Every Breath He Takes

Matt Joyce's lungs were failing him. Then two strangers came forward.

Cystic Fibrosis

Floating just off the sandy crescent of California coastline at La Jolla Shores Beach, Matthew Joyce straddles a surfboard, facing out to sea. He studies the rhythm of the water -- a gentle rise and fall, so similar to the rhythm of breathing -- spots a wave he likes, turns the board and paddles hard. In two seconds he is standing, legs strong but thin as saplings, his curly brown hair lit up like a halo in the early morning sunlight. As the wave lifts him, he steps toward the board's nose, hangs ten, and then turns and dances in the other direction, a spider in a wet suit performing the difficult move surfers call a "walkback."

He turns forward again and, as the wave dies in a burst of froth, expertly flips his board and drops into the water.

The whole performance takes less than a minute and looks like something Neptune's choreographer developed to demonstrate the joy of being alive. For 20-year-old Matt, surfing is a celebration of life. Three years ago he lay in a Los Angeles hospital "about as close to death as anybody I've seen who didn't die," says his doctor, Mark Pian. He was breathing with the help of a machine that pushed air into his diseased lungs. His weight had dropped to 70 pounds, and he was coughing up blood.

Unlike the three dozen other surfers in the water on this perfect morning, Matt has cystic fibrosis, a fatal genetic disease that afflicts about 30,000 Americans. But through a combination of medical technology, devoted doctors, the support of family and friends, Matt's own determination, and the remarkable sacrifice of two strangers, the young surfer is much more than just alive. On this fine day, Matt Joyce is breathing easy -- through someone else's lungs.

A few hours after his morning surf, Matt sits in the backyard of his grandparents, Anthony and Frances Montisano. He pricks a finger to test his blood sugar level, and says, "All I wanted is what every other kid wanted, to be normal."

But normalcy is the first thing taken from people with cystic fibrosis. The disease has a simple cause: a defective gene that interferes with the normal movement of salt and water in and out of cells. The consequence of this -- thick, sticky mucus -- seems relatively benign until one considers the essential role mucus plays in the functioning of the lungs, pancreas and other organs. Just how essential may be evident very early. Many CF sufferers are diagnosed as infants because the thick mucous lining keeps pancreatic enzymes from getting to the intestines. Nutrients can't be properly absorbed and, in medical terminology, the infant "fails to thrive." This problem can be addressed with a regular intake of enzymes and vitamins, but, in later years, some people with CF develop painful bowel obstructions or, like Matt, diabetes.

Mucus plays an even more critical role in the lungs. Certain species of bacteria -- harmless to healthy people -- thrive in CF patients, forming infectious colonies that damage the lung tissue to the point where even a cold can be life-threatening. Eventually, the patient either gets a set of new lungs or suffocates.

There are at least 1,000 known mutations of the gene that causes CF, and every patient's symptoms are somewhat different. While there have been steady advances in treatment, some patients still die in their teens; others live into middle age, hold jobs, have children -- even run marathons.

Spiraling Downward

Matt was luckier than most, for a while. "I always knew I had CF, but I hid it," he says. Until eighth grade he was the fastest miler in his school. His father, Kevin, got him interested in bicycle motocross racing, and between the ages of 6 and 14, Matt and his brother, Joseph, were addicted to it. He played basketball, was on the cross-country team, learned to surf.

Then, in the summer after ninth grade, he was hospitalized for what he calls "a tune-up" -- three weeks of IV antibiotics to control a lung infestation of a common bacterium in people with CF. In the tenth grade he was infected with a rare and more insidious bacterium called Burkholderia cepacia (only three percent of CF patients ever get it). He'd given up running, and now, stick-thin and weak, had to give up surfing.

By his senior year, Matt was on oxygen. He stopped going to classes, barely left his room. It was clear that the disease had progressed to the point where the only option was a lung transplant. But, looking at the poor survival data for patients with B. cepacia, physicians at his local hospital were unwilling to offer Matt the expensive transplant procedure. The circle was closing around him.

What happened next was a series of coincidences that Matthew's mother, Debbie, describes as a miracle. First, she took part in a CF walkathon and happened to meet a young woman who'd had a successful double-lung transplant, in spite of being infected with B. cepacia. Debbie and Kevin -- who divorced when Matt was five years old but still remain friendly -- sought a second opinion and met with Dr. Pian of the University of California, San Diego School of Medicine. Pian was more optimistic: He knew that surgeons at Childrens Hospital Los Angeles, two hours away, had done transplants on CF patients with B. cepacia.

In the midst of this hopeful news, Matt continued to spiral downward. On October 15, 2000, he was taken by ambulance to Childrens Hospital L.A., but it was obvious he wouldn't live to see his name at the top of the long list of people awaiting a new set of lungs. His only hope was a living-donor transplant, a procedure performed less than 30 times a year in America. The human lung is made up of two or three lobes; a living donor can give up one and still have nearly normal breathing capacity. To turn the hope of a transplant into reality, however, Matt's family had to find two compatible donors, each willing to endure the risks of major surgery.

Back to the string of coincidences. Frederick Phillips, a 55-year-old lawyer from San Diego, happened to be a regular customer at the La Jolla supermarket where Debbie worked. Debbie told Fred that Matt was sinking, and Fred, whose own stepson has CF but was doing well, volunteered to donate a lobe. Another donor -- a woman who remains anonymous -- also materialized, and for a brief moment it seemed Matt would have his second chance at life. But when she failed the last of a long list of pre-surgery tests, Matt's family began to prepare for his death.

Fortunately, a backup donor was being tested at that very hour. His blood type was compatible, he met the height and fitness criteria, and his pulmonary function tests were off the charts. This man, Dave Manglos, had come forward by a route some would call coincidental, others an act of providence.

Reborn

Manglos, then 40, was a special agent with the U.S. Customs Service. Driving back to San Diego a few days earlier after a morning assignment in L.A., he started feeling drowsy and pulled off the highway near the beach at San Onofre. There, he noticed a cluster of surfers in the water. He walked a ways, picked up some stones and started skipping them. Because the stone-skipping reminded him of being at church camp as a boy, he began to pray. And then: "I had this strong feeling that I was supposed to do something. I didn't know what it meant." He told no one -- not his wife, children, or co-workers -- about his moment on the beach. "I'm a cop," he says. "I deal in concrete things. I thought people who had experiences like that were a little off the deep end."

The next night, he and his wife, Rhonda, watched the TV news, something they seldom do. Matt's cousin Jenny LaRocco came on to make an appeal for donors. Dave says, "Almost audibly I got this huge voice -- you couldn't ignore it -- saying, 'Didn't we talk about this yesterday?' " Jenny said they needed someone five-ten or taller, nonsmoker, O-positive blood. Dave looked at Rhonda. "That's me," he said.

Five days later, Dr. Vaughn Starnes, who pioneered the living donor lobar transplant in 1990, made a looping "clamshell" incision across Matthew Joyce's chest, sawed his patient's sternum in half, lifted the top of his chest wall as if it were the hood of a car, and went to work. He removed the ruined lungs and replaced them with a lobe each from Phillips and Manglos. The surgery went perfectly. Not long after he awoke from the anesthesia, Matt asked for paper and pen and scrawled 30 exuberant pages of notes. "I'm so happy," he wrote. "I have been reborn."

Three years have passed, and Matt is three inches taller and 40 pounds heavier. He still has cystic fibrosis, and will have to battle to keep bacteria from infecting his new lungs. But the transplant has given him the precious gift of time (some recipients are still doing well ten years after the operation), during which he and everyone associated with CF hope a cure will be found. Matt often counsels other CF kids who are considering transplants, and saves up money from his job at Mitch's Surf Shop in La Jolla to make junkets to Fiji, Australia and Costa Rica. He spends time with his parents and grandparents, his brother and surfing pals, his girlfriend, Amelia. When asked what he gets upset about now, he says, "Only one thing: people who abuse their bodies."

He stays in touch with his donors too. On a warm San Diego night recently he could be found at a Moroccan restaurant, joking with Dave Manglos about whose drainage tubes had come out faster after the surgery (Matt's), who had complained more (a matter of debate), and whether his lung is having more fun now in Matt's body, traveling the world.

The music is turned up. From the back room steps a belly dancer, who works her way among the tables clacking her finger cymbals and shaking her hips. As if prompted by some mysterious intuition, she stops next to Matt's seat, motioning for him to get up and dance with her. And he does, his body language surfer-cool, his face expressionless except for a tiny smile at the corners of his lips, a glint of gratitude, perhaps. Or just pure joy.

For more information, contact the Cystic Fibrosis Foundation at 800-FIGHT CF or visit cff.org.