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Getting back on track

Remember that food is a part of life for all of us. A new diet means changes to long-held personal associations and new eating patterns can feel like a loss. Give yourself the best chance to make your treatment work and go easy on yourself.

Always have low-Phenylalanine (Phe) food within easy reach for snacks.
Plan ahead. Divide freshly prepared food into appropriate portion sizes. Store individual servings in plastic containers.
Freeze portions for quick, microwaveable meals during the week.
Don’t forget breakfast. Pre-calculate your favourite cereal, then store it in plastic sandwich bags for quick breakfasts throughout the week.
Make sure you’re taking your special supplement or substitute, which contains the nutrients your low-protein diet lacks.
Prevent excessive weight gain by being active and doing exercise.
Choose foods low in salt.
Drink plenty of water and take alcohol in moderation.

See PKU treatment information for adults and refer to your PKU specialist for a personalised treatment plan.

Guide for adults with PKU

Best practices guide for adults with PKU

PKU specialist, dietitian -or nutritionist, a supplier, or PKU association in your area.

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.

Neurotoxic

A substance or agent which is damaging to the brain.

Nutritionist

See dietitian/nutritionist.