Neonatal hemochromatosis

A 5-day-old baby who received a new liver cut down to one-eighth its original size has become the world's youngest liver transplant patient.

Dark-haired Baebhen Schuttke, born with a disease that killed her two brothers, laughed and gurgled Monday through a news conference called to announce the operation, which took place in July.

"She's just like any normal baby . . . it's almost easy to forget how close we came to losing her," said her Irish mother, Ita Schuttke. Ita Schuttke, 30, and her German husband, Jurgen, 33, were referred to King's College Hospital in London, the largest transplant center for children in Europe, as first one son then another died. Two days after her birth in Dublin, Baebhen was diagnosed with the same condition, neonatal hemochromatosis, which causes a buildup of iron in the liver. She was flown by air ambulance to King's College Hospital. There, doctors told the Schuttkes there was a chance for Baebhen. On the fifth day of her life, the family of a 10-year-old boy who died outside London gave permission for the liver transplant. "This is the youngest child to get a liver transplant because she was lucky enough to get a donor," said Mohammed Rela, the surgeon who led a 20-member medical team that performed the six-hour operation. "She has done very well, and the liver is now adapting to her body and is growing normally. She will not need another transplant when she is older," Rela said. Baebhen whose name is Gaelic for "sweet, beautiful woman and melodious" takes a light dose of anti-rejection drugs. The Children's Hospital in Pittsburgh, the biggest pediatric transplant center in the United States, said the youngest baby given a liver transplant there was 2 weeks old. Younger children have received heart transplants. Canadian Paul Holc, now 10, received a heart at Loma Linda University Children's Hospital in California three hours after birth. During the surgery at King's College Hospital, one group of doctors took two hours to prepare a 4-by-3-inch section of liver for the transplant. Other doctors removed Baebhen's diseased liver. The cut-down liver still was slightly too big for the 6-pound infant, hospital spokesman Nick Samuels said, and for a few days "the wound was left open while the liver adjusted to the size of the patient." "We want to thank them (the doctors) from the bottom of our hearts," said Ita Schuttke. "Because the donor is anonymous, it is impossible to thank them for what they have done. But we would like to give our heartfelt gratitude to donors of all organs." The Schuttkes also have an elder daughter, Aodhbha, 5, who was born healthy.

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