Six-year-old Arif Ahmed was admitted to a private clinic on August 12, 2012 with jaundice for 22 days. Tests showed no presence of Hepatitis A, Hepatitis B or Hepatitis E viruses. After a few days of bed rest, Arif felt quite well but the cause of his jaundice was not discovered.
Four years later, Arif was again hospitalised on July 15, 2016 in the Pediatric Gastroenterology unit of Dhaka Shishu (Children) Hospital with the same complication along with generalised swelling of whole body. This time detailed investigation was done.
His diagnosis was chronic liver disease with portal hypertension due to Wilson’s Disease.
Specialists say lack of awareness about Wilson’s in Bangladesh means that it often remains undiagnosed.
Jaundiced patients should be tested for the disease every time, because when a viral cause is absent in patients over five years of age, Wilson’s is very likely, said Dr Salahuddin Mahmud, assistant professor of pediatric gastroenterology, hepatology and nutrition at Dhaka Shishu Hospital.
According to European Association for the Study of Liver Disease, the worldwide prevalence of Wilson’s is 1 in 30,000 and 1 in 90 persons carry it in their genes. However, the extent of prevalence of Wilson’s Disease in Bangladesh is not known.
Former director of Institute of Epidemiology, Disease Control and Research (IEDCR) Mahmudur Rahman told the Dhaka Tribune that the Wilson’s Disease is not common here.
However, between 2015-2016, 125 children got admitted at Dhaka Shishu (Children) Hospital with acute liver disease, and among them 20 (16%) were found to have Wilson’s.
In 2012-2013, 76 children were admitted with chronic liver disease to Bangabandhu Sheikh Mujib Medical University (BSMMU) and among them 47 (61%) were diagnosed with Wilson’s Disease.
Another study found that 43.7% children had the disease, of whom 58% were patients of chronic liver disease, 19.4% had acute hepatitis, 19.4% had liver failure and 3.2% were asymptomatic Wilson’s Disease cases.
BSMMU Associate Professor Dr Rukunuzzaman said about one-third of children presented with liver diseases were diagnosed as Wilson’s patients, and about 76% of the Wilson’s patients presented with chronic liver disease.
“People do not know about the disease. Physicians diagnose a jaundice patient with hepatitis viruses and when all results come out negative, they just prescribe the patient with bed rest and do not investigate further because physicians themselves are not conscious about Wilson’s,” said Dr Salauddin Mahmud.
“In chronic liver disease, Wilson’s is more common than Hepatitis B and Hepatitis C,” Dr Salahuddin added.
To avoid this dilemma Dr Rukunuzzaman suggested that any child presented with jaundice after the age of three years should be investigated for Wilson’s.
What is Wilson’s Disease?
Wilson’s Disease is an autosomal recessive condition, meaning it is an inherited disease that one can get if it is carried in both parents’ genes.
The disease affects copper metabolism, causing copper to accumulate in the body, damaging liver, brain and other vital organs. Children mostly suffer from liver and brain damage.
A study on “Wilson’s Disease in Bangladeshi Children: Analysis of 100 Cases” found that in 30% cases parents of the patients were first cousins, meaning both of them inherited and carried the gene for the disease.
Symptoms of the disease are almost similar to Hepatitis as it affects the liver.
An obvious and unusual symptom of Wilson’s is the Kayser–Fleischer ring, a bright circle of copper around the iris due to copper deposition in part of the cornea.
“When diagnosed early, Wilson’s Disease is treatable, and many people with the disorder live normal lives. That is why we need to investigate jaundice cases to know its root, otherwise it may cause liver failure,” Dr Salahuddin said.
After diagnoses, physicians suggest to avoid food which contain copper. In this disease, liver fails to excrete copper through urine and stool, and it can travel through blood into eye and brain.
A Wilson’s patient has to adopt a specifically copper-free diet.
Physicians prescribe Penicillamine to Wilson’s patients and in Bangladesh, this medicine comes from India and can be expensive. One brand of the drug costs Tk20 per tablet. This medicine helps copper excretion through urine and stool.