Wilson Disease

Wilson disease is an inherited disorder in which the body cannot get rid of copper. The liver keeps absorbing copper from food but cannot excrete it into bile, so copper builds up first in the liver and then spills into the blood and settles in the brain, the cornea, the kidneys and red blood cells. Untreated, it is fatal. Treated early and taken consistently, medication controls it, which is why the nursing work in Wilson disease is mostly about three things: recognizing the pattern in a young person, keeping a lifelong medication plan safe and continuous, and spotting the client who is sliding into acute liver failure. The 2022 AASLD practice guidance describes symptomatic presentation mainly between about 3 and 55 years of age, so neither early childhood nor middle age rules it out. Children and adolescents usually present with liver disease; adults more often present with neurologic or psychiatric problems, with or without obvious liver disease. Because early findings are non-specific — fatigue, mildly raised liver enzymes, a drop in school performance, a new tremor, low mood — diagnosis...