Maternal PKU: Pre-Pregnancy & Kuvan

This is the first in a series of posts related to Maternal PKU. These will be post-dated since pregnancy made me a bit behind on my blogging. I am not a medical expert and this is my experience only. Pre-pregnancy I was already on Kuvan for about five years with a tolerance of 30 grams protein/day. (Pre-Kuvan my tolerance was about 500 mg phe/day or 10grams of protein)

~Pre-Pregnancy~
If your reading this, you may already know that one of the most important things for any PKUer before getting pregnant is to have levels under control. We knew we wanted to start trying to have children sometime around August (2013), so we talked to my geneticist and dietitian months before that. On Kuvan my levels were under control for the most part, but they ranged from about 3-8mg/dl (a little to high for pregnancy).

My geneticist wanted levels every other week before we started trying to ensure I could keep them in range. For my clinic, acceptable range was seen as 2-6mg/dl. (I have heard some clinics prefer no more than 4mg/dl). Since I was already under control with my levels, it didn't take long to ensure I didn't have levels over 6mg/dl. She also had me start taking pre-natal vitamins at this point and ran a complete amino-acid panel. She sent us with some information to give to my OBGYN and requested we fill him in on PKU so that he is ready when the time comes. When I had a couple months of good levels, we felt comfortable trying.

Kuvan or Not to Kuvan?

~Kuvan~
As far as the decision to stay on Kuvan goes, this was a difficult choice. As I mentioned above, I had already been on Kuvan for five years. Pre-Kuvan I was rarely able to keep my levels consistently low (8-14mg/dl was the normal level). After five years of not eating any low-protein foods, I could not imagine trying to go back. My husband and I met a few years into my Kuvan use, and he didn't even know what life before Kuvan was like for me. We moved to a new town once we were married, so literally no one in my everyday life knew what my "real" PKU diet would look like. I knew I would have my husband and friends' support if I wanted to try to stop taking Kuvan, but I wasn't sure if it was the best choice.

My husband and I both attended the National PKU Alliance conference in 2012 to learn as much as we could about Maternal PKU. Although there was not a lot of published information, we were able to meet with people who had successful PKU pregnancies. We were able to actually ask questions to doctors who had done Kuvan Maternal PKU studies. We were connected with the Maternal PKU Facebook page (a must for any Maternal PKU lady in my opinion). Through that, I was actually able to interact and ask questions with some of those ladies who were otherwise anonymous parts of those studies. Bottom line is we decided the risks of Kuvan were low, and the risks of no Kuvan for me personally were high.