How do doctors treat Wilson disease?

Doctors treat Wilson disease with

  • medicines that remove copper from the body, called chelating agents
  • zinc, which prevents the intestines from absorbing copper

In many cases, treatment can improve or prevent symptoms and organ damage. Doctors may also recommend changing your diet to avoid foods that are high in copper.

People who have Wilson disease need lifelong treatment. Stopping treatment may cause acute liver failure. Doctors regularly perform blood and urine tests to check how the treatment is working.

Chelating agents

Penicillamine (Cupramine, Depen) and trientine (Syprine) are two chelating agents used to treat Wilson disease. These medicines remove copper from the body.

Penicillamine is more likely to cause side effects than trientine. Side effects of penicillamine may include fever, rash, kidney problems, or bone marrow problems. Penicillamine may also reduce the activity of vitamin B6, and doctors may recommend taking a vitamin B6 supplement along with penicillamine. In some cases, when people with nervous system symptoms begin taking chelating agents, their symptoms get worse.

When treatment begins, doctors gradually increase the dose of chelating agents. People take higher doses of chelating agents until the extra copper in the body has been removed. When Wilson disease symptoms have improved and tests show that copper is at safe levels, doctors may prescribe lower doses of chelating agents as maintenance treatment. Lifelong maintenance treatment prevents copper from building up again.

Chelating agents may interfere with wound healing, and doctors may prescribe a lower dose of chelating agents for people who are planning to have surgery.

Zinc

Zinc prevents the intestines from absorbing copper. Doctors may prescribe zinc as a maintenance treatment, after chelating agents have removed extra copper from the body. Doctors may also prescribe zinc for people who have Wilson disease but do not yet have symptoms. The most common side effect of zinc is stomach upset.

How do doctors treat Wilson disease in women who are pregnant?

Pregnant women should continue treatment for Wilson disease throughout pregnancy. Doctors may prescribe a lower dose of chelating agents for women who are pregnant. Since the fetus needs a small amount of copper, lowering the dose may keep copper at safe levels without removing too much copper.

In most cases, doctors recommend that women continue to take the full dose of zinc during pregnancy. Experts recommend that women with Wilson disease do not breastfeed if they are taking chelating agents. Penicillamine is present in breast milk and can be harmful to a baby. Experts have little information about the safety of trientine and zinc in breast milk.

What are the complications of Wilson disease?

Wilson disease may lead to complications, but early diagnosis and treatment can lower your chances of developing them.

Acute liver failure

Wilson disease can cause acute liver failure, a condition in which your liver fails rapidly without warning. About 5 percent of people with Wilson disease have acute liver failure when they are first diagnosed.5 Acute liver failure most often requires a liver transplant.

Acute kidney failure and a type of anemia called hemolytic anemia often occur in people who have acute liver failure due to Wilson disease.

Cirrhosis

In cirrhosis, scar tissue replaces healthy liver tissue and prevents your liver from working normally. Scar tissue also partly blocks the flow of blood through the liver. As cirrhosis gets worse, the liver begins to fail.

Among people who are diagnosed with Wilson disease, 35 to 45 percent already have cirrhosis at the time of diagnosis.6

Cirrhosis increases your chance of getting liver cancer . However, doctors have found that liver cancer is less common in people who have cirrhosis due to Wilson disease than in people who have cirrhosis due to other causes.

Liver failure

Cirrhosis may eventually lead to liver failure. With liver failure, your liver is badly damaged and stops working. Liver failure is also called end-stage liver disease. This condition may require a liver transplant.

How do doctors treat the complications of Wilson disease?

If Wilson disease leads to cirrhosis, doctors can treat health problems and complications related to cirrhosis with medicines, surgery, and other medical procedures.

If Wilson disease causes acute liver failure or liver failure due to cirrhosis, you may need a liver transplant. A liver transplant cures Wilson disease in most cases.

Last reviewed by a Cleveland Clinic medical professional on 11/01/2018.

References

  • Bandmann O, Weiss KH, Kaler SG. Wilson’s disease and other neurological copper disorders. Lancet Neurology. 2015;14(1):103–113.
  • Coffey AJ, Durkie M, Hague S, et al. A genetic study of Wilson’s disease in the United Kingdom. Brain.2013;136:1476–1487.
  • Kanwar P, Kowdley KV. Metal storage disorders: Wilson disease and hemochromatosis. Medical Clinics of North America. 2014;98(1):87–102.
  • Kim JW, Kim JH, Seo JK, et al. Genetically confirmed Wilson disease in a 9-month old boy with elevations of aminotransferases. World Journal of Hepatology. 2013;5(3):156–159.
  • Schilsky ML. Liver transplantation for Wilson’s disease. Annals of the New York Academy of Sciences.2014:1315;45–49.
  • Schilsky ML. Wilson disease: diagnostic tests. UpToDate. Available at https://www.uptodate.com/contents/wilson-disease-diagnostic-tests . Updated December 1, 2015. Accessed May 14, 2018.
  • National Institutes of Health; National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/

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