Suffering from rare Wilson disease, 8-year-old girl in coma undergoes lifesaving liver transplant

New Delhi, Apr 20: An 8-year-old girl from Bihar underwent a lifesaving liver transplant procedure here after being diagnosed with a rare disease which has a 100 per cent mortality rate in children who develop coma, Indraprastha Apollo Hospitals said on Tuesday.

Wilson's disease is a genetic disorder in which excess copper builds up in the body leading to liver damage. Its treatment includes lifelong medication to bind copper.

However, some children develop coma due to liver failure, it said. Anshika was admitted to Indraprastha Apollo Hospitals on March 25 in a state of coma, the hospital said in a statement, adding that she was being treated at another hospital where her condition had deteriorated. The child was immediately put on a ventilator and advised a liver transplant. She was parallely put on dialysis and plasma exchange therapy to remove copper and buy time for a pre-transplant evaluation, the hospital added.

Due to non-availability of the same or compatible blood group donor, an ABO-incompatible emergency live donor liver transplant was planned, with the child's mother as the donor, it added.

"Within 36 hours of admission, on March 27, liver transplantation was done," Anupam Sibal, Group Medical Director and Senior Pediatric Gastroenterologist, said.

"The complexity of the case required us to support the child on ventilator immediately post her admission. Both her pre-surgical management and the seamless administration of the liver transplant surgery (with her donor being ABO Compatible) were a challenge," he said. "Of the 432 pediatric liver transplants performed at the Apollo Liver Transplant Programme, this is the first case of an emergency ABO-incompatible living related liver transplant," he added.

Two days later, the child was removed from the ventilator, and she regained consciousness.

eerav Goyal, senior consultant, Liver Transplant at Indraprastha Apollo Hospitals, said, "A rare complication, in case of Wilson's disease, children typically manifest deteriorating symptoms and if not treated within a specific duration can cause life threatening problems.

This child had come to us with acute liver failure, swelling in the brain due to ammonia content, was unconscious and in a state of coma.

"At Apollo, we immediately counselled the family for a liver transplant. Her recovery has been uneventful and the child was discharged on April 12, post-operative day 17," he said.

What is Wilson disease?

An inherited disorder that causes too much copper to accumulate in the organs.

In Wilson's disease, copper isn't eliminated properly and instead accumulates, possibly to a life-threatening level. Symptoms typically begin between the ages of 12 and 23.

Symptoms include swelling, fatigue, abdominal pain and uncontrolled or poorly coordinated movements.

Treatment often includes medication that can prompt the organs to release copper into the bloodstream. Once it's in the bloodstream, it can then be eliminated from the body through the kidneys.