India’s first domino liver transplant saves lives of two kids

1. Doctors remove 20 per cent of Mukta's liver. This portion is transplanted into Shourya, who suffered from Maple Syrup Urine Disease.
2. Shourya's liver in turn given to Siya. The girl was battling terminal liver failure due to a rare condition called Langerhan's Cell Histiocytoma (LCH).
3. Siya is India's first and the world's youngest recipient of a domino liver transplant. She is also the first kid to be cured of LCH with a transplant.

While 22- month- old Shourya Verma was cured of the Maple Syrup Urine Disease ( MSUD) — a metabolic disease — by a transplant performed using 20 per cent of his aunt Mukta’s liver, his own liver was, in turn, transplanted into two- year- old Siya Thakur. Siya was battling terminal liver failure due to a rare condition called Langerhan’s Cell Histiocytoma ( LCH).

The unique transplant has also made Shourya India’s first

and the world’s youngest recipient of a living donor liver transplant for MSUD, and Siya the country’s first and the world’s youngest recipient of a domino liver transplant. Siya is also the first child to be cured of LCH with a transplant.

A team of 20 surgeons and anaesthetists led by Dr A. S. Soin, the chief liver transplant surgeon and director of liver transplantation, performed the surgery on January 31. The surgery took more than 16 hours.

Siya’s mother Swati Thakur said: “ My child has got a new lease of life. She was diagnosed with the disease when she was 19 months old. She was suffering from a cancer, which has a different type of protocol. It was a big challenge for us but we derived strength from God and our family.

It’s good to see her eat food of her choice,” she said.

Domino transplants are rare because there are very few conditions in which you can cure the patient with a transplant and then transplant his or her organ into someone else without passing on the disease. MSUD is one such disease and Shourya was suffering from it since birth.

In this disease, toxic levels of branched chain amino acids called leucine, isoleucine and valine build up due to the lack of the detoxifying dehydrogenase enzyme. Soon after birth, such children develop metabolic crises with vomiting, poor feeding, weight loss, convulsions and coma.

“ Before the operation, Shourya suffered from such attacks until his parents Niti and Prashant Verma moved to the US and put him permanently on the MSUD diet. Apart from the difficulties in procuring the diet and measuring amino acid levels in India, it was too restrictive on Shourya’s life. His family then decided to go ahead with a liver transplant and his aunt Mukta was found to be a suitable donor,” said Dr Neelam Mohan, the chief pediatric hepatologist.

High levels of acids in the urine result in a smell of burnt sugar, hence the name Maple Syrup.

To survive, patients need a special diet that is low in protein and devoid of amino acids, along with regular monitoring of leucine levels.

MSUD is caused by lack of an enzyme in the body, so the patient who receives a liver transplant is able to make enough of the enzyme in the new liver to overcome the shortage elsewhere in the body. In turn, an MSUD patient’s liver — which is otherwise normal — can be transplanted into someone else without passing on the disease because that patient’s other cells still make the enzyme lacking in the liver.

“ The domino procedure requires careful planning for several weeks.

This surgery was crucial as it had never been performed on such small children. For the surgery, we divided ourselves into three teams and prepared Mukta’s liver, Shourya’s liver and Siya’s diseased liver for removal sequentially in three separate operation theatres,” said Dr Soin.

“ First the donor liver and then Shourya’s liver were removed and prepared for transplantation by re- constructing the vital blood vessels with complex vein grafts.

Shourya was given Mukta’s liver first, and then his liver was transplanted into Siya. Both transplants kicked in immediately and the children are making excellent progress,” Dr Soin added.

The biggest challenge was to control amino acids, glucose and sodium levels in blood before and during surgery until the new liver started working.

“ We specially arranged to measure the amino acid levels at our hospital and Shourya’s father Prashant arranged special oral and intravenous MSUD diets. But the boy won’t need any of that now. It is such a joy to see Shourya on normal diet for the first time in his life,” Dr Mohan said.

Siya underwent many chemotherapy sessions to contain the LCH disease. Though the disease went into remission, her liver was damaged irreversibly, due to which she needed a life- saving transplant.

The two- year- old girl had become very weak due to the side- effects of chemotherapy and making her fit for the transplant was a challenge. “ Swati wanted to give a part of her liver but Shourya’s liver could be successfully used in this unique domino operation,” added Dr Mohan.