News Release: University News

Aug. 21,  2009

Emory University Hospital Performs Georgia's First Domino Liver Transplant

Very rare organ transplant procedure used to treat separate disorders in two different patients - restoring quality of life in both

News Article ImageDomino liver transplant procedures are aptly named for the sequential, one-after-the-other nature of the process in which a viable liver from a deceased donor is transplanted into the first recipient, and the first recipient¿s organ is then transplanted into a second recipient. The procedure is still extremely rare. Video from press briefing -- doctor and patients discuss the story.

Transplant surgeons at Emory University Hospital are the first in the state of Georgia to perform a very rare domino liver transplant procedure. In effect, the procedure is saving two lives and curing one rare disease (Maple Syrup Urine Disease - MSUD) in a 24-year-old patient, and dramatically improving the quality of life in the other patient, who has suffered a lifetime battle with hemophilia, and more recently hepatitis C.

Patient Profiles:
Robert Massie and Jean Handler

Domino liver transplant procedures are aptly named for the sequential, one-after-the-other nature of the process in which a viable liver from a deceased donor is transplanted into the first recipient, and the first recipient's organ is then transplanted into a second recipient. The procedure is still extremely rare, with less than 100 done in the United States since the first in 1996. Outside of Florida, Georgia is the only state in the southern region (including Alabama, Louisiana, Mississippi, Arkansas, Florida and Georgia) to have performed the procedure.

The 10-hour procedure at Emory took place on July 10, and involved two separate surgical teams working in adjoining operating rooms. In fact, when factoring in the time to retrieve the deceased donor organ from another hospital, the dramatic procedure took more than 14 hours. Both patients still remain in Atlanta in order to be monitored for complications and signs of organ rejection, but are very close to returning home.  

According to Stuart Knechtle, MD, professor of surgery in Emory School of Medicine and director of the Emory liver transplant program, domino transplants are a rare but effective way of overcoming the national shortage of organs available for transplant. In most cases of domino liver transplants, one of the donated livers is transplanted from a patient with another type of disorder that does not affect the organ recipient.

"Domino transplants are still quite rare because, in part, there are few illnesses that may impact one liver, but if that organ were to be given to another donor, would fail to negatively impact the health or quality of life in the recipient. In the case here at Emory, one patient suffered from a rare disease called Maple Syrup Urine Disease, and it happens to be one particular challenge that would not impact the recipient," says Knechtle.

The MSUD that so badly impacted the donor will not be passed along to the other patient receiving the donated liver, Knechtle says. And because Maple Syrup Urine Disease does not exclusively affect the liver (the disease is rather caused by a lack of a particular enzyme in all cells of the body) the second patient will not acquire the disease since their non-liver cells make the enzyme.

"Both patients are now, in effect, living with new, perfectly functioning livers," he says.

Maple syrup urine disease (MSUD) is caused by a rare gene defect, and the patient's body cannot break down the branched-chain amino acids leucine, isoleucine, and valine - all found in most typical foods including even trace amounts of protein. This leads to a buildup of these chemicals in the blood. Over time and in its most severe form, MSUD can damage the brain during times of physical stress (such as infection, fever, or not eating for an extended period), and often causes slow or retarded mental development.

A special low protein liquid formula diet must be strictly adhered to and monitored over the life of a patient with MSUD.

In this case, a liver from a deceased donor was transplanted into Jean Handler, a 24-year-old from Boca Raton, Fla., who has suffered since birth with MSUD. Handler's liver was then transplanted into Robert (Bob) Massie, a 53 year-old from the Boston suburb of Somerville, Mass., who was born with hemophilia, and has suffered through a lifetime of pain and illnesses that accompany the rare disorder. In addition, he contracted hepatitis C and HIV in the early 80s as a result of donated blood products that had not undergone the same level of scrutiny or screening that is standard for today's blood donors, products, and recipients.  

According to Christopher Hughes, MD, one of the surgical transplant team members, liver transplants are typically one of the most challenging types of organ transplant procedures because of the incredibly vascular nature of the organ. The organ is connected by a large artery - the hepatic artery - and a network of large veins, including the portal vein. In this case, Hughes and his team needed to actually reconstruct the vascular pathways in Handler's liver.

"Usually in a liver transplant, a diseased organ is removed from the recipient without the attached veins and arteries, with the new donated liver being attached to the recipient using his or her own tissue. In this case, because we had two recipients, and one of the recipients in turn also becoming a donor, we had to ensure that there were enough vascular structures to reattach both organs," explains Hughes. "Because we had to leave Jean with some of her own vessels, we needed to reconstruct the vessels from her liver in order to transplant it into Bob."

According to Knechtle, a transplant procedure as still-rare and complicated as a domino procedure requires a tremendous amount of coordination, education, and skill to ensure the best outcome for both patients.

"This successful domino liver transplant is something that simply does not start or end in a hospital operating room," says Knechtle. "This procedure, which saved two lives, and will impact both families for many years to come, is the end result of a long chain of special events, starting with the decision by one person to donate the gift of life upon his untimely demise, which in turn allowed Jean to then donate to Bob. The incredible combined efforts of so many on the transplant team, including our surgeons, hepatologists, nursing staff, OR techs, anesthesia team, and outreach coordinators, truly makes today a special day in medicine at Emory."  

Patient Profiles

Robert (Bob) Massie
Age 53
Hometown: Somerville, MA

Bob Massie could write a book on overcoming adversity. In fact his parents Robert and Suzanne Massie did write Journey in 1975 detailing the difficulty of raising a child with a severe bleeding disorder.   Now a new, more positive chapter began on July 10 at Emory University Hospital in Atlanta, when Massie received a new donated liver as part of a unique medical procedure known as a domino transplant.

Massie was born with severe factor VIII hemophilia causing frequent internal bleeding. Over the course of his childhood years this would become a painful affliction, corroding and stiffening his joints.  "Growing up, even small childhood injuries could be of great concern for me," says Massie. "Many times, my parents would need to rush me to a hospital in order to seek treatment, so we were tethered to hospitals and doctors when I was a child."

When he was 12 years old, plasma-based concentrates that could be kept at room temperature became available to treat conditions like Bob's, allowing him to live a more typical childhood.

After graduating from Princeton University in 1978, Massie was hospitalized with an undiagnosed illness while a graduate student at Yale Divinity School. It would not be until 1984 that he learned he had contracted the HIV and Hepatitis viruses from contamination in the same plasma products he had relied upon for so many years to maintain his life.

Massie says "I had a great upbringing from my parents and I relied on a strong faith in God. I made the decision to live life as fully as possible and to keep working for those things I believed in."

Living life to the fullest for Bob included being the director of a national environmental organization, an author, a professor at Harvard, a minister and a candidate for public office - he won the Democratic primary for Lt. Governor of Massachusetts in 1994.  He is the father of three children - two sons and a daughter.

While Massie has never developed AIDS, he is an "elite controller", he finally succumbed to Hepatitis C in 2002, which over the course of the last seven years has slowly destroyed his liver.

"The same incredible immune system that helped me to survive for so many years, and even stave off the AIDS virus, finally let me down with hepatitis C. But this transplant has literally cured the hemophilia and cirrhosis. I feel hopeful about the future.

"We are enormously grateful to Jean Handler, her family and the extraordinary team at Emory Hospital for this incredible gift which has already changed my life in ways I never would have imagined."

Jean Handler
Age 24
Hometown: Boca Raton, FL

Within hours of her birth in South Florida, Jean Handler's parents Kathie and Henry knew that something was simply not right with their new baby because of the unusual sound of her crying. Their fears and suspicions were confirmed within two days when the baby simply would not eat.

After many weeks and doctor visits, the diagnosis was in - Maple Syrup Urine Disease.

"Unfortunately, more than two decades ago, most hospitals did not screen for many genetic abnormalities - if any," says Kathie. "Early diagnosis of MSUD is essential to prevent neurological damage and death in infancy. In Jean's case, however, it took many weeks, after visits to pediatricians and experts across the state before the disease was finally confirmed. By that point, however, Jean's situation worsened severely to the point her stomach had closed completely. We were lucky that Jean's condition was diagnosed before we lost her. After that, we learned how to live with this disease, and how to provide the very best care for her possible, knowing there would be challenges."

Maple syrup urine disease (MSUD) is caused by a rare gene defect, and the patient's body cannot break down the branched-chain amino acids leucine, isoleucine, and valine - all found in most typical foods including even trace amounts of protein. This leads to a buildup of these chemicals in the blood. Over time and in its most severe form, MSUD can damage the brain during times of physical stress (such as infection, fever, or not eating for an extended period), and often causes slow or retarded mental development.
A special low protein liquid formula diet must be strictly adhered to and monitored over the life of a patient with MSUD.

Through her early childhood, Jean managed to function well, but soon she would endure the age-old rite of passage of school children everywhere, when she learned to endure the teasing from kids because she was a little "different."

"It was kind of tough because I was sick a lot, and the kids always wanted to know why I had to eat differently. But I had a lot of good friends, and every year I attended metabolic camp at Emory - still do," says Jean. "But I have also lost some friends from similar diseases, and have seen others suffer. It has definitely inspired me to do everything I could to survive."

That included researching, with her parents, similar cases and treatments, where they learned about the possibility of transplant.

"By the time I was in high school, the disease was really starting to impact me to the point that I was having frequent seizures, memory loss, difficulty concentrating. During the seizures, I would literally freeze up, my eyes would cross, and every muscle in my body would freeze," says Jean.

The seizures were the result of too much leucine in her body, which would require an infusion of the liquid formula that had been a staple of her life for so long. Ultimately, the disease would cause Jean to stop being functional, and possibly face an early death.

"This transplant is a wonderful gift for me, as well as Bob. I have my life back now, and look forward to someday helping other kids with similar challenges to those I have faced," says Jean. "I look at this as an opportunity to really give back from my own experience. I would like to stay in Georgia and eventually attend college here in the Atlanta area where I have a lot of good friends."

What does she think of her liver now residing in another person's body?

"I think it's great. I am so happy for Bob and that he'll get to spend time with his children. He has suffered for so long, and I am just glad I was able to help in the same way someone helped me."

And now that she is finally able to eat solid foods again, what is her favorite dish? Steak? Hamburgers? Pizza?

"Ice cream!"

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