Wilson's disease is treated with medications, or "drug therapy." This must be continued for life. For patients who have symptoms, the first goal of treatment is to remove as much copper from the body as possible. This is done with medicines called D-penicillamine or trientine hydrochloride. It may be weeks before it is clear whether the treatment worked.
These drugs may have some serious side effects, including:
- Pain in the joints
- Neurological problems that affect mental abilities
- Problems with blood clotting
- Allergic reactions
Your doctor will monitor your child's drug therapy through physical exams, measurement of copper in urine collection, blood tests that show the amount of copper in the blood, measurement of liver function and blood cell counts.
For patients without symptoms, and for patients initially treated with medications to reduce the amount of copper in the body, the focus becomes preventing the body from taking in too much copper. Taking extra zinc may help block the body's absorption of copper. Patients also will need to take vitamin B6 and follow a low-copper diet, which means avoiding mushrooms, nuts, chocolate, dried fruit, liver and shellfish.
When this drug therapy is not successful in getting rid of the high amounts of copper in the body, liver transplantation may be required for those with very advanced disease.
Liver transplantation is very effective in the treatment of patients with advanced liver disease caused by Wilson's disease. If a transplant is the best treatment option, the care team will focus on preventing complications and treating symptoms while awaiting a donated liver.