Fighting for air: A lung transplant patient’s difficult journey

Diagnosed as a newborn with cystic fibrosis, a hereditary chronic disease that clogs the lungs and obstructs digestion, Stanley was an active child and teenager. She loved to dance. She skated, rode her bike, performed cheerleading.

That active lifestyle — she was on the dance team all four of her years at Bartlett High School — kept symptoms at bay. But after graduating in 2003, she stopped dancing. Breathing became more labored.

Stanley attended Elgin Community College for two years, then transferred to Loyola University Chicago, where the privacy of her single dorm room allowed her to administer two liquid feedings a day through a button in her stomach.

Her condition worsened and she moved in with her parents, transferring to Aurora University. She spent 14-day stints in the hospital to receive intravenous antibiotics and to clear her airway, but her health continued to deteriorate. The day after Thanksgiving 2007, Stanley was on a ventilator at Loyola University Medical Center and placed on a lung recipient waiting list.

Donor lungs arrived less than a day later. Stanley endured about eight hours of surgery in which doctors trimmed her left atrium, sewing the lungs to her heart. Recovery was traumatic. She suffered an allergic reaction to medication, endured bleeding in her brain and, for a time, was unable to speak. Her muscles atrophied and she was forced to relearn to walk.

Stanley returned home shortly before Christmas and continued an arduous rehabilitation regimen. Slowly, her health improved. She went on a cruise in April 2008 and took in a handful of Cubs games at Wrigley Field. She returned to Aurora University, earned a degree in English and planned to become a teacher. She got engaged to her longtime boyfriend, but it ended in early 2009.

By May of that year, her body started rejecting the lungs and her health began a steady descent. She lost weight and underwent surgery for acid reflux. Doctors determined her body’s rejection of the lungs was chronic and put her on the wait list in December 2009.

Now Stanley is 30 pounds below normal weight, even though a tube to her stomach pumps in supplemental nourishment designed to boost her caloric intake. She wakes about 11 a.m. and needs nearly two hours to shower and get ready for someone to carry her to the first-floor den. She takes 15-20 medications a day and is on oxygen at all times. She rarely leaves home.

“I miss the little things,” Stanley said, like being able to get up and leave, or drive a car. “I just want to be able to do things for myself.”

For years, patients received lungs primarily based on how long they were on a waiting list. That changed in mid-2004 to the Lung Allocation Score, or LAS, a national approach aimed at providing them to patients with the most urgent need.

But when lung transplants shifted to the LAS system, with its mathematical calculations that produce a numeric rating for each patient, nearly 40 percent of those who had been on the waiting list were dropped, said Dr. Robert Love, who has performed more than 700 lung transplants and serves on the national board for the United Network for Organ Sharing. The nonprofit organization based in Richmond, Va., manages organ transplants in the U.S.

They were dropped because their risk of dying of their specific lung diseases in the next two years was infinitesimal, added Love, a surgeon at Loyola University Medical Center who performed Stanley’s surgery in 2007 and who hopes to perform a second.

Controversy stirred and some viewed the LAS “as an unwelcome complexity added to a system that was already working OK,” Love said.

“It took a little while to get (LAS) explained to the community,” he added.

Still, LAS achieved the desired results, getting lungs transplanted to the sickest people, Love said. “It’s never going to be perfect,” he added, “but it should get better over time.”

Time is precious for Stanley and the estimated 30,000 people in the U.S. with cystic fibrosis. Although life expectancy, barely six years in the 1950s, now stretches past 40, individuals with the disease and others waiting for lungs endure a psychological suffering distinct from patients needing other organs, Love said.

“They’re all breathless, but not sick enough to be placed in hospitals and made better,” he said. That status generates an enormous amount of anxiety, Love said, “and not knowing what the next hour basically is going to bring really makes it a pretty miserable experience.”

For Stanley, anxiety erupts in the shower, where humidity makes it extremely difficult to breathe and the tight quarters make her claustrophobic. Some days, it’s too much. She routinely finds herself succumbing to pessimistic thoughts and is taking antidepressants. She said she’s scared.

If she gets the second set of lungs, they likely will be her last. Trimming the atrium a third time almost always ruins the heart, Love said, and scar tissue from two previous transplants often is unmanageable. The encouraging news is that cystic fibrosis would be absent from her new lungs and not likely to return there, the doctor said.

Medical concerns can be overwhelming but Stanley said she has grown from this ordeal. She’s learned patience and to “never take anything for granted, to make every day count.” Over the past five years, she, her family and friends have participated in the Cystic Fibrosis Foundation’s annual Great Strides Walk and raised more than $10,000 to fight the disease.

After her last hospital stay in late December, Stanley rallied, committing to walking around the house for at least 10 minutes a night to stay as strong as possible while she waits.

“Every day,” she said, “I’m going to increase that time.”

Doctors performed the first lung transplant in the U.S. in 1983 and the first double-lung transplant in 1986, the year after Stanley was born, according to the United Network for Organ Sharing.

Survival rates improved as the number of transplants increased. Surgeons became more skilled at the technical aspects of performing transplants and the medical community has done a better job of matching donors with recipients, Love said.

In 1988, the one-year survival rate was less than 43 percent. In 2008, the latest year of available figures, that rate was nearly 83 percent. Five-year survival rates stand at more than 50 percent, up from 24 percent in the late 1980s.

But second-time lung transplant recipients, less than five percent of lung transplants, have a lower survival rate: 68 percent survive one year and about 50 percent make it to three years. The overall, three-year survival rate is 61 percent.

Those odds are unsettling, but they are Stanley’s only odds. Apart from the statistical criteria that place her high on the LAS, she said she believes she deserves a second set of lungs in part because, “I still haven’t lived a normal life, like everybody else.”

It’s not her fault, she said, that her body rejected the lungs. “So, why can’t I get a second chance?” she said, tears welling in her eyes.

Statistically, Stanley is unlikely to survive another year without a transplant. But she tries to keep those thoughts at a distance, tries to keep a sense of humor, tries to keep breathing.

She said she’d like to return to school, become a dental hygienist, “move out and try to get married — all that good stuff.”

Stanley turned 27 this month and her family had a small party at home. A friend brought balloons. Her parents presented her with flowers and a white cake with 27 lit candles.

She inhaled and blew on them and then she did it again and again.

“I couldn’t even blow out the candles,” Stanley said a few days later, laughing. “My brother had to help me.”

And then, too quickly, her laughter faded.