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.
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.
Sep. 23, 2011
- Cox DW, et al. Wilson disease. In: Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2010. http://www.mdconsult.com/books/page.do?eid=4-u1.0-B978-1-4160-6189-2..00075-5&isbn=978-1-4160-6189-2&uniqId=270505025-3#4-u1.0-B978-1-4160-6189-2..00075-5. Accessed Aug. 2, 2011.
- Ferri FF. Wilson's disease. In: Ferri FF. Ferri's Clinical Advisor 2011: Instant Diagnosis and Treatment. Philadelphia, Pa.: Mosby Elsevier; 2011. http://www.mdconsult.com/books/about.do?eid=4-u1.0-B978-0-323-05610-6..C2009-0-38600-6--TOP&isbn=978-0-323-05610-6&about=true&uniqId=230100505-53. Accessed Aug. 2, 2011.
- Huster D. Wilson disease. Best Practice & Research Clinical Gastroenterology. 2010;24:531.
- Wilson disease. National Institute for Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/wilson/. Accessed Aug. 2, 2011.
- Kaplan MM. Treatment of Wilson disease. http://www.uptodate.com/home/index.html. Accessed Aug. 2, 2011.
- Kaplan MM. Diagnosis of Wilson disease. http://www.uptodate.com/home/index.html. Accessed Aug. 2, 2011.
- Picco MF (expert opinion). Mayo Clinic, Rochester, Minn. Aug. 11, 2011.
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