Every year dozens of infants and young children in need of a liver transplant die on the organ donation wait list because a donor organ small enough for their body didn’t become available in time. But there is hope—a surgical technique that lets surgeons split donor livers into two unequally sized segments, a small one for infants and a larger one for older patients. If the process were adopted more widely it could save many of these children, just as it saved Reagan.
An uncertain beginningA young Reagan and Dr. Kim
When Reagan Devlin was born in 2004, her parents Teresa and Bryan were overjoyed. But that joy quickly turned to concern when nurses noticed her dark complexion and irregular heartbeat. The family was transferred to a nearby pediatric hospital in their home state of Florida, where the skin around Reagan’s lower abdomen started to turn greenish in color, leading doctors to believe she had a rare liver disorder called patent ductus venosus. Because the condition is seen so rarely only a few medical centers have any experience in treating it, but doctors from Boston Children’s Hospital’s Vascular Anomalies Center are considered world experts in these types of diseases. Within 48 hours, the Devlins were boarding a flight to Boston.
But just a few hours after meeting their new care team at Boston Children’s, the Devlins received yet another shock: the disease Reagan had traveled hundreds of miles to receive care for wasn’t to blame for her failing health. After closely reviewing her case and speaking with colleagues, Maureen Jonas, MD, director of Boston Children’s Center for Childhood Liver Disease, determined that Reagan actually suffered from neonatal hemochromatosis—a liver condition even more rare than her original diagnosis. Unfortunately, this only increased the urgency of her case. The family was quickly introduced to Heung Bae Kim, MD, director of Boston Children’s Pediatric Transplant Center, to discuss next steps.
“After talking with Dr. Jonas and looking over her charts, Dr. Kim said he didn’t think Reagan would live more than 48 hours without a liver transplant,” Teresa remembers. “Everything was happening so fast it was hard to process, like a bad dream.”
Saving two lives with a single organReagan and her brother
Because she was so sick, Reagan was fast tracked to the top of the organ transplant wait list. Even with elevated status, Reagan faced a serious hurdle to treatment: her size. Reagan not only desperately needed a new liver, but she also needed one that would fit inside her infant body.
Most donated organs (also called grafts) come from adults, so when an infant or small child requires a transplant they typically have to wait for a size-appropriate graft to become available, often increasing their wait time and seriously endangering their already fragile health. In Reagan’s case, it was time she simply didn’t have.
However, as Kim explained to the Devlins, there is an alternative for children in Reagan’s situation.
When an infant is experiencing acute liver failure and a size-appropriate graft may not become available in time, a specially trained surgeon can split a donated adult liver into two unequally sized segments—giving the smaller portion to the infant and the larger segment to an older patient. Because the liver has the ability to regenerate (re-grow) these partial livers eventually grow larger, taking on the work normally done by a whole liver.
Fortunately for the Devlins, Kim and his team had been performing split liver transplants since the early 2000s, so they were readily equipped to accept the first donor liver to become available, regardless of its size. Less than 24 hours later, an adult liver was available and Kim was rushed to the donor hospital. After a difficult four hour operation, the liver had been split and the larger portion was reallocated to an adult patient while Kim returned to Boston Children’s to transplant the smaller segment into Reagan.
The operation was a success, and couldn’t have happened any sooner. “When Dr. Kim took Reagan’s liver out, he said it was all but dead. He described it as being pitch black, just like a crayon,” Teresa recalls. “But he performed everything just in time and she made a full recovery. Everything occurred just in the knick of time.”
Split livers present opportunity for young children in need
Split liver transplantation presents an opportunity to save both an infant as well as a larger patient with a single organ and one would expect that the practice would be universally accepted. Unfortunately this is not the case, partially because of outdated information that still causes some in the medical community to question the procedure’s effectiveness.
From the mid 1990s to the early 2000s, when the split liver transplantation was in its infancy, graft failure was a real threat for recipients of partial pediatric livers. However, thanks to the increased experience of the surgeons who perform split liver transplantation, such risk no longer exists. This was recently demonstrated in a new study from investigators at Boston Children’s who showed that since 2006, partial liver transplantation has become just as safe as whole liver transplantation in children.
“Our research shows that these days young patients who receive a partial graft face no greater risk of graft loss than a patient who receives a whole one,” says Kim, also one of the paper’s senior authors. “Based on these data, more donated livers should be considered for being split. Doing so could significantly reduce the number of young children on the waitlist for a liver, possibly even reducing the number of infants and small children who die on the waitlist to near-zero.”
“Based on these data, more donated livers should be considered for being split. Doing so could significantly reduce the number of young children on the waitlist for a liver, possibly even reducing the number of infants and small children who die on the waitlist to near-zero.”
Reagan is living proof of the power of split liver transplantation. Once a critically ill baby with just hours to live, she’s now a happy, healthy 8-year-old who wants to be a nurse when she grows up. She and her family still travel to Boston once a year to be seen by Kim and her liver transplant team, and they usually time the visit to coincide with the New England apple picking season in early October. (Reagan is a big fan of apple picking!)
“These days, Reagan is just so full of life,” Teresa says. “She’s smart, compassionate and funny. It’s amazing to see how far she’s come when you consider how close we were to losing her. We can’t thank Dr. Kim, Dr. Jonas and the rest of her team at Boston Children’s enough for their dedication to split liver transplants. It saved our daughter’s life and we’re hopeful more adult livers become available for splitting so doctors can help other families the way we were.”