Glutaric Aciduria Type 1 (GA-1)

Glutaric Aciduria Type 1 (GA-1) is a rare life-threatening genetic disorder present from birth. In GA-1, the body is unable to break down 3 amino acids called lysine, hydroxlysine and tryptophan, which then builds up in the body and can cause problems.

At present, the treatment for GA-1 involves 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 thats helps the body get rid of harmful substances.

During times of illness, the body will break down its own tissue  to provide energy (catabolism). This can be dangerous for people with GA-1 as levels of glutaric acid and other harmful substances will increase quickly. Patients will have an individualised emergency plan to use when they are unwell.

Many countries screen newborns for this condition and we are hoping to add GA-1 to our National Newborn Screening Programme in the near future. At NCIMD, we have been treating patients with GA-1 for a number of decades and have significant experience of managing this disorder. Patients with GA-1 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 and we have also presented our experience of management of GA-1 at various international conferences.

For patient stories click here

Printable Resources
GA1 Dietary Handbook
GA1 Pharmacy letter 2017
1g Exchange list 2017
Slightly higher exchange list 2017-for those on more than 7 exchanges
Super high exchange list 2017-fore those on more than 20 exchanges
Low Protein Manufactured Food List 2017
Reading food labels for other protein disorders
Reward Charts
Weaning your baby on a GA1 diet

Other resources
George and Gina have GA-1 Storybook-a colourful and informative storybook about George and Gina who have GA-1 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 copy please contact us directly or ask for a copy at your next out-patient appointment. George and Gina have GA 1