Jan. 13, 2000 (Boston) -- With typical New England thrift, surgeons from the Lahey Clinic in Burlington, Mass., last week performed two rare "domino" transplants, implanting livers from two cadavers into two patients with liver damage from a metabolic disease. The diseased but still functional livers removed from the recipients were then given to two other patients with livers irreparably damaged by hepatitis C infections.
The novel domino transplant technique, which has been performed only about 40 times throughout the world, holds the promise of a new lease on life for patients who might otherwise not be good candidates for liver transplants, and at least partially addresses the critical problem of a shortage of donor organs, says Frederick Gordon, MD. Gordon is the director of hepatology and medical director of the liver transplant program at the Lahey Clinic.
"The number of patients on the transplant waiting list is increasing exponentially, but if you look at the number of donors nationally and in New England, it's the same thing year after year," says Gordon in an interview with WebMD. "Any way that we can increase the donor pool is beneficial, whether it's with domino transplants, living donor transplants, or split livers, it can certainly have a positive affect upon the organ supply."
The donor-recipients were a 66-year-old man and a 28-year-old woman with familial amyloid polyneuropathy (FAP), a rare inherited disorder in which a genetic mutation within the liver causes the organ to manufacture a mutated version of a normal protein called amyloid transthyretin (ATTR). The mutated protein builds up within the liver, nervous system, and other organs, causing numbness and tingling in the arms and legs, dizziness on standing, and diarrhea, among other symptoms. Removing the liver and replacing it with a donor organ -- the only treatment known to be effective for FAP -- removes the source of the mutated protein and allows the donor liver to manufacture the normal protein, thereby halting disease progression. It is still not known, however, what happens to existing ATTR deposits in the bodies of patients with FAP.
At last report both the patients with FAP and the recipients of their organs -- both men with hepatitis C, one age 53 and the other age 68 -- were doing well, says Dave Lewis, MD, director of Lahey's liver transplant program and associate professor of surgery at Tufts University School of Medicine in Boston.
Although some people might question whether it is ethical to give patients with a critical illness a "diseased" organ, there is good therapeutic rationale for the experimental surgery, says Martha Skinner, MD, an expert on FAP and other amyloid protein abnormalities.
"The livers taken out of these patients are very good looking livers; they don't have the usual end-stage liver disease like people who usually get liver transplants," Skinner, professor of medicine and director of the amyloid program at Boston University School of Medicine, tells WebMD.
She says that although it is still unknown how livers from donors with FAP will affect the recipients, "these people are not ideal candidates for transplants because of problems like hepatitis C that would make a donor liver more likely to fail, so they may not be at the top of the transplant lists, but this clearly is giving several years of life to these people that they would not have."
Bioethicist Jeffrey Kahn, PhD, MPH, tells WebMD that for patients with hepatitis C or other life-threatening illnesses, the prospect of receiving a less-than-perfect liver almost certainly beats the alternative. "Why limit this to patients with hepatitis C? There are others waiting for liver transplants who probably will die if they don't have one. Would they not rather accept a liver with this inborn error of metabolism than none at all?" says Kahn. He is director of the Center for Bioethics and an associate professor in the department of medicine at the University of Minnesota Medical School in Minneapolis.
"There is already a 'second-tier market' in livers from people who are hepatitis C-infected but don't even know it when they die," Kahn notes. "So those livers will not be transplanted into healthy people, but they will be transplanted into people with hepatitis C who need a liver transplant, which in a way expands the pool of useable organs." A liver from someone with hepatitis C can function for many years before failing.
Lewis tells WebMD that all patients involved are likely to benefit from the domino procedure. "To my knowledge, the longest-surviving domino FAP liver recipient is about two-and-a-half years out and has not developed, to this point, any symptomatic amyloidosis," he says.
He also tips his surgical-scrub cap to the patients with FAP for their willingness to undergo a difficult procedure -- a typical liver transplant can last up to 16 hours -- while helping others at the same time. "Neither of the amyloid patients even blinked when we presented this whole science-fiction kind of scenario to them," he says. "Both of their responses were essentially 'I don't want this thing any more and if someone else can use it, please, please give it to them.'"