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Prader-Willi syndrome.

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  • Chief Cushie

A Hunger That Can Never Be Satisfied




What: Inside NZ: Insatiable Hunger


Where: TV3


When: 9.30pm, Thursday




NEW Zealand may have a growing obesity problem, but for the young people in Inside NZ: Insatiable Hunger, their weight issues really aren't their fault.


They suffer from a disease where their stomachs are unable to tell their brains that they are full, and they always think that they are starving. Insatiable Hunger follows four Kiwi families with children suffering from the devastating effects of Prader-Willi syndrome.


Despite the obvious shortcomings of a disease that puts sufferers in danger of literally eating themselves to death, Prada-Willi syndrome is coupled with other cruel side- effects. Low muscle tone limiting exercise and a slow metabolism make overeating even more dangerous, while behavioural problems make life even harder for sufferers' loved ones, who are already dealing with padlocking food in the cupboard and keeping sufferers out of rubbish bins.


Each year two Kiwi children are diagnosed with this non- inherited lifelong and life-threatening disease. It does not discriminate between race or gender, and it is not known what causes it. Ironically, the disease which will cause these children to balloon begins with stunted growth, and stunted development.


Though Prada-Willi syndrome has no known cure, regular growth hormone injections have been proven to reduce fat and improve muscle tone, significantly helping with obesity and related complications. Yet though these injections are funded in other countries, Kiwi kids face an ongoing battle trying to qualify for the treatment in New Zealand. Pharmac, the government drug-governing agency, has strict criteria for funding the treatment, which many Prada-Willi syndrome kids do not fit into.


Inside New Zealand: Insatiable Hunger follows four young people suffering from the disease at different stages of their lives. Tiny, doll-like 18-month-old Amelia Reid, only sat up by herself at 14 months of age. Amelia needs to have the growth hormone treatment so she can have a chance at developing normally.


Three-year-old Cameron O'Reilly's parents have been paying more than $10,000 a year for him to have the treatment. Cameron has finally nailed the art of walking, and with his growth hormone treatment has been able to graduate to the big kids' section at kindy. However, this may be Cameron's last year with free access to food and treats because the insatiable appetite will kick in sometime between now and five years old.


In Northland, 11-year-old Darcy Harris faces being shifted out of the mainstream education system because of the behavioural problems caused by the disorder. Stealing his classmates' lunches and his violent outbursts are putting a lot of pressure on the staff at his small rural school.


Francie Thornton, 22, is of the old era of Prada-Willi syndrome sufferers. She is not eligible for the growth hormone treatment, and will be forced out of her sheltered flat if she can't control her eating -- and her temper. She is strictly monitored by minders, and should be on a 1000-calorie a day diet, but Francie is resourceful.




? 2008 Dominion Post. Provided by ProQuest Information and Learning. All rights Reserved.





Published: 2008/07/08 15:00:12 CDT


? RedOrbit 2005

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I have worked with children affected by Prader-Willi Syndrome (PWS). It is actually one of the childhood disorders that has been genetically mapped. The deletion or translocation of the gene occurs on the proximal arm of chromosome 15, and occurs from the genetic material from the paternal chromosome (it is interesting that at this same location, deletions and translocations from the maternal chromosome result in another syndrome, which is very different, Angelman Syndrome). PWS is much more than just a disorder that affects weight (much like Cushings), the children who are affected can have mental retardation and behavioral and learning difficulties, but generally can function in society to some degree. From infancy they can have feeding and muscle tone issues, and I have even seen severe cases where children ended up on ventilators due to the severe lack of muscle tone in the muscles necessary for breathing. It is really a sad syndrome. What is amazing to me is that I have worked with both children with PWS and Angelman Syndrome, and even though the problem is at the exact same location on the chromosome, the two syndromes are almost opposite. Children with Angelman syndrome are typically very thin, they have severe and profound mental retardation, suffer from seizures, and very tight and spastic muscles which causes them to walk in a very characteristic pattern for which the disease was first named "happy-puppet syndrome." This is one of those cases that makes me very interested in genetics, and makes me think that we don't really know a "speck" about how the human body really works!!

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Prader-Willi Syndrome was featured on one of the cable health channels a couple of years ago. There is a special "institution in Pennsylvania where doctors and staff try and help the kids who suffer from P-W, to learn to coping skills so they can better deal with life. It's sad---these kids look like "fat" kids---and they act like "brats". As hard as it would be to have to take your child to a place like this---it is probably better than having to deal with a lot of misunderstanding at home.


I take GH---and it says that it should not be given to folks with P-W---not quite sure why.


It's a devastating thing to deal with---I know parents have had to literally put locks on their kitchen cabinets and the fridge and freezer---but some kids have taken a screwdriver to the hinges or just ripped the cabinets apart to get to food.

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I knew a little girl that ended up with Pica (pretty much means you eat non-food things), because she had PWS and her parents kept everything locked up. Her favorites were books and puzzle pieces, anything paper or cardboard actually. I loved her, but she was a terror of a girl, non-verbal, would bite, head-butt, but could you imagine living life feeling like you were hungry all of the time?? And then add not being able to express yourself on top of that!! I used to give her huge bear hugs to help calm her down, when she got to be about 10, she was too big for me to fit my arms around anymore. Man I loved those crazy days!!

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You have the patience of Job!!!

I couldn't manage to work with these poor kids---and I know it's not their fault!

It's an incredible challenge for all of those who are involved---you are an amazing person!

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