Blood Levels · Living with PKU · Promotion/Awareness · Science

Kuvan – the what and how

In my last post I shared that I have begun Kuvan treatment. Because some of my audience are people that don’t have PKU and are not familiar with Kuvan, I thought I’d take a minute to explain what Kuvan is and how it works.
First, a refresher on PKU I general: in PKU patients, the enzyme hat normally breaks down Phenylalnine, or Phe, in the body is not working properly. There are different genetic mutations that cause PKU, and different PKU patients have different tolerances for Phe. The regular treatment is a low-protein (low-Phe) diet and a formula with protein and nutrients minus the Phe, with the goal of lowering blood Phe to safe levels.
In the body there is a cofactor or coenzyme that helps the PKU enzyme to function. Kuvan is saproterin dichloride, a synthetic form or this cofactor. For treatment, Kuvan is ingested every day to help with metabolizing Phe. There are pills and there is powder. The amount is prescribed based on body weight. I take 11 pills at dinner each day.
I take 11 pills each night at dinner
A person taking Kuvan has to start with a four week trial to see if they respond — meaning Kuvan effectively lowers their blood Phe levels without them changing anything else about their diet. Kuvan works for many, but not all PKU patients.
So I was grateful that I responded and am appreciating the added freedom I have now with eating normal pasta, wheat bread, and a little bit of cheese. We will see how things go as I continue to try new foods and send blood levels in to my clinic.

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