Kashmir boy with rare disorder gets new liver

NEW DELHI: Faisal Farooq Shah, a 15-year-old boy from Srinagar, was extremely excited as he tasted an egg for the first time in his life just two days ago. He was just four when doctors diagnosed him with Type I citrullinemia, a rare disorder that causes ammonia and other toxic substances to accumulate in the blood.
When he was younger, Shah would vomit a lot and was slower to develop compared with other children.The disorder is seen in 1 in 60,000 children, said Dr Neelam Mohan, director, department of paediatric gastroenterology, hepatology and liver transplantation at Medanta Medicity Gurgaon.
“In these cases, patients lack a specific enzyme in the liver that helps metabolise amino acids. So, if they consume food rich in protein, it leads to accumulation to of ammonia in blood and further causes brain edema or fluid build-up” she added.
Dr Mohan said Faisal had been on a protein-free diet, like rice, vegetables and potatoes, ever since he was diagnosed. “Recently, his family approached our hospital with a request for liver transplant. Shah said he yearned to have normal food like his friends,” the doctor said.
As there is no other cure for citrullinemia and sustaining life on a protein-free diet is tough, Dr Mohan said they agreed to the procedure. Shah’s father donated part of his liver and the transplant took place on June 27.
“The surgery is tricky in such cases. The fasting period needs to be minimised, hypothermia has to be prevented and special drugs are needed as ones with proteins must be avoided,” said Dr A S Soin, chairman and chief surgeon of the Medanta Liver Institute, who operated on Faisal. “He is doing well post-transplant.”
Zahoor Ahmed, Faisal’s uncle, said the 15-year-old finally got to eat eggs. “Yesterday, he also had some chicken,” he said.