Interview with a PKU teenager
Phenylketonuria (PKU) is a rare inherited condition in which there is a build up of phenylalanine in the body. Phenylalanine is a natural substance; it is a building block of protein. PKU is looked for in all newborns in the United Kingdom by measuring phenylalanine levels in the heel-prick blood test. All babies should have this test as it allows treatment to start early in life.
PKU is a treatable condition. Phenylalanine is found in the protein part of the food we eat. The treatment is a low protein diet. This means that high protein foods such as meat, cheese, poultry, eggs and milk are not permitted. Instead the diet is supplemented with artificial protein which contains no phenylalanine. This diet is very effective at lowering levels of phenylalanine and allows normal growth and development of the child.
The Child with PKU - this gives an excellent introduction to phenylketonuria. Although aimed at the parents of newly diagnosed children with PKU, it describes the condition, it's diagnosis and treatment, and gives an overview of the genetic implications of PKU. As such it is a useful reference source for students.
Food lists of Phenylalanine Exchanges - one of the prime services that the NSPKU provides to PKU clinics across the UK is the production and maintenance of lists giving the weight of food that contains 50mg of phenylalanine (equivalent of one "exchange"). The lists are regularly updated and are routinely used by the vast majority of PKU people in the UK and their families.
Management of PKU - a document produced with a consensus of opinion from the leading medical authorities on PKU in the UK. External links
Technical Description of PKU - this is a very detailed document produced in the USA. It would be suitable for students undertaking advanced research into PKU. It contains many references to other information sources
Molly has had PKU from birth and it will always be with her. For day to day living it means a very restrictive diet. Eggs, dairy, breads, pasta, rice, meat, and many many other food items are all to high in protein to have. It means no ice cream on the beach, no popping into a fast food place, no just picking at food, no restaurants unless you want just vegetables.
This interview is from a 15 year olds point of view.
Interview with Molly, aged 15.
WHAT IS PKU ?
It means your not born with something that breaks down protein. So you need to measure out your daily allowance of protein.
SO WHAT WILL HAPPEN IF YOU HAVE PROTEIN ?
If you have too much you can get brain damage, the protein builds up inside of you so you will have affects from that.
SO, WHAT FOODS ARE YOU DENIED BY HAVING PKU ?
milk, bread, cheese, eggs, flour, meat, most things have protein
WHAT FOOD THAT YOUR NOT ALLOWED TO HAVE, WOULD YOU LIKE TO HAVE ?
Eggs and real flour, cakes with real flour taste much better.
WHAT IS WRONG WITH PKU FLOUR?
It has a different taste, the cakes made from protein free flour are not as good as shop bought normal flour cakes.
TELL ME ABOUT A DAY IN THE LIFE SOMEONE WITH PKU.?
You wake up and 1st thing I do is have one of my supplements, then work out what I want for breakfast and work out how many exchanges it will be. At this time I make my packed lunch, again it's all worked out as I cant have school dinners, I've never had a school dinner. Get home from school and have tea, again, its carefully planned and weighed out. I cant just eat what I want, when I want.
SUPPLEMENTS, HOW MANY AND WHAT ARE THEY LIKE.?
I am on 3 ( cooler 20's) they have various flavours and some people like the berry flavour but I think they are disgusting so I have the normal non flavoured one.
DO YOU FIND TAKING THE 3 SUPPLEMENTS A DAY A CHORE ?
Sometimes, depending on where you are and what your doing but you just get used to having them.
WHAT FOODS THAT HAVE PROTEIN, HAVE YOU TASTED BUT IT TASTES DISGUSTING TO YOU ?
Normal cheese & milk, I drink pro zero milk, always have, so your so called normal milk is disgusting
HOW DOES PKU AFFECT YOU WITH FRIENDS ?
Parties, you cant eat what everyone else is eating, you have to take your own food, then its face all the questions as to why I'm bringing my own food, you have to then explain, its annoying.
WHATS SO ANNOYING ABOUT IT ?
Just being asked about it, having to explain the why's of it, just not being able to be normal.
SO DO YOU CONSIDER YOURSELF NOT "NORMAL"?
Yeah, when your with people who are eating the "normal" everyday day food, I'm the odd one out.
GIVE AN EXAMPLE OF WHAT THEY WOULD EAT AND WHAT YOU EAT ?
Regular party food, buffet stuff, cocktail sausages, crisps, if I wanted them I'd have to weigh some out , others can just pick at it as and when they want.
DOES THAT MAKE YOU SAD ?
Its annoying and does make me sad at times
IF SOME HIGHER POWER SAID TO YOU " MOLLY I'LL TAKE AWAY YOUR PKU SO YOU NO LONGER HAVE IT, BUT FOR YOU NOT TO HAVE IT I'D HAVE TO GIVEBIT TO SOMEONE ELSE, WHO HASNT GOT PKU " WOULD YOU GO FOR THAT DEAL.?
Probably not, I'm used to having it, its easier for me to keep than giving it to someone who hasnt got it, they wouldn't know what their doing and would have to learn it all.
WHAT IF IT WAS A NEW BORN BABY ?
Honestly yes then, because they would know no difference. having PKU is annoying not being able to eat what you want and not being able to even try foods because they are so high in protein.
WHAT FOODS WOULD YOU LIKE TO TRY THAT YOU HAVENT ?
It's not so much what I havent tried, it's like going out for a meal with your friends and not worrying about what I can and cant have.
SO DO YOU THINK PKU IMPACTS ON YOUR SOCIAL LIFE.?
yes.
HAVE YOU HAD TO SAY NO, BECAUSE OF PKU ?
Not recently but definitely when younger, like not going to parties or someones house because you have to explain to them and their parents all about the diet.
DO YOU HOLD A GRUDGE ABOUT HAVING PKU ?
What's the point, you just have to get on with it.
WHAT DO YOU HOPE FOR IN THE FUTURE ?
Kuvan, a drug which would help me tolerate more protein, I can then have a more varied diet.
HOW MANY EXCHANGES ARE YOU ON ?
16. Which I know is more than lots of others, but there was a time I was on far less, it would be a bigger struggle now if I was only on 3 or 4 exchanges.
WHAT IS THE SINGLE WORSE THING ABOUT HAVING PKU ?
Not being able to eat what you want, when you want to.
DO YOU THINK IN ANY WAY THAT HAVING PKU HAS HELPED YOU.?
No.
DO YOU THINK THAT WHEN YOU LEAVE HOME YOU WILL CONTINUE WITH YOUR STRICT DIET ?
I'd have to if I dont want to be affected in a bad way.
HAVE YOU READ ABOUT PEOPLE NOT ON DIET ?
Mostly about people who are pregnant and how important it is for them to be on diet.
HOW OFTEN HAVE YOU SNEAKED FOOD AND WHEN WAS THE LAST TIME ?
A few times, probably last year sometime, cant even remember what it was.
WHY DO OR DID YOU FEEL THE NEED TO SNEAK FOOD ?
You know it's there, you know you cant have it, its tempting when hungry. If I could eat whatever whenever there would not be any temptation.
HOW DO YOU THINK THE PEOPLE AROUND YOU DEAL WITH YOUR PKU ?
Always trying to find new foods, searching shops daily, trying to make new things for me to eat. Its nice.
WHATS BEEN THE BEST MOMENT IN YOUR LIFE CONCERNING PKU ?
My 15th birthday, going to pizza hut for the 1st time. Going out with family and a friend and eating from the menu.
DO YOU SIT DOWN AT DINNER AND BEGRUDGED OTHERS AT THE TABLE FOR EATING SO CALLED NORMAL FOOD ?
Not really, you sometimes wish you could eat the same but it's not often I think that. We eat different things virtually everyday, it's normal.
DO YOU THINK OTHERS TAKE YOUR PKU INTO CONSIDERATION WHEN EATING.?
Yes, when I go to other people's houses or sleepover at a friends I think they may not grab the chocolate or whatever it while I'm there, because they know I cant have it also.
YOUR PRESCRIPTION FOODS, WHATS THE BEST AND WORSE FOODS ?
Something I used to love was strawberry chocolate ( duo bars) but cant get them anymore. The pasta is quite nice and useful and the cake mix is handy. The worse is some of the biscuits and the chocolate is horrible (vitabite) .
ANYTHING YOU'D LIKE TO ADD ABOUT PKU ?
I just want this kuvan to be made available and for it to work for me.
If you would like to know more about PKU, click the link below.
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