Thursday, May 2, 2013


Welcome to May, Prader Willi Awareness month. Man I am hungry; I've been foraging through the kitchen looking for something to sate my appetite (not much luck though; we're low on groceries and there's not much for a non-grain-eater to ingest, grrr). When I eat, this will go away until I need food again. But it never goes away for Z-Man or others with Prader Willi Syndrome, no matter what or how much they eat. How would I deal with a hunger that was worse than this, and that was constant, unremitting, insatiable? How would you? And what if this hunger came with a host of other insults and difficulties?

Prader Willi Syndrome (PWS) is caused by a defect of the long arm of the 15th chromosome in which the paternal copy of the gene is silenced. It has a sister syndrome, Angleman Syndrome, in which the maternal copy is silenced.
The hallmark feature of Prader Willi Syndrome is hyperphagia, from the Greek for excessive eating, though in PWS it is the literally constant urgency of being starving hungry caused by a brain that physically lacks the ability to turn off hunger and resulting in a truly insatiable appetite. Thus a person with PWS will do anything to get food and will actually eat themselves to death, making food dangerous and meaning they must be protected from it.
There are many other aspects of PWS, which causes major disorder of the entire endocrine system and the hypothalamus, but I won't get started on anything else today; the hunger was enough.
My grandson the Z-Man lives with Prader Willi Syndrome, and in a sense so do his mother, siblings, grandfather and I. We all live with Prader Willi Syndrome here in this lovely crowded old house. It isn't easy for any of us, it certainly isn't easy for our irresistable little Z-Man. PWS has been a central feature of all our lives, even before his diagnosis, since the day he was born, and we and the entire Prader Willi community are Still Hungry for a Cure.

May is Prader-Willi Syndrome Awareness Month! Sharing a different fact about PWS each day of the month - Did you know that approximately 70% of people with PWS are walking around undiagnosed? Can you imagine? How can this be?  PWS is a spectrum disorder. Some people have so few symptoms or such mild symptoms that they don't know they have. Sometimes, doctors and therapists know that something is going on, but they haven't figured out what. To further complicate the issue, there are a few different kinds of genetic tests that can be run to detect PWS, but depending on the test used and the subtype of PWS, the test could miss a PWS diagnosis.
  I'll let these say the rest.
Please share any of the following videos you like
and help spread Prader Willi Syndrome awareness.
The Doctors
Two video segments on Prader Willi Syndrome from a recent episode

 Prader Willi syndrome (PWS) is a congenital condition that significantly reduces muscle mass and causes cognitive delays. A common symptom of PWS is insatiable hunger, which often leads to obesity and can be life-threatening if left uncontrolled.
Something odd is happening with the rest of the vids I added here. I'll sort it later, meanwhile they're just missing. There's still documentaries below.

Can't Stop Eating

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