Wilson’s disease, sometimes referred to simply as Wilson disease, is a congenital disorder that affects the liver and other internal organs. Children inherit Wilson’s disease from their parents, one both of them are carriers of a specific DNA mutation. It is an extremely rare condition that causes a dangerous buildup of copper in the blood, which then gets deposited in the liver, eyes, brain, and other parts of the body.
There is no way to prevent Wilson’s disease, and there is also no permanent cure. There are, however, treatments that can reduce the amount of copper in your body, thereby taking away the dangers of liver failure and other complications of Wilson’s.
It is important to start treatment as soon as you start displaying symptoms of Wilson’s disease, which does not happen until enough copper builds up in the body. This can be anywhere from five to forty years of age and beyond. Treatment will focus on reducing copper buildup in the body, and you will need to continue treatments for the rest of your life.
If you have Wilson’s disease, chelating agents will be the first treatment option. Chelating agents are medications that remove copper from the body, thereby reducing its risk to you. Penicillamine and trientine are the two most common types of chelating agents, as well as the most effective.
Typically, doctors will start you on a low dose of whatever chelating agent is right for you. Then, as your body adjusts to less copper, your doctor will gradually increase the dose until you have the right amount of copper. After all, the body needs a small amount of copper to remain healthy and functional, but excess copper can be deadly.
In addition to chelating agents, your doctor may also prescribe you zinc. Zinc is a mineral that the body needs for certain functions. In people with Wilson’s disease, zinc will keep the intestines from absorbing copper, which can protect your internal organs and digestive system.
Zinc can be prescribed for one of two reasons. You may need zinc because you have tested positive for Wilson’s disease but are not yet showing symptoms. You may also need zinc after chelating agents have removed the right amount of copper from the body.
If you have Wilson’s disease, you will need to change your diet and avoid foods that are high in copper. Liver, shellfish, dried fruits, peas, and beans, mushrooms, nuts, and chocolate are all high in copper and should be avoided.
If chelating agents and dietary changes are not enough to keep your copper levels at bay, you will eventually experience liver failure. When this happens, the only way to save you will be to have a liver transplant. During the transplant, all or part of your defective liver is removed and replaced with healthy liver tissue. In many cases, symptoms of Wilson’s disease go away after a transplant.
If you are pregnant, the treatment for Wilson’s disease will be similar to how it would be in a non-pregnant person. However, because the foetus needs small amounts of copper, as does the human body, treatment will be more conservative. You do not want to remove as much copper as you otherwise would, so the zinc or chelating agent dosage will be smaller.
While Wilson’s disease can be a frightening condition to have, and there is no cure for it, proper treatment can prevent liver failure. However, in the event that your liver fails and needs to be replaced, a transplant is an option. A transplant is a worst-case scenario treatment, however, and you should do everything in your power to reduce copper levels and keep your current liver as healthy as possible.