Community Discussion Forum That’s PKU life! How do you keep your level down? Reply To: How do you keep your level down?

Avatar of Breanna

It is wonderful to hear that you are responding so well to Kuvan! However, I think it is important to keep in mind that everyone responds differently to Kuvan and unfortunately some people do not respond at all.
Like you, I have been on Kuvan for over a year. My phe tolerance has increased from 300 mg of phe to 1000 mg of phe. Although we have both clearly benefited from Kuvan, I still must remain on a low protein diet in order for my levels to stay in the safe range (2-6mg/dl). Unlike you I have only added a few new foods to my diet. Besides eating regular bread and tortillas I eat the same as I always have, except I do not have to be as strict with what I eat (ie when I go out to eat I have a few more options to chose from).
I am not sure if you are still required to drink your formula, but I know that I and a few other people I know do still drink the metabolic formula even while taking Kuvan. I have decreased the amount of formula I need to take, just a little bit, because I am able to eat more natural protein, but I still have to drink it.
While taking Kuvan, it is also very important to continue monitoring how much phe you are consuming each day. By doing this you will be able to insure that your phe levels stay consistently between 2 and 3 mg/dl like you mentioned in another thread. If you do not monitor how much phe you are eating your phe levels could bounce around. For example if you do not eat a lot of phe one day your level may drop below 2mg/dl, which is not safe. On the other hand if someone else who is on Kuvan does not monitor what they are eating they may eat too much and their phe level could get very high. By consistently eating the same amount of phe you are able to be sure that your phe levels are not going to be low one day and high the next. I hope I am making sense .
Like I said, it is great to hear that Kuvan has given you so many more options. It is just important to remember that not everyone responds the same and many people I have spoken to who are taking Kuvan do still need to remain on a low protein diet (even if it is not as restrictive) to ensure their levels are where they need to be. Even with Kuvan high phe levels are dangerous!
I just thought I would share my side of the story!
Take care!

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