Isovaleric acidaemia

Isovaleric acidaemia is a rare inherited metabolic disease. People suffering from isovaleric acidosis have insufficient levels of the enzyme that breaks down the amino acid leucine. If left untreated, the accumulation of isovaleric acid occurs which can lead to a range of clinical symptoms from neurological impairment and even death. A low protein diet and drugs such as carnitine and glycine are used to minimise the amount of isovaleric acid in the body. They may need extra supplementation, based on glucose polymer powders during acute illness. People diagnosed with Isovaleric acidaemia therefore must carry on board and/or checked luggage:

• Special protein substitute formulas and/or vitamins (liquid, powder or pills)
• Glucose polymer powders
• Low protein foods
• Pharmaceutical products dedicated to Isovaleric acidaemia treatment