Thursday, January 22, 2015

Day 10 - Prader-Willi Syndrome

Well, when I look at the fact that this is day 10 in my 100 day challenge, yet it's already the 22nd, I realize that I haven't been keeping up with my writing.  I know it's been because I've been a little busy in the evening, or just too tired to write. 

Today I read an article about Prader-Willi Syndrome.  I have close friends whose daughter has PWS so I try to keep informed about this syndrome.  It's such a scary way for a child to live.  

If you're not familiar with PWS I'll explain what I understand about it.  Their little Pixie is almost 6 years old now. When she was born they knew something was going on with her.  She was diagnosed within a few days of her birth.  For little Pixie she was unable to suckle so she never nursed or drank from a bottle, never.  She wasn't strong enough.  So, low muscle tone is a major symptom.  She was never hungry and rarely cried.  I honestly don't remember her crying.  

So, PWS children have many struggles in their lives.  Low muscle tone, learning disabilities, speech delays, slowed physical development, no appetite when they're young and many other issues.  Usually around 2-3 yrs of age their appetite switch is turned on, and never turned off.  This means that they will never feel full, ever.  Can you imagine feeling hungry all the time, no matter if you just ate or not?  I can't. 

So, they are always hungry, but their body doesn't process or metabolize their food properly so they are limited to the amount of food they can eat.  This little Pixie eats only 900 calories each day!  900!  Seriously, try to watch how much you eat in one day.  The average person consumes about 1200 - 1500 calories each day.  Many people eat much more than that.  To give you an idea a McDonald's Cheeseburger, yeah, the little burger we give kids is 290 calories. Now, that's just the burger.  Kids, and adults, should eat 5-6 small meals each day.  For a 900 calorie diet that would mean you could consume 180 calories each meal, for only 5 meals.  Ok, that's about one apple and one piece of string cheese (just under 200 calories). 

PWS causes obesity in children especially if the parents do not have control of the food in the house.  These children find ways to eat, breaking the locks on the fridge and pantry, food in the garbage and sometimes things that aren't food.  The signal that tells them to stop eating doesn't work so they can overeat.  Sometimes that's horribly sad because they can eat so much that their stomach can rupture.  They also struggle with apnea, breathing problems and inability to maintain their body temperature.  So if they have a fever, it's off to the ER. Same goes if they vomit.  

PWS is scary.  I've been privileged to know this family and see how amazing they are.  Pixie is being raised by some awesome parents who want to have knowledge of what their daughter is going through.  They are also helping others become aware of Prader-Willi Syndrome.  

This link is the story of one girl and a short part of her journey as well as more information about PWS.

Having the internet at our fingertips has helped provide information and knowledge about PWS for people.  Take a look at what you can learn.

I hope you have a wonderful day!

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