor muscle tone, so they can’t suckle a bottle, said Julie Frisbie-Knudsen, Arc nursing coordinator. Their appetite is almost nonexistent.
The hunger comes later.
Prader-Willi patients develop their insatiable appetite at about 8, said Dr. Jennifer Miller, an associate professor at the Univerisity of Florida’s College of Medicine, who has studied the disease for the past 13 years. “They go from eating nothing to eating everything.”
Miller, whose specialty is pediatric endocrinology, said there are no limits to what they’ll eat: pet food, garbage — anything.
Drago said he doesn’t buy Halloween pumpkins anymore for this reason.
“All you’ll find is the stem.”
At 8, Elchert developed the hunger.
She would skillfully slide a butter knife into the top of the locked pantry door and pop it open, slipping her thick arm inside, said her sister Amanda Elchert.
Prader-Willi results from a chromosomal mutation on chromosome 15. The mutation also inhibits hormonal development, preventing proper brain development.
This is detrimental, Miller said, as it prevents patients from understanding they can help themselves with self control.
Because Prader-Willi is now diagnosed in infancy, patients take growth hormones that enable proper brain development, Miller said.
Miller said Elchert is one of her smartest patients. With her quick wit and jokes, Elchert is far from slow.
Elchert, who was diagnosed at 2, is able to comprehend why she can’t give in to her stomach. She said the knowledge has helped her control her urges, but it’s still a struggle.
“People say, ‘You can’t have this. You can’t have that,” she said. “It’s hard.”
It’s always been hard for Elchert. As a child, her classmates teased her mercilessly, she said. They would call her names and throw her lunch in the trash.
“They were very much cruel to me — very much,” she said.
The teasing made her change her name. Elchert’s childhood nickname was “Delly.” She said she didn’t like her name associated with a deli.
Elchert said she felt like an outsider growing up — that no one understood her. But that’s changed. The Arc allows patients to live in group apartments with a staff member if they’re responsible enough.
Elchert celebrated her one-year apartment anniversary in October.
“I feel like I’m normal,” she said.
Elchert cherishes her autonomy. She said she loves buying her own groceries and cooking her own food. She works at the ARC, doing filing and organizing the office. Her boyfriend, James Sharp, is also being treated at the Arc.
Elchert’s apartment is neat and clean with its dark couches and cream carpet. She immediately heads to the kitchen to start dinner. She forks chicken into a pan with a bag of mixed veggies and places it in the oven to bake. It’s 3:30 p.m. She won’t eat for two hours.
She heads to her room. An entire kingdom of stuffed animals canvases her bed. Sitting in the center of them all, she looks a bit like a child in a fancy red dress.
But she is far from the obese little girl with the butter knife and discarded lunch scraps. That was Delly.
Here, in the center of her room in her apartment is Adele: The independent woman with the unlocked fridge and radiating pride.