We are pretty sure if you are on this site, you know exactly what PKU is. Many of you are either living with PKU, raising a child with PKU or are interested because you know someone with PKU. If you are living with PKU you don’t need us to explain what it is. If your child has PKU, we hope that Cook for Love will become a tool to help you cope with PKU…and you WILL cope. If you know someone with PKU, thank you for being a good friend/family member. Being on a special diet is especially hard in social situations — and being aware of that difficulty (and ideally making some accommodations in your menu) will help to make things a little easier for those that live with these restrictions every day.
There are many sites that give wonderful descriptions of PKU. We’ve posted some of the links in our resources section. We’ll provide just a brief technical overview here.
PKU (phenylketonuria) is a rare inherited metabolic disorder that, if untreated, can cause mental retardation and other neurological problems. PKU affects approximately 1 in 12,000 people. There is no outward sign of a problem until it is too late and brain damage has occurred. Fortunately, newborn screening allows for early diagnosis and treatment. If detected at birth and a diet is maintained, people with PKU can live normal lives.
Individuals with PKU cannot break down one amino acid in protein called phenylalanine (phe) due to missing or insufficient liver enzyme activity (phenylalanine hydroxylase or PAH). Without treatment, phenylalanine builds up in the bloodstream and is toxic to the brain.
As a result, people with PKU must follow a very restricted low protein diet. This does not involve something as simple as being on a vegetarian diet. Protein — and therefore phenylalanine — is found in all foods except water, sugar, oil, vinegar and pure starches. Grains, beans, dairy, nuts, and soy are too high in phe and therefore off limits. Fruits and vegetables are allowed in the PKU diet, but only in measured amounts. As grains are usually too high in phe, specially-made pasta, rice and cereals are needed. Baked goods (including breads) are made with fragile starches instead.
In order to obtain the nutrients that their bodies need, people with PKU must consume synthetic phenylalanine-free protein drinks throughout the day. The formula and special foods are very expensive and often unpalatable. In addition, frequent blood monitoring is required to make sure the blood phenylalanine levels are maintained in good control.
So What Does This Really Mean?
Food takes center stage in almost all social events. It is something that most of us are lucky enough to just take for granted. When we are hungry, we can go grab a bite to eat anywhere, depending on our mood.
Imagine that not being the case.
Imagine having to plan and account for all the food that you eat. Imagine all spontaneity being taken away. Imagine watching everyone else partake in an activity on a daily basis and always feeling excluded. Imagine just wanting to fit in.
Imagine the toddler at pre-school snack time. The little goldfish crackers are being passed out and she wants desperately to have a taste. Instead she has to say “No thank you.” Imagine how the little boy feels watching all of his friends enjoy an ice cream cone on a hot day after a softball game. He wants desperately to have a lick but has to say “No thank you.”
Imagine these children grown a little. They’ve stayed after school a little later than expected and all of their friends decide to go for pizza. They want desperately to hang out but have to say “No thank you.”
Now imagine these kids have grown and gone to college. There’s no late night pizza delivery while they’re cramming for exams. Their nights out on the town end at the diner, and they have French fries as their friends devour burgers and milkshakes.
Finally they’ve reached adulthood. Imagine him finally saving enough money for his dream vacation to Italy but he cannot enjoy the food. Imagine her joy at finding out she is pregnant being overshadowed by her fear that something might go wrong. If she eats the wrong food, she’s putting her unborn baby at risk for mental retardation.
Imagine the will power needed for all of these scenarios. If you have PKU, you don’t have to use your imagination – this is your reality. And at certain times this has been our children’s reality, as we attempt to understand the challenges they face being on such a restricted diet.
The PKU diet is complicated: it requires not only severe dietary restrictions, but careful measurement, calculation and record keeping. As a result, more time is needed for meal planning and food preparation – and all too often these efforts are met with less-than-great results.
As a parent, imagine the frustration of just trying to feed your child, and failing to meet that most basic need. Imagine the exasperation when you have spent countless hours making dinner and your child simply will not eat what you have prepared. Imagine how intense the pressure is when the threat of mental retardation is looming over your child’s beautiful little head.
Poor dietary compliance – whether resulting from misunderstanding, unpalatable food options, lack of variety and/or social pressure – is a regularly-encountered problem in the PKU community. Given the dire consequences, everything must be done to provide people with the necessary tools to stay on diet for life.
Cook For Love’s Role
Now imagine providing a snack for the preschool class that no one knows is made for a special diet -they just know it is delicious! Imagine low protein ice cream that you and your friends can enjoy. Imagine penne a la vodka that would impress Mario Batali. Imagine pizza nights that you look forward to. Imagine delicious low protein crackers stashed on your bedside table to ward off morning sickness.
This is what we imagined and now it is becoming a reality. Cook for Love has these and many other wonderful recipes. Check them out. We promise they will impress everyone from the pickiest toddler (Molly at 3yrs) to the finicky pre-teen (Liam at 12yrs), and perhaps the most difficult of all, the disillusioned cook (us).
As parents, there is nothing we want more than to take away the difficulties our children will face having PKU. That is something we cannot do, but we can make it a little easier. Improving the taste of low protein food is our way of trying to help.