Drugs Used to Treat Wilson's Disease

A number of drugs are available for the treatment of Wilson’s disease, including the chelating agents D-penicillamine and trientine, as well as zinc salts. Chelating agents work by increasing the removal of copper in the urine, whilst zinc salts work to reduce the amount of copper absorbed. Treatment must be lifelong and monitoring is necessary to make sure symptoms and copper balance are controlled.1,3
The aim of the treatment is to reduce the copper content in the body by removing it through the urine and to prevent the progression of the disease.1,3
It is very important to attend regular follow-up doctor visits to allow your doctor to monitor your condition. You should not stop taking your medication without speaking to your doctor. If you stop taking your medication, copper may build up rapidly, which can be life-threatening.1,3
Liver transplantation can be necessary for patients with severe liver failure due to delayed diagnosis or to treatment inefficacy, often due to poor treatment compliance. In such individuals, liver transplantation can be lifesaving despite the lifelong risk for graft rejection.1,3

Treatment Phases

For people with symptoms, treatment with medications usually occurs in 2 phases.

Phase 1:

Removal of copper from where there has been a build-up in the body – chelating agents (trientine or D-penicillamine) will be used in this phase.1,3


Phase 2:

Maintaining copper levels within acceptable levels. Chelating agents (trientine or D-penicillamine) or zinc therapy can be used.1,3

Diet & Nutrition

Copper enters our bodies through the food we eat and the water we drink, which is important because our bodies need copper to function. However, when you have Wilson’s disease and your body can’t rid itself of excess copper, it’s important to limit your copper intake by avoiding foods with a high copper content.1,3,5
Foods you should avoid (or limit) include:1,3,5
Organ meats (e.g., liver)

    People with Wilson’s disease should also avoid using copper containers for cooking, serving, or storing food.1,3,5

    Tap water that runs through copper pipes or comes from a well may contain higher copper levels. Water should be run for a while before using, as this will reduce any copper residues to acceptable levels.1,3,5

    Talk to your doctor before taking a multivitamin. If they approve, ask your pharmacist to recommend one that does not contain copper. You should also discuss any other dietary supplements or herbal preparations you are taking or would like to take, to make sure they will not interact with your medications or worsen problems in your liver.3,6

    If you are a woman with Wilson’s disease and pregnant, or planning to become pregnant, you should ask your obstetrician to consult with your Wilson’s disease specialist before prescribing prenatal vitamins.6

    As trientine has been found to reduce iron levels in the blood, your doctor may prescribe you iron supplements. As iron and trientine may inhibit the absorption of each other, iron supplements should be taken two hours after taking trientine.7,8

    It is essential to read the information that is provided with all medicines and follow your healthcare professional’s advice when it comes to any potential sources of copper or drug interactions that could be harmful to you.

Have a Question About Wilson's Disease?

If you have a question or would like to learn more about the treatment of Wilson's disease, please contact one of our regional advisors by visiting the link below.