How is Wilson disease treated?
Lifelong penicillamine chelation therapy is the preferred choice because it can prevent or improve virtually every manifestation of Wilson disease. Side effects are common (30%). Other treatment options include trientine (chelator) and zinc salts (bind copper in the gut). Foods rich in copper such as organ meats, nuts, chocolate, and mushrooms need to be avoided. With early, effective chelation, most patients can live normal, healthy lives. Patients presenting with fulminant hepatic failure do not respond well to chelation and require urgent liver transplantation.
