About PKU

PKU Basics

I am living with a metabolic disorder called Phenylketonuria, or PKU. Unless you studied genetics in college, you probably haven’t heard of it, and that’s okay. Here’s the basic crash course:

  • PKU is a genetic condition in which a person lacks a certain liver enzyme that is needed to break down a harmful part of protein, called phenylalanine (or phe).
  • In regular people, the phe gets broken down and leaves the body, no harm done. But in people with PKU, there is nothing to break down the phe, so it goes straight to the brain and causes all sorts of issues.
  • The only way for us to avoid these issues is to eat a low protein diet (no meat, no poultry, no fish, no beans, very limited dairy, limited grains…) and take a protein supplement (formula) to give us all the protein we need without the harmful phe.

Formula

This is a big enough challenge for everyday life, but traveling takes it to a new level. Toting the formula around is one of the biggest burdens. It is not available in stores or even in pharmacies. A lot of it is in premeasured liquid pouches, which means lots of extra weight and extra hassle going through airport security. Other formulas come in powders that you mix with water right before drinking- a bit conspicuous in addition to tedious, finding a safe place to measure and mix and drink and rinse and repeat. I need to get 60 grams of protein per day from this formula which means I have to bring A LOT of it on vacation with me. Which in turn means VERY careful packing.

Formula

I recently switched formulas and now I take it in the form of teeny tiny pills called MicroTabs. I need six capfuls a day, which comes out to a little less that one whole bottle. Six total bottles give me about a week’s worth. It was the BEST decision I EVER MADE! My other formulas were gritty and heavy and smelly, loaded with calories and always gave me stomach aches. They were difficult to travel with, cumbersome to carry, and overall a pain. These new tabs are much lighter, all I need to take a capful is about two sips of any liquid. One bottle fits into the smallest of my purses, and if it happens to pop open all I need to do is turn the purse upside down to rid it of the tiny white dots- no more throwing bags away because formula leakage has caused stains and stenches in varieties I never thought imaginable!

Microtabs3 Microtabs1

Food & Travel

When it comes to eating, I pick my battles. I eat A LOT of potatoes, be they French fried, home fried, baked, or mashed, when I travel. Sometimes, I have French fries two meals out of the day because they are so easy to find. Salads and vegetable side dishes are usually a safe bet for me as well. Every now and then I eat pasta, though it is actually much higher in protein than potatoes so I do need to be careful there. I’ll also eat a roll or bagel every now and then. There are a lot of waiters and chefs out there willing to accommodate and it never hurts to ask, but always expecting it can lead to disappointment. As such, one thing I am definitely not afraid of is chain restaurants. A few of my travel companions teased me for seeking out a McDonald’s in places like Cairo, Paris, and Prague rather than trying the local cuisine, but when you have as many restrictions as I do it is sometimes more efficient to go someplace where you know you’ll be able to find something safe and filling to eat. For many travelers, trying the local food is part of the experience- for me as a PKUer, that is only the case some of the time, and I’m okay with that. For example, during our time in Switzerland, I had to forgo the famous schnitzel (meat is always a red light for me, just way too much protein) but I got to try three different varieties of the local rosti potatoes.

After a quick explanation of my restrictions, the amazing chefs at a restaurant in Giza, Egypt put together this wonderfully PKU friendly meal for me.

After a quick explanation of my restrictions, the amazing chefs at a restaurant in Giza, Egypt put together this wonderfully PKU friendly meal for me.

Finding Out More

PKU.com | The links on the left-hand side of the main page will help with a basic understanding of what PKU is and how PKU is diagnosed and treated.

National PKU Alliance & European Society for PKU | For the latest news and advocacy efforts.

Cook for Love | Started by a PKU parent, this site gives recipes that are safe for PKUers, including lower phe versions of classics like mac & cheese, brownies, pizza crust, etc.

4 thoughts on “About PKU

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