Wilson's Disease

Treatment

Treatment for Wilson's disease is effective if diagnosis occurs before the onset of life-threatening symptoms. Typically a combination of medications and dietary changes is needed to halt disease progress. If treatment is stopped, the disease can be fatal.

Medications

Medications are used to reduce or eliminate excess copper from the body and prevent it from reaccumulating. Useful medications include:

• Penicillamine
• Trientine
• Ammonium tetrathiomolybdate
• Zinc acetate

Both penicillamine and trientine act by binding copper and increasing urine output, ultimately reducing the copper content in the body. Due to many known side effects from penicillamine, Mayo Clinic doctors favor trientine as a binding agent.

Tetrathiomolybdate has shown to provide similar binding results while also proving useful in alleviating neurologic symptoms.

For long-term care, zinc acetate is preferred. Zinc acts by blocking the absorption of copper in the intestinal tract. A major advantage of zinc therapy is its lack of serious side effects.

Dietary changes

In the first year of treatment people are asked to avoid foods with high levels of copper, such as shellfish and liver. Thereafter, intake of these foods should be limited to once per week. Other copper-rich foods such as chocolate, mushrooms, or nuts, are not commonly restricted. A water purification system is advisable if your water supply is high in copper.

Liver transplantation

If Wilson's disease progresses to the point where the risk of liver failure is high or symptoms involve your central nervous system, then usually a liver transplant is recommended. Mayo Clinic is one of the most experienced centers for liver transplant in the United States.

Read more about Wilson's disease treatment on MayoClinic.com.