Outlook (Prognosis)
Wilson's Disease requires lifelong treatment. If the disorder is detected early and treated correctly, a person with Wilson's disease can enjoy completely normal health, but without proper treatment, Wilson's is generally fatal.
Disease that presents early in life has the worst prognosis than that which presents later in life. Wilson’s Disease has been diagnosed in children as young as 3 to 5 years. If the patient stops the drugs the disease can rapidly lead to deterioration and death in 1 to 3 years.
Early diagnosis and treatment can mean a normal life span. If the brain has been affected before the start of treatment then the manifestations such as dysarthria and dystonia may continue in mild form. Prognostic factors depends on blood serum aspartate aminotransferase, bilirubin and prothrombin levels at the time of diagnosis to determine if the disease is likely to be fatal or not (Called Nazer score)
Even with treatment some residual cirrhosis that has developed is likely to stay though liver function improves to give normal or near normal function. Features of portal hypertension if developed due to cirrhosis will not disappear and patients with dilated veins in the esophagus or stomach can bleed from these and may require treatment. If a patient has had one such episode of bleeding the risk of second such bleed is high and can lead to death.
Death in this disease is generally due to the liver, kidney or blood related problems and occurs generally at age of 30 years. In adults the first sign of the disease maybe neurological or psychiatric symptoms in nature.
