Second Life

Richard Norris was just a normal 22-year-old in 1997, a young man from rural southwest Virginia who liked fly fishing and the outdoors. But leaving his parent’s home one day, heading out to meet up with friends, something askew caught his eye. He noticed that the shotgun in the family gun cabinet was leaning awkwardly against the glass door. An avid deer hunter, Norris went to safely secure the firearm, but as he unlocked the cabinet, the Remington 12-gauge shifted, and a single shell exploded through the barrel, striking him in the face.

Two weeks later, awakening from a medically induced coma, he opened his eyes. The rest of his face was gone. A blob of scar tissue surrounded what was left of his mouth. His lips, teeth, jaws, nose, and part of his tongue were no more.

Over time, he underwent roughly 30 surgeries in the hopes of even marginal improvement to his disfigured face. Nothing worked. At home, he removed all the mirrors in the house to avoid seeing his image. He shunned his friends and rarely ventured out in public except at night. To avert scowls and shrieks from strangers, Norris wore a black surgical mask to cover his face. Once, someone in a store believed him to be a robber.

He slumped into depression, eventually contemplating suicide.

Meanwhile, in Baltimore, at the University of Maryland Medical Center, Dr. Stephen Bartlett, the medical system’s surgeon-in-chief, had begun researching how to help wounded soldiers in need of facial transplants. Fourteen years since the accident, after nearly a decade of research, Bartlett was convinced he could transform Norris’s face—and life—through a revolutionary surgery.

“The amount of disability he had was just phenomenal,” says Bartlett, describing Norris’s situation at the time as “heartbreaking.” “He truly was a hermit.”

Norris had to consider the possibility of more disappointment, of his body rejecting the transplant, of not waking up from a long surgery. Just the 23rd person worldwide ever to undergo a partial or full face transplant, his would be the most extensive effort ever. In his mind, there was nothing left to lose.

Then, in March of 2012, the call came. A 21-year-old White Hall man had been struck and killed by a car and his family consented to donate his face. After 36 hours in an R Adams Cowley Shock Trauma operating room, Norris, literally, would smile again.

“People used to stare at me because of my disfigurement. Now they can stare at me in amazement and in the transformation I have taken. I am now able to walk past people and no one even gives me a second look,” Norris, now 38, said afterward. “My friends have moved on with their lives, starting families and careers. I can now start working on the new life given back to me.”

Even at one of the busiest transplant centers in the county—the University of Maryland Division of Transplantation replaced 297 kidneys, 91 livers, 28 lungs, 17 hearts, one solo pancreas, and 13 simultaneous kidney/pancreases last year—Norris’s surgery stands out as an extraordinary accomplishment. Bartlett calls the transplant the greatest achievement of his career. However, what has stunned Bartlett and his team since the operation is not Norris’s radical cosmetic improvement. As they tell it, the bigger story is how Norris’s body accepted the transplant—rejection always a daunting obstacle to overcome. Ultimately, it’s Norris’s remarkable tolerance for his transplant that Maryland doctors predict will be a game-changer for transplant recipients—all types of organ transplants. And not just at the University of Maryland Medical Center, but everywhere.

“Tolerance in transplants is the Holy Grail,” says Dr. Si Pham, the University of Maryland’s director of heart and lung transplantation. “We’ve all been trying to work on that for a long time now. The field started in the 1950s, but I think now there’s some light at the end of the tunnel.”

Bartlett believes Norris’s immune system’s ability to tolerate the transplant so well is likely linked to what’s called “vascularized bone marrow”—within his donor’s jaw bones. Organs, such as hearts and kidneys, for example, have always been transplanted on a one-for-one basis without accompanying bone structure. In Norris’s case, however, he needed an entire jawbone. Pre-clinical research suggested that when a donor’s marrow-rich bone is transplanted intact along with an organ, the recipient shows more tolerance and needs fewer immunosuppresion drugs. (Organ recipients require a life-long regimen of immunosuppresion drugs, often with considerable side effects, to prevent their bodies from rejecting donor organs.)

“There’s something very unique about the environment of the whole bone, and what we saw on the animals is a remarkably low immunosuppression level,s and now we’re seeing that in Richard,” Bartlett says. “You look at him and go, ‘How is this graft working with so little immune suppression?’”

“We’ve been prepared for this for almost a decade in the lab,” says Dr. Rolf Barth, 42, the University of Maryland’s director of liver transplantation, who managed Norris’s immunosuppression regimen. “This was such a fantastic example of why research is so important and how it lets us treat and come up with new cures in medicine.”
Now, with the help of two grants that kicked in Oct. 1, more vascularized bone-marrow research is underway.

“There are very few people who have looked at giving vascularized facial and bone marrow and we really have one of the only models, the only preclinical model for vascularized bone marrow that exists, to our knowledge,” says Barth.

Adds Bartlett: “We are going to change the whole world by saying we don’t need all that immune suppression, what you need is vascularized bone.”
 
But at least one big question remains. Can doctors find a way to use vascularized bone marrow in all transplants? There isn’t a ready answer with heart and lung transplants, but for abdominal transplants, such as the kidneys, liver, or pancreas, the solution may rest in our ribs. Think Adam and Eve here.

“You could take a rib out [from the donor]. A rib bone has a lot of bone marrow in it and has the artery and the vein attached to it,” says Dr. Jonathan Bromberg, chief of the University of Maryland’s abdominal transplant program. “You could take that rib, attach it to a nearby artery or vein so that now you’ve transplanted the bone marrow, it’s got blood flow to it, and you have a very advantageous situation.”

Research at Maryland to improve organ recipient acceptance, as well as other aspects of transplant surgery, is not limited to vascularized bone marrow, however.

Bromberg, for example, is studying a subtype of white blood cells, T-lymphocytes, which travel in the body and can weaken or strengthen the immune response. A weakened immune response isn’t good if it comes in contact with the flu virus, but it’s beneficial for organ recipients.

“If we can learn how to use these, we might be able to learn how to suppress the immune response to the graft, and we might be able to get
better graft survival and get tolerance,” Bromberg says.

In the area of heart and lung transplantation, advances in bioengineering have long been of interest to Dr. Bartley Griffith, professor of surgery at the University of Maryland School of Medicine.

Griffith, who paved the way for the medical center to be the first in Maryland to use an artificial heart while waiting for a donor heart, recently developed a “pump-lung” device with his team that could be revolutionary for lung-transplant patients. “We now have this device that we want to introduce this heart-lung machine developed in the lab, which you could say is maybe an artificial lung,” he says. “It does all the work of the heart and lungs and that’s something that drives all of our interests here with end-stage lung disease.”

Griffith is also the principal investigator for a separate national trial, which he reports is very encouraging, that strengthens donor lungs not healthy enough for a transplant.

Of course, none of this is possible, the doctors all note, without the true lifesavers in transplantation: donors. The medical center works with The Living Legacy Foundation, a locally based nonprofit that supports patients and families considering donation and coordinates hospital process. [In Norris’s case, five other patients received organs from his donor Joshua Aversano, including his heart, liver, pancreas, kidneys, and one lung.]

“Our relationship with the University of Maryland is excellent,” says Charlie Alexander, Living Legacy’s CEO. “It’s one of the most successful and aggressive transplant centers in the country. They’re great partners, and they’ve been incredibly gracious participants in the process for years.”

In the area of liver transplantation, the hospital’s program is flourishing under Dr. John LaMattina, who arrived at Maryland three years ago and directs the living donor liver transplant program. Living donors have become a critical option for patients with end-stage liver disease in recent years, and Maryland is one of the few places capable of performing the surgery. “For someone in the U.S., if they get put on the transplant list, about half of them get a transplant, which is great, but that means half of them don’t, which isn’t great at all, and they die without a transplant,” he says. “So if you have a living donor, this is your best option as far as long-term survival goes.”

All transplants are equally rewarding, he says, but an operation in February of 2012 sticks out in his memory. The patient: Jen Dietrick.
 
It was Dietrick’s boyfriend, Rob Hopkins, who first noticed something was wrong at his 25th birthday celebration. Over dinner at the popular Canton restaurant Nacho Mama’s, Hopkins watched as Dietrick’s behavior grew more and more boorish and bizarre.

“She was very rude to the waiter and it was very uncharacteristic,” Hopkins says. “She didn’t drink any of her drink or have any of her meal and it was very weird. I didn’t realize what was going on. I actually thought she was on drugs or something.”

A day or two passed and Hopkins couldn’t get in touch with his girlfriend of two-and-a-half years. He called her father, who rushed to Dietrick’s apartment. He found her disoriented, and hurried her to the hospital.

Dietrick, it turned out, had been taking acetaminophen on top of prescribed Percocets after a recent car accident. The potentially lethal cocktail was overwhelming her liver, sending toxins to her brain and affecting her behavior. More critically, doctors determined her liver was failing.

With hours to live, LaMattina bumped her to first on the transplant waiting list.

“She looked pretty ill at that point. Her brain wasn’t working particularly well, and her liver wasn’t working at all,” says LaMattina. “We had looked all around the region for potential donors for her, and there was nothing. We called every center that we can draw from and they all had nothing even close to pending for her. So I called her dad and said we have no other choice, we need to move forward with a living donor.”

LaMattina looked to family members as potential donors, but the only eligible person who matched Dietrick was her boyfriend. Hopkins was told he could save his girlfriend’s life by donating 60 percent of his liver to her.

He didn’t hesitate.

“I wasn’t too worried. I didn’t have anything else to do that night,” says Hopkins, jokingly. “I think anyone in my position would have done it. I’d do it again tomorrow.”

After 13 hours of surgery, LaMattina’s quick action paid off. Hopkins was discharged after just two days, and his liver has since regenerated to its full size. Dietrick, now 26, spent another 11 days in the hospital before she eventually returned to work at a local accounting firm.  The two, of course, remain a couple, happily planning their future.

“It’s very touching to know that not only did he love me,” she says, “but that he was willing to do that for me, to give me a second chance.”

She admits though, since her recovery, she’s struggled with the side effects of the immunosuppression drugs that keep her body from rejecting her boyfriend’s liver. “The recovery process hasn’t been the easiest,” she says. “Getting adjusted to the anti-rejection medicine has some not-so-pleasant side effects. It makes you very jittery and tired.”

Though human trials remain years away, doctors hope vascularized bone marrow—which so dramatically helped Norris’s body tolerate his new face—will make the recovery process less taxing for liver recipients like Dietrick, as well as those receiving new kidneys, pancreases, and other organs.

The doctors at Maryland say it’s the constant search for improvement and scientific advancement—from inside the lab to the operating table—that has pushed the medical center to the forefront of transplantation medicine. It’s also what makes the University of Maryland an attractive and challenging place to research and ply their trade.

“It’s become the place that you want to work, [with] all the innovations here,” says Pham, the director of heart and lung transplantation. “It’s the kind of environment that allows you to bring transplant to the next stage.

“I gave up a professorship at the University of Miami, I gave up beautiful weather,” Pham adds with a smile. “I came here for that reason.”