Category Archives: Prader-Willi Syndrome

Top Sources on Prader-Willi Syndrome

Readers: I’ve noticed a dramatic increase in traffic to my posts on Prader-Willi, as well as people trying to search for facts about Prader-Willi on this blog, i.e. “Why are those with Prader-Willi always hungry?”

Firstly, thanks for reading, watching and commenting. It is great to know that my posts are being looked at by others. Also, I welcome any sort of feedback, be it negative or positive. Feel free to email me at klandergan@globe.com.

I wanted to provide some resources on Prader-Willi that I’ve found particularly useful. I hope this will help those who want to learn more.

Prader-Willi Syndrome Association USA

This is by far the best source on Prader-Willi Syndrome, and includes basic facts, personal stories and events.  If you or someone you know is trying to find a psychiatrist/physician who treats Prader-Willi, use the site’s search tool.

Q&A on Prader-Willi Syndrome

This is from the Prader-Willi Syndrome Association’s website, but I wanted to highlight this because their site can be a bit hard to navigate. The above link is a question and answer page about the syndrome.

Foundation for Prader-Willi Research

Cutting-edge research and treatment options for those with Prader-Willi: a must for those with Prader-Willi or for those who know someone with the syndrome.

“Can’t Stop Eating,” A British Documentary on Prader-Willi Syndrome

This British documentary takes an in-depth look at a clinic that serves Prader-Willi patients. It’s pretty informative and definitely pulls at your heartstrings. But, the film tends to overgeneralize and sensationalize the syndrome by failing to acknowledge that this is a spectrum disorder. Not everyone is this extreme. That said, I think this is worth a watch if you are interested in Prader-Willi. It’s 47 minutes long, so make sure to set aside a chunk of time.

And because I got so many hits on the slideshow, I wanted to include the stills from it. Enjoy, and thanks for reading.

 

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Raising a daughter who is perpetually hungry: “I can never leave her alone”

The night after she was born, Mady Wells was asleep in the hospital when she stopped breathing.

“Another mother found her and she was gray,” said Cindy Wells, who works for the quality and patient safety division at the Steward Health Care System in Massachusetts.

Wells said that at first, the doctor told her and her husband, Jarrod, that their daughter may have a form of muscular dystrophy that is fatal at the age of two. When they were told it was Prader-Willi syndrome they were actually relieved, she said.

That was, until they went online.

“We started reading these horror stories…of parents with Prader-Willi kids who skin pick, and how their child picks their skin, butt, and has soars…Or how their kid could never walk: they were five and not walking, not talking,” Wells said. The national Prader-Willi group recommends not to search the syndrome online because of what’s out there, she said.

According to the Prader-Willi Association of the USA, Prader-Willi syndrome is the most common genetic reason for obesity in children, but it is still quite rare: estimates range from one in every 8,000 individuals to one in every 25,000. They syndrome causes short stature, low muscle tone, lack of sexual development, and a low IQ which results in learning issues.

But the most frightening aspect of the syndrome is that those with Prader-Willi have a defect in their hypothalamus, the part of the brain that controls appetite. Consequently, they have a chronic feeling of hunger for which there is no cure. And on top of never feeling full, those with Prader-Willi have a slow metabolism, and their low muscle tone makes exercise extremely difficult.

Mady, who is now 4-years-old, started gaining weight around the age of two and a half, Wells said. When she was an infant, Mady was underweight. But around the fall of 2010 to the spring of 2011, her weight had skyrocketed to the 95th percentile for her height and age.

“We can’t eat in front of her at all if she’s not eating,” Wells said. “She will say, ‘what are you having, what are you having, what are you having?’ She will stay right by you and stare at you. You can’t even leave a drink out if you go to the bathroom because even if she is doing something else, she will go right for it.”

Wells and her husband have cracked down on Mady’s eating, and she is now at a healthy weight, Wells said. She only eats one snack at school, whereas all the other children get two snacks. For breakfast she eats a half a cup of Honey Nut Cheerios and a banana with skim milk, and her snacks are almost always fruit. For dinner she eats whatever the family is having, but her portion is under 500 calories.

But to ensure that Mady isn’t sneaking food is really a 24 hour a day operation. All of the cabinets are locked and there is a baby gate in front of the kitchen entrance.

“It’s difficult in the sense that we always have to keep an eye on her,” Wells said. “I can never leave her alone.”

And although raising a daughter with Prader-Willi can be tough, Wells said that it is also a really amazing experience.

“For the first couple of years we had some bitter times where our friends’ kids can say the ABCs and count to 10, and our daughter is just learning to crawl,” she said. “But then it just makes it so much more special, just seeing her accomplishments and seeing how far she’s come; she has to work so hard to get where she is.”

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Raising a Prader-Willi child: “We knew there was something wrong right away”

For the past few years, Meg Comeau, director of the Catalyst Center at Boston University, has had to lock her cabinets and refrigerator so her 24-year-old daughter, Sarah, won’t binge eat when she’s not looking.

Sarah has Prader-Willi syndrome, a rare disorder that prevents sufferers from feeling full. Comeau said her daughter had enjoyed eating, but it wasn’t until her teenage years that she started sneaking food.

“It’s always been challenging,” Comeau said. “I think it’s a very natural maternal reaction, that feeding is nourishing your child. Over time we sort of figured out that this is the way we need to operate.”

When Sarah was born the hospital staff called her “the floppy baby” because of her low muscle tone, which is a symptom of Prader-Willi, Comeau said. But Sarah wasn’t diagnosed until she was 13 months old, after she had undergone an extensive series of tests for various neurological disorders. In retrospect, Comeau doesn’t know why it took so long.

“We knew there was something wrong right away,” she said.

Comeau describes Prader-Willi syndrome as “a triple whammy” against trying to maintain a healthy weight. There is “hyperphagia,” which is the dysfunction in the hypothalamus that causes individuals with Prader-Willi to feel perpetually hungry, a symptom for which there is currently no cure. Those with Prader-Willi also have a slow metabolism, so it’s harder for them to exercise and burn calories.  And on top of that, it’s harder for individuals with Prader-Willi to exercise because of their low muscle tone.

Sarah has had issues with maintaining a healthy weight, Comeau said. It’s the hunger aspect of Prader-Willi that is particularly scary because the results can sometimes be deadly.

“The overwhelming majority of folks with PWS can die from either one of two things,” she said. “The result of complications from obesity, or choking.”

The choking, she said, happens during a binge. Another deadly consequence of binge eating is it can cause a stomach rupture, though those incidents are quite rare.

Many parents of children with Prader-Willi need to lock their cabinets, but sometimes that won’t be enough, Comeau said.  She knows of some children who actually scour for scraps in the garbage.

Like many with Prader-Willi, Sarah has learning issues, Comeau said. According to the Prader-Willi Syndrome Association USA website, the average IQ of an individual with Prader-Willi is 70. Sarah’s IQ, Comeau said, is only in the low 60s. Having this intellectual disability makes it hard for Sarah to understand people’s emotions.

“She gets confused by other people’s emotions,” Comeau said. “It makes it really hard for her to relate to other people.”

Because of this, dating can be a real challenge. Sarah had a good relationship with a guy in high school, but if someone else were to enter into her life, Comeau said she would worry that he would take advantage of her daughter.

“I would be really suspicious of sexual abuse,” she said. It’s not only a big problem for people with Prader-Willi, but for those with intellectual disabilities as well, she said.

Comeau said she planned to become a history professor, but that after Sarah was born she went back to school to study healthcare financing policy. Now she heads the Catalyst Center, an organization out of Boston University that helps states improve services for kids with disabilities.

“There have been some real positives that have come out of it as well,” she said. “I wouldn’t be doing this work if I wasn’t Sarah’s mom.”

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Mentions of the “hunger disease”

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Prader-Willi Syndrome: “Can’t Stop Eating” …well, sort of

Imagine if you felt perpetually hungry. No matter how much you ate, you were still left unsatisfied. This is the nightmare that many with prader-willi syndrome, a chromosomal disorder, must face on a daily basis. And they may never experience this feeling of fullness, as there is no cure for the syndrome.

Eileen Rullo, president of the Prader-Willi Syndrome Association of New England, has a son who is diagnosed with prader-willi syndrome. Luckily, his symptoms are mild. Rullo doesn’t have to lock her cabinets like some parents do to prevent their child from binging. The disorder, she said, has a wide range of severity. And this is what goes unreported on talk shows and in documentaries, Rullo said.

“They sensationalize (prader-willi syndrome) in the news, and that’s hurtful,” she said. “And then they misrepresent it; they don’t get the facts right.”

Take this British documentary, on prader-willi syndrome, which is entitled “Can’t Stop Eating.” There is no mention that symptoms of prader-willi vary from person to person. Instead, the documentary focuses on two individuals with very extreme cases, and throughout the piece the narrator  makes very broad claims about the syndrome.

“People with prader-willi syndrome never feel full,” the narrator says. “Left to their own devices, they would eat themselves to death. This film is about people who are trapped by their love of food.”

According to the Prader-Willi Syndrome Association of New England’s website, the cause of prader-willi syndrome is unknown, though it is most commonly found in individuals who are missing the 15th chromosome. Symptoms include short stature, incomplete sexual development, low muscle tone, and chronic hunger which can lead to life-threatening obesity.

But it is the symptom of chronic hunger that is really blown up in the media, Rullo said.

“I believe that there’s a spectrum of this hunger piece,” she said, and this is what is mostly misrepresented.

“Can’t Stop Eating,” opens with scenes of individuals with prader-willi, who are  stuffing their faces with food. The film centers around a clinic in Great Britain that treats dozens of prader-willi patients who are there as a last ditch effort. But the narrator speaks about the syndrome as if it is this extreme in every case.

For instance, Tamara, who is portrayed as a typical sufferer of the syndrome, has a case which is actually more out of the ordinary.

“Neither Tamara nor her mother understood why she always had a craving for food,” the narrator said. “Their relationship was torn apart. She turned to stealing to feed her addiction. By the time she was 23 she weighed 34 stone (476 pounds) and was finally diagnosed with prader-willi.”

 

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