She recently posted a selfie on Facebook with this caption:
“I don’t really give in to HIM,” she says, “but there are some days that my stomach hurts more than other days. It’s hunger pains. I’ve had them all my life and unless something is found that can help it, they’re always going to be there. They’re not going to just up and disappear.”
When Vanessa was born, she had poor muscle tone and appeared undernourished. The term is failure to thrive. She had to be tube fed and spent 13 days in the neonatal intensive care unit at Maine Medical Center, where she had multiple tests to figure out what was wrong.
After ruling out several possible causes, including muscular dystrophy, doctors told her mother, Veronica, that Vanessa likely had a genetic condition called Prader-Willi Syndrome. “She was probably threeish when she had a chromosome analysis, explains Veronica. “It cemented the diagnosis. Up until that point, she had only been clinically diagnosed.”
What is Prader-Willi syndrome?
Prader-Willi Syndrome (PWS) is a rare genetic disorder — approximately one in 15,000 births. In the majority of cases, it’s caused by deleted or missing genes from the father on chromosome 15.
Those genes play an important role in regulating appetite.
Normally, when a person eats a signal is sent to the brain that he/she is full. With PWS, the part of the brain that registers that information — the hypothalamus — doesn’t get the message.
Other parts of the body are also affected by the missing genes. In addition to a lack of muscle tone, difficulties feeding, poor growth, sensitive skin and developmental delays, Vanessa showed classic physical characteristics.
“She had most all of the general characteristics,” says Veronica, “right down to physical traits like an almond shaped face, small hands, small feet, and blond hair and blue eyes — another thing that most of these kids have.”
Vanessa says the disorder also affects her emotions and prevents her from doing things most people take for granted. “I can be happy one day and end up upset or angry on another, “she says, “and I am smart, but because of PW, I can’t do a lot of things most people can do, such as drive or even something as simple as living on my own.”
As a child, every night she got a human growth hormone (GH) injection to, among other things, increase her muscle strength, improve her cognitive functions and help her grow. “I was deficient in GH as my body didn’t produce it in an appropriate way or in appropriate amounts,” she says. “If it wasn’t for GH I would still be just a little over 4 feet tall, so among the other benefits I got from the injections, I grew a foot and a half taller and I’m still happy about that.”
But there is no cure for PWS and when Vanessa was around three, its most profound characteristic kicked in. Suddenly, she was always hungry. Insatiable. Throughout her childhood, cupboard doors had to be locked and she had to be watched constantly because she would sneak food and eat and eat and eat.
PWS may be a rare disorder, but it is the most common genetic cause of obesity. Not only do people with it always feel hungry, they have less muscle mass and tend to burn fewer calories.
By the time she was in her 20s, Vanessa weighed 254 pounds. One day, she decided it was time to climb into the ring with the monster. His voice might still live in her head, but she was going to put up a fight and was determined to win.
Her red boxing gloves are symbolic in more ways than one. Her 23-year-old cousin Lacy, who died of cancer in November 2012, had a pair that she would use to help keep her strong. A month after her death — shortly before Christmas — Lacy came to Vanessa in a dream and presented her with a pair of boxing gloves. On Christmas morning, Vanessa opened a gift from Santa. “I open it,” she says, “and sure enough the gloves from my dream were sitting in my lap!”
The last time she visited her cousin, Vanessa had already started losing weight. Lacy told her she knew she could do it and made her promise to visit again when she hit her goal of under 200 pounds. “I never got the chance to tell her, but it makes my promise more meaningful because she passed away before I got under 200 pounds. I was one day shy of being under 200 pounds three months to the day that she passed.”
Vanessa needs every bit of motivation she can get because to this day, her monster is unrelenting. “In order for me to take the weight off,” she explains, “I had to find something that would keep me from listening to him because those pains are going on all the time. A normal person when they eat too much would get a stomach ache, but we wouldn’t know. If you put me in a room with food and nobody else, I couldn’t promise you I wouldn’t eat any of it and we can overeat. We can’t stop ourselves and what would actually end up happening — and I know this has happened — is we can eat to the point of stomach rupture.”
In February 2013, Vanessa reached her “ONE-Derland” — a term that means someone has lost weight and gotten below 200 pounds. Besides keeping the monster at bay, she exercises by walking several miles every day. “I don’t burn calories as fast as the typical population,” she says, “so I have to work twice as hard as a normal person to lose and not gain any of it back — which makes me that much happier that I have managed to lose a ton of weight (70 pounds) and I have kept it off.”
At 26, Vanessa often dreams of freedom and independence. When she graduated from high school and was considered an adult, services that used to be available through the school system went away. She doesn’t qualify because although people with Prader-Willi usually have a lower than average IQ, hers is too high and doesn’t meet the cut-off point — it’s 75 instead of 70 or below.
A highlight of her summer is going to Pine Tree Camp, but she’d also like to participate in Special Olympics and she misses old friends.
Don’t feel sorry for Vanessa though. She may be fighting a monster, but she does it with determination, grace and joy. All she asks for is support and understanding.
Way to go, Vanessa. I think your monster is down for the count.