What happens when vital minerals like copper, which is required by the body for developing strong bones and for building of proteins in the blood, turns excess? An adult human body contains 1.4 to 2.1 mg of copper per kilogram of body weight. This small amount is essential and enough for the well-being of the body. But when there is excess of copper due to genetic mutation, it causes Wilson’s disease which damages the liver and the brain.
The biggest problem for those suffering from this disease is detecting the copper in the body. The first and easy sign can be found in the eyes.
Dr M.S. Sridhar, senior consultant ophthalmologist, explains that through a Kayser-Fleischer ring in the cornea, it is possible to detect copper in the body and every ophthalmologist must be trained to detect it.
What is the role of an ophthalmologist in detecting this disease?
Diagnosis of the liver by injecting a needle to check how much copper it has is very dangerous as it can lead to internal bleeding and even death. Even the brain scans do not show the amount of copper in a particular body part. The only successful method is the Kayser-Fleischer ring at the peripheral cornea which gives an idea of the amount of the copper in the body. It appears as a greenish-yellow or golden-brown ring. It is formed by the copper particles that infiltrate into the membrane.
The diagnosis of the disease is important. This requires proper training and also counselling of the patient. The ring is formed in the eye after substantial amount of copper poisoning. Most of the cases that have been detected in terms of liver are towards the end-stage. And, the cases where the brain is affected are realised when the patient’s hands shake involuntary. Hence, our main aim is to detect the disease so that the patient can be put on the right treatment, with an integrated approach by three specialists.
Do the patients come for a check-up during the early stage of Wilson’s disease?
The patients come at a critical and a very crucial stage. Many people go to a psychiatrist as they think it is a mental problem. If there is no result despite medication, then some even consider it as evil spirit. The involuntary shaking of the hand is linked with being possessed. By the time it shows in the eye, the disease is already at a crucial stage.
At a crucial stage, are there chances of recovery?
Most of these patients are children or young adults. Hence if we detect it in the eye, it is very important for them to stop consuming food that has copper. If the amount is reduced, the degeneration of the liver and brain can be controlled via different medications.
Food items to avoid
- Copper is available in dietary foods like seafood, organ meat, whole grains, legume, chocolate, peanuts, wheat, lemon, raisins, cereals, potatoes, peas, red meat, mushrooms, coconut, papaya and apples.
- Rice, tea and chicken do not have large quantities of copper. Hence the diet has to be changed completely.
- Patients must not drink water or eat food which is served in copper utensils.
What is Wilson’s disease?
Wilson’s disease is caused when there are two abnormal genes in the body which mutate, leading to excess copper in the body, which leads to copper poisoning. Studies show that when people from the same family, who are carriers of the genes, marry each other, the child is born with this defect.
- If the brain is affected then there is involuntary shaking of the hands and later arms and then it spreads to other parts of the body.
- If the liver is affected, it is detected only towards the end-stage, when there are chances of liver failure.
Incidence of Wilson’s disease
- It is found in one in 30,000 people to one in 1,00,000 people worldwide. In India,
- it is found in families who marry within the same family as the disease is genetic in nature.
- One in 90 people are carrier of the genes.
