Treatment of Wilson Disease

All patients diagnosed with Wilson Disease, irrespective of type and severity of symptoms, will have to receive life-long therapy for their disease.

The treatment goal in Wilson Disease is to reduce and maintain free copper at normal levels and as a result to improve the patients’ symptoms. The drugs available today for the treatment of Wilson Disease are penicillamine and trientine, which are so called copper chelators, binding and reducing the body’s copper levels by increasing urinary copper excretion, and zinc. Zinc reduces the dietary uptake copper in the gut. In the United States, all three agents (i.e., penicillamine, trientine and zinc) are approved, but in the EU only zinc (Wilzin) is approved in all of the EU member states. Access to penicillamine and trientine varies considerably between the European countries.