According to wikipedia…PKU (Phenylketonuria) is an autosomal recessive metabolic genetic disordercharacterized by a mutation in the gene for the hepatic enzyme phenylalanine hydroxylase(PAH), rendering it nonfunctional.:541 This enzyme is necessary to metabolize the amino acid phenylalanine (Phe) to the amino acid tyrosine. When PAH activity is reduced, phenylalanine accumulates and is converted into phenylpyruvate (also known as phenylketone), which can be detected in the urine.
Sounds fancy but what does it really mean? It means if Tevin has too much protein it will build up in his blood and fry his brain. However, he also has to have a certain amount in order to maintain proper growth and development. Protein is in nearly every food.
Tevin’s current limit of phenylalanine is 350 mg per day. This is approximately equal to 7 grams of protein. Protein is listed on the back of all foods. Peanut butter has 7 grams of protein in 2 tablespoons. If Tevin was allowed to eat regular food he could have 2 T of peanut butter per day and that would be it for the entire day.
Currently all food or drink that goes into Tevin’s mouth is measured. In order to determine how much protein he is getting we measure everything he eats or drink in grams. There is a book that has the phenylalanine for nearly all foods. It has different brands of each and every major food. Different brands of the same product may have different amounts. We record all of Tevin’s food for the day. We have pages and pages of Tevin’s food.
Most of Tevin’s food is special ordered. There are a couple of sites online where we can order from. Cambrooke foods has a lot of frozen foods that are really convenient. However, they are pricey and must be stored in the freezer. PKU perspectives has a large selection of shelf stable foods but they usually involve a little more prep. Below is Tevin’s current food supply. We are a little lower than normal as he has been at his mother’s for part of the summer.
There are also many recipe books and sites. My favorite is http://www.cookforlove.org/ . A chef started this site after her daughter was diagnosed. She strives to use supplies that are easy to find and are more reasonably priced. However, the recipes often involve many steps and can take all day to make.
We strive to make Tevin’s meals as similar to his siblings as possible. I have learned to shape his foods into things similar to our meals. We have also pushed Tevin hard to eat new and different things. Its been a struggle.
In order to supplement tyrosine Tevin must drink formula. We are very lucky that our insurance covers this formula. I believe it costs over $1000 a month.
The other major part of PKU is measuring the phenylalanine in Tevin’s blood. This means getting a blood test done every 2 weeks to once a month. We have the materials to do this at home. However, by the time we ship it it is sometimes no longer good and we have to redo it. For awhile we were going to the hospital to get it done. When they had to get blood from Tevin’s arm he would scream. The last time we did it that way he cried the entire way home. We are so thankful that Children’s Hospital of Colorado has opened an outpatient lab in the Springs because they will do a finger prick. It really makes our lives so much easier.
PKU is hard. It makes almost any meal related activity difficult. We have to preplan for everything. We have found a lot of support thru different facebook groups. It is nice to have others with similar lives. And its nice to have others to answer questions we may have.