Pune: Suffering from rare metabolic disease, toddler gets new liver

Swara Shinde, parents Suhas, Reshma and Dr Bipin Vibhute. Express

Swara, a two-and-a-half-year-old girl who was suffering from a rare metabolic liver disease, progressing kidney failure and a hint of liver cancer, received a liver transplant at Sahyadri Hospital recently. But two weeks after the transplant, she had a heart attack.

Her father, 29-year-old Suhas Shinde from Chikhali village in Sangli, never gave up hope that she would survive. “My daughter is a fighter,” Suhas said, fighting back tears and recalling the ordeal.

Suhas’s wife Reshma, who donated part of her liver, said they were clueless about Swara’s medical condition and ran from pillar to post till doctors at Sahyadri Hospital diagnosed her condition as tyrosinemia, a rare metabolic liver disease that causes liver failure and kidney failure if not corrected in time.

Dr Bipin Vibhute, head of liver and multi-organ transplant department at Sahyadri Hospital, said Swara had developed signs of advanced liver failure at 1 year of age with ascites (accumulation of fluid in the abdomen), jaundice and failure in gaining weight.

Her condition deteriorated when the body became deficient in vital electrolytes and nutrients like sodium, potassium, calcium, magnesium, phosphorus and vitamin D. Because of this, she developed deformities of all the bones, mainly of the upper and lower limbs and the chest leading to multiple fractures, Vibhute said.

“But Swara’s father was very determined that his daughter should survive and travelled to several cities to raise funds for the medical treatment,” said Dr Snehawardhan Pande, paediatric hepatologist at Sahyadri Hospital.

At two-and-a-half years of age, Swara was very sick, weighing 5.9 kg with deformed arms, legs and chest. She was extremely irritable with a distended abdomen, severe dehydration and pneumonia, doctors said. She was admitted in the paediatric ICU, where she was treated “aggressively”, doctors said.

According to Dr Sheetal Mahajani, director, hepatology and GI endoscopy department, Swara underwent a liver transplant in November last year after 10 days of hospitalisation and stabilisation.

The surgery lasted for 14 hours, doctors said.

“Post transplant, she developed severe acute rejection due to the body’s immune mechanism, which tried to reject the new liver. This was managed by medication and other immune suppressants. She was stable but developed high grade fever due to an abdominal infection, which did not seem to be responding to any antibiotics. Her condition worsened one day when she went into shock and had to be put on the ventilator. It was a tricky situation for the transplant team in balancing the doses of immune suppressive medications on one side, which lowers immunity, to protect the new liver from the rejection and the antibiotics, which increase the body’s immunity to fight the infection,” said Mahajani.

Dr Ketan Apte, Sahyadri Hospital, said their dedicated unit for paediatric transplants provides financial assistance to economically challenged patients under an initiative.