PKU.com Community Discussion Forum Generation PKU NEW MEDICAL ADVANCEMENT Reply To: NEW MEDICAL ADVANCEMENT

#6806
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BreaMarie91
Member

I don’t know if Phebloc is the same as this, but Kuvan (more scientifically called sapropterin dihydrochloride) was approved by FDA on December 13th. Basically it is a drug that the enzyme PAH work better so that our bodies can break down more Phe (this is the enzyme that is lacking or defiecient in those with PKU). I started Kuvan back in early October and I have gone from 300mg of phe to 880mg of phe. I have been doing weekly levels to figure out what my phe tolerance is with Kuvan and I should know the results from my level from Sunday by today or tomorrow because we mailed our levels in late (we do finger pokes).
I spoke with my doctor and she said that as of right now there have been people who have been on Kuvan for years in places such as Japan. They aren’t showing any side effects, though that is something that taunts me about new medical advances. Also in the past BH4 or Kuvan has been used to treat other disorders. nThe only side effect I had because of Kuvan was a hard time sleeping. At the time I was adding any extra phe that I was being allowed by adding milk to my formula at the end of the day. We figured that my sleeping problems was caused by me consuming 380mg of phe (at the time) right before bed and I was kind of spiking my phe I guess and not spreading it out. Either way we aren’t even 100% sure if that was related to Kuvan or not and it has gone away.
The bummer about Kuvan is that not all people with PKU are responders. I have a friend who has CPKU like me and she is allowed about the same amount of phe as me without Kuvan, but she was not a responder. There are so many different PKU mutations that it is hard to pin point who will or won’t respond without giving Kuvan a try. nBre 16 CPKU

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