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Phenylketonuria management
is lifelong1

What are the options for PKU patients?

In combination with a phenylalanine (Phe)-restricted diet, there are pharmacological options which may help reduce blood Phe levels for patients with PKU.

Dietary therapy

Globally, the approach for phenylketonuria (PKU) management has been a highly phenylalanine (Phe)-restricted diet and medical nutritional therapy supplements.1-3

Protein icon

Nutrient requirements in phenylalanine hydroxylase (PAH) enzyme deficiency

Restriction of natural protein intake and replacement with a protein (amino acid mixture) devoid of Phe1

Formula icon

Medical foods in PAH deficiency

PKU medical food/formula supplements provide protein, calories, and other nutrients and help facilitate compliance with dietary Phe restriction1

Dietary control with PKU icon

Modified low-protein foods1

Modified low-protein foods help provide satiety to patients

Pharmacologic options exist for patients with PKU

In combination with a low-Phe diet, prescription PKU medications are available, which may help reduce blood Phe levels.

Learn more about PKU

Symptoms of uncontrolled Phe levels icon

Do you know the symptoms of uncontrolled Phe levels?

Dietary control with PKU icon

88% of patients with PKU struggle to maintain blood Phe control with a Phe-restricted diet alone4

Stay connected with the latest PKU information

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  1. Vockley J, Andersson HC, Antshel KM, et al; American College of Medical Genetics and Genomics Therapeutic Committee. Phenylalanine hydroxylase deficiency: diagnosis and management guidelines. Genet Med. 2014;16(2):188-200.
  2. van Spronsen FJ, van Wegberg AMJ, Ahring K, et al. Key European guidelines for the diagnosis and management of patients with phenylketonuria. Lancet Diabetes Endocrinol. 2017;5(9):743-756.
  3. Rocha JC, MacDonald A. Dietary intervention in the management of phenylketonuria: current perspectives. Ped Health Med Ther. 2016;7:155-163.
  4. Enns GM, Koch R, Brumm V, Blakely E, Suter R, Jurecki E. Suboptimal outcomes in patients with PKU treated early with diet alone: revisiting the evidence. Mol Genet Metab. 2010;101(2-3):99-109.
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