What is PKU?

Phenylketonuria (PKU) is a rare but potentially serious inherited disorder.1 People with PKU can’t break down the amino acid phenylalanine (Phe), which is in all foods containing protein.1,2 If left untreated, and levels of Phe get too high, serious medical problems can occur.2

How is PKU managed?

PKU can be managed with:3-5

  • A very limited diet
  • Specially manufactured low-protein foods
  • Phe-free protein substitutes

Guidelines recommend that the majority of people with PKU undergo treatment for life4,5

What can happen if PKU is poorly managed?7

If patients don’t stick with their PKU diet it could cause:

Problems with concentration; Depression; Anxiety; Tiredness; Mild learning difficulties; Problems with behaviour

Not sticking with the PKU diet is commonly attributed to a lack of palatability.5,8,9 That’s why we’ve developed a range of palatable protein substitutes designed to help people with PKU stick with their diets and stay healthy.

Take a look at our PKU EASY range of products to find out more
Click here


  1. NHS Choices, https://www.nhs.uk/conditions/Phenylketonuria/, accessed 13 June 2018.
  2. PubMed Health, https://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0024675/, accessed 13 June 2018.
  3. The National Society for Phenylketonuria, 2017, http://www.nspku.org/publications/publication/prescription-guidelines, accessed 13 June 2018.
  4. van Wegberg AM, et al. Orphanet J Rare Dis 2017; 12:162.
  5. Hagedorn TS, et al. Orphanet J Rare Dis 2013; 8:191.
  6. van Spronsen FJ, et al. Lancet Diabetes Endocrinol 2017; 5(9):743-756.
  7. The National Society for Phenylketonuria, 2017, http://www.nspku.org/sites/default/files/publications/NSPKU_HoC2017_Event_Newsletter_ A4_4pp_3.pdf, accessed 13 June 2018
  8. MacLeod AL and Ney DM. Ann Nestlé 2010; 68:58-69.
  9. European Society for Phenylketonuria and Allied Disorders, PKU: Closing the Gaps in Care. An ESPKU benchmark report on the management of phenylketonuria within EU healthcare economies, https://www.espku.org/ projects/benchmark-report, accessed online 13 June 2018