"To be set free"

By TINKER READY
The News & Observer

April 19, 1994, ninth floor, Duke University Medical Center, Durham, N.C.

Shelby Parker sits in the lotus position in the middle of a hospital bed, her slender arms resting on the pillow in her lap. Cystic fibrosis is catching up with her.

At 23, she stands 5-foot-2 and weighs 70 pounds. She seems impossibly thin, her limbs so frail it's a wonder she can lift a glass or walk to the bathroom.

Just a year ago, Shelby was a full-time college student, but now she spends day and night tethered to an oxygen tank. That hose runs into her nose; another pumps intravenous antibiotics into her arm. An infection almost killed her in January, and nothing short of a lung transplant will save her.

But Shelby doesn't want to talk about being sick.

"You have to meet my cats!" she says.

There is Annabelle, she explains, and Abraham, who is named for President Lincoln.

"He just sort of reminds me of him," she says.

A tiny, doe-eyed woman with a child's voice, Shelby is in a life-and-death showdown with her disease. Her lungs, scarred by repeated infections, are failing. Ten years ago, death would have loomed certain. There was no cure then, and there is none now.

Today, there is hope, albeit fragile hope. Just a few miles from where Shelby waits, doctors at the University of North Carolina at Chapel Hill are leading a national effort to cure cystic fibrosis by repairing the faulty gene that causes the disease. But that prospect is still far off.

For Shelby, the only real hope lies in the hands of the doctors who have the power to remove her weak, scarred lungs and replace them with a healthy set.

So she waits. It may be a year before someone else's misfortune becomes her salvation. She could very well die before an accident leaves another patient brain-dead, a family makes a difficult decision and a set of donor lungs becomes available to her.

Until then, she tries hard to live not just to stay alive, but to have a life. She divides her time between her father's home in Raleigh and Duke Hospital a scary, unfamiliar place for most people her age. Even so, Shelby seems oddly at home here.

Even during her relatively healthy periods when she rode a bike, climbed mountains and went to college Shelby came to Duke for regular "tune-ups." Over the years, these sessions in which she spends several days hooked up to a bag of intravenous antibiotics have become more frequent.

"At first, it was once a year," she says. "It got to be twice a year. Now it's three or four times a year."

So, she knows the nurses on the ninth floor, which houses Duke's pulmonary wing. She knows her way around the mazelike building. And she's become an expert at dealing with doctors, nurses, technicians, therapists, social workers and hospital aides.

When she thinks she needs a particular drug, she asks for it. She's always polite, but when she doesn't like something, she says so.

In January, a doctor toured some medical students through the intensive care unit, and she heard him describe her as close to death. She resented the comment and asked them to leave.

"You have to be that way, or they just walk all over you," she says.

April 27, ninth floor, Duke University Medical Center

"I'm starting to feel good," Shelby says. "I haven't felt good for a long time."

A week later, she's still in the hospital, still cross-legged on the bed. She searches for a way to describe how she feels today compared with how she felt last week.

"I just come alive," she says. "I revive."

Today, she feels good enough to go to "the gym," but not quite up to walking there. So, a nurse helps Shelby into a wheelchair, loads up her oxygen tank and they set off down the hall to the elevator.

Downstairs, in a room filled with dumbbells and exercise bikes, physical therapist Fran Zabolitski waits by the treadmill.

"How are you feeling today?" she asks.

"Pretty good," Shelby says in her squeaky little voice. She sounds so much like a small child that pizza restaurants won't take her order over the phone.

She gets out of the wheelchair, reaches for the railing and plants both Bass moccasins on the treadmill belt. She walks. Then, she coughs.

The coughing sounds awful, but Zabolitski says it helps. Cystic fibrosis destroys the lungs by allowing a thick, sticky mucus to build up inside them, clogging the airways and causing infections. The coughing strips it away.

The gluelike mucus is a breeding ground for infection, and these patients have to work so hard to breathe through it that it eventually destroys their lungs.

"Exercise makes people breathe harder, and it helps mobilize the secretions," Zabolitski says. "It helps keep their lung function up."

Meanwhile, Shelby keeps walking and coughing. A band holds her straight, shoulder-length brown hair off her face, and her favorite green T-shirt swings on her frail frame. Yesterday, she went for 25 minutes. She doesn't go for quite as long today.

"I did real good," she tells Zabolitski.

"You did ...," the physical therapist responds, "and you coughed a lot today."

But it's hard. Shelby looks pale and drained. She knows she should do this every day, but she confesses she's not good about it. She needs to build up her strength, but she doesn't eat much. So, she has little energy to spare.

"If I had to walk down to the gym, it would have worn me out," she says.

The focus may be on her lungs, but the biggest threat may come from Shelby's failure to gain weight. It's not just that she has little appetite. Cystic fibrosis affects her pancreas, as well as her lungs, making it harder for Shelby to absorb nutrients and gain weight.

At 70 pounds, she may lack the stamina needed to withstand the stress of a transplant, if she survives until then.

So, after her January brush with death, Shelby agreed to begin using a feeding tube. Now, every night, after she brushes her teeth, she attaches a plastic tube to a special port implanted in her groin. Then, a machine pumps liquid nutrition into her stomach while she sleeps.

She hates it.

Earlier that day, she heard the doctors outside her door say, "She's as well as she's going to get." So, she goes home the next day.

May 18, Park Drive, Raleigh, N.C.

Shelby shuffles into the living room, trailing a plastic hose behind her. The extra-long tube on the oxygen tank lets her move freely around her father's house from the spacious kitchen to the dining room and into the well-appointed living room. If she wants to go out, she can hook her hose up to a portable tank and take it with her.

It's a beautiful day in Raleigh's Cameron Park neighborhood, and the shopping center is just two blocks way.

But Shelby's not interested. She's been home from the hospital for about three weeks now, and she's not in a very good mood.

The tube feedings make her feel lousy, and the oxygen hose makes her feel self-conscious. Walking around with a plastic tube running up your nose is akin to leaving a hair salon with a really, really bad haircut, she says.

"And I really haven't had the energy...," she says, "not that I'm planning to stay cooped up in the house."

For now, though, she does. Like yesterday and the day before, Shelby will pass all day here, much of it alone.

With its antique furniture and Oriental rugs, the Parker home feels formal, but comfortable at the same time. Still, Shelby sits at the edge of the couch. She seems ill at ease.

Maybe it's that she didn't grow up here. She lived in Greensboro with her mother, Winnifred "Winks" Parker, until she went off to college. Only then did she make her summer home here with her dad, John, and his second wife, Lynette Parker.

More likely, she's simply uncomfortable with her new status as a sick person. For years, Shelby could pretend she didn't have a life-threatening disease. Now, the illness she once ignored has taken over her life.

Just last year, she was enrolled at Campbell University, majoring in religion. She lived in the dorm. She went to parties. She dated. She was even engaged for a while.

"I loved it," she says. "I'm very independent."

She wanted her life to stay that way, but her lungs did not cooperate. Each day, Shelby had to work harder and harder to breathe. It took all she had. She lost 20 pounds in 1992. When her doctor, Wayne Samuelson, told her she needed to begin tube feeding to gain weight, Shelby resisted.

"That was unacceptable to her," Samuelson says. "It was tough for her not to be like everyone else."

Just before she was to start classes in January, she went to Duke for a check-up.

"She asserts that she will be able to take excellent care of herself," Samuelson wrote in his notes. "I am somewhat pessimistic about her prospects for being able to complete the semester."

She never even started. Two weeks later, her family sent her not to school in Buies Creek, but to the intensive care unit at Duke. The infection in her lungs stole so much of her strength, they doubted she would survive.

Somehow, Shelby made it back from the edge.

After that episode, she agreed to the feeding tube and to the scary prospect of a lung transplant. Now she tries to cope with the former while she waits for the latter.

Doctors often learn how to keep people like Shelby alive long before they find a way to save them, but it's not much of a life on a feeding tube. She feels horribly nauseous. She vomits every morning. She tries to eat, but has no appetite.

"Zero," she said. Because she cannot keep the liquid food down, though, she's not gaining any weight and has no strength. Just getting up, bathing and dressing wears her out.

She's crabby. But she's trying.

"It's just a matter of adjusting," she says. "You either do it or you don't. If you're going to sit around and be depressed, it's going to affect your health."

June 7, Park Drive

Shelby has always had a man in her life, according to her stepmother. The first was Keith.

Just a mention of him brings a rare smile to Shelby's face.

"He was the first boy I kissed, and I was the first girl he kissed," she says.

It's about 4 p.m. and Shelby is just starting to feel normal. By late afternoon, she feels well enough to sit in the living room and chat about her "first love."

For much of her life, Shelby tried to convince people that she wasn't really sick. Apparently, she did a pretty good job, keeping up both her pace and her spirits.

As she reached her teens, though, she could no longer fool herself.

"When you're 10 years old, you don't care," she said. "By about 14, your body is changing and you suddenly care about what you look like. I was skinnier than everyone."

She knew why, but locked her fears inside until she befriended a classmate and neighbor named Keith.

"He was the first person I ever talked to about it," she said. "To suddenly realize you have this disease it's incredible He was the first person I didn't have to pretend with."

Keith and Shelby have remained friends over the years, although he now lives in New Hampshire. She has new friends who live nearby, but in many ways she remains isolated.

Shelby still spends most of her time in the house. As always, her hair looks neat and shiny. She's swapped her hospital T-shirt for a crisp polo shirt, but she still worries about her appearance.

She passes the day reading or watching television Court TV and the Arts & Entertainment network and two soap operas whose titles echo her own drama: "General Hospital" and "One Life To Live."

"I haven't been bored, but I haven't been doing anything," she says. "I'm wondering if I'm becoming boring."

She finds the wait frustrating. She finds the overnight tube feeding unbearable.

"I wake up all the time," she says. "I don't sleep soundly."

The gray liquid that flows into her stomach all night never settles quite right. In the morning, she gets up and vomits.

"I feel bad, and it takes me all day to feel better," she says. "I can't really describe it. It's just yuck."

All this has left Shelby feeling rather ragged. One day, she says, she was sitting with her father in the living room and burst into tears, something she rarely does.

She was upset that her stepmother, Lynette, had gone outside to smoke a cigarette. Most of her family members smoke, and usually it doesn't bother her. But that day, Shelby couldn't help wondering why someone would do damage to a good set of lungs.

Her tears upset her father, and they had a talk. When he began to cry, Shelby apologized for getting so upset.

"I just can't talk about sad feelings with my dad," she says. "He can't handle it."

She pauses for a minute and sighs.

"I get so tired of being strong."

The next day, she's back in the hospital.

June 15, law office, John Parker, downtown Raleigh

John Parker's office is not unlike his home traditional, but comfortable. He works at a big, broad desk surrounded by diplomas, degrees and artwork. A set of windows offers a view of the Fayetteville Street Mall.

A true son of the South, John is a solid man who tends toward seersucker, bow ties and little round glasses. He's talkative without being chatty. He's friendly, but intense. He walks into the room with none of the airs of a former judge.

Today, he seems troubled. After 23 years, he's come face to face with the cold fact that his sweet little girl could die.

From the day she was born when it took seven minutes to get her breathing John Parker has struggled with the feelings unleashed by his daughter's illness. He's a man who likes to be in control, a trait he's passed on to Shelby. Since the day she was born, though, he's felt powerless to help her.

John and Winks were former high school sweethearts who already had given birth to a healthy baby when Shelby was born. The mother and father were a rare combination. Although niether has cystic fibrosis, each one unknowingly carried a gene that could cause it. Shelby fell victim to the 1-in-4 chance that they would produce a child with the disease, the most common genetic illness in the United States.

Baby Shelby was small and sickly, and she had constant diarrhea. She spent much of her infancy in the hospital.

"I would get sick physically ill and throw up when I would go to visit her," John says. "I would cry and I was not much help to Winks at that time, I'm afraid to say."

Shelby survived, but the marriage didn't. Winks moved to Greensboro with her children, and John became a long-distance dad. He thinks back on those years, struggles to hold back his tears, and fails.

He pauses, regains his composure and continues his recollections.

"One of the strongest people I have ever known is Shelby Parker," he says. "She was constantly told in school that she was not going to be able to go to college, that her grades weren't good enough, and that made Shelby very angry. ... I know she's my daughter and I'm biased. I just feel that there is a lesson to be learned through what Shelby has had to deal with."

Shelby did go to college and, thanks to John, she climbed mountains, traveled cross-country and toured Britain. He taught her how to play the guitar and after she learned 12 chords bought her a one of the best, a Martin.

Most people with cystic fibrosis don't live past the age of 30. If Shelby's life had to be short, John wanted to make sure it was full.

Sometimes, in the early evenings, Dr. Samuelson would hear the strains of John's guitar coming from Shelby's room when he made his hospital rounds. The doctor would pause at the door. He didn't want to disturb them.

So, now that Shelby can't go hiking, she and John spend time together in the hospital. He doesn't get sick anymore, but it's never easy. Shelby's tenacity and honesty continue to amaze him and, sometimes, infuriate him. They bicker all the time. Their squabbles are the stuff of family legend. They adore each other.

Still, Shelby accepts the reality of her condition in a way her father cannot. She knows her death and her cure lie ahead like the prongs of a fork in a long, hard road. She's let her mind wander down both paths. John tries, but hesitates.

"I was sitting with Shelby and she informed me about how it was she wanted to die," John says. She was specific. There would be no machines, no lingering in a hospital. If her life had to end, she wanted it to be at home.

He is always astounded by how frankly his daughter deals with the prospect of dying. He responded not just as a father, but as a lawyer. He helped her write a will.

Until recent successes with lung transplants, death was the only path open to someone like Shelby. Back then, Samuelson says, most cystic fibrosis patients preferred to pass on quietly rather than linger on life support without hope. Now, they want to hold on for a set of lungs, no matter how ill they become.

Because organs are in short supply, there are more tears than happy endings. For each person who gets a new set of lungs, two die waiting.

Strong patients decline so quickly they no longer qualify for the transplant list. Others don't make it through the operation. Or, they die before their new lungs ever show up.

Samuelson, who treats about 85 cystic fibrosis patients at a time, has seen it all. The uncertainties of science and fate cloud the life-and-death decisions each of his patients must face.

"If it were as simple as pulling the plug or not pulling the plug, it would be a lot easier," Samuelson says.

So for now, Shelby hovers in that limbo. Every time she has a bad episode, John prepares himself for the worst, his wife, Lynette, said later. When Shelby gets better he finds hope, and when she gets sick again he loses it.

"He relives that death a thousand times," she says.

June 29, ninth floor, Duke University Medical Center

Shelby sits on her hospital bed in her usual lotus position. Her mom, Winks, has come from Greensboro for a visit, but a trip to the gynecologist has left Shelby in a bad mood.

A nurse pokes her head in the room and asks how the exam went.

"Uncomfortable," Shelby says, not looking up.

She's not getting her periods a common problem for a woman so thin. She doesn't buy that. "It's because of the stress and because I've been sick," she says with a scowl.

Her mom says nothing.

A small woman with curly hair, a firm voice and a patient expression, Winks is a special education teacher who spends summer vacations working in her garden and relaxing.

Her life seems settled, but it wasn't always that way. The task of caring for Shelby as a child fell to Winks when she was a single mother working toward her graduate degree. It wasn't easy.

When the doctors told Winks that her baby had cystic fibrosis, she knew nothing about the illness.

"I thought they would give her something and it would go away," she says.

Instead, little Shelby only got sicker. She had several serious infections as a toddler and nearly died. After Winks and John split up, the chloramycetin triggered Shelby's blindness. On top of her lung problems, she had chronic ear infections and wet her bed until the age of 9. Her teachers identified her as learning disabled, and at 11 she was diagnosed as clinically depressed.

Shelby lets out a long, chest-rattling cough, but Winks just talks over it.

Raising a sick daughter was not without its rewards, she says. Shelby helped her gain the courage she needed to move away, go back to school and start a new life after her divorce.

"Being around that kind of personality really helped me take stock," she says. "She helped me believe in my life because she believed so much in hers."

At the same time, the qualities that allowed Shelby to persevere also made her difficult to deal with at times. In a 1985 paper Winks wrote for a graduate school course on childhood development, she gave credit to Shelby's resilience, but also described her as an "inflexible, obstinate, stubborn, willful, opinionated, uncompromising child to discipline."

It's not Shelby's favorite piece of writing.

"I can't believe you said some of the things you said about me," she tells her mom.

By now, Shelby has settled down over the pelvic exam and has some good news to report. She's not feeling so sick all the time and she gained two pounds.

"The other morning I was up to 72," she says. "74 at night."

And, she is going to start a flower garden.

They send her home a week later.

Privately, Winks says she can't bear to see her daughter suffer much longer. She wants relief for her child, one way or another.

"I'm not afraid for Shelby to die," she says. "I don't think she's afraid to die. It's living like this that I have problems with. I have a hard time seeing her in distress."

July 25, ninth floor, Duke University Medical Center

Shelby and her father have just had an argument, an ugly one as she tells it. They are at odds over why she's back in the hospital. He says she's overexerting herself. She says it's just the disease.

"Shelby was feeling real frightened this week because she couldn't get her air," John says.

She felt good until she went out to the movies with some friends, and then stayed up late the next night, he says.

"I just couldn't cough," she says.

John thinks Shelby pushes herself too hard, and Shelby thinks he's the one who does the pushing. Sometimes, she puts her head in her hands when he talks.

"She did too much too quick," John says. "She wants to have a normal life."

Shelby looks annoyed.

"At night, though, I feel so good," she says.

Now she's hooked up to the IV again, and it may take a little longer for this infection to clear up, they say. The doctors are reserving a few antibiotics for after the transplant. That way, Shelby won't have much of a resistance to them.

In the meantime, Shelby has launched her gardening project.

"I have a nursery in the bathroom," she says, nodding toward the tray of dirt just beyond the open door. The pansy seeds should sprout in a few days.

After her father leaves, Shelby sighs and apologizes for the tension in the air.

"I don't think he looks at my disease very realistically," she says. "He pushes me to do things I can't do and it makes my life hard."

The discussion they had about this got so heated, she says, John was about to walk out of the room.

"Maybe I didn't say it in a nice way," she says. "I guess we have to be patient with each other."

He finds comfort in denial, she says. She finds comfort wherever she can. Often, she turns to God and prayer. She believes in the afterlife and often dreams of heaven. Her mother says that at age 2, Shelby delighted family members by saying she could feel God inside her because "his beard was tickling."

Shelby has always had a spiritual side that is both emotional and intellectual. She majored in religion. In poems and prayers, she often pleaded with God to explain why she had to carry such a burden.

Sometimes she dreams of an afterlife when she will carry that burden no more. During a conversation in the hospital, she says she had a dream of heaven. "I was in a field and it was huge and I could fly and I felt totally at one with God," she says.

For now, she wants God's help in this world.

"In high school, I used to pray to God to cure me of my disease," she says. "When the transplant came, that was an answer to my prayers. It's a miracle they can do that kind of operation."

August 31, Park Drive

Shelby's been home for about three weeks now. She's in a real holding pattern. She watches television. She reads the Bible. She saw a documentary on Eleanor Roosevelt and got a large-type book from the library about the former first lady's life.

Today, she sits at the dining room table with a portable phone, looking glum. She still doesn't go out much. Some of her father's friends try to get her out of the house, but she doesn't really enjoy the outings.

"They come and see me and take me out and tell me all about their lives," she says. "I just don't have anything in common with them."

Then there's Brad. She met him a few months ago, when a friend brought him by the house. A student at N.C. State University, Brad took a liking to her and visited her in the hospital.

He's taken her to the mall. Now he wants to take her to Charlie Goodnight's, a comedy nightclub where he and a group of friends meet once a week. She wants to go but has her doubts.

It's not that she's easily exhausted. It's not the oxygen tank. She's uncomfortable with the way strangers relate to her.

"I was afraid that they would see me the way he did in the beginning," she says. "He was enchanted by my disability and my strength and how I dealt with this."

So, she had a long talk with him about that. He promised to try to see her as a person, not a patient. Since then, she's stopped referring to him as a "boyfriend," but she still gets that moony-eyed look when she talks about him.

"He's so sweet," she says. "He's very romantic. He would be a very good boyfriend."

Meanwhile, she's up to 77 pounds. Her medical records still note "significant malnutrition," and she continues to struggle with the tube feeding. Dr. Samuleson says her inability to gain weight is both a cause of her decline and a yardstick of its severity.

She is now No. 11 on Duke's lung transplant waiting list. She's hanging her hope on new lungs, but she sometimes worries that she may grow too frail to handle the operation. So do her doctors.

"The longer I wait, the more I know that possibility is there," she says. "But I believe God wants to give them to me."

September 26, Duke University Medical Center

Shelby lies on her side in her hospital bed as a nurse pats her back, trying to loosen the mucus. Her legs, without her lap pillow to cover them, look bone-thin. She's just been moved back from the intensive care unit. Winks sits at the end of the bed.

"My lung collapsed," Shelby announces. "I have this tube in my chest. It's very irritating."

About five days ago, doctors think, a cyst in one lobe of Shelby's lungs burst during a coughing spell. The fluid flowed out of the cyst, pushing her lung up against the chest wall. Luckily, she was already in the hospital.

As doctors were trying to sedate her so they could treat her lung, she blacked out and all of her vital signs, including her pulse, disappeared.

They were able to bring her back.

When she came to, she was in the intensive care unit. She spent five days there, hooked up to even more tubes and hoses than usual. She didn't much like it, and was glad to be moved out.

"It's real loud," she says. She doesn't know the nurses down there and feels she needs to check them out before they put in her IVs.

"She hate IVs," Winks says. "She's always interviewing people. 'How long have you been doing this, how many have you done?'"

Now she's hooked up to something even worse. A long yellow tube runs from an incision near her ribs, out from under her nightgown into a bubbling pump on the floor. It is siphoning the fluid out of her lung, and it hurts.

An intern knocks on the door, sticks his head in and asks how she is doing. Shelby asks him for Tedral, a drug that helps open the airways in the lungs. It also contains a low dose of phenobaritol, a narcotic.

He seems a little put off by her request, but she politely persists.

"I'm just having a hard time getting comfortable," Shelby says. The intern looks blank.

"I guess we'll have to deal with it," she says.

For Shelby, one good thing has come out of this episode. She's been jumped to the top of the transplant list. The next small set of lungs that comes in is hers.