PKU.com Community Discussion Forum Generation PKU NEW MEDICAL ADVANCEMENT

Viewing 6 posts - 1 through 6 (of 6 total)
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  • #6664
    Avatar of Maddy
    GotPKU
    Member

    hiya, so i have classical PKU and i herd that a new system has come out that enables classical PKU kids to eat twice as much protein. does anyone know the name? my doctor keeps on telling me about it but i dont understand her. HELP?

    #6665
    Avatar of Breanna
    BreaMarie91
    Member

    She might be talking about a drug called Sapropterin. If FDA approves it then it will be called Kuvan. It’s suppose to help your body break down more Phe. I’m in a study for it right now and I have classical PKU. I found out on Monday that it is working for me, after four weeks of being on Kuvan my levels went from 2.5mg/dl to 0.1mg/dl. So my dietitian is having me add 70mg of phe to my diet by mixing milk into my formula. She was pretty surprised that it worked for me because she said in a lot of Classical PKU it either doesn’t work or it doesn’t work as well as it does for people with hyperphe. It also depends on the person, for some people it might triple their phe intake, but for others it might only add like 50mg of phe. nBre

    #6804
    Avatar of Meaghan
    mayrogers13
    Member

    It is called Phenyl Block I believe but it is not for everyone. My PKU physician does not give it out to patients because the long term effects are not known since it was only developed about 10 years ago. Honestly if you are doing wlel on the diet, I wouldn’t bother with it.

    #6806
    Avatar of Breanna
    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

    #7099
    Avatar of christopher
    c_pickle
    Member

    i heard that they have approved

    #7100
    Avatar of Breanna
    BreaMarie91
    Member

    Yeah, it was approved December 13th. I know because the Kuvan study is over now and my parents insurance started covering it for me about a month ago. They only have to pay $50 every three months.

Viewing 6 posts - 1 through 6 (of 6 total)
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