- Live in constant fear that your 19 month old is becoming hyperphagic?
- Count every poop because gastroparesis is a very real condition that can lead to death?
- Jam a needle full of growth hormone into your child’s leg every night?
- Cry at the sight of a “typical” child eating, sleeping, walking, or functioning?
If so, your offspring might have Prader-Willi Syndrome!
Don’t get me wrong, not every day is like this, but a lot of them are. I want Kyler to get better, I want his missing chunk of chromosome to magically reappear, and I don’t want him to hurt. He’s quickly approaching 2, and with that are coming some big personality changes. I probably call my mom or message my sister about a thousand times a week in constant fear that his PWS is advancing.
“OH MY GOD. Kyler is crying when he’s hungry, do you think he’s becoming hyperphagic already?!”
“No, Megan. He’s just getting stronger and turning into a two-year-old.”
“Oh, ok. But he’s also whining and crying all of the time. Is he sick?! You know he doesn’t get fevers and I have no way of knowing!”
“No, Megan. He’s just getting independent and wants his way.”
“Crap! I can’t afford organic food, and I accidentally fed him too many carbs. This will surely kill him, right?!”
“No, Megan. Stop reading crazy people’s blogs.”
“Ok, thanks. I’ll talk to you tomorrow when the world is ending again.”
I have to say, I don’t know what I would do without them.
PWS is a constant battle, in part, because of its rarity. Most of the “treatments” that exist (supplements, diet, etc.) are largely anecdotal since no one knows much about, or will fund the research to officially approve them as treatments. I think the worst part for us (and many other families of children with special needs) is that supplements are rarely covered by insurance. PWS management is a very expensive endeavor.
Kyler is now on five medications/supplements, not including the oxygen and other medical equipment we have in case of emergency. When we drive to Florida in a month, I have no idea how we’re going to haul all of this. I’ve said this before, but traveling in general is rough. Even if all of our supplies, food, medication, and equipment are completely intact (and not forgotten), Kyler is completely worn out for two full days after, which puts him at higher risk for getting sick.
Speaking of food, since he’s been eating more complex purees (and we have been increasingly short on time) we’ve relied on those organic baby food pouches. For a “typical” baby, these things are convenient and truly the bee’s knees. Unfortunately, they’re pretty full of bad carbs, and I’ve had some equally bad guilt about feeding them to Kyler. Despite going to school and having absolutely no spare time, I’ve committed myself to making all of his food again. How badly I WISH he was eating solid table food. So, three hours after roasting squash, mashing beans and lentils, sautéing onions with ground beef and turkey, and sweating… a lot, I have ten days of Kyler-food prepped and ready. Did I mention that NONE of it is organic (gasp!) Yeah, don’t be too quick to call me supermom, apparently that lower grade stuff is poison. Along with all of this comes the realization that anything having to do with food is never going to be easy for our family.
Can I talk a little bit about that? Well, I’m going to because it’s important. The pain of hyperphagia is not funny nor is it something that can EVER be taken lightly. I’m going to quote a segment out of my One Small Step speech here, because it truly is the only way I know how to describe what Kyler will likely experience:
“Imagine having to fast for 24 hours before a blood test. You’re starving, and can’t wait to have that first meal to make the pain and discomfort go away, but it never does. You eat a big cheeseburger, fries, maybe even a milkshake. You want to take a nap and relax, but your stomach still hurts- there’s a gnawing in your brain that tells you to eat more. You want to scream and cry because it hurts and you want it to stop. That will be Kyler’s life.”
I tear up just typing that.
Kyler is, and always will be on a special diet, and will never be allowed many of the foods you and I are used to. We hope, and hope, and HOPE that by the time Kyler reaches the hyperphagia stage of PWS, there will be some kind of intervention for this awful condition. Unfortunately, even if there is some type of “fix”, he also (likely) has slow gastric motility (food does not pass as quickly through his digestive system). He also cannot vomit. Both of these issues are recipes for disaster, or even death if he is allowed to eat too much, or if the warning signs of GI issues are not addressed immediately. Lastly, the low muscle-tone and potential severe behavior and emotional issues are, well, scary. Let’s face it, Prader-Willi Syndrome is just downright scary.
But you know what? In spite of all of this, Kyler is amazing. I know it sounds like I’m constantly worried, upset, and bitter, and maybe I am. Maybe we all are. Kyler is loving, strong, intelligent, and downright cute, and it seems like a cruel joke that he should have to go through much. Despite what many parents say, I am a logical being, and I don’t feel “blessed” that PWS came into our lives. I do, however, think that a genetic mutation is a really unfortunate thing that could have happened to any of us, and the only way we can survive is to make the best out of it. I love Kyler unconditionally. He has taught me more than I could have ever dreamed to know, I will always fight for him. After all that we have been through, and all that we have yet to endure, I am ever grateful to have my little superhero: the most resilient and awesome kid I know.