Pharm'Up 
Wilson disease is a rare, inherited condition that disrupts how the body processes copper. While copper is an essential mineral for health, the inability to eliminate excess amounts leads to toxic buildup, which can be life-threatening if left untreated.
How It Affects the Body
In a healthy body, the liver filters excess copper and excretes it through bile. In individuals with Wilson disease, this mechanism fails:
- Liver Accumulation: Copper first builds up in the liver, causing damage and eventually scarring (cirrhosis).
- Systemic Spread: Once the liver is saturated, copper is released into the bloodstream, where it travels to and damages the brain, kidneys, and eyes.
Symptoms and Diagnosis
Although the disorder is present from birth, symptoms typically do not manifest until copper levels reach a tipping point, usually between the ages of 5 and 35.
Key Warning Signs
- The Kayser-Fleischer Ring: The most distinct sign is a rusty-brown ring around the cornea of the eye caused by copper deposits.
- Neurological Issues: Tremors, difficulty speaking, or problems with coordination as the central nervous system is affected.
- Liver Issues: Jaundice (yellowing of the skin), swelling, or abdominal pain.
Lifelong Management
Wilson disease requires consistent, lifelong treatment to keep copper levels within a safe range.
Medical Treatment
Doctors prescribe chelating agents, which are drugs that help “grab” copper from the tissues so it can be excreted by the kidneys.
Dietary Restrictions
Patients must adhere to a strict low-copper diet.
- Always Avoid: Shellfish and liver (extremely high copper content).
- Avoid During Initial Treatment: Chocolate, mushrooms, and nuts.
- Environmental Safety: It is important to test home drinking water for copper and avoid multivitamins containing the mineral.
About the Author
