Savannah and PWS: the horrible, honest to goodness truth

Some of you have asked about Savannah.... well here ya go :)

Savannah was born on November 1. I was 21 years old. VERY young by most standards. Ok all standards, but that is old compared to the babies I take care of. She was initially diagnosed with Russell Silver Syndrome. There are many traits and characteristics and I will only list those of Savannah. She was tiny. They call it IUGR (intrauterine growth retardation). They told me it was nothing that I did. That was comforting. She has her left side shorter (and smaller) than the right. You can see the physical mostly. She has a shoe lift about 9cm and brace on her left ankle to keep her from falling and breaking it. She has low muscle tone and tires easily (you would NEVER know this if you have seen her in 'action.'

She learned to roll over at 9 months, sit up at 12 months, stand at 2 years, and walk with a walker at 3. She shed the walker at 4.5 years old and has been moving EVER since. She had her first surgery when she was 6 months old. And has since had 3 others.

When she was about 4 years old, she was diagnosed with Prader Willi Syndrome. It is the UNCONTROLLABLE UNSATIABLE desire to eat. There are other characteristics, but this is the main one. Not only are they ALWAYS hungry, but they burn less calories because of their low muscle tone. So NOT only is she on a limited diet, but she gets 70% the calories that other children her age get. Sounds fair right? One might think that it would behoove someone like Savannah to hit the nearest buffet and load up. Truth is, PWS children and adults have been known to eat themselves to death. Literally. Their hearts, guts, and overall body just can't take it.

Typically it is late diagnosed and by that time the kids are too old and too overweight. Not that it can't be fixed but it is VERY hard. Trust me I did it and it was torture for us all and she was only 4.

What does this mean for us? We lock our pantries, my mom, my aunt, me. There were times when we would wake up in the middle of the night and find her scooping ice cream, cool whip, popsicles, anything she could find. She didn't even realize what she was doing until we found her, but she was shoveling food in like she would never see another bite. This sets us back LOADS of calories when she does this because I have to make up for it by decreasing her already LOW calorie diet.

I have gates up in my hallway to my bedroom and from their bedroom, that way they can only come into my bedroom. I had to do this because I would find her doing random impulsive things (very common. the impulsions for PWS) like emptying ALL my lotion (really it was like 10) onto the bathroom floor. Opening all the toilet paper (from costco) and then unraveling it all over the floor, painting my bathroom walls with nail polish, and oh did I mention buzzing her hair? And she was 6.5 years old when she did all this.

This is all to be blamed on the PWS. Really. She isn't being 'naughty' per se, it is more the uncontrollable impulsiveness and not enough will to stop. When she is caught, she feels terrible, but sometimes she just can't stop. This is the maddening part. I can't get TOO mad at her, you know like slap her silly or yell because ten minutes from now, she isn't going to remember what you are mad about.

Back to the food. There can't be one morsel ANYWHERE, gum, candy, crumbs, food. ANYWHERE. She will sniff it out, find it, and eat it. No exceptions. I don't know how she does it. So all food is in pantry and fridge under lock and key. NO EXCEPTIONS. After each meal, the food is cleaned up, dishes done (because she will see if there is anything in the sink) within minutes of finishing. There is no food in any bedrooms of any house she visits regularly. We have certain snack times and meal times and if there is any wavering, the day is shot to you know where.

Thing is, Savannah is NEVER full. Despite the large plates of fruits, veggies, low carb items that I feed her. NO amount of food is enough. She is NEVER full. Someday this will be a big problem for me. Some PWS kids are not uncommonly found sneaking out the window to run to the local grocer and get some food. It is not uncommon they will do favors for people in exchange for food. I have to know where Savannah is at ALL times. Not a minute goes by I don't know exactly where she is and exactly what she is doing. Did I mention that NO amount of food will fill her up? NO AMOUNT. She goes to school 'hungry,' she goes to bed 'hungry,' she leaves the table 'hungry.' ALWAYS. She always will. It is the fact of her life.

It is the most horrible, awful way to live life. I have been hungry and it sucks. To feel that way all the time because me brain is broken in that area? I just don't know if I could bare it. One might ask is the appetite suppressants work or if there is some special "yoga" to do to decrease hunger. NOPE. NOTHING. The part in the brain that says you are full is broke. BROKEN. Someday (hopefully never) she will have to be put in a home where PWS adults live. Where they lock all food away. Where they have to lock all the doors and windows. Just to keep them safe. I hope it doesn't come to this. But one day it might.