Teen with rare disorder undergoes West India's first auxiliary liver transplant

After being confined to home since birth, a 14-year-old Oshiwara boy suffering from a rare genetic disorder underwent the first auxiliary liver transplant in Western India at Global hospital in Parel.

Yash Ranjit had Crigler-Najjar syndrome (CNS), which affects 1 in 1 million newborns worldwide. It's a severe condition where a high level of toxic substance bilirubin is found in the body. "CNS is divided into two types. This child had CNS Type 1 which is very severe. CNS is an inherited disorder in which bilirubin — made by the liver — cannot be broken down," said Dr Ravi Mohanka, chief liver transplant surgeon, Global hospital.

He added that CNS occurs when the enzyme that normally converts bilirubin into a form that can easily be removed from the body does not work correctly.

"Without this enzyme, bilirubin can build up in the body and lead to jaundice and cause damage to the brain, muscles and nerves, and even loss of life in extreme conditions," said Mohanka.

According to doctors, in Yash's case, CNS was diagnosed when he was 14 days old. He was on photo therapy for which his parents imported a photo therapy machine. "Alternative to liver transplant is photo therapy for treating this disorder. Yash had to undergo daily photo therapy for six hours a day, which increased to 12-14 hours a day as he grew up, to keep his bilirubin under control. If not, he would have suffered brain damage," said Mohanka. The photo therapy sessions led to Yash living indoors.

It was an episode of seizure that made Yash's parents go for a liver transplant. With the parents' livers not matching with Yash, his aunt agreed to donate a part of hers. "We decided to go for auxiliary liver transplant, where we remove a part of the liver and replace it with a part of the donor's liver. Once the patient's own liver regenerates, the need for the donor liver no more exists. In most cases, the donor liver shrinks and becomes a small tissue. In some, another surgery is needed to remove it," said prof Mohamed Rela, group director, Institute of Liver Diseases, Global hospital.

Yash underwent surgery on May 28 and got discharged on June 10. "He is free of the disease and can lead a normal life. With the successful transplant, the other part of his liver has started producing the desired enzyme and his liver function has become normal. He will need to be on immunosuppressant medications," said Mohanka.