Treatment of Wilson disease
Wilson disease is a very treatable condition. With proper therapy, disease progress can be halted and oftentimes symptoms can be improved. Treatment is aimed at removing excess accumulated copper and preventing its reaccumulation. Treatment for Wilson disease is a lifelong process. Patients may become progressively sicker from day to day, so immediate treatment can be critical. Treatment delays may cause irreversible damage.
Chelation therapy drugs approved for treating Wilson disease include penicillamine (Cuprimine® and Depen®) and trientine (Syprine® and Trientine Dihydrochloride) Both of these drugs act by chelation or binding of copper, causing its increased urinary excretion.
Zinc acetate is approved for treating Wilson disease under the brand names (Galzin™) in the U.S. and (Wilzin®) in Europe. Zinc acts by inducing metallothionein and blocking the absorption of copper in the intestinal tract. This action both depletes accumulated copper and prevents its reaccumulation. Zinc is used primarily for maintenance treatment and to treat asymptomatic patients and may also be used in conjunction with chelators. Its effectiveness has been shown by more than 40 years of considerable experience in the USA and Europe. A major advantage of zinc therapy is its lack of side effects.
Patients with severe hepatitis or liver failure may require liver transplant. Patients being investigated or treated for Wilson disease should be cared for by specialists in Wilson disease or by specialists in consultation with their primary physicians. Stopping treatment completely will result in death, sometimes as quickly as within three months. Decreasing dosage of medications also can result in unnecessary disease progression.
Taking your medicine as prescribed is extremely critical to the success of Wilson disease treatment. One of the advantages of having WD is that it is VERY treatable with effective, safe medications leading to a normal life expectancy . . . IF . . . you faithfully take your medications, as prescribed, LIFELONG. There are varying reasons for non-adherence in patients with WD and your physician has probably heard them all. Adherence is especially difficult in patients who were asymptomatic as diagnosis. These patients often do not see a cause and effect relationship.
