By Joe Martinec
For parents of children with some inborn errors of metabolism, like phenylketonuria (“PKU”) the daily drama of putting condition-appropriate foods on the table can be a real chore. In 2014 there are (fortunately) a variety of formulas for kids with PKU and vast online food databases with all of the nutrients listed to the 5th decimal point. There are also commercial PKU foodstuffs available, many of which taste … almost natural. In 1982, when my wife, Jen, and I welcomed our daughter Ant, kids with PKU were subjected to chemistry experiments, “formula”, that removed all of the phenylalanine and most of the flavor from edible protein. The resultant products relied heavily on the infant's lack of experience with ordinary food to make the formula seem palatable. The parents were required to suppress their reaction to the odors of the formula, forcing a smile and a hearty “yummm!” to entice the little one to drink up. As a result of my daughter's too frequent snippy-cup-full-of-formula spills, my pickup had a peculiarly rank smell. My friends refused to ride in my truck, the rumor being that I was hauling goats in the cab on the weekend
As my daughter grew older we began buying mail order "low-phe" bread, cookies and pasta. The bread was as devoid of flavor as wheat starch, its principal ingredient, and the texture was gummier than wet Wonder Bread. We began to realize that we would have to take charge of providing food for Ant that was healthy but would not leave her wondering why people claimed that food could actually taste "delicious ", a term rarely used to describe the components of the PKU diet at that time. We all know how adventurous kids are about new food suggestions. Fortunately, Jen has always been good at baking, so she began experimenting with different recipes and created excellent bread and cookies for Ant that even passed muster with Ant's non-PKU friends. While I had always enjoyed cooking, the physics and chemistry I set into play was utterly mysterious to me, and I was profoundly ignorant of the constituent nutrients of the foods I prepared. Yet we knew that keeping Ant "on diet" for a lifetime would require a bit more attention to the food we were preparing, not only for nutrition, but so that the limitations on allowable foods did not turn eating into a burden rather than a joy.
PKU diet regimens usually limit the intake of phenylalanine to a certain number of milligrams per day (referred to as “phes”). In those pre-Google days finding that information for each food was difficult, and you had to have a digital scale. (When we took the 7 year old Ant to Europe, we were certain that the milligram scale and the white powder – her formula - would get us pulled aside at the airport by the DEA.) In addition to careful tracking of phes, we had to boost her caloric intake to meet the needs of a growing child. In addition to fruits and vegetables, she had to eat fried foods and candy. Really. We found ourselves scouring food data bases for foods that would keep it interesting, and Ant and I became experts at preparing vegetable tempura; we were the “Tempura Team”.
A result of all this “food science” was that I developed a better understanding of why certain foods tasted as they did, we tried vegetables that we might not have eaten otherwise-hearts of palm anyone?- and we became more competent in the kitchen generally. I now love to cook and I will try anything, and I probably know more about what I am eating than most. Thanks to PKU.
Joe Martinec is a member of the NGECN Consumer Advisory Group. He represents the Mountain States Genetics Regional Collaborative.
