“I Can Never Let My Guard Down,” Kevin Alexander’s Phenylketonuria Story

Phenylketonuria (PKU) is a rare disease which causes phenylalanine (an amino acid) to build up in the body, leading to serious health complications. Since amino acids are obtained through the dietary intake of protein, those with PKU must maintain a strict diet which is low in protein. Most patients are only able to ingest 10 grams of protein a day. However, a medicine called Kuvan has allowed PKU patients to eat up to 60 grams of protein each day, significantly increasing many patient’s quality of life. Of course, this medication is extremely expensive, leaving some patients without access.

However, a generic form of the drug, created by Par Pharmaceuticals, has just recently received approval by the FDA (May 10, 2019). This drug is called Saprpopterin Dihydrochloride, and it could help improve the quality of life of more PKU patients.

Unfortunately, neither Kuvan or Saprpopterin Dihydrochloride are completely effective.

Kevin Alexander

When you live with a rare or chronic disease it’s often easy to assume that you have everything under control. Perhaps you get in a routine and think to yourself, “I’m good”, and then relax a little bit.

Well, count this as a reminder that I can never let my guard down and must always remain vigilant regarding my PKU treatment. I’ve been on Kuvan for 8 years now, and it has significantly increased my tolerance. For a long time now I’ve understood my tolerance to be 60 grams, and haven’t been diligent with submitting blood samples because “I’m good”. I keep track of everything I eat, I count my grams, I feel fine. So I’m good, right?

Well, I just got the results from my latest blood sample, and my levels were elevated. It looks like I’ve been eating 10 more grams of protein per day than I should have. My tolerance is more like 50 grams.

Which means my diet is going to have to change. My routine is going to have to change. I’m going to have to be diligent and submit blood samples again, and not assume that everything is all good.

If you follow my posts, you’ll know that I always try to stay positive. But I’ll be honest right now. I’m super bummed. It’s just a reminder that I have to remain diligent, day after day, every day… for the rest of my life (or until there is a cure).

And it’s a wake up call that even though Kuvan has been incredible for me, there’s a limit to what it can do. It’s not a license for me to eat whatever I want– not that I eat whatever I want. But ever since, I’ve been consistently eating more than I should have, and that realization reminds me of my limitations.

I’m trying to be positive and remain thankful that this won’t be a severe shift in mindset. Going from 60 grams to 50 grams is a lot easier than going from 20 grams to 10 grams. Still, I’d be lying if I said I wasn’t bummed right now.

The only reason I’m posting this is because of all my fellow PKUers who know exactly what I’m talking about, and understand how I’m feeling right now.

Today is one of those days when it’s a bit tiresome and overwhelming to realize this struggle hasn’t gone away and isn’t going away any time soon.

About the Author, Kevin Alexander: “I am a professional videographer and adult living with Phenylketonuria (PKU). Since 2011 I have been sharing my story online, traveling across the world, and producing videos about PKU. I also host the PKU Life Podcast where I interview people in the PKU community. Even though I love producing videos, my true love is playing music. I have played guitar for over 20 years, and my guitars keep me company after a long day at work!”


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