Jeremy Laurance, Health Editor, reports on a remarkable alliance of parental determination and surgical skill.
The parents of Baebhen Schuttke had already suffered a double tragedy when she was born last July. Their two sons both died soon after birth from a rare condition that resulted in liver failure.
Baebhen had the same condition and collapsed with liver failure 24 hours after she was born. She was flown to King's College Hospital in London where doctors said an immediate transplant was necessary to save her life.
By chance, the liver of a 10-year-old child became available and surgeons led by Mohamed Rela arranged to conduct the delicate and complex operation three days later.
Yesterday, Ita, 30, and Jurgen, 33, presented their sleeping daughter to the press and declared that she was a "completely normal five-month- old baby".
Accompanied by their five-year-old daughter, Aodhbha, they thanked their doctors - Mr Rela was the "surgeon of the century", Mrs Schuttke said - and appealed for more donors.
Mr Schuttke, who is originally from Westphalia, Germany, said: "The main reason we decided to go public was to thank the donors. Mr Rela has done an excellent job but without the organs you can't do anything.
"A tragedy in somebody's life may bring someone else happiness. It has made our Christmas."
Behind the celebratory tone, however, lies a harrowing story. When their first son died in Dublin, where the family lives, Ita, who is Irish, and Jurgen, were distraught - and perplexed by the absence of any clear diagnosis.
They searched medical libraries and the Internet for clues. They found a specialist in Pittsburgh, in the United States, who suggested the diagnosis neonatal haemochromatosis, an extremely rare condition caused by an excess of iron whose cause is unknown, and told them that King's College Hospital in London had the expertise that they needed.
When their second son was born, he was flown to King's and had experimental drug treatment but it failed. When Baebhen was born last summer the Schuttkes, who have another daughter, five-year-old Ava, knew that a transplant was their only option. Ita said: "We didn't think whether she was the youngest or the oldest [transplant patient]. Saving her life was our main concern."
The seven-hour operation involved transplanting a single lobe from the donor liver, one eighth of the liver's total size, but even this was too big to fit into the baby's tiny abdominal cavity. For two weeks the surgical wound was left open, covered by a dressing, until the liver had naturally shrunk to fit.
Mr Rela said: "The liver is an amazing organ. It will shrink to fit or grow to fit whatever cavity it is placed in."
The liver is made up of eight segments and the first part of the operation, which has been pioneered at King's, involved reducing it to one segment, a process that took over two hours.
Once the liver segment had been prepared, a second surgical team transplanted it into the baby in a lengthy, delicate procedure in which the surgeons practised microsurgery under magnifiers to attach the tiny blood vessels.
No signs of rejection have been seen and Baebhen is taking only minimal doses of immunosuppressant drugs because, at such a young age, before the immune system is fully functional, foreign organs are not recognised as alien. The liver will grow with the child, removing the need for further transplants as she gets older, and doctors expect to be able to withdraw the drugs altogether by the time she is five.
Surgeons at King's have performed 13 liver transplants on children below the age of three months and five below one month. The hospital is the largest liver transplant centre in Europe, carrying out more than 180 transplants a year, including 50 in children.
Mr Rela added: "This is the youngest child to get a liver transplant because she was lucky enough to get a donor. She would have died if she had not got a transplant within two weeks. She has done very well, and the liver is now adapting to her body and is growing normally."Reuse content