PLEASE NOTE: I am not a medical professional, this is all my own work and do not base your diet off things I say here. This is simply a layman explanation of the diet with some of my own perspective thrown in.
Phenylketonuria (PKU) is a genetic condition. It is passed down through a family and will last for your whole life; those who pass it down don’t always have the condition, and don’t need to be treated. Both parents need to be carrying PKU to pass it down to a child.
PKU is easily treatable. To some new parents or your friends, the restricted diet that many PKUs are put on seems horrifying. Counting your protein every day? No meat, ever? Blood tests???
It’s honestly not that bad. I’ve been on the diet my whole life and it’s just something I do. You are so used to counting the protein and checking the menu for what you can eat that it’s second nature – bring your child up to have it as second nature and that it’s not a big deal, and they won’t treat it like a big deal.
The diet, for a child, involves some foods you mustn’t have: meat, fish, eggs, cheese, nuts, normal bread and cakes; some foods you should only had a weighed portion of, like potatoes, broccoli, baked beans; and some foods you can eat in normal quantities, most vegetables and fruits for example. Your diet should be a mixture of the last two kinds of foods and the weighed portions should correspond to the amount of protein you are allowed in a day.
So, for example, say tonight I’m having potatoes, carrots and broccoli. I need to think about how many grams of protein I’ve eaten already today, and how many I can eat in a day. You dietician should help you with this. Say I’m on 14 grams a day (which I’ve always called exchanges), and say I’ve had 10 already. Tonight, I’d weigh out 160g potatoes (2 exchanges/grams of protein) and 2 of broccoli. Then I couldn’t have any chocolate for pudding, but I could have an apple*. After that, I would have my supplement.
There are a lot of kinds of supplement on the market and really it’s best if a dietician or consultant helps you with how many supplements to drink and when. I drink Lophlex while my sister drinks PKU Coolers but we are both classed as adult PKUs now. For children, there are other ranges available.
The number of exchanges I and any other PKU has a day is determined by their latest blood test. Again, these sound horrible, but once you’re used to them, they’re not so bad. They’re never fun, but it’s not awful. They won’t take much blood, and now they sometimes just prick your finger. The dietician will (eventually) get back the result and will translate this into how many exchanges you should be on a day. When you are little, they are stricter about the amount of phenylalanine that can be in your blood, and take blood more often. Now I’m an adult, my phenylalanine levels can be higher and lower an I have blood tests less frequently.
This is because the main problem with having too much phenylalanine in your system is that it hinders brain development. While your brain is developing, it is crucial that you don’t have too much phenylalanine in your blood. Now my brain has developed, I can have a bit more – they used to take people off the diet at 8 then at 18, but now the recommendation is to remain on it for life (especially for girls). High phenylalanine levels have an effect on mood, coping ability and temper, all of which I’ve noticed in myself. Your concentration span reduces and other effects have been noted.
Girls should remain on the diet simply because if/when they get pregnant, they have to be on the diet or their baby will be, I’m sorry to say, mentally and physically deformed.
I am not looking forward to being pregnant.
*I can’t list all the different foods and their exchange rates here, but these two links to the Exchanges Information Leaflet and the Dietary Information Leaflet should help answer any questions you may have. If you want answers in plainer English from me, please just comment and I’ll do my best to answer!
PKU Cookery 