A New Breath of Life

updated 07/06/1992 AT 01:00 AM EDT

originally published 07/06/1992 AT 01:00 AM EDT

IT WAS DAYS BEFORE SHILOH AVERY FULLY UNDERSTOOD HOW completely her world had changed. At first, following her double lung transplant at St. Louis Children's Hospital, the outgoing 9-year-old was consumed by a need to talk—but was stymied by the breathing tube in her throat. Frustrated, Shiloh motioned for paper and scribbled, "Get this tube out!" Five days later, when it was finally removed, Shiloh's attention turned to the ache in the pit of her stomach. But even as Shiloh spoke her first words—"Mom, I'm hungry!"—she realized that more than just an appetite had been awakened in her. With her green eyes widening in amazement, she excitedly whispered, "I can breathe!"

For Shiloh, who has suffered from cystic fibrosis since birth, being able to breathe—the most fundamental, and essential, of pleasures—has opened up a thrilling new world. No longer a spectator to her own confined life, she can, for the first time in three years, look forward to a summer of fun—riding her bike, climbing on the jungle gym and going fishing with her dad.

For most children, such pleasures are taken for granted. But for three years, no part of Shiloh's life could be carefree; "fun" was always accompanied by an oxygen tank and a long breathing tube. So it was that on March 9, after doctors had told her family that Shiloh's lungs were failing and that a transplant was her only hope, Shiloh received the lungs of a 5-year-old donor—and entered largely uncharted medical territory. According to the St. Louis International Lung Transplant Registry, about 70 percent of the 1,300 patients worldwide who have received a lung transplant in the past five years have survived at least a year. But because lung transplants are so new, especially in children, it is difficult to predict long-term survival.

Lung transplants are performed only as a last resort for cystic fibrosis patients, with the hope that the transplants will both prolong the patient's life and improve its quality. The new lungs are not affected by cystic fibrosis, and other complications of the disease can usually be controlled with medication. However, complications of the transplant itself—rejection of the lungs, or infection—can be fatal. Says Robert Beall of the Cystic Fibrosis Foundation: "We're hopeful that two new drug therapies, plus gene therapy in the near future, will someday prevent the need for lung transplants."

"I don't think you can call an organ transplant a cure," says Dr. Thomas Spray, who performed Shiloh's surgery. "It will extend the life of patients, but you are trading one kind of disease for another. Having a transplant is a chronic illness."

Shiloh is fully aware of her precarious condition. She knows that she came close to death and may merely have been given a reprieve. Still, the guarded prognoses of doctors seem, for the time being at least, somewhat beside the point. Consider the past three years. Shiloh's father, Charles, who recently retired as an Army staff sergeant and now works as a security guard, would wheel her oxygen tank up to the back door of their one-story home at Fort Hood, Tex., so she could splash, still attached by her plastic hose, in an inflatable pool. When she rode a horse at cystic fibrosis camp, she had a small portable tank hanging off the saddle.

"Now I can go through the tunnel in McDonald's playground," says Shiloh, beaming with delight at the thought of an unencumbered life. "I never could before because of my oxygen. Now I won't even have to ride the disease bus," she adds, referring to the special bus that carried her to Pershing Park Elementary School in Killeen, Tex., for two years.

Shiloh was born March 22, 1983 in Fort Benning, Ga. Four days later she began vomiting and turning yellow as a result of an intestinal blockage and was rushed to a nearby medical center. The blockage was surgically corrected, but its significance could not be dismissed: It indicated that Shiloh probably had cystic fibrosis, a genetic disease in which a thick, sticky mucus increasingly clogs and disables the respiratory and digestive systems.

A sweat test, administered when she was three months old, revealed a high salt content in her perspiration and confirmed the diagnosis. She was given enzyme therapy to help digest her food but, at the time, received no further treatment.

In 1984 the Averys—Chuck, his wife, Sharon, and Shiloh—moved to Hawaii, where Chuck had been reassigned. Shiloh seemed to flourish there; she grew and gained weight, spending all her time outdoors, swimming at the beach with friends or riding her skateboard. But in October 1988, three months after the Averys were transferred to Fort Hood, Shiloh began to suffer from severe breathing problems. She was in and out of the hospital for the next year and a half.

Three years ago, Shiloh's breathing became so labored she was put on oxygen. It was then that Dr. Dennis Schellhase, who treats cystic fibrosis patients at Scott & While Memorial Hospital in Temple, Tex., told the Averys that their daughter's only chance for a normal life was a transplant. Says Sharon: "We asked Shiloh, 'How would you feel if you could ride a bike, run and play softball again—if you could do everything your little sister does?' "

In May 1991, on Schellhase's advice, Sharon called St. Louis Children's Hospital to see if Shiloh would be eligible for the hospital's well-known transplant program. The Averys were so determined to find relief for her that they didn't even wait for formal acceptance. Chuck went ahead and petitioned the Army for a "compassionate" transfer, which was granted, and in July 1991 he moved his family to Fort Leonard Wood, 120 miles from St. Louis.

The people at Children's Hospital were amazed—and still are. "The Averys weren't going to take no for an answer," says Dr. George Mallory, who is medical director of the transplant program. Most families, he says, remain home until they are accepted, using the interim period to raise money for the transplant—up to $250,000. But Chuck and Sharon believe that Army insurance will eventually cover most of it. In any event the family now qualifies for Medicaid as well.

Last August, Shiloh spent two weeks in St. Louis undergoing medical tests and was accepted into the program. She went home to wait and managed to go to school—with her oxygen tank in tow—for several months. One Friday morning in December, the emergency room at Fort Leonard Wood hospital called Children's Hospital to say Shiloh was in critical condition. Children's immediately sent a helicopter to pick her up.

Dr. Mallory remembers Shiloh's condition when she arrived in St. Louis. "She had such a thick volume of mucus in her lungs it was amazing she could breathe," he says. Usually when children are that sick in the intensive-care unit they neither talk nor eat, but Shiloh did both. Mallory recalls thinking, "This girl is a go-getter."

Her mother's resolution, meanwhile, was weakening. While visiting Shiloh in the ICU, Sharon witnessed firsthand the desperate struggles of other patients. "There were kids dying right and left," says Sharon. "Finally one died from a transplant." Frightened, she told Dr. Mallory she was having second thoughts about the operation. "I never realized what kind of risks and suffering were involved," she says. "My husband and I were really thinking of pulling out."

But Shiloh wouldn't hear of it. She told her parents she wanted the transplant despite the risks—if it meant she might be able to go outside and play like the other kids.

"If you didn't know Shiloh," says Mallory, "the idea of a 9-year-old making this decision is absurd. But Shiloh knew what was happening—she knew she was dying and she knew she loved being alive."

On Feb. 12, Shiloh again went home to wait. By then she was the sickest child on the list. On March 9 the Averys received an early-morning call from Lynn Shaw, one of the coordinators on the transplant team. She said, "Don't feed Shiloh. They have found a donor." At 8:20 the hospital called again to tell the Averys to be there in an hour. Chuck took the call because Sharon was crying in the shower. "I was crying from happiness," she says. "I can't cry in front of Shiloh because she thinks something is wrong."

Shiloh remembers being scared that morning when her mother told her the news. But she calmed down on the way to the hospital and sang along with country and western songs playing on the radio.

Lung transplants were first attempted in the 1960s. But it was not until 1983 that the first successful operation was performed on an adult in Toronto. Whereas adults have transplants for conditions such as emphysema and pulmonary fibrosis, children are usually transplanted for cystic fibrosis and blood vessel disease within the lungs. "In general," says Dr. Spray, "children tend to be sicker, and their cases are more unusual."

Shiloh entered the operating room at about 4:30 P.M. Dr. Spray inserted each lung separately in her small chest through a clamshell incision from armpit to armpit, using the heart-and-lung machine to support circulation and respiration. "Shiloh had a lot of internal scarring," says Spray. "Because of repeated infection, the linings of her lungs stuck against her chest wall, and all those adhesions had to be removed."

When Shiloh awoke from the six hours of surgery, she had two IVs in her left arm, another in her neck, four drainage tubes in her chest, a catheter, and a breathing tube down her throat. She was stuck like a pincushion but accepted her condition bravely. According to Mallory, children with cystic fibrosis are often exemplary patients, for the most heartbreaking of reasons. "CF patients are acquainted with suffering," he explains. "They are familiar with taking multiple medications, and they know all about the ups and downs. They are no longer coughing, and they seem to delight in the fact that their wait is over."

Indeed, one of the few times Shiloh vented her displeasure was when the tubes in her chest were removed. As the resident approached, she screamed to her mother, "He's going to hurt me!" When the tubes were finally out, she turned to the resident and said with a pout, "You're not my buddy anymore."

Currently, Shiloh is fighting an infection around a tube used for administering medication and taking blood samples, but it will soon be removed. A recent lung biopsy, X rays and other tests show that her condition is improving. So far she has had just one episode of rejection, which sent her back to the hospital for two weeks soon after her transplant. Twice surgeons have had to open an airway in her right lung when it swelled shut, and in May she entered the hospital with pneumonia, but she has made a strong comeback. "Nothing holds Shiloh down, even when she is sick," says Bobbie Mackay, a pulmonary clinical nurse with the lung transplant program.

Children's Hospital has become Shiloh's home away from home. She returns three days a week for physical therapy and is currently walking at a brisk pace on the treadmill for up to half an hour. When in residence, she likes to hang around the nurses' station to help them answer the call buttons and chart data on the computer. She thinks one day she'd like to have a job using computers. "Shiloh is a tough little girl," says Mackay. "She captures everyone's heart."

Shiloh is so appealing, in fact, that some of the nurses have needed to steel themselves against her. "She is one of my favorite patients," says one nurse. "I could just eat her up. But I know she could be back on the unit tomorrow. So I put up my guard against getting my hopes up."

Of the first five patients in the St. Louis Children's Hospital program—who received their transplants from July 1990 to January 1991—two have died and three are doing well. "Our ultimate goal is long-term survival," says medical director Mallory. "That is what we are in it for."

Shiloh, meanwhile, is too busy living in the now to think about tomorrow. Sure, she has had to put up with a tutor this spring. And come fall she's going to have to repeat the third grade because she has missed so much school. But Chuck has removed the training wheels from her bike and oiled its rusty chain so she can finally learn to ride a two-wheeler.

In recent weeks, Shiloh has been making up for lost time in other ways as well. She has been to the St. Louis Zoo, a country and western concert by Alabama, a rap fest by Hammer and a St. Louis Cardinals baseball game, where she walked all the way to the top of the stadium and never once experienced shortness of breath. Where to next? This summer Shiloh is going to Six Flags Over Mid-America, near St. Louis, with other transplant kids and their families.

All that activity can become a bit daunting. On a sunny day some weeks back, Shiloh and her sisters, Shawna, 5, and Charyl, 2, went over to the playground across the street from the family's apartment complex. Shiloh sat quietly on a swing but did not want anyone to push her. Finally she climbed to the top of the jungle gym and grinned down at Shawna and Charyl.

"This is fun," she said, walking barefoot through the playground. "I can run. I can play. I can swing. I can swim in the pool. I can even fight with my sisters."

What more could a 9-year-old ask?


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