Ben Blake underwent his first liver transplant before he was out of diapers — an operation that kept him going for the next 20 years. But during his third year of college, Blake’s donated liver suddenly started to fail and he began to waste away.
With their son far down the wait list for another transplant, his frightened parents scrambled to find options to save his life. Then, Blake’s doctors at Massachusetts General Hospital suggested another, experimental option. Would he allow surgeons to transplant a liver from a donor infected with hepatitis C — a procedure that had rarely been attempted before. They would then give Blake a powerful new drug to — hopefully — keep him from becoming infected.
Blake and his father, Duane, readily agreed, and his mother, Jackie, who had initial doubts, soon came around. During a five-hour transplant operation that started at midnight on Dec. 8, he became one of a very few hepatitis C-negative patients in the United States to get a liver from a donor who has the disease.
Blake said he felt so exhausted and malnourished without a functioning liver that any risk of infection was worth it. “We were willing to think outside the box,’’ said the 26-year-old, who is recovering at his parents’ home in Westwood.
He had been on the wait list at Mass. General for five years, but once he agreed to this unconventional approach, he had a new liver in three months.
He began taking the hepatitis C drug Epclusa before his surgery and will continue it for three months — similar to the course of treatment for a person with the disease. The idea is that it keeps the virus from spreading outside the liver and shuts it down within the organ itself.
So far, he shows no signs of infection.
“We were all very worried about Ben,’’ said Dr. James Markmann, chief of transplantation at Mass. General.
One week after his transplant, Blake was on his feet at home and talking animatedly about his experience. Doctors say he is recovering well, though he’s been admitted to the hospital for two brief stays because of possible infections. He’s also experienced anxiety at the hospital, where it’s hard to sleep, and he has had his blood drawn multiple times.
The severe shortage of donated organs has led many transplant centers to use livers they would have rejected in the past — particularly for desperate patients. Surgeons routinely transplant kidneys and livers from donors with hepatitis C into recipients who also have the disease and, therefore, are not risking a new infection.
But transplants like Blake’s are rare. Intermountain Medical Center in Utah said last month that it had recently performed what it believed to be the first such liver transplant in the United States. As a result, there are no scientific studies about how patients fare.
That may soon change. Teams from Johns Hopkins Hospital in Baltimore and the Perelman School of Medicine at the University of Pennsylvania in Philadelphia early last year began pilot studies of 10 patients each, all people without hepatitis C who received kidneys from donors with the infection.
The number of organ donors with hepatitis C may be rising because of the growing number of overdoses among intravenous drug users, many of whom also have the infection, which can be transmitted by contaminated needles.
Johns Hopkins has performed eight transplants as part of the pilot so far, and early testing shows none of the recipients has contracted hepatitis C — though it is too soon to know for sure, said Dr. Niraj Desai, director of kidney and pancreas transplantation at Johns Hopkins. He said the approach would not be possible without new drugs that came on the market three years ago, and actually cure the chronic illness in more than 95 percent of people.
But not everyone is convinced. Dr. Charles Rosen, head of transplantation at the Mayo Clinic in Minnesota, said there is a risk that a transplant recipient will contract hepatitis C and that the new drugs won’t cure it. It’s a dangerous illness that can cause cirrhosis and liver cancer. There is also the cost of the drugs — three months of treatment run $50,000 to $75,000. Mayo has plenty of patients on the liver transplant waiting list who have hepatitis C and need the expensive treatment for their disease anyway. Giving the drugs to a hepatitis C negative transplant recipient is an added cost.
Still, he said, “It’s a very reasonable thing to do on a patient-by-patient situation.’’
Mass. General was developing a protocol to use livers, kidneys, and hearts from hepatitis C positive donors when Dr. Karin Andersson, a hepatologist, suggested the team seek approval immediately from the hospital’s institutional review board for Ben Blake.
“They were eager, and they didn’t have any other options,’’ she said.
Blake was born on May 23, 1990, at Albany Medical Center in New York, with what turned out to be an incurable condition called biliary atresia. His bile ducts were inflamed and blocked, trapping bile in his liver and rapidly destroying it. Four days after the family moved to Boston, they found out 7-month-old Ben needed an urgent liver transplant.
The following years weren’t easy — Blake has had close to 1,000 doctor appointments, takes dozens of medications, and developed diabetes as a result of his condition. But his parents tried to keep his childhood as normal as possible. He played baseball for elite teams through high school and was accepted to a five-year sport management program at Drexel University in Philadelphia.
It was during visits home from college that his parents began to really worry. He was having trouble digesting food and lost large amounts of weight. He contracted strange infections and developed night blindness. They watched the muscles in his arms and legs disappear and severe fatigue set in.
Mass. General put him on the wait list right away, in August 2011. But the particular way end-stage liver disease manifested itself in Blake put him at a disadvantage. Doctors give patients a “medical urgency score,” based on laboratory tests, from 6 to 40; the higher the number, the more likely the patient will die within three months — and the higher the ranking for a transplant.
Blake fell into the small group of patients whose lab tests did not reflect the severity of his illness, doctors said. “Wasting syndrome’’ is not included in the calculation. His urgency score usually hovered around 12 or 14. His situation was complicated by the fact that as a re-transplant patient he needed a full liver — he could not accept a partial organ from one his parents.
When the family set up a website to seek liver donors — allowed under US transplant policy — the dozens of people who offered to donate either remained alive or their livers could not be used because they were too elderly or had another risk factor. Trips to the Mayo Clinic in Florida, where waiting lists are generally shorter, were fruitless as well. The hospital would not list Blake on the transplant wait list until his urgency score climbed to at least 18, the family recalls.
By this past June, Blake had lost about 50 pounds — nearly 30 percent of his body weight — and was put into an induced coma for a week in the Mass. General intensive care unit so doctors could treat internal bleeding and a serious infection.
Around that time, doctors decided to petition the region’s transplant review committee to grant Blake an exception to the traditional ranking system and raise his score to 32, based in part on his severe weight loss, which would have shot him to the top of the list. The committee of the United Network for Organ Sharing, which oversees transplants in the United States, rejected the request twice. After an appeal by Mass. General that included a personal letter and photos from his parents and a conversation among Mass. General physicians and committee members, the group agreed to give him a score of 22.
Blake’s parents argued that biliary atresia should be considered an exception to the traditional scoring system, and that patients who’ve had previous transplants and complied with medication and lifestyle restriction should be given extra points. The committee did not share a reason for their rejection of the request for 32 points, Markmann said. “Everybody has a lot of sick patients and they are reluctant to give points,’’ he said.
But the combination of the 22 score and the agreement to take a liver from a hepatitis C positive donor ended up getting Blake what he needed. “This was in the nick of time,’’ his father said.
As Blake waited on a gurney outside the operating room late on Dec. 7, he said it was the only time he’s ever prayed. The minutes crept by as he waited for the doors to swing open.
“It’s going to fall through like the other times,’’ Duane Blake remembers his son remarking.
“I think this is for real,’’ he replied.
Liz Kowalczyk can be reached at firstname.lastname@example.org.