Wilson's disease is a rare genetic disorder that causes copper to accumulate in various organs of the body, primarily the liver and brain. If left untreated, it can lead to severe liver damage, neurological problems, and even death. However, with early diagnosis and appropriate treatment, individuals with Wilson's disease can lead normal lives. The main goal of treatment is to reduce copper levels in the body and prevent further damage.
Chelating agents: The first-line treatment for Wilson's disease involves the use of chelating agents, such as D-penicillamine and trientine. These medications bind to excess copper in the body and help eliminate it through urine. Regular monitoring of copper levels and liver function is essential while on these medications.
Zinc: Zinc acetate or zinc sulfate is often prescribed as a long-term maintenance therapy. Zinc blocks the absorption of copper in the intestines, reducing its accumulation in the body. It is usually taken orally and needs to be separated from meals and other medications to ensure optimal absorption.
Low copper diet: Individuals with Wilson's disease are advised to follow a low copper diet to minimize copper intake. This involves avoiding high-copper foods such as shellfish, liver, nuts, chocolate, and mushrooms. It is important to consult a registered dietitian to ensure a balanced diet while restricting copper intake.
High-protein diet: Wilson's disease can lead to impaired liver function, which may affect protein metabolism. Consuming a diet rich in high-quality proteins, such as lean meats, fish, eggs, and legumes, can help support liver health and overall nutrition.
In severe cases where liver damage is extensive or if medication therapy fails to control copper levels, liver transplantation may be considered. This involves replacing the diseased liver with a healthy liver from a donor. Liver transplantation can be a life-saving option for individuals with advanced liver disease caused by Wilson's disease.
Regular follow-up appointments with a healthcare team experienced in Wilson's disease management are crucial. Monitoring copper levels, liver function, and neurological status is essential to ensure treatment effectiveness and adjust medications if needed. Genetic counseling may also be beneficial for individuals and families affected by Wilson's disease.
While Wilson's disease is a lifelong condition, it can be effectively managed with appropriate treatment. Medications such as chelating agents and zinc, along with dietary modifications, play a key role in reducing copper accumulation. In severe cases, liver transplantation may be necessary. Ongoing monitoring and support are vital to ensure optimal management and improve the quality of life for individuals with Wilson's disease.