Topic: Wilson Disease
Question: What are the treatments for Wilson Disease ?
Answer: A health care provider will treat Wilson disease with a lifelong effort to reduce and control the amount of copper in the body. Treatment may include
                
- medications  - changes in eating, diet, and nutrition  - a liver transplant
                
Medications
                
A health care provider will prescribe medications to treat Wilson disease. The medications have different actions that health care providers use during different phases of the treatment.
                
Chelating agents. Chelating agents are medications that remove extra copper from the body by releasing it from organs into the bloodstream. Once the cooper is in the bloodstream, the kidneys then filter the copper and pass it into the urine. A health care provider usually recommends chelating agents at the beginning of treatment. A potential side effect of chelating agents is that nervous system symptoms may become worse during treatment. The two medications available for this type of treatment include
                
- trientine (Syprine)the risk for side effects and worsening nervous system symptoms appears to be lower with trientine than d-penicillamine. Researchers are still studying the side effects; however, some health care providers prefer to prescribe trientine as the first treatment of choice because it appears to be safer.  - d-penicillaminepeople taking d-penicillamine may have other reactions or side effects, such as      - fever    - a rash    - kidney problems    - bone marrow problems
                
A health care provider will prescribe a lower dose of a chelating agent to women who are pregnant to reduce the risk of birth defects. A health care provider should consider future screening on any newborn whose parent has Wilson disease.
                
Zinc. A health care provider will prescribe zinc for patients who do not have symptoms, or after a person has completed successful treatment using a chelating agent and symptoms begin to improve. Zinc, taken by mouth as zinc salts such as zinc acetate (Galzin), blocks the digestive tracts absorption of copper from food. Although most people taking zinc usually do not experience side effects, some people may experience stomach upset. A health care provider may prescribe zinc for children with Wilson disease who show no symptoms. Women may take the full dosage of zinc safely during pregnancy.
                
Maintenance, or long term, treatment begins when symptoms improve and tests show that copper is at a safe level. Maintenance treatment typically includes taking zinc or a lower dose of a chelating agent. A health care provider closely monitors the person and reviews regular blood and urine tests to ensure maintenance treatment controls the copper level in the body.
                
Treatment for people with Wilson disease who have no symptoms may include a chelating agent or zinc in order to prevent symptoms from developing and stop or slow disease progression.
                
People with Wilson disease will take medications for the rest of their lives. Follow-up and adherence to the health care providers treatment plan is necessary to manage symptoms and prevent organ damage.
(Source: NIDDK)