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  • Writer's pictureSam Balenzano

Treatment: Kuvan

Although there is no cure for PKU, there are a few treatments for it, one of them being Kuvan. The following is taken from Kuvan's Website:

"People without PKU can break Phe down into tyrosine with an enzyme called PAH and its helper, BH4. People who have PKU were born with PAH that’s missing or not working properly, which makes it hard to break down Phe. This causes a buildup of Phe that can have toxic effects in the body and brain. KUVAN acts like the body’s own BH4 to help break down Phe in people with PAH that is missing or not working properly."

So basically it does the work of breaking down protein that our bodies can't do on their own. Not all people with PKU respond to this treatment though, that's why if you are starting Kuvan, you're required to go on a trial to see if it works. People with Classic PKU (me), usually don't respond.

When I was in high school, I never wanted to try Kuvan, but eventually when I was 20, I decided I could give it a try. PKU blood levels are supposed to be between 2 and 6, and my levels were at 10 and 11, so it was worth a shot to get my levels down.

To everyone's surprise, my levels were down to 2 and 3 within a few days of being on Kuvan! I responded!

Kuvan has been such a blessing in my life because I can eat more of what I already eat, such as another slice of pizza, or an extra slice of toast. But it has also been difficult because it has allowed me to try new foods that I've never had before like mochas, bagels, real pasta, etc, and it's so hard to have a bowl of low protein pasta once you've had a bowl of regular pasta.

My protein intake was at 3-5 grams of protein a day, and now it's at 8-12 grams a day! Not only that, I can think clearer, I can remember things better, my hands don't shake nearly as much and I don't have mood swings nearly as much. People with PKU often talk about living with a fog in their head, but the kuvan lifts the fog and makes everything clearer!

Kuvan is EXPENSIVE, no matter what your dosage is. Mine is $150,000/year. I was covered under my dad's insurance until I was 25, and was afraid I would only be able to choose jobs based on the type of insurance they had to cover my Kuvan, and what kind of company would want to cover someone who needed $150,000/year! However, because I've been responding since I was 20, and I'm a classic, I'm a good advocate for the company and they've decided to support me whenever I don't have a company that will pay for my kuvan so the stress is off!

I take my Kuvan in bed before I fall asleep and I take 12 pills, although I've read that in America they have them in sachets like my formula, so one day I could just mix it in with my formula! That would be a good day. The only side effects I have noticed in myself is that because the pills are so acidic, the top layer of the skin on my tongue has burned off a little bit, and sometimes it's more sore than other times, and I also have a little rash on my lower back that has never gone away since I started it. But it's TOTALLY worth it. If you have PKU and haven't tried Kuvan yet, I would highly recommend giving it a shot! I'm not sure about everywhere but in BC, Canada the trial was free!

These are just my experiences with Kuvan, everyone's experience is a little bit different!

Here's a picture of me the first time I received my Kuvan Trial in the mail:

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