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Managing your baby’s special PKU diet

Preparing your baby’s Phe-free formula

Your dietitian or nutritionist will prescribe the exact amount of special Phenylalanine (Phe)-free formula to meet your baby’s requirements. Take care to measure the amount precisely.

Download a full list of steps and tips for preparing your baby’s food.

How to calculate Phenylalanine (Phe) levels in food

Once your baby begins to eat solid food around the age of six months, your dietitian or nutritionist will teach you how to count protein consumed in the diet and will provide a food list, which indicates how much protein common foods contain. This will enable you to keep a record of the different food your child has eaten. The amount of Phenylalanine (Phe) in the diet will be adjusted according to blood Phe levels.

There are various ways to count protein. Some systems measure protein in units and others measure it in grams. In most food, one gram of protein is equal to 50mg of Phenylalanine (Phe).

The nutrition information panel on the food label will list all the nutrients inside. From this, you’ll be able to compare different products and choose the one with the least protein. When in doubt, talk to your dietitian or nutritionist.

When planning a daily menu, match one higher Phe food like cereal or corn with a lower Phe vegetable, like carrots or peaches.

Also see Establish your blood Phe range and Taking blood samples at home

Treatment information for newborn babies with PKU

Learn about the treatment options for your newborn baby

Manage your everyday life with your newborn baby and download special tips.

Receive the latest information about PKU, treatment options and tips on how to manage your family and social life.

Neurotoxic

A substance or agent which is damaging to the brain.

Nutritionist

See dietitian/nutritionist.

PAH (Phenylalanine Hydroxylase)

An enzyme which works together with Tetrahydrobiopterin (BH4) to convert Phenylalanine (Phe) to Tyrosine (Tyr) in the liver. A deficiency in PAH results in high Phenylalanine levels in the blood, which is the biochemical hallmark of Phenylketonuria (PKU).

Phenotype

An observable characteristic or trait of an organism. Phenotypes result from the expression of an organism’s genes as well as the influence of environmental factors.

phenylalanine (Phe) tolerance

The quantity of Phe a patient may consume without a significant increase in her or his target blood Phe levels. Usually expressed as daily intake.

Phenylalanine (Phe)

A building block of protein, and one of the 10 essential amino acids. An inability to metabolize excess phenylalanine is the characteristic feature of Phenylketonuria (PKU). When the blood concentration of Phenylalanine exceeds what is considered a healthy level, excess amounts may build up in the brain, causing mental retardation.

Phenylalanine Hydroxylase

See PAH.

Phenylketonuria

See PKU.

PKU (Phenylketonuria)

An inherited metabolic defect in protein metabolism. In PKU, the Phenylalanine Hydroxylase (PAH) enzyme is unable to convert Phenylalanine to Tyrosine, resulting in a build-up of Phenylalanine (Phe) in the blood that eventually passes into the brain, causing mental retardation and other neurological problems.

Protein

A type of molecule produced by the body from Amino acids.