Jenn, we do not get the National PKU Newsletters (anymore) for personal reasons. In the past my family was very involved with the PKU community because my mother was the president of the PKU Organization of Wisconsin for about eight years and we are still are. We do keep ourselves up to date with PKU related information, especially Kuvan, but we choose not to receive our information from the newsletter (It has nothing to do with Virginia herself). Although, I have read bits and pieces of the articles that you are talking about. To be honest I struggled with the decision to begin Kuvan and so did my parents, so believe me we did as much research on our own as we could and we talked in great depth with our clinic before we choose this route.
The main reason we choose Kuvan was because as a child and into my pre-teen years I struggled A LOT with the diet. I was a completely different person then I am now and everyone who knows me will admit to that. Right before I started high school I finally started taking charge of my diet. I tried very hard to drink my formula and write down what I am, but my levels were still not where they needed to be, so when Kuvan was offered to me we thought it would be best to take Kuvan. If taking Kuvan meant that my levels would consistently be between 2-6mg/dl rather than only half of the time, then that was what we were going to do. I began Kuvan a year ago it was believed that because I have Classical PKU I would not respond, so we didn’t even think I would be on Kuvan today.
With the long-term risks we discovered that BH4 (Kuvan) has been used for over ten years for other disorders in other parts of the world and our geneticist backed up this research. I do recognize that these other disorders are not PKU, but those who have been using BH4 are not growing extra arms and legs . I also trust my life with my PKU staff (quite literally). If they saw even the slightest issue they would have me in the clinic in two seconds. Last Christmas I was seriously considering discontinuing taking Kuvan because controversy that I heard on the Listserv that had me terrified. My doctor and dietitian came in two days before Christmas (the day after we called), during their holiday vacation, to speak with me when my mom told them how freaked out I was. In no way did they push we in either direction, they made it very clear that whether or not I stayed on Kuvan they would always support me. They were very informative and did, and still do, listen to my concerns and they helped me threw those concerns.
Believe me insurance was a huge concern because not only do I have PKU, but I have two sisters who also have PKU and five other siblings. We estimated that without insurance my Kuvan would cost approximately $55,000/year (the average estimated cost is $57,000). Our co-pay is $50 every three months and we were guaranteed that they would continue paying for as long as I take Kuvan. Also, Biomarin has been WONDERFUL throughout my whole ‘Kuvan Journey.’ When we received our co-pay information Biomarin actually contacted our family and told us that if that even that co-pay was too much for our family they would help cover part of it, but we did not need the assistance so that was not an issue. When I am 25 I plan to use the same insurance company that my parents use. They have been using this insurance company since before I was born and even when the company was transferred to another owner they continued.
So yes, all in all taking Kuvan might be a bit risky but…I already knew that. I knew that before I even said yes to Kuvan. I am also well aware that Virginia Schuett is completely against Kuvan, but if I listened to everyone when they were against one of my crazy ideas in life I wouldn’t get myself anywhere LOL. Besides if the day comes that, for some reason, I cannot continue Kuvan I know (and my family members would vouch for this) that I am stubborn enough and have enough will power that I could bring myself back down to 300 mg of phe.