Wilson's Disease

Treatment

Treatment for Wilson's disease is effective if diagnosis occurs before the onset of life-threatening symptoms. The goal of treatment is to remove excessive copper from the body and prevent it from reaccumulating. If treatment is stopped, the disease can be fatal.

Drug Treatment

Drugs used for the initial treatment of Wilson's disease include penicillamine and trientine. These act by chelation or binding of copper, causing increased urinary excretion and rapidly reducing the copper content in the body. Tetrathiomolybdate is another chelating drug that will soon be approved by the FDA for initial treatment of Wilson's disease. Thus far, it seems very effective as the initial treatment for patients with neurologic symptoms. Due to many known side effects from penicillamine, trientine is the only chelating agent currently recommended in Mayo's Wilson's Disease Clinic.

For long-term care, zinc acetate is preferred. Zinc acts by blocking the absorption of copper in the intestinal tract. This action depletes accumulated copper and prevents its re-accumulation. Its effectiveness has been demonstrated in more than 30 years of use overseas. A major advantage of zinc therapy is its lack of serious side effects.

Dietary Therapy

Dietary changes require avoiding certain foods, such as shellfish and liver, which contain high amounts of copper. This is important especially for the first year of treatment and after that, limiting these foods to no more than once per week. Other foods such as chocolate, mushrooms or nuts, which are also rich in copper, are not commonly restricted. A water purification system is advisable if copper content is high in the patient's water supply. Mayo offers a handbook of dietary suggestions for its Wilson's disease patients, which includes a checklist for keeping track of copper content in the daily diet.

Liver Transplantation

In some cases, the condition is diagnosed at the stage of acute liver failure and involvement of the central nervous system. When this happens, there is nearly 100 per cent chance of death unless the patient is treated. A liver transplant is required in most cases. Mayo has performed many liver transplants for Wilson's disease patients and performs more liver transplants than any other medical center in the United States.

Complications

Many complications result from Wilson's disease. Specialists work together so that patients have the best treatment for these related conditions.