I’m a Dietitian as many of you may know. I work in quite a specialist area called Inherited Metabolic Disorders which are basically genetic conditions where a patient cannot process a specific component of food.One condition we look after is called Phenylketonuria or PKU.
This is where a person cannot break down one of the amino acids found in protein – phenylalanine. Think of protein looking like lots of beads on a chain. Each bead is a different amino acid. Remember when your 5 day old baby had their heel pricked and you watched in horror? They were screening for PKU. The only treatment currently in the UK, if diagnosed. is diet. If not followed as advised, a baby’s brain and development can be severely affected. A massive shock for any new parent and lots of support is always needed.
Diet is a low protein diet. Protein is everywhere. Places you never thought. Meat, fish, eggs, nuts, beans and dairy are the obvious choices and must be avoided. However, pasta, rice, bread, cereals and even vegetarian products are still very high. It is found in the sweetener aspartame, so many diet drinks and chewing gums have to be avoided. So what do people do? They have special drinks with all the essential amino acids, except phenylalanine and their vitamins and minerals. This provides their protein. They have special low protein foods on prescription; bread, pasta, rice, cereals, biscuits, flour, milks and meat alternatives.They eat naturally low protein foods such as fruit and vegetables, sugars and fats and oils. Depending on their severity they may have some medium protein foods in weighed amounts; rice, cereals, potato and snack foods like crisps or cereal bars.
|Low protein shop|
In my role I look after adults. Some are still very strict with diet, some are off diet and some are in between. The brain has fully developed by adulthood and people used to be taken off diet but now diet for life is recommended. It is a very strict regime to follow and is very challenging, especially for anyone going back to diet after a long break. Imagine going out to eat low protein. What would you have in a pub? Veggie options have cheese – not great. Most foods are all meaty. Imagine explaining your diet to others? If you were going to a wedding or party. A lot of planning, organisation and support is needed.
It is International PKU day on 28th June and my work colleague and I are following the PKU diet for a week; 7 days and 7 grams of protein a day. That’s one egg in total per day if you want an equivalent. Hard stuff!
It will be really interesting to see what our patients do on a daily basis. How they eat out, cook different meals to other family members and keep themselves full.
Check back next week to see how I got on.