What is tyrosinaemia?
Tyrosinaemia is caused by the reduced activity of an enzyme that breaks down the essential amino acid tyrosine. The substances that build up because of the lack of enzyme are toxic and can cause damage to the liver and kidneys.
How common is tyrosinaemia?
Tyrosinaemia is very rare, affecting 1 in 100,000 births.
How is tyrosinaemia treated?
Tyrosinaemia can be treated using a low protein diet using a 1g protein exchange system (i.e. the weight of food which provides 1g of protein) so that one food can be swapped for another to vary the diet. There are many low protein food alternatives available such as bread, biscuits, all purpose flour for baking, pizza bases and pasta. These products provide the diet with variety and a useful source of energy.
A drug called NTBC is also used in the treatment of tyrosinaemia. An amino acid supplement free of tyrosine and phenylalanine is usually given too. Blood levels of tyrosine and phenylalanine are monitored regularly and dietary treatment is altered on the basis of these levels, in discussion with the dietitian.
The Juvela Low Protein Team are also doing our best to develop low protein recipes to make your diet as varied as possible, so that you are not missing out at mealtimes and snack time!