About PKU
Discover more about the science of PKU, and understand what’s happening to your body.
PKU treatment information
PKU management: controlling blood Phenylalanine (Phe) levels
The mainstay of treatment for Phenylketonuria (PKU) is management of Phenylalanine (Phe) levels in the blood to avoid damage to the brain while maintaining appropriate nutrient intake. Phe is a building block of protein and is found in most food. The goal of management is to maintain blood Phe levels at a low level and within a target range. Talk to your PKU specialist about how best to control Phe according to your lifestyle and stage.
Your dietitian or nutritionist will help devise a special low-Phe diet. Foods that are Phe rich, such as meat, fish, eggs, dairy and nuts must be avoided, a selection of vegetables, fruit and special low-protein breads and pastas being the main allowed diet.
In addition to the Phe-restricted diet, the remaining nutrition needed by people with PKU is provided by special medical food and supplements or substitutes. These are made especially to contain a balance of amino acids (excluding Phe), minerals, vitamins and other nutrients.
Find a clinic or special food and supplement or substitute supplier in our PKU directory
Maintaining a food diary and performing regular blood test to measure blood Phenylalanine (Phe) levels is essential.
| Food | Approximate Phe content (mg) |
|---|---|
| Banana (100g) | 50 |
| Broccoli (30g) | 50 |
| Rice (45g) | 50 |
| Baked potatoes (90g) | 100 |
| Milk (90mL) | 150 |
| Ham (100g) | 500 |
| One egg | 700 |
Some people with PKU may also benefit from Sapropterin Dihydrochloride, (also known as BH4), a prescription drug approved in the European Union, Liechtenstein, Norway, Iceland, Switzerland, the United States and Japan.
The medication functions exactly like BH4, a substance found naturally in the body, which ultimately helps the body break down Phe by making the defective Phenylalanine Hydroxylase (PAH) enzyme more active.
Owing to the many different defects possible in the PAH enzyme, not all people with PKU will be helped. Ask your PKU specialist for advice.
In summary, the full range of treatment options includes:
- Phenylalanine (Phe) free food.
- Supplements or substitutes (including LNAA).
- Medication.
Ask your PKU specialist for a personalised treatment management program.
Also see The science of PKU, for a medical explanation of PKU, and Establish your blood Phenylalanine (Phe) range to find out about how to establish your individual blood Phe targets and learn about levels of PKU.
A commitment for managing Phenylalanine (Phe) levels for life
To get the best out of life and protect your brain, Phenylketonuria (PKU) specialists generally agree that people with PKU should manage Phenylalanine (Phe) levels not only during childhood but also throughout life. Your PKU specialist – assisted by your dietitian or nutritionist - can help design a personalised treatment management program.
This treatment program will help you keep your blood Phe levels under control for a better life. But the important thing is to stay on track. If you’ve strayed from your treatment goals you may need to spend time with your PKU specialist to review your treatment management. Find out more in this section about PKU management and the scientific advances that have brought positive changes to people with PKU.
