Maternal PKU: Weeks 1-8

This is part of 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)

~Positive Test!~
The entire time my husband and I were trying to get pregnant, we sent in blood levels every other week. Then one Saturday in early December, we saw a positive test. We were officially having a baby! After the joy and excitement, I started to freak out a little. Thanksgiving was only a week earlier and I was fairly certain I had done some "cheating". We took a level the day after we found out I was pregnant, and it was 2.5 mg/dl. I was safe (granted, I didn't know that until almost two weeks later because the results took forever to get back!).

First thing Monday morning, I e-mailed my geneticist and called my OBGYN.

The geneticist was apparently out of the country when I tried to contact her, so it was over a week before I heard back. As soon as I did, she scheduled an appointment with me the next day (at about week 5). At the appointment, we went over my levels, she requested weekly levels instead of very other week, she ordered another Amino Acid panel, and we discussed having follow up appointments every other month. We also discuss morning sickness and the important of keeping my formula down. At this point, I had no issues yet, but she suggested we discuss it with the OBGYN and make him aware.

We had already told my OBGYN about PKU and he had basic information about Maternal PKU. We scheduled an appointment with the OBGYN at what would be 6 weeks. For the most part, it was just a typical appointment anyone pregnant would have. We were already able to hear our babies heartbeat!
He went ahead and prescribed Ondansetron (Zofran) just in case I needed it for morning sickness later on. The only  other PKU-related issue was discussing and confirming that the OBGYN and the geneticist were communicating with each other.

~Morning Sickness~
A week later the Zofran became an essential part of life. Morning sickness is a deceitful name. I was sick all day long. I started taking Zofran and the second it wore off, I would feel sick again.

In hindsight, I learned a few things. First, the prenatal vitamins I was on made me sick. I switched brands a few months later and felt better. Than I switched back to the past vitamin and was sick immediately. I guess I had never noticed before how often I would get sick within about 30 minutes of taking them. It may not be a bad idea to try different brands if you are having problems.
The second thing is that formula almost always made me feel sick and the Kuvan did as well. This made it very difficult to keep food down. I learned to time the formulas well- to be sure to take my formula at least an hour after taking the medicine or an hour before it wore off, or else the formula would be lost! (This continued from Week 7 until Week 19). and I learned to avoid certain foods (tomato sauce on anything for example), and managed to keep formula and food down most of the time even if I still felt ill. It was a long journey!

Levels (mg/dl)

Week 2- 2.1

Week 4- 2.5 (Positive Test)

Week 5- 4.1

Week 6- 7.7 (no idea what happened there?)

Week 7- 5.1 (morning sickness started)

Week 8 - 4.6

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.