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.
PA Dietary Handbook
Exchange list 1 gram 2020
Slightly Higher Exchange List 2020 -for those on more than 7 exchanges
Super high exchange list 2020-for those on more than 20 exchanges
Manufactured Food List 2020
Reading food labels for other protein disorders
Weaning your baby on a MMA-PA diet