Wilson's Disease

Topics

• What is Wilson's Disease?
• What Causes Wilson's Disease?
• Who is at Risk?
• Symptoms of Wilson's Disease
• Wilson's Disease Diagnosis
• Treatment for Wilson's Disease
• Side Effects from Medications
• Long Term Prognosis
• Suggested Articles

What is the Treatment for Wilson's Disease?

Once a diagnosis of Wilson’s Disease is confirmed, lifelong treatment will be necessary in order to prevent copper from accumulating in the liver again.

Treatment for Wilson’s Disease includes the following:

• Low Copper Diet. Avoiding foods rich in copper will most likely be recommended such as shellfish, nuts, chocolate, mushrooms, dried fruit, liver, dried peas, beans, lentils, avocados, and bran products. Also, eliminating the use of copper pots and pans and storage containers is suggested.
• D-penicillamine. A chelating agent sold under the trade names Cuprimine and Depen, D-penicillamine removes excess copper from the body by binding to the copper and increasing urinary output to rid the body of excess amounts of copper.
• Trientine. Also a chelating agent sold under the trade name Syprine, Trientine works much the same as D-pencillamine by binding to the copper and increasing urinary output to rid the body of excess amounts of copper.
• Zinc acetate. Zinc acetate blocks the absorption of copper and increases copper excretion in the stool. Often used for people who have been successfully treated with chelation agents, zinc acetate is often used to maintain healthy levels of copper in the body and for asymptomatic individuals to prevent progression of the disease.
• Ammonium Tetrathiomolybdate. An agent previously used to treat copper toxicosis (poisoning) in animals, ammonium tetrathiomolybdate is also effective in treating Wilson’s Disease. However, it is still considered an experimental drug, and is not readily available. 
• Liver Transplant. For individuals with cirrhosis, end-stage liver disease and sudden-onset liver failure caused by Wilson’s Disease, a liver transplant may be necessary.

Symptomatic treatment for symptoms of muscle spasm, stiffness, and tremor may include anticholinergics, tizanidine, baclofen, levodopa, or clonazepam (parasympathetic nerve blockers, muscle relaxants, anti-spasmodics, and anti-convulsants).

For individuals who have been diagnosed but are asymptomatic, chelating agents may be administered to reduce the amount of liver damage. Once symptoms are under control, a lower dosage of drugs may be prescribed to control the amount of copper retained by the body.

Wilson's Disease Diagnosis Side Effects from Medications