Tag Archives: PKU

MDD awareness month

MAY is Metabolic Dietary Disorders awareness month and Warrandyte’s own Grand Hotel have announced a very special program — in what is believed to be a world first; they are offering a special menu to cater for people living with this rare group of diseases.

The Warrandyte Diary spoke with the President of the Metabolic Dietary Disorders Association (MDDA) about the most common of this rare disorder Phenylketonuria (PKU) and what this menu initiative means for people living with PKU.

What is it like living with PKU?

Phenylketonuria is considered a rare disease and I guess the significance of this disease is it is one of the things they pick up with the heal prick test.

The heal prick test has been administered for about 50 years and most newborn babies are tested, so PKU is usually diagnosed within the first ten days of life.

My son is seven and he’s got PKU — when he was diagnosed, I had never heard of it before and it came as quite a shock.

However, as a parent you learn — over time —it is a manageable disease as long as you are diagnosed early, are on top of it, and you teach your child as they are growing into a teenager and an adult.

They live a relatively normal healthy life — the biggest challenge is the diet, because it is all about your metabolism.

What are the symptoms and restrictions?

To have PKU means you have got a faulty enzyme in your liver that prevents you from metabolising a particular amino acid which is found in protein — Phenylalanine.

It can’t be metabolised and therefore creates toxic levels in your body which effect the brain — so a young child, if they are not diagnosed early, just simply breastfeeding is getting more  Phenylalanine than they need.

It sends their levels sky high and potentially within 6-12 months, they are brain damaged, so it is all about avoiding brain damage.

When they are young that is when it is most critical, as they get older their brain gets to a point where it is considered to be developed, which they now say doesn’t happen until they are 25, but they advocate diet for life to protect the brain for life so what that means it they have to have a very strict low protein diet.

My son Charlie is allowed to have 12 grams of protein, kids his age with a metabolism his age would probably have about 40 grams, that doesn’t sound too bad because he is a child, but as he gets older that 12 grams won’t change, most adults will have around 70 grams of protein a day.

For example, an egg is six grams or a glass of milk is 10 grams of protein, a potato is two grams, so it is fruit and vegetable as well — everything has protein in it, even nuts legumes soy; we have to avoid all those things too because they are too high in protein, so what we have to counteract this is a specialised synthesised protein, it is like a protein shake — Charlie will have that three times a day and that gives him all of the other protein amino acids his diets is missing out on.

We also have special protein free rice, pasta, milk, cereals, cheese, the whole lot to make up a lot of the staples of the diet and then depending on what their actual allowance is we will incorporate normal foods in to their diet — I am always to the supermarket now reading labels.

It is a very complex diet and because it is not an allergy, or something like diabetes, it doesn’t have that instant effect so it is very hard for people to understand. oIt doesn’t really fit into any category of special diet either, people say, oh you are just vegan — and then they will bring you out a vegan meal with all these sesame seeds or lentils or something.

So what happens is people with PKU often don’t dine out because it is too hard to explain and prepare a meal and so most places you go generally the staple they have is hot chips, but we generally have to weigh them, because one potato is two grams of protein so if someone is going out trying to find a two gram meal for their kid they have to weigh them and they say: Charlie you can have that many — so it is tough.

 The Grand Hotel are doing a special PKU menu, how important is that?

With dining out, a lot of people say it’s just too hard so they take their food everywhere and they often eat the same bland stuff, that is the other sad thing they have their special free pasta they boil it up they put a bit of their free cheese they might sprinkle some herbs on top, you think how much food is a part of our lives and it so bland – even drinking — I mean beer has protein so to even go and have some beers with your mates, to have that social aspect, for families with kids and teenagers and for adults to be able to come out and have that dining experience and just eat some beautiful foods.

What it will mean is that families that are in the area can come and have a dining experience with their kids or as adults and actually be able to order something off a menu without having to explain it — it is just brilliant to be able to eat something and feel like everyone else.