Phenylketonuria (PKU)

Phenylketonuria (PKU for short) is a rare inherited metabolic disorder. It is caused by the lack or insufficient level of activity of the PAH enzyme (phenylalanine hydroxylase). The enzyme (PAH) breaks down the amino acid phenylalanine (phe) to tyrosine within the liver. Without normal function there is an excessive accumulation of phe within the body. This has a toxic result, particularly in the brain for a person diagnosed with PKU. PKU is an incurable disease, and a strict low-protein, and low-phe diet, supplemented by a protein substitute drink or use of pharmaceutical products is required. Failure to comply with this guidance can cause severe cognitive impairment, intellectual and mental disability. Therefore, depending on the therapy PKU patients are undergoing, during their travels, they must carry on board and/or checked luggage:

• Special protein substitute formulas (liquid, powder or pills)
• Low protein foods
  
• Pharmaceutical products dedicated to PKU treatment (these might include, according to the personalized treatment: syringe, pills.)