Wilson’s disease, also known as hepatolenticular degeneration, is a rare genetic disorder caused by the buildup of copper. Small amounts of copper exist in the body and are released through the urine. In Wilson’s disease, the copper cannot leave the body so it accumulates in certain organs. Most copper builds up in the liver and brain. Copper accumulation in the brain causes neurological problems. Buildup in the liver can result in liver failure and kidney dysfunction. Though treatable, early diagnosis is crucial in stopping the progression of Wilson’s disease. Delayed treatment or no treatment at all can result in liver failure, brain damage, or even death.
Risk factors for Wilson’s disease are genetic. Parents who carry the gene are at risk for passing the disease on to their children. A genetic test can be performed if a child shows symptoms of Wilson’s and has one or both parents who have the disease.
Certain drugs may be used to release copper from organs into the bloodstream. Most of the copper is then filtered out by the kidneys and excreted in urine. But these drugs may cause neurologic symptoms to become worse. Zinc may be used to block copper absorption from food in the digestive tract. Zinc may be safe to use at full dosage during pregnancy. Your health care provider can help decide what amount is best for you. Maintenance therapy begins when symptoms improve and tests show that copper has been reduced to a safe level. Maintenance therapy often includes taking zinc and low doses of a medicine that releases copper into the bloodstream. Blood and urine should be monitored by a health care provider to make sure that treatment is keeping copper at a safe level. It also is important to reduce your dietary copper intake. You should not eat shellfish or liver. These foods contain high levels of copper. Your health care provider may tell you to avoid other foods as well. It is important to have your drinking water checked for copper content. Avoid taking multivitamins that contain copper.