Propionic Acidemia (PA) is a rare genetic disorder present from birth. In PA, the body is unable to break down 4 amino acids (methionine, threonine, isoleucine and valine) and certain fatty acids. This causes a build up of propionic acid and other harmful substances in the body and can cause problems.
At present, the treatment for PA is a protein restricted diet for life and taking regular dietary supplements which contain amino acids, vitamins and minerals. Supplementation of L-Carnitine may be recommended. This is a natural substance that helps the body to get rid of harmful substances. Antibiotics taken by mouth may also be recommended to lower the amount of propionic acid made in the intestine by naturally occuring bacteria.
During times of illness the body will break down its own tissue to provide energy (catabolism). This can be dangerous for people with PA as levels of methionine, threonine, isoleucine and valine and other acids will increase quickly. Patients will have an individualised emergency plan to use when they are unwell.
At NCIMD, we have been treating patients with PA for a number of decades and have significant experience in managing this disorder. Patients with PA are managed by the multidisciplinary team and we liaise closely with the Metabolic Laboratory to ensure close monitoring of these patients. We follow international best practise guidelines in managing our patients with PA.
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
Storybook
Penny and Paul have PA Storybook-a colourful and informative storybook about Penny and Paul who have PA 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.