COPPER AND WILSON’S DISEASE7Medical management involves the use of chelating agents to remove extra copper in the body. The chelating agents release the extra copper in the bloodstream and enhance its clearance in the kidney (Harada, 2012). The most common agents are trientine, d-penicillamine, and zinc and ammonium tetrathiomolybdate. Trientine has a polyamine structure that chelates copper using its nitrogen in its structure. The drug is very effective, but it is associated with worsening neurological symptoms. D-penicillamine mobilizes intercellular copper into the circulation thus enhancing excretion by the kidney (Hay, 2009). The drug also increases the production of metallothionein that chelate copper into a nontoxic form. The drug has severe adverse effect, andits use has raised much debate. The adverse effects include bone marrow depression, kidney disorders, rash and fever. The drug is also associated with neurological related symptoms acceleration.Zinc induces the release of metallothionein, which is released by the duodenal enterocytes (Schilsky, 2009). The copper is bound in the enterocytes and released into the alimentary system during routine shed off. Another chelating agent is the ammonium tetrathiomolybdate, which chelates copper using sulfur groups in its structure. The agent forms a complex with copper and prevents it from being utilized in the body (Brewer et al., 2009). The drug has fewer side effects compared to other chelating agents. Medical regime also involves treatment of symptoms associated with Wilson's disease. Parkinsonism is treated using L-dopa, anticholinergic and dopamine antagonist. Tremors are treated using beta-blockers while dystonia is treated using dopamine antagonists (Schilsky, 2009). The adverse effects of drugs are managedsymptomatically.Liver transplantation is considered if medical management fails. The transplant is necessary for patients who develop acute liver failure, poor prognosis and cirrhosis leading to
COPPER AND WILSON’S DISEASE8liver failure (Dong & Wu, 2012). The transplant involves replacing the diseased liver with a healthy one. Research and evidence-based practice have demonstrated that liver transplant is an effective way of managing the disease.Women suffering from the Wilson disease can get pregnant if copper levels are controlledprior to pregnancy. Use of chelating agents is not discontinued since risks of discontinuing are high than teratogenicity (Dong & Wu, 2012). The dosage is increased in the second trimester andthen reduced in the third trimester to enhance wound healing and avoid fetal copper insufficiency. However, the doses of the chelating agents are reduced especially for the first trimester. Breastfeeding is not recommended if the patient is using chelating agents. The patients suffering from the disease are put on medication for a lifetime course (NIH, 2014). Cases of medication non-compliance are common and thus need for follow-up clinics. The future of Wilson’s disease is more likely to have a breakthrough due to promising research based on hepatocyte transplantation and genetic engineering (NIH, 2014). Studies on animals have shown successful hepatocyte transplantation.
- Spring '08
- Hepatology, Wilson Disease, Wilson’s Disease