Rare surgery optimizes limited organ supply
Russell Ward greets Donald Guyton, who recently donated his liver to Ward.
The University of Maryland Medical Center is the first in Maryland to perform a "domino" or sequential liver transplant, an unusual procedure in which one man received a new liver from a deceased donor to cure a paralyzing disease and then his liver was transplanted into another man suffering from liver failure. Both men are recovering well from their simultaneous transplants, which began in the morning of May 19, 2005, and ended 18 hours later.
The first patient, 64-year-old Donald Guyton of Middletown, Md., had a rare, genetic liver disease called familial amyloidosis, also known as familial amyloidotic polyneuropathy, which gradually causes paralysis throughout the body. The only effective treatment is a liver transplant. While the man's native liver produced an abnormal protein, it functioned well otherwise and would not cause symptoms of the disease in another person for at least 30 years.
After surgeons at the University of Maryland Medical Center gave Mr. Guyton his new liver from a deceased donor, his original liver was implanted in 74-year-old Russell Ward of Salisbury, Md. Mr. Ward was very ill from another type of liver disease and, due to his age and condition, had a slim chance of receiving a conventional liver transplant from a deceased donor.
"The domino procedure is technically challenging and requires good coordination of two transplant teams. It takes much longer than a single transplant and is more complicated because the liver from the patient with amyloidosis will need to function well in a second patient," says Benjamin Philosophe, M.D., Ph.D., head of the University of Maryland Medical Center's Division of Transplantation and associate professor of surgery at the University of Maryland School of Medicine.
"At the same time, the domino procedure enables us to help two very sick patients and make efficient use of the limited number of donor organs," says Dr. Philosophe, who led the two transplant teams. Other team members included transplant surgeons Luis Campos, M.D., and Eugene J. Schweitzer, M.D., and anesthesiologists Obi R. Udekwu, M.D. and Vadivelu Sivaraman, M.D., M.B.B.S., all of whom are on the faculty of the University of Maryland School of Medicine.
Dr. Philosophe says the surgeons who removed the liver from Mr. Guyton had to cut the connecting blood vessels in a way to provide enough length to attach the liver he received from the deceased donor. At the same time, they needed to make sure the vessels from the amyloidotic liver would be long enough when they were attached to Mr. Ward.
Mr. Guyton, the liver transplant recipient/donor, has been an electrical contractor for 40 years. He says he was diagnosed with familial amyloidosis last September after DNA testing. There was no known history of the disease in his family, so when symptoms began to appear in his late 30's, no connection was made to the disease. He had tingling and numbness in his hands, and he began experiencing gastrointestinal problems. By his late 50's, the symptoms included weakness, burning and tingling in his feet, and difficulty walking.
"After the diagnosis of familial amyloidosos, we found out that a liver transplant could save my life. On the other hand, if my old liver stayed in place, the condition probably would have continued to progress and would eventually become fatal," says Mr. Guyton.
The abnormal protein produced by the liver in people with familial amyloidosis is deposited throughout the body and eventually affects the nerves and organs such as the heart. Removal of the liver eliminates the source of the abnormal protein and halts the progression of damage. This genetic defect is estimated to occur in about one out of every 100,000 Caucasians in the United States, but there is a higher incidence in particular geographic, ethnic and family groups. As Mr. Guyton and his wife, Louise, gathered more information about the disease, they learned that there is cluster of families with amyloidosis in the area of Frederick and Washington counties in Maryland.
"We knew there was a disease among people in our area, and it was usually called by the family name of the victim," says Mr. Guyton, who has since learned that several members of his family also may be affected.
Dr. Philosophe says patients sometimes called the disorder "sitting down disease," because it affects mobility throughout the body. He adds that many of those affected may not realize they have this rare disorder because it requires sophisticated DNA analysis to make a diagnosis. Also, they may not know that a liver transplant can stop its progression. According to UNOS (the United Network for Organ Sharing), only 51 domino liver transplants have been performed in the United States since 2000.
The recipient of Mr. Guyton's liver, Russell Ward, who has worked as a building manager, says he was told about three years ago that he had the beginnings of a liver disease, but that it would take some time before it became a serious problem.
"It came on like a ton of bricks," says Mr. Ward. In the weeks before the transplant, Mr. Ward was hospitalized repeatedly for confusion due to an ammonia buildup in the blood from his liver failure. "The disease was causing chemical imbalances that were totally wiping out my mind. I knew what I was talking about, but I couldn't make you understand it," Mr. Ward says.
When he was placed on the liver transplant waiting list at the University of Maryland Medical Center, Mr. Ward knew his odds of getting a transplant were slim because of a shortage of donated organs and his age. "At one point I was number 186 on the list," he says. "My condition would have to get much worse before I would be moved up the list. Also, they said liver transplants are rarely done in anyone over age 75. So now I was down to the wire. If the transplant didn't happen soon, I could forget it," Mr. Ward added.
A patient's rank on the liver transplant waiting list depends on what is called the MELD score (Model for End-Stage Liver Disease), a numerical scale ranging from 6 (less ill) to 40 (gravely ill) as determined by routine blood tests. "Mr. Ward's score was not high enough," says Dr. Philosophe. "His only hope was something out of the ordinary."
That extraordinary solution came when the transplant team paired the needs of Donald Guyton with those of Russell Ward. In contrast to Mr. Ward's concerns about a prolonged wait, the plan came together after he had been on the transplant list for just over three weeks.
Mr. Guyton says he learned about the plan to do the domino transplant on the day of surgery. But before the procedure began, both men understood the details. "I am grateful that I was able to have a transplant to stop the amyloidosis," says Mr. Guyton, who was discharged from the hospital on May 31. As for Mr. Ward, "The doctor said that in 30 to 40 years, I might start to pick up the donor's problem. Well, good. I'll worry about that 40 years from now," says Mr. Ward, laughing. Two days after surgery, Mr. Ward said he was already feeling like a new person. He was discharged from the hospital on May 29.
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