The treatment

The medicinal treatment: Orfadin®

Patients must follow a very strict diet low in animal and vegetable proteins called a hypo proteinic diet.

In addition patients must take the Orfadin treatment. This treatment prevents an enzym to produce substances responsibles for the Tyrosinemia and it avoids as well their accumulation.

Despite this treatment, the tyrosin rate is still present in the blood. 

This is why a dietary treatment low in tyrosin and phenylalanine must be obligatory associated to this medicinal treatment.

Orfadin® is prescribed at the beginning when the diagnosis is established, associated with an hypo proteinic diet low in natural proteins it helps to recover quickly from hepatitis.

The dietary treatment: the hypo proteinic diet

Hypo proteinic diet is also a treatment for patients. In other words patients must follow a strict dietary diet low in tyrosin and phenylalanine which are contained into vegetable and animal proteins.

A hypo proteinic diet doesn’t contain :

  • meat
  • fish
  • eggs
  • bread or cakes
  • dairy products
  • cheese
  • tofu

or any derivatives.

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Patients must also control the level of proteins content in food. It determines and makes sure that the number of protein portions authorized per day are respected. These portions depend on the patient’s weight and age and on his medical results.

In addition to this hypo proteinic diet, patients are constrained to a daily treatment:

  • To take synthetic amino acids twice a day. The amino acids are naturally contained in animal and vegetable proteins. Yet, as the patient follows a hypo proteinic diet he must take substitutes in order to keep his organism developing well.
  • To take a nitinisone-based medicine called Orfadin (for the patients suffering from the Tyrosinemia type 1).

Patients must do regular medical exams in hospitals with specific structures for rare diseases.

In France, the Filiere G2M (a group for metabolism diseases) has ranked the different treatment centers and the specialists for Tyrosinemia. There is 1 coordinator’s site, 7 constitutive’s sites and 19 centers of expertise in the country (cf. map below).