A PKU Family


March Dan and Michelle 004

My husband, Daniel, has Phenylketonuria also known as PKU. It is a rare inherited genetic disorder where the amino acid phenylalanine (also called PHE) cannot be broken down. This causes a build up of phenylalanine which is bad since too much of it can cause brain damage, behavioral problems, and developmental delays. If diagnosed early (babies are tested before they go home from the hospital Рremember the heel prick test?) , a person with PKU can live a normal life by maintaining a low protein/low phe diet and drinking a specially made formula. My husband has been on the diet all of his life. Being strictly on diet he is now one of many PKU adults that have shown what PKUers can do!  He has a PhD in Physics and minor in mathematics.

In order for a baby to have PKU, he/she has to get both PKU genes from their parents, even if one or both parents don’t have it. With two gene carrier parents the chances are 1 in 4 that their offspring will have PKU. My husband is the only one in his family that has it.

As I said before, Daniel has been on diet all of his life and thus is faithful to eating low. He doesn’t crave foods that would be dangerous to him. A low protein diet consists of vegetables, fruits, and specially made low protein pastas and bakery items. No meats, dairy, nuts, beans or grains.

PKU does have its challenges. Date nights usually consist of looking at menus before we go out to see if a place we want to go to has food for him or we go to the old standbys that we know he can do (Sweet Tomatoes/Soup Plantation is great for PKUers). It also helps having restaurants that are able to do custom food orders for those with dietary restrictions. At home, we do a lot of made from-scratch cooking and baking. I came from a family that LOVES food and cooking so this comes in handy especially when a lot of PKU foods requires basic knowledge of the kitchen. Part of my blogging is going to be on PKU friendly recipes, including adapting recipes for the PKU diet.