So, next week I go for a PKU clinic visit. This means that I need to send in a sample of blood beforehand so they can measure my PHE levels and we can discuss them when I come in. I'm kind of just getting back onto my PKU diet full force, so they are working with my protein/PHE allowance right now.
For anyone who doesn't know what PKU is (which is most likely everyone), it is a genetic disorder. Protein is made of amino acids, there are 22 of them. My body can't break down one of those 22, called Phenylalanine, or PHE for short. For some unknown reason, buildup of PHE in the bloodstream is toxic. In babies or anyone whose brain has not fully developed, it will cause mental retardation if the baby is not placed on a low-protein diet immediately after birth. They test for it at birth as a part of Newborn Screening. For older adults, the effects of high PHE are not fully known, but they think neurological problems can occur. They used to allow people to go off diet around age 18, however, it is now recommended that people with PKU stay on the diet for life. The diet consists of extremely low protein intake. No meat, dairy, eggs, poultry, legumes, and very limited grains. Mainly fruits and vegetables and low-protein foods made specially for people with PKU. It also means drinking a supplement, which provides the nutrients that we miss in normal food.
As I said, I am just getting fully back on diet. I was mildly off since age 18. When I say mildly, I mean that I did not take the supplement and I did eat grains, which I am not supposed to, but I never ate meat, dairy, eggs, or any of the other really bad things. I never experienced any of the symptoms that are expected when being off diet (poor concentration and attention span, headaches, etc). So I was lucky. The main reason I'm going back on is because when I get pregnant I need to be EXTREMELY strict because whatever my PHE levels are, the baby's are doubled. So I need to keep them 2-6 mg/dl.
The last sample I sent in my level was a 9, which is actually really good for someone who isn't on diet and not keeping track of their protein daily. After that, my dietician told me as a starting point I should eat 16 grams of protein a day. Let me just say, this is NOT a lot. Pasta, which is one of my favorite foods, is totally out. Luckily they make low-protein pasta which is actually really good. So I've been eating a lot of that and also lots of fruits and veggies and I ordered some vegan cheese as well, which is not bad. Since I have now fully cut out grains, I expect that my level will be better. How much better is hard to say, she may have to lower my allowance again depending on how the level is.
The thing that sucks about all these low-protein foods is they are extremely high in calories. I've been trying so hard to maintain my protein intake but also not go over on my calories. I weighed myself yesterday and I'm now down to 177 from my starting weight of 185, so I'm really excited to hit 10 lbs lost!! As hard as it is to track both my protein and calories everyday, luckily it is becoming more of a routine for me now. The more I do it, the easier it gets. The hardest things to count are usually when we eat out. My PHE book has some like fast food restaurants in it as far as counts but I rarely eat fast food and there's no like actual restaurants so I sometimes have to track down the nutritional information via the internet or sometimes I just estimate.
I'm crossing my fingers for low levels when I go next week. I really want to get between 2-6. 2-10 is actually acceptable but 2-6 is ideal. Hope everyone is having a great week.
