Treatment

Your doctor might recommend medications called chelating agents, which bind copper and then prompt your organs to release the copper into your bloodstream. The copper is then filtered by your kidneys and released into your urine.

Treatment then focuses on preventing copper from building up again. For severe liver damage, a liver transplant might be necessary.

Medications

If you take medications for Wilson's disease, treatment is lifelong. Medications include:

  • Penicillamine (Cuprimine, Depen). A chelating agent, penicillamine can cause serious side effects, including skin and kidney problems, bone marrow suppression, and worsening of neurological symptoms. Penicillamine should be used cautiously if you have a penicillin allergy. It also keeps vitamin B-6 (pyridoxine) from working, so you'll need to take a supplement in small doses.
  • Trientine (Syprine). Trientine works much like penicillamine but tends to cause fewer side effects. Still, neurological symptoms can worsen when taking trientine.
  • Zinc acetate (Galzin). This medication prevents your body from absorbing copper from the food you eat. It is typically used as maintenance therapy to prevent copper from building up again after treatment with penicillamine or trientine.

    Zinc acetate might be used as primary therapy if you can't take penicillamine or trientine. Zinc acetate can cause stomach upset.

Your doctor might also recommend other medications for symptom relief.

Surgery

If your liver damage is severe, you might need a liver transplant. 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 the donor's liver.

Aug. 03, 2017
References
  1. Shilsky ML. Wilson disease: Clinical manifestations, diagnosis and natural history. https://www.uptodate.com/home. Accessed June 22, 2017.
  2. AskMayoExpert. Wilson disease. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2017.
  3. Pazirandeh S, et al. Overview of dietary trace minerals. https://www.uptodate.com/home. Accessed June 22, 2017.
  4. Wilson disease. National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/liver-disease/wilson-disease. Accessed June 22, 2017.
  5. Shilsky ML. Wilson disease: Treatment and prognosis. https://www.uptodate.com/home. Accessed June 22, 2017.
  6. Bandmann O, et al. Wilson's disease and other neurological copper disorders. The Lancet Neurology. 2015;14:103.
  7. Wilson disease. American Liver Foundation. http://www.liverfoundation.org/abouttheliver/info/wilson/. Accessed June 22, 2017.
  8. Riggin EA. AllScripts EPSi. Mayo Clinic, Rochester, Minn. June 23, 2017.