Isovaleric Acidaemia (IVA) is a rare, inherited disorder of protein metabolism caused by a deficiency in the enzyme isovaleryl-CoA dehydrogenase. In IVA, the body cannot break down the amino acid, leucine, causing a build-up of isovaleric acid, other organic acids and toxic chemicals, including ammonia. The severity of IVA can vary from mild to severe, with some cases presenting in the neonatal stage and others later in childhood.

The current treatment for IVA is a protein restricted diet for life and taking a regular dietary supplement called synthetic protein which contains all amino acids except leucine, in addition to vitamins and minerals. Carnitine and glycine supplementation are also used in the management of IVA to help excrete toxic substances.

During times of illness, patients with IVA must adhere to an individualised emergency plan. This plan aims to prevent catabolism, where the body breaks down its own tissues to release energy.

For exchange lists, manufactured foods list, low protein shopping guides and information for school visit our Low Protein Corner

For a variety of low protein recipes click here

If you would like to use a reward chart to help your child with their eating or taking their synthetic protein then we have a selection here

Printable Resources

Weaning Your Baby on a Diet for IVA

Reading Food Labels for Protein

Low Protein Foods Guide

Fridge Pin Up

Storybook

Izzy and Ian have IVA Storybook-a colourful and informative storybook about Izzy and Ian who have IVA has been developed by Jenny McNulty, Dietitian and Jill Moore, Play Specialist. This book focuses on the importance of the synthetic protein. If you would like a printed copy please contact us directly or ask for a copy at your next out-patient appointment.

Izzy & Ian have IVA

Storybook