Change Is Good

First off, I want to say I am sorry for not having posted lately. As many of you know, I had an appointment with my metabolic specialist on Tuesday, February 7th, to talk to her about Kuvan. Turns out Kuvan wasn't what I had originally thought it was. It doesn't make it so that I can eat anything I want, it is just like a vitamin (in a way). But there is still hope that it can make me feel and eat better.

Protein goes into the liver and is broken down into tyrosine and many other amino acids. Well the enzymes that break down the protein in the liver is where Kuvan is meant to help. In someone with PKU, those enzymes are nonexistent or close to it, depending on the patient. I have a mild/ moderate case of PKU which means I do have some of those enzymes.

OK, so having given you a little lesson on that, here is how Kuvan works. It acts like a vitamin in a way that it helps the enzymes that I do have work harder and better. So, basically it improves my protein toleration by a bit but doesn't allow me to totally eat what I want. I still have to drink my formula, but I might not need almost all of my daily protein to be from a medical supplement.

So, everything else in my appointment went well. My labs weren't great, but that was to be expected considering I had been off the diet for a long time. But they were not as bad as we had thought they would be. Instead of having me back in 6 months to get set up for Kuvan (which is what we had discussed in the appointment), after getting my lab results back they want to see me back in 3. I am excited about that.


As part of my appointment, they took me in to the medical food store in OHSU/Dornbecker's Children's Hospital to help me pick out formula. Man, things have definitely changed since the last time I had been up there. There are so many things to choose from now, formula and medical food! For someone with PKU, medical food can allow a lot more freedom with their diet by supplementing some medical food in for high protein foods.


I got to choose between so many things! I got to bring a lot of medical food home to try, some are okay, some not so much. For example, they have these things called Tweekz. They are the PKU alternative for chicken nuggets. My husband and I tried them last night, awful. They taste like mushroom paste coated in bread crumbs, they smell as bad as they taste, too.


I, also, got to bring home some Camburgers, the PKU alternative to a hamburger. I haven't tried those yet but I will let you know how those are. I brought home some low protein pasta, which I had as a kid, delicious. The list goes on with Mac & Cheese Powder, Alfredo Sauce Powder, Cheese and Broccoli Soup, Portobello Mushroom Ravioli, and Cheese Ravioli. I haven't tried any of those, either. But the best thing is, I can order them through email and my insurance pays for it!! :)



When they handed me formula samples I was amazed by how many choices I had. I got a small box that was packed! It had everything from a Gatorade type drink to powder you sprinkle on your food. It had the formula I use to drink in there as well as many others. The only one I have tried is the Gatorade type drink and it made me sick, almost like it was poisoning me. I will let you know about the others I got as I try them.


Well that's all for now. If you have any questions about anything you can email me at: lauragoode@hotmail.com
Or you can comment to any post, or ask (and follow) me on my Twitter : Phe_ThePKUGirl


Until Next Time,
PHE