Wednesday, May 1, 2013

PKU Awareness Month

As I have spoken about before, I have PKU.

What is PKU? It is actually short for phenylketonuria (which to this day I still have to look up the spelling for) and in plain English it means I can't have a diet high in protein. I have had it since I was born and every newborn is tested for it when they get their heel prick (which tests for more than 240 metabolic disorders).

Basically the science behind it is that protein is made of 20 amino acids my body cannot break down the amino acid phenylalanine (phe). What does that mean? If I eat a hamburger will I start seizing on the floor, no. The effects of PKU are a long-term pain in the butt.

Maintaining a strict diet was extremely important when I was growing up and my brain was developing. See, having high phe levels in my blood levels affects my brain functioning. If I was not treated from birth, I'd be severely mentally retarded today. So thank you science, for figuring this out before I was born so I can function normally from day to day.

This graphic courtesy of shows it in very simplistic terms.

Wait a second, you need protein to survive right?! Yes, you do. I get my protein the form of 60 pills a day. Pills that contain the other 19 amino acids my diet lacks. You think 60 pills is bad? When I was growing up I had to drink a formula that was so bad I had to drink it in a thermos with a straw through high school because I would gag at the smell of it.

This is what 20 pills looks like, I do this three times a day.

Have they improved the formulas today? Surely they have. But when I was recently going back on diet (more on that in a minute), the red carpet was rolled out to me and I was sent a million different samples. Forumlas in the form of powders you could sprinkle on your food, forumlas that came in fruit flavored shot form (so that you could get it done quickly), forumlas you could shake in a bottle of water (Crystal Light style) and forumlas that looked like cool sports drinks. Did any of them appeal to me? No. Because I had the old science formula growing up, I knew that distinct awful amino acid flavor. I can't describe it but I can tell you that no matter how they try to mask the flavor, my tricky taste buds will find the flavor immediately. I'm sure its easier for children who get to try the new formulas right away.

What does this mean now? Well from about late high school through about the last few years, I was off diet. Eating things I should not have been, being rebellious, enjoying food. Not thinking about what I was eating every ten seconds.

Well as I have just turned 30 and I have begun thinking about having children, there is this wonderful thing called Maternal PKU. Basically I have to go and have the most strict and stringent diet before I even start TRYING to have a child.

So for the past two years I have been cutting bad things out of my diet and getting back on diet. I spent the better part of the beginning of the year figuring out where my blood phe level needed to be through pregnancy and before I want to start trying.

How do I manage the diet? Well everything is based on a point system, very similar to Weight Watchers. When I was growing up I was allowed 22 points a day. A banana is 3 points. A large french fry from McDonalds is 13 points. So all real world food (except sugary candy) pretty much has some sort of points attached to it. You literally have to think about everything you put in your mouth and you have to keep track of what you eat.

Well I found out that the maternal PKU diet is even more restricted, EVEN MORE. So I dove into the world of low-protein food. And basically for the three months before I get pregnant and throughout pregnancy I will be eating low-pro engineered foods with some low-phe veggies and fruits added in. It's not great, but that kid better be worth it!

So why am I sharing all these good bits? Well since it is PKU Awareness month I thought I would write a post explaining my weird freakazoid issues about what I eat. If you've shared a meal with me, you know all this already but if not I hope I've enlightened you a bit.

Why do people need to be aware of PKU? Well for starters it is expensive! Thankfully I live in California and insurance is required to provide 90 percent of the cost of my formula. They are SUPPOSED to pay for my low-pro food too, but I can't seem to make that happen.

Thus people needing to be aware of PKU. The insurance coverages and laws vary from state to state and country to country. In Europe, where health care is more socialized coverage issues are a lot easier to deal with. Here in the United States we have to fight continuously until we get what the law says is our right. Currently people are fighting for having a national PKU provision law in the United States which would help a lot of other states out. Personally for me, it took me close to six months to gain access to my pills via insurance. I had to appeal the insurance three times and they didn't actually pony up for the pills until I told the California Medical Review Board my story about their refusal to pay their share.

Anyway, thanks for making it this far. I hope you leave a little more educated about what PKU is, and what it means for me personally. It is a daily struggle, and I'm not going to lie, every day isn't perfect. But every day is a new day and a new opportunity to get my phe levels where they need to be.


  1. This was a great read. Very informative with the personal touch. Loved it!

  2. Thanks for sharing this. I had no idea this existed! It is always interesting to me to take a step back and see what other people's struggles are. Puts things into prospective and makes me realize that you never really know what someone else is going through! You sound like a very strong person to be challenged with this your whole life and to have the strength to share your story with others! Good luck with the baby too :)