TreatingPKU

Diet

The main treatment for PKU is a low-protein diet that completely avoids high-protein foods such as meat, eggs and dairy products and controls the intake of many other foods, such as potatoes and cereals.

In addition, people with PKUmust take an amino acid supplement to ensure they're getting all of the nutrients required for normal growth and good health.

There are also a number of specially designed low-protein versions of popular products such as flour, rice and pasta specifically designed for people with PKU and related conditions to incorporate into their diets. Many of these are available on prescription.

If a high phenylalanine level is confirmed,a baby will immediately be started on a low-protein diet andamino acidsupplements. Phenylalanine levels in the blood are regularly monitored by collecting blood from a finger prick onto a special card and sending it to a laboratory.

Your dietitian will draw up a detailed dietary plan for your child that can be revised as your child grows and their needs change.

As long as a person withPKU sticks to alow-protein diet throughout childhood and their phenylalanine levels stay within certain limits, they'll remain well andtheir natural intelligence will be unaffected.

Aspartame

People with PKU also have to avoid food products that contain aspartame, because it's converted into phenylalanine in the body. Aspartame is a sweetener found in:

• sugar substitutes such as artificial sweeteners often used in tea and coffee
• diet versions of fizzy drinks
• chewing gum
• squashes and cordials
• some alcopops

All food products that contain aspartame or a related product should be clearly labelled.

There are alsomedications that contain aspartame, such as some children's cold and flu remedies. It's a legal requirement for any medication that contains aspartame to state it on the patient information leaflet that comes with the medication.

Regular blood tests

Achild with phenylketonuria will need regular blood tests to measure levels of phenylalanine in their blood and assess how well they're responding to treatment. Only a drop of blood is needed, and it can be collected at home and sent to the hospital by post.

You may be able to have training so you can carry out your child's blood tests, or be able to test yourself if you have PKU. This will make testing more convenient.

It's recommended that children who are:

• six months of age or younger should have their blood tested once a week
• between six months and four years of age should have their blood tested once every two weeks
• over four years of age should have their blood tested once a month

Someone with PKU will usually need to have regular blood tests throughout their life.