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Treatments and drugs
By Mayo Clinic staff
If you've been diagnosed with Wilson's disease, your doctor may recommend medications to reduce the amount of copper in your body. Once that is achieved, treatment focuses on preventing copper from building up again. When liver damage is severe, a liver transplant may be necessary.
Medications that remove excess copper from your body
Medications called chelating agents prompt your organs to release copper into your bloodstream. The copper is then filtered by your kidneys and released into your urine.
Treatment for people with signs and symptoms of Wilson's disease usually begins with a chelating agent. Once your signs and symptoms are under control, your doctor may recommend a lower dose of medication to maintain a safe level of copper in your body.
Doctors sometimes also recommend chelating agents to people who've been diagnosed with Wilson's disease, but don't have signs and symptoms. For these people, a chelating agent can reduce the risk of liver damage.
Side effects of chelating agents depend on the specific medication:
- Penicillamine (Cuprimine, Depen). Penicillamine can cause serious side effects, including skin problems, bone marrow suppression, worsening of neurological symptoms and birth defects.
- Trientine (Syprine). Trientine works much like penicillamine, but tends to cause fewer side effects. Still, there is a risk that neurological symptoms can worsen when taking trientine, though it's thought to be a lower risk than is penicillamine.
Medication to maintain healthy copper levels
Zinc acetate prevents your body from absorbing copper from the food you eat. Zinc is sometimes used in people who've had successful treatment with a chelating agent. In these people, zinc may help maintain a healthy copper level. Zinc is also used in people who've been diagnosed with Wilson's disease, but don't have any signs or symptoms.
Zinc acetate causes few side effects, but can cause upset stomach.
Liver transplant
For people with severe liver damage, a liver transplant may be necessary. During a liver transplant, a surgeon removes your diseased liver and replaces it with a healthy liver from a donor. Most transplanted livers come from donors who have died. But in some cases a liver can come from a living donor, such as a family member. In that case, the surgeon removes your diseased liver and replaces it with a portion of your family member's liver.
- Cox DW, et al. Wilson disease. In: Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 8th ed. Philadelphia, Pa.: Saunders Elsevier; 2006. http://www.mdconsult.com/das/book/body/153549812-5/0/1389/0.html. Accessed Aug. 11, 2009.
- Roberts EA, et al. Diagnosis and treatment of Wilson disease: An update. Hepatology. 2008;47:2089.
- Wilson disease. National Institute for Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/wilson. Accessed Aug. 11, 2009.
- ATP7B. Genetics Home Reference. http://ghr.nlm.nih.gov/gene=atp7b. Accessed Aug. 11, 2009.
- Copper, Cu (mg) content of selected foods. USDA National Nutrient Database for Standard Reference, Release 21. http://www.nal.usda.gov/fnic/foodcomp/Data/SR21/nutrlist/sr21w312.pdf. Accessed Aug. 18, 2009.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Sept. 14, 2009.
