Home » Everything About Phenylketonuria

Don’t Panic, PKU Is Treatable

You became a parent and your baby has been diagnosed with Phenylketonuria. Naturally, you’re wondering: “My God, why this had to happen to me? What shall I do now?” Don’t worry, this is a perfectly normal reaction to any unexpected situation. But good news is that this is a treatable disease and if you stick strictly to a diet prescribed by your doctor, your baby will be all right.

After a diagnosis, the most important thing is to put the baby on a low protein diet as soon as possible. Your pediatrician will give you all the necessary information on the implementation of a diet. But the most important thing he’ll tell you is that your baby mustn’t eat the high protein food! Specially produced food with low protein content is available on the market, as well as a powder supplement whose purpose is to substitute all other amino acids except phenylalanine, which a child can’t get with regular nutrition. Along with amino acids, this supplement also contains all the necessary vitamins and minerals which body needs.

Only a limitted amount of phenylalanine per day can enter a child’s body, so it is important to carefully calculate this amount. You have to be aware that everything you eat have a certain ammount of phenylalanine (Phe) in its content. Phenylalanine is an essential amino acid, meaning it’s a constituent part of every foodstuff. Only the food that is pure carbohydrate, such as sugar or honey, doesn’t have Phe as their component.

Every child is an individual and  it’ s necessary to make an appropriate and personalized diet for each of them. The amount of Phe that a child is allowed to consume can vary from only 100 mg/day, up to 1000 mg/day. This range is so large because children develop different levels of Phe tolerence, along with different side effects which can differ from child to child.

Roughly, there are three groups of PKU:

– classic  – type 1 (max. concentration of PHE >20 mg/dL)
– mild variation of classic PKU – type 2 (max. concentration of PHE 10-20 mg/dL)
– benign – type 3 (max. concentration of PHE <10 mg/dL)

Depending on a group your child belong to, they will be allowed to consume a restricted amount of Phe.

Sometimes it will be hard to stick to a diet and you’ll wish to give your baby something that they aren’t allowed to eat, thinking: “I’ll give it to them just this time, nothing bad will happen.” Don’t do that. By giving your child food they aren’t allowed to eat, you aren’t doing them any favor and you can only cause damage.

If you carefully organize a diet for your child and don’t give them the high protein food, he or she will grow into a strong and healthy person who will only differ from other children by the food they eat.

Remember, this is not a terrible, untreatable disease. By taking into account your eating habbits and taking a powder amino acid supplement, a person can live a normal life. Nowadays, this is much easier than twenty years ago. In the beginning, there wasn’t much low protein food you could give to your baby. But over the years, this part of food industry has developed and now you can find a low protein products of all kinds, which can make a life  of your child much easier. Phenylketonuria is a disorder you can normally live with, if you accept it as a way of life.

Want to learn more?


“The PKU Paradox: A Short History of a Genetic Disease” by Diane B. Paul and Jeffrey P. Brosco.

“Robert Guthrie–The Pku Story: Crusade Against Mental Retardation” by Jean Holt Koch.

“Low Protein Cookery for Phenylketonuria” by Virginia E. Schuett.

“Low Protein Food List for PKU” by Virginia E. Schuett.

“Low Protein Bread Machine Baking for PKU” by Virginia E. Schuett.

Share/Bookmark this!


Leave a reply

Add your comment below, or trackback from your own site. You can also subscribe to these comments via RSS.

Be nice. Keep it clean. Stay on topic. No spam.

You can use these tags:
<a href="" title=""> <abbr title=""> <acronym title=""> <b> <blockquote cite=""> <cite> <code> <del datetime=""> <em> <i> <q cite=""> <s> <strike> <strong>

This is a Gravatar-enabled weblog. To get your own globally recognized avatar, please register at Gravatar.


Recent Posts