Rare surgery saves kid’s life

In a rare surgery — only the second time in the world — surgeons at Apollo Hospital used the appendix of a child to treat Byler’s Disease, a genetic disorder that causes the buildup of bile in the body, leading to liver failure, reports Sanchita Sharma.

In a rare surgery — only the second time in the world — surgeons at Apollo Hospital used the appendix of a child to treat Byler’s Disease, a genetic disorder that causes the buildup of bile in the body, leading to liver failure (cirrhosis).

In such cases, liver transplantation, a major surgery, is the only treatment.

Four-and-a-half-year-old Triveni Pawar had come to Apollo from Jodhpur last November with an itch so acute that her whole body was covered with welts caused by scratching. Even tufts of hair had fallen out because of secondary infections on scratch wounds on her scalp. “The itching was so bad that she couldn’t sleep, eat properly or go to school. She didn’t wear clothes at home because it irritated her skin,” said mother Gunjan, a housewife.

Triveni was born with Byler’s Disease (also known as progressive familial intrahepatic cholestasis), the genetic disease which is rare in India though fairly well documented in the US.

Bile — which helps the body digest fats — is produced in the liver from where it goes to the gall bladder and eventually into the intestines. In Triveni’s case, the bile did not get drained from the liver cells into the intestine. To drain the excess bile, surgeons used her appendix to create a bypass from her gall bladder to the surface of the skin through a 2-mm hole on her right abdomen just below the panty line.

“The surgery is conventionally done using a section of the small intestine to construct a bypass for the bile from the gall bladder to the large intestine or the skin surface. For us, the appendix was a natural choice because it has no function in the body, is narrower than the intestine and so needs a smaller opening (stoma) on the skin’s surface, and best of all, it prevented surgical shortening of the intestine,” said Dr Sujit K. Chowdhary, senior consultant in paediatric surgery at Apollo.

“She was cured by a relatively simple surgery. If her condition had not been treated, she would have needed a liver transplantation, a major surgery for both donor and recipient. She would also have had to take drugs to suppress her immunity all her life to prevent the rejection of the transplanted liver,” said Dr Anupam Sibal, senior consultant paediatric gastroenterologist at Apollo who is treating her.

Three months on, Triveni looks like any other girl her age. “We have rediscovered what it feels like to sleep through the night. All these years, the itching kept her up,” said father Rajesh, an advocate.