The amino acid phenylalanine is converted to tyrosine in the body, thus the intake of phenylalanine also has to be restricted. To obtain sufficient amounts of all other amino acids, protein substitutes specially designed for hereditary tyrosinemia type 1 patients must be used. All other aspects of nutrition like energy, fat, vitamins, minerals should be adapted to the needs of the child to grow normally. In addition, regular blood tests are needed to monitor the blood levels of tyrosine and phenylalanine.
It's important to make sure that your child gets the right type of protein to help growth and development. So, infants with HT-1 will need to adhere to a special diet that includes specialty formula. As children with HT-1 mature, they must take protein supplements and keep to a special diet. Working with a dietitian can help you develop a diet that works best for your family.
Some foods to avoid include:
There are a lot of little- and no-protein foods that can be eaten, such as low-protein pasta, breads, pizza, fruit, vegetables and sweets. There are also low-protein food options that can be eaten in measured amounts, such as low-protein pasta, rice, cereals, peas, and sweet corn. Your doctor may refer to these foods a "protein exchanges."
Dietary treatment is life-long and requires a great deal of understanding and commitment from those affected, their parents and relatives, and their schools. Professional support and nutritional advice is often available in this respect.
Orfadin is a synthetic reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase indicated for use as an adjunct to dietary restriction of tyrosine and phenylalanine in the treatment of hereditary tyrosinemia type 1 (HT-1).