Wilson's Disease

Alternate Names : Hepatolenticular Degeneration

What are the treatments for the disease?

Treatment is started right after the diagnosis is made, even if there are no symptoms. It continues for the rest of the person's life. If treatment is stopped, the symptoms return. Treatment includes avoiding foods rich in copper. Examples of such foods are dried beans, peas, whole wheat, chocolate, and organ meats.

Medicines are also needed. Penicillamine is usually used to treat Wilson's disease. It binds copper and allows it to be excreted in the urine. Pyridoxine, a component of vitamin B6, is also given. Other medicines such as trientine dihydrochloride and zinc may be used in some cases.

In rare cases, Wilson's disease may cause rapid, severe liver damage. The only option in such cases is a liver transplant.

What are the side effects of the treatments?

All medicines have side effects, such as allergic reactions or stomach upset. Penicillamine may cause a rash, low blood cell counts, or joint pains.

What happens after treatment for the disease?

Someone with Wilson's disease will need lifelong treatment.

How is the disease monitored?

Copper levels in the body are measured to check the effectiveness of treatment. Routine blood and urine tests are also done. Any new or worsening symptoms should be reported to the healthcare provider.