How is the Wilson disease treated?
Wilson disease, a genetic condition that leads to the accumulation of copper in the body, primarily affects the liver and brain. Its treatment involves several medications and therapies aimed at reducing the copper levels, preventing further organ damage, and managing symptoms. Here are the main treatment approaches for Wilson disease:
1. Medications:
- Chelating Agents: These medications bind to copper in the body and help remove it through the urine. Common chelating agents used for Wilson disease include:
- Penicillamine (Cuprimine)
- Trientine (Syprine)
- Tetrathiomolybdate (Thiomolybdates)
2. Zinc Supplementation:
- Zinc, an essential mineral, reduces the absorption of copper from the intestines and promotes its elimination from the body.
- Zinc acetate or sulfate may be prescribed to lower the intestinal absorption of copper.
3. Diet Modification:
- Maintaining a low copper diet is crucial in managing Wilson disease.
- Foods high in copper, such as liver, shellfish, nuts, seeds, and cocoa products, should be restricted.
- Dietitians can provide guidance on creating a balanced and copper-controlled diet.
4. Liver Transplantation:
- In cases where Wilson disease has caused severe damage to the liver, a liver transplant may be considered.
- Liver transplantation replaces the damaged liver with a healthy one, allowing for the restoration of normal liver function and the removal of excess copper.
5. General Supportive Care:
- Regular monitoring and testing of liver function, copper levels, and other related parameters are essential for tracking the disease progression and response to treatment.
- Medications to manage symptoms such as pain, nausea, or neurological issues may be prescribed as needed.
- Psychological and emotional support can be important due to the chronic nature of the condition and its impact on daily life.
It is crucial for individuals with Wilson disease to closely follow their treatment plans, as recommended by their healthcare team. Compliance with medication schedules, regular monitoring, and lifestyle modifications are essential for effective management and preventing long-term complications.