When Emma Lowney was diagnosed with jaundice a day after her birth last December, her parents assumed it would quickly vanish on its own like it did in Emma’s older brother, Henry. Instead, Emma turned out to have a rare life-threatening liver disease called biliary atresia, and she recently celebrated her first birthday waiting for a liver transplant.
“The odds of Emma making it to age 2 without a transplant are very low,” said Dr. Maureen Jonas, the girl’s doctor and medical director of the liver transplant program at Boston Children’s Hospital.
Jonas and the rest of the medical team hope the toddler, who’s been on the waiting list since April, will soon get a portion of a liver by means of a relatively new procedure — called a split liver transplant — that is aimed at spreading the limited supply of organs among more recipients. The organ from a deceased donor is sliced in two, with the larger portion going to a teen or adult and the smaller slice to a baby with a liver disease such as biliary atresia. The liver is the only vital organ in the human body that can regenerate and grow to full size, which also enables living donors to give a piece to a child in need.
But the number of these transplants is small, and surgeons pushing to do more of them on children are meeting resistance from adult transplant doctors.
Biliary atresia, a rare condition affecting just 1 in 18,000 newborns, occurs when the bile ducts in the liver become inflamed and scarred, which shuts their normal openings; this causes the digestive fluid to become blocked in the liver, leading to irreversible damage to the tissues and eventual liver failure.
About three-quarters of the 250 American infants born with this condition every year require liver transplants by the time they reach nursery school age. Boston Children’s began offering split liver transplants from deceased donors in 2001. “We now do about 7 to 10 of these procedures every year,” said Dr. Heung Bae Kim, director of the hospital’s pediatric transplant center. He’d like to do more to avoid having to turn to living donors as a last resort, because such donors risk serious liver complications.
Increasing the number of split liver procedures performed nationally from 100 to 200 a year, Kim said, would save the life of virtually every child waiting for a new liver — while accounting for less than 4 percent of total liver transplants. Armed with new studies highlighting the safety and effectiveness of the procedure, Kim and other pediatric surgeons have been pushing to change how livers are allocated through the United Network for Organ Sharing, a nonprofit group that works with the federal government.
They are trying to make the case that healthy livers from young deceased donors should be split whenever possible to save two lives instead of one. Adult transplant surgeons, however, have pushed back against such policies, because organ retrieval and transplantation are less complex when using whole livers.
“Surgeons aren’t turning down split livers for adult patients,” Kim said, “but if their patient is allocated a liver and has the potential to give a piece to a child, that almost never happens. They don’t want to split when they’re offered the whole organ first.”
Transplant surgeons prefer to use a whole liver for adult patients largely because early studies evaluating the procedure more than a decade ago found that those who received split livers were more likely to experience a failure of the organ within a few years after the transplant compared with those who had a whole-
liver transplant. Young children, on the other hand, must receive a small section of a liver to match their body size when pediatric donors are not available.
The tide may be turning, however, in favor of performing more split liver procedures. In an August study published in the Journal of the American College of Surgeons, Kim and his colleagues reviewed the medical records of more than 62,000 adult liver transplant recipients and found that those who received split livers from 2002 onward had only a 10 percent higher risk of losing the organ compared with those who received whole livers. Patients who received split livers from 1995 to 2002 faced a 45 percent higher likelihood of losing the transplanted organ. Children who received split livers had no increased risk compared with those who received a trimmed whole liver where surgeons can pick the best blood vessels to keep, according to a July study published by the same researchers.
Two-year-old Ian Rutherford and 18-year-old Anthony Cote share a liver received from the same deceased donor in May 2012. “We call them liver brothers,” said Anthony’s mother, Melissa Gould, of Winterport, Maine. The teenager and then-8-month-old baby bonded while recuperating at Children’s Hospital after their surgeries were performed on the same day. Cote, who developed liver cirrhosis in his teens as a result of being born with the lung disease cystic fibrosis, is now majoring in business management at Eastern Maine Community College.
“He’s been extremely well,” Gould said, “with a few bumps here and there that has to do with getting his medications regulated.” The anti-rejection drugs that Cote takes to keep his immune system from attacking the donated liver also make him more susceptible to life-threatening lung infections that often occur with cystic fibrosis.
Rutherford has been “running around the house like a regular 2-year-old,” said his mother, Heidi Thoe, who is expecting her second child. He’s tolerating the immunosuppressive drugs well and has only needed regular checkups. “His doctors predict he’ll be fine until adulthood,” Thoe said, though they worry about future side effects from his long-term use of the immunosuppressive drugs.
Like with adults, children who have the greatest risk of dying in the next 90 days are placed at the top of the liver transplant waiting list, and the list changes day by day depending on the ups and downs of patients’ health status. While the organ-sharing network encourages surgeons to split healthy livers in two, they have no policies mandating that all qualifying organs be shared upon retrieval — which many pediatric transplant surgeons would like to see implemented.
That, however, will likely never happen for practical reasons. “I really don’t believe there are enough surgeons with the kind of expertise needed to split a liver during the retrieval process,” said Dr. Goran Klintmalm, chairman and chief of Baylor University’s Annette C. and Harold C. Simmons Transplant Institute in Dallas. Transplanting a divided liver into an adult is a far more complicated surgery that requires additional training for the surgeon and more time on the operating table for the patient.
While conceding that split liver surgeries are highly successful when performed in the right hands, Klintmalm said a national mandatory policy would be unsafe. “Instead of getting two livers, we could lose two patients.”
About 482 children in the United States are currently waiting for a liver transplant, according to the latest statistics from the organ-sharing network. “Children die on the waiting list,” Jonas said. But, she concedes, that rarely happens because they can also receive a segment of liver from a living donor, often a parent in good health with the same blood type.
Baby Ian had the option of receiving a piece of his father’s liver, but a deceased donor’s liver became available before he urgently needed it. “My husband had been willing to donate,” Thoe said, “but Ian’s doctors told us the last thing they wanted was to put two lives in jeopardy with the surgery. We’re glad it never got to that point.”
Emma could also potentially receive a transplant from her father if a split liver doesn’t become available within the next few months. “It’s a possibility that we would entertain if we need to further down the road,” said her mother, Melissa Lowney.
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