Treating GA1


Children diagnosed with GA1 are referred to a specialist metabolic dietitian and given a low-protein diet. This is tailored to reduce the amount of amino acids your baby receives, especially lysine and tryptophan.

High-protein foods need to be limited, including:

  • meat
  • fish
  • cheese
  • eggs
  • pulses
  • nuts

Your dietitian will provide detailed advice and guidance, as your baby still needs some of these foods for healthy growth and development.

Breastfeeding and baby milk also need to be monitored and measured, as advised by your dietitian. Regular baby milk contains the amino acids that need to be restricted, so a special formula is used instead. This contains all the vitamins, minerals and other amino acids your baby needs.

People with GA1 may need to follow arestricted protein diet for the rest of their life to reduce their risk of a metabolic crisis. As your child gets older, theyll need to learn how to control their diet and stay in contact with a dietitian for advice and monitoring.


Your child will be prescribed a medication called L-carnitine, which helps to clear some of the excess glutaric acid.

L-carnitine is given as a tablet and needs to be taken regularly, as directed by your doctor.

Emergency treatment

If your baby develops an infection, such as a high temperature or cold , their risk of having a metabolic crisis increases. Its possible to reduce the risk by changing to an emergency diet while theyre ill.

Your dietitian will provide detailed instructions, but the aim is to replace milk and food containing protein with special high-sugar drinks. Medication should still be taken as normal.

Your dietitian may provide you with a feeding tube (nasogastric tube) and show you how to use it safely. This can be useful in an emergency if your baby isnt feeding well while they are ill.

If your baby cant keep down their emergency feeds, or develops repeated diarrhoea , you should contact the metabolic team at the hospital to let them know youre heading straight to the accident and emergency (A&E) department .

You should also have been provided with a leaflet to bring with you in case of an emergency, in case the doctors havent seen GA1 before.

Once in hospital, your baby can be monitored and treated with intravenous fluids (given directly into a vein).

You should also take your baby to hospital if they develop symptoms of a metabolic crisis, such as irritability, loss of energy or breathing difficulties.

Content supplied by the NHS Website

Medically Reviewed by a doctor on 21 Dec 2018