How is Wilson disease treated?
Treatment for Wilson disease focuses on lowering toxic levels of copper in your body and preventing organ damage and the symptoms you get when your organs aren’t functioning normally. Treatment includes:
- Taking medicines that remove copper from the body (chelating agents, D-penicillamine, tetrathiomolybdate).
- Taking zinc to prevent your intestines from absorbing copper.
- Eating a diet low in copper.
People who have Wilson disease need lifelong treatment. Stopping treatment may cause acute liver failure. Your healthcare provider will regularly perform blood and urine tests to check how the treatment is working.
What medications treat Wilson disease?
Your healthcare provider may offer several different medications to reduce the amount of copper in your body.
Chelating agents
Chelating agents remove copper from your body. Types of chelating agents include:
- Penicillamine.
- Trientine.
When treatment begins, doctors gradually increase the dose of chelating agents. People take higher doses of chelating agents until the extra copper in their body goes away. When Wilson disease symptoms improve and tests show that copper is at safe levels, your provider may prescribe lower doses of chelating agents as maintenance treatment. Lifelong maintenance treatment prevents copper from building up again.
These medications can have side effects and you should ask your provider if you need to take dietary supplements like vitamins or take any precautions before having surgery.
Zinc
Zinc prevents the intestines from absorbing copper. Your healthcare provider may prescribe zinc as a maintenance treatment after chelating agents remove extra copper from your body. Your provider may also prescribe zinc if you have Wilson disease but don’t have symptoms.
What types of treatment are available for someone who is pregnant and diagnosed with Wilson disease?
If you’re pregnant, you should ask your provider how to continue treatment for Wilson disease throughout your pregnancy. Your provider might prescribe a lower dose of chelating agents because the fetus needs a small amount of copper. Having an obstetrician who is familiar with Wilson disease can be very helpful.
Also ask your provider if it’s safe to breastfeed (chestfeed) while you’re being treated for Wilson disease.
What should I avoid eating with Wilson disease?
Your healthcare provider may recommend changing your diet to avoid certain foods that are high in copper if you have Wilson disease. You should specifically avoid:
- Shellfish.
- Liver.
Other foods that are high in copper include:
- Chocolate.
- Dried fruit.
- Dried beans and peas.
- Mushrooms.
- Nuts.
After treatments lower your copper levels and you begin maintenance treatment, talk with your healthcare provider about whether you can safely eat moderate amounts of some of these foods.
If your tap water comes from a well or runs through copper pipes, have the copper levels in your water checked. You may need to use a water filter to remove copper from your tap water.
If you plan on taking dietary supplements like vitamins, talk with your healthcare provider before taking them. Some dietary supplements contain copper.
Are there complications of the treatment?
Wilson disease may lead to complications, but early diagnosis and treatment can lower your chances of developing side effects. The medications used to treat Wilson disease also may have side effects, so ask your provider what to watch for.
Acute liver failure
Wilson disease can cause acute liver failure, a condition where your liver quickly stops functioning without warning. About 5% of people with Wilson disease have acute liver failure when they receive their diagnosis. Acute liver failure could require a liver transplant.
Acute kidney failure and a type of anemia called hemolytic anemia often occur in people who have acute liver failure due to Wilson disease.
Cirrhosis
Cirrhosis is a condition where scar tissue replaces healthy liver tissue and prevents your liver from working normally. Scar tissue also partially blocks the flow of blood through your liver. As cirrhosis gets worse, the liver begins to fail.
Among people diagnosed with Wilson disease, 35% to 45% already have cirrhosis at the time of diagnosis.
Cirrhosis increases your chance of getting liver cancer. However, healthcare providers found that liver cancer is less common in people who have cirrhosis due to Wilson disease than in people who have cirrhosis due to other causes.
Liver failure
Cirrhosis may eventually lead to liver failure. With liver failure, your liver is badly damaged and stops working. Liver failure is also called end-stage liver disease. This condition may require a liver transplant.
How are the complications of Wilson disease treated?
If Wilson disease leads to cirrhosis, your healthcare provider may be able to treat your complications with medicine or surgery.
If Wilson disease causes acute liver failure or chronic liver failure due to cirrhosis, you may need a liver transplant. Some people who receive a liver transplant will completely recover from Wilson disease, but your healthcare provider will carefully monitor you to make sure the transplant was successful.
How soon after treatment will I feel better?
Treatment for Wilson disease is lifelong. The timeline for when you’ll feel better depends on the severity of your symptoms, but your symptoms could reduce significantly after four to six months of treatment followed by regular maintenance treatment. Wilson disease requires regular testing by your healthcare provider to understand if the treatment is successful and they will adjust your medicine dosage to meet your body’s needs.
