What's the treatment for Wilson's disease?
Without treatment, Wilson's disease is fatal. The longer the copper poisoning continues, the harder it is to successfully treat, so early diagnosis is important. The aims of treatment are to reduce the amount of copper in the body and control the
symptoms. Treatment must be lifelong.
Treatment of Wilson's disease generally consists of anti-copper agents to remove excess copper from the body and to prevent it from re-accumulating. Most cases are treated with the drugs zinc acetate, trientine, or penicillamine. Penicillamine and trientine increase urinary excretion of copper, however, both drugs can cause serious side effects. Zinc acetate - which blocks the absorption of copper, increases copper excretion in the stool, and causes no serious side affects - is often considered the treatment of choice. Tetrathiomolybdate, an experimental drug, also shows promise in treating Wilson's disease. In rare cases in which there is severe liver disease, a liver transplant may be needed.
Careful monitoring is necessary. When patients have side effects from penicillamine, the dose can sometimes be lowered to an effective level that causes fewer difficulties. Alternatively, steroid medications may be required to reduce certain sensitivity reactions. Trientine has fewer potential side effects, but must still be carefully monitored. |
