Tyrosinaemia Type 1

Tyrosinaemia Type 1 is a rare life-threatening genetic disorder present from birth. In Tyrosinaemia Type 1, the body is unable to break down an amino acid called tyrosine. This causes a build up of tyrosine and another chemical called succinylacetone in the liver, kidneys, eyes and brain and can cause problems.

At present, the treatment for Tyrosinaemia Type 1 consists of a medication called nitisinone (NTBC) together with a protein restricted diet for life and taking regular dietary supplements which contain amino acids, vitamins and minerals.

Patients with Tyrosinaemia Type 1 are managed by the multidisciplinary team and we liaise closely with the Metabolic Laboratory to ensure close and frequent monitoring of these patients. We follow international best practise guidelines in managing our patients with Tyrosinaemia Type 1. We collaborate with our international colleagues in the management of our patients with Tyrosinaemia Type 1.

Printable Resources
Tyrosinaemia Dietary Handbook
1 gram Exchange list 2023
Slightly Higher Exchange List 2023-for those on more than 7 exchanges
Super High Exchange List 2023-for those on more than 20 exchanges
Low Protein Manufactured Food List 2024
Reading food labels for Tyrosinaemia
Reward Charts
Weaning your baby on a diet for TYR

Other resources
Tess and Tom have TYR Storybook-a colourful and informative storybook about Tess and Tom who have TYR 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. Tess and Tom have TYR