It was a day we knew would come, sooner not later -- the day my little sister died. The whole family knew the odds of survival. Doctors level with all patients with cystic fibrosis, a hereditary disease that slashes at the lungs until they are too scarred to work. It is a death sentence present at birth. There is almost no chance at clemency. The reward for good behavior is a little more time.
Leslie Doyle Bowen had more time than most. She was first diagnosed at the U.S. Public Health Hospital in Baltimore at the age of 12 and she lived beyond her 32nd birthday. Life expectancy at the time of her diagnosis was 17.
The first comprehensive report of CF as a separate disease wasn't made until 1938, only 22 years be fore Leslie was born. Therefore, many doctors in the 1960s were unfamiliar with the particulars of the illness, and Leslie's childhood symptoms were so mild they might not have seen them had they known what to look for.
The symptom that tipped off the public health doctor was pneumonia, a chronic problem for CF sufferers. Our mother said that, at the time, she was relieved to be given a reason for Leslie's difficulties, ignorant of the disease's consequences. After all, her daughter didn't appear to be in danger.
However, there were many clues that she was. There were the hacking cough, the expanded rib cage on an otherwise skinny frame (a sign of chronic breathing difficulties), and the digestive problems (cystic fibrosis also hinders the work of the pancreas). Doctors couldn't piece together these puzzling symptoms, and prescribed only a change in diet to foods that didn't contain wheat. This change -- to bologna roll-ups (no bread) and rice cakes -- set her apart from the peanut butter-and-jelly crowd and brought ridicule in the school cafeteria.
Her toilet troubles, brought on by the digestive problems, were pointed out to her in the not-so-gentle way children have of elevating themselves in the schoolyard pecking order. The effect was to make her maddeningly defensive and too embarrassed to use a public restroom for the rest of her life.
Dr. Beryl Rosenstein, director of the Cystic Fibrosis Center at the Johns Hopkins Hospital, confirmed the diagnosis. But even when he spelled out the grim scenario she faced, there was no shattering of a preteen's belief in her own indestructibility. Leslie was going to beat this disease for which there is no cure, or at least live a full and normal life.
Doctors helped her in her pursuit, giving her pills to replace digestive enzymes her body would not produce. She took them by the handful with each meal, leaning her head back like a hungry baby robin and swallowing as many as a dozen at a time. These allowed her finally to take sandwiches in her lunch box. Potato chips were devoured by the bag, her need for salt providing a handy excuse for overindulging. (CF sufferers secrete two to five times more salt in their sweat than those not afflicted.)
Leslie gave herself a mandate to live, and cited it to waive off remonstrations that she should take care of herself. Rather than become melancholy over her outrageous fortune, she honed a sense of humor that would make her a delight to be around. She left no joke untold, no dance undanced, no curfew unmissed. That made her not atypical of other teens of the 1970s, except that others' indiscretions might not tear the pages off the back of a thin calendar.
She had been taught to perform chest therapy -- pounding on her chest and back to loosen the thick, sticky mucous that clogs the CF patient's lungs and hinders breathing -- but in those years she rarely felt badly enough to do it.
Leslie played her way through Towson High School, even spending two years on the badminton team -- proving our dad, a collegiate tennis player, passed something on to her besides the gene for CF. (He died of cancer in 1966, leaving our mother alone to raise two boys and two girls between the ages of 5 and 15.
This was before Leslie's illness was identified.) Her athletics were something others similarly afflicted could only dream about.
She once told me she didn't set any long-term goals because she didn't expect to live long enough to see them realized. The gravity of her situation was impressed upon her during nearly every visit to the Johns Hopkins Hospital, as the number of her peers decreased. But still she carried on like a person with a future.
She enrolled at Villa Julie College, a two-year school in Stevenson, studying to become a medical secretary. She also joined the disco craze, catching Saturday Night Fever several evenings a week.
"She was my most disobedient child," our mother says affectionately. "She was just a fun lover."
"She liked to party and used to tell us about it, show us pictures of her trips to the Caribbean," Dr. Rosenstein says. "Every experience was a wonder to her. She didn't let things keep her down. She wanted to do everything."
One of two big breaks in her life happened at the end of her time at Villa Julie, when she interned in the office of a doctor who was not put off by her disease.
"We were well aware of her physical condition," says Dr. Lee Gresser, a family practitioner in Towson. "The school told us about it, that she was a fair-to-good student who would need supervision for awhile, and would we take on such a person to train."
Dr. Gresser hired Leslie after her internship and employed her the rest of her life, even though she took a lot of sick days and needed long lunch breaks so she could do her now-essential chest therapy.
"There were times she would be ill and we would compensate for it," Dr. Gresser says. "We understood Leslie's problems and respected that."
Leslie's problems allowed her to empathize with the doctor's patients, and so she treated them as she wished to be treated. "She thought she was Dr. Gresser," the doctor says. "She would treat people on the phone; she was trying to help people in her own way. She let people cry on her shoulder, never let her disease cry on theirs. She never talked to people about her disease. Patients wondered why she coughed so much.
"Her one problem was she was so nice to patients she would spend too much time on the phone. . . . Despite Leslie's minor faults, she had such a great personality that we tolerated it."
Her second big break was meeting Albert "Duke" Bowen when she was in her early 20s. Duke knew the sacrifices he would need to make to live with her, that there was no chance of growing old with her. Still, he says, "I didn't see it as an undertaking. I thought of it more as selfishness on my part. I loved everything about Leslie, she had such a strong spirit. She taught me a lot of things. I miss her so much. She was my everything, everything I see as positive in life."
He picked the beach at Ocean City, one of Leslie's favorite places, to make his marriage proposal but she wouldn't cooperate. They were there with an other couple and she resisted his pleas to go off somewhere by themselves.
"Why are you being so gooey?" she would cackle when telling others this tale.
"She finally agreed to take this mysterious walk down to the water," Duke says. "On our way to the water I put the ring on the pinky finger of my right hand, and tried to flash it to get her to notice. As we sat by the water, I put my hand on her knee and thigh, but still no response.
"I said, 'Do you notice anything?' " and said the ring was for her. I think she forgot to answer. . . . When I proposed, she just dropped her jaw, said, 'Oh my gosh,' " and took off [up the beach]. She wanted to spread the news. . . . She did remember to take the ring.
They were married at Second Presbyterian Church in Guilford on June 18, 1988, so Leslie had a chance to taste all of the flavors of adult life except parenthood. Her maternal instincts were lavished instead on animals. She would stop her car to admonish people who were too harshly disciplining their pets. Her boss would bring his golden retriever to the office, and the dog would spend the day at her feet. She adored the family dog, a black Lhasa Apso who returned her affection. On the morning of her wedding, the photographer tried to shoo the dog from his accustomed position at her feet, only to be told by the bride the pose was perfect. It was Leslie's favorite photo of the day.
But not all was picture-perfect. In 1986, one of her lungs had collapsed, and with it her confidence in her ability to beat CF. If that were to happen to an otherwise healthy person, doctors would simply reinflate the lung. Leslie required surgery. Doctors created adhesions in the reinflated lung so it would stick to the chest cavity, then stapled the holes closed so it wouldn't collapse again.
The lung collapse brought her face-to-face with her future, and with it the fear of the hardships she had seen her late friends suffer at the CF clinic. Worse yet was the removal of the tubes inserted through her chest during surgery. Leslie had asked to be tranquilized during the procedure.
"She hated procedures as a patient. Hated most anything," Dr. Rosenstein says. "She admitted she was a coward."
While one of the nurses was getting the medication, a tired intern grew impatient with the wait and yanked out the tubes without warning.
Hurting and furious, Leslie cried and cried. "It was the worst experience she had," our mother remembers.
A far more cruel blow was delivered just three weeks before her wedding. Her other lung collapsed. She required more surgery, and there was concern she wouldn't be healthy enough to make the walk down the aisle. When Leslie arrived at the rehearsal dinner, our family's worries multiplied. Her face was the color of cream sauce, the smile absent. She left while everyone was still on the entree; we finished eating subdued, without appetite.
No one could have imagined then the joy of the next day. Mom said Leslie seemed to get a shot of adrenaline from having her attendants, which included our sister, Laurie, and my 3-year-old daughter, around her. Cliches such as "radiant bride" fit her as well as her wedding dress. When the new Mrs. Bowen triumphantly faced the rice-clutching crowd at the top of the church's steps, I greeted her with a shower of tears.
"The circumstances around her wedding were unbelievable," says Dr. Rosenstein. "It was incredible she was able to recover from her surgery, get on her feet and walk down the aisle as a beautiful bride. It was amazing."
The doctor attended almost as a proud father who had guided Leslie through life's trials. "Being able to go to her wedding was a reward," he says, "because years back patients didn't have weddings."
The reception was held at the Baltimore Museum of Art. Leslie stayed until the end, requiring only a short rest to make it through the celebration.
Leslie and Duke waited months to go on their honeymoon to the Caribbean, and in that time her condition worsened. She could hardly walk down the hall of their hotel without getting winded, but she pushed herself because she didn't want to spoil the occasion. She came home with pneumonia, which forced her to go on oxygen in early 1989; she could never get by without it again.
She didn't allow her spirit to drop with her condition. Instead, she kept up the pleasant demeanor that gave patients such a good first impression of Dr. Gresser's practice.
"She didn't dwell, or tried not to dwell, on the ultimate problem she would have to face," says Judy Gresser, the doctor's wife and Leslie's first office manager. "She didn't ask for sympathy. . . ."
If there was tension in the office, Leslie broke it, carrying laughter with her, along with the oxygen bottle that perpetually hung on her shoulder.
"Her facial expressions could just break you up," Mrs. Gresser says. "It was like a running comic strip, her wacky kind of offbeat humor. You could tell she was having a bad day if she was quieter."
Sometimes even the bad days didn't slow her down. The family took Leslie to see "Alien 3," and she needed several rest stops just to navigate the theater lobby. The effort of the evening took its toll: By the end of the less-than-memorable movie, her skin had turned blue, and everyone's mood matched. Still, she delivered a comic critique of the film far more devastating than two thumbs down, contorting the family's looks of concern into convulsions of laughter.
But inside, no one was smiling, because we knew the statistics. Cystic fibrosis is the most common fatal genetic disease in the United States. Every year, one in 2,000 babies are born with it, and a total of 3,000 cases are diagnosed annually, but there are only 30,000 people alive with the disease today. That number is much larger than in years past: When Leslie was diagnosed, the life expectancy was 17 years; when she died it was 27. The increase was brought on by the use of antibiotics that fight the damaging infections.
The infections grow as the thick, sticky mucous clogs the lungs more and more. The CF patient submits to pounding of the chest and back to loosen the mucous enough to spit it into a bucket. There are drugs to thin the mucous so it can be expectorated faster. But all of the treatments so far only delay the patient's demise, they don't prevent it.
In her late 20s, Leslie was spending as many as five hours a day doing the nauseating therapy. She reluctantly had a gastrotomy feeding tube inserted so she could keep on the weight needed to fight on. She required quarterly "tuneups" in the hospital, a two-week intravenous course of antibiotics -- stronger than you can get by mouth or aerosol -- and more intensive chest therapy.
There was no such thing as popping out of bed in the morning; she needed two hours for pounding and then time to force some breakfast into her roiling stomach. She worked a noon-to-10 p.m. shift that gave her two hours at lunch for more therapy.
The good-time girlfriends from the disco days were gone now, leaving behind a loyal group that would gather at Leslie and Duke's apartment in Northeast Baltimore, play Monopoly, fix meals and watch the pets play while Leslie would go through her treatments.
People were always waiting for her. She never ate a holiday meal with her gathered relatives. After work and more therapy, she would make a grand entrance during dessert and entertain us while eating our leftovers.
We began gobbling up news of the latest medical advances, hoping that in the tortoise and hare race between research and the breakdown of Leslie's body, the tortoise would miraculously win. While we watched this race being lost, she won our hearts.
"Leslie was a role model for the other patients who knew her; the nursing staff and medical staff had a lot of love for Leslie," Dr. Rosenstein says. "She wasn't just waiting for 32 years to die. She was determined to make something of her life and she did it."
"She was intensely proud that she was a physician's personal secretary, proud this was her responsibility," Judy Gresser says. "If I felt the way she felt, I could never have dragged myself in [to work]. It had to be a tremendous effort."
And then, in 1992, there was a ray of hope. There was a doctor at the University of North Carolina at Chapel Hill Medical Center who was having success with lung transplants. Leslie talked on the phone to a man in his late 20s who had undergone the procedure; he had just finished playing tennis and was having fun filling the time he used to spend in chest therapy.
I begged her to apply for the surgery right away, but Leslie was leery and afraid of the rehabilitation. "She had more fear of pain and painful procedures than of dying," Dr. Rosenstein says. She also feared dying on the operating table and preferred to live all the days her lungs would allow rather than take a chance on giving up even one.
She took months to gather her courage, but finally she, Mom and Duke headed for North Carolina. There, she was subjected to days of grueling tests to determine if she was a candidate for a transplant. In the worst of these, an incision was made in the femoral artery in her leg, where a catheter was inserted and pushed all the way to her heart. When the catheter was removed, the surgeon stopped the bleeding by applying so much pressure to the wound that it caused severe bruising. Then, a 10-pound bag of sand was placed on her leg and she was told to lie still, on her back, for eight hours. "This made it very difficult for her, because she didn't breathe well lying flat," Duke says. "That was very scary to her when she couldn't get a breath."
This pain paled next to what she felt when the doctors turned her down. She was relieved at not having to go through the surgery, but devastated that there was nothing more that could be done for her.
Surgeons had seen through the catheter a heart damaged by the stress of two lung collapses. Removing lungs attached to her pleura cavity would cause so much bleeding her weakened heart couldn't cope. The surgery she couldn't live without she wouldn't live through.
I had a week off from my job as a sports copy editor at the Buffalo (N.Y.) News at Thanksgiving, a few months after her rejection, and drove with my family from Buffalo to Baltimore. My mother's four children used her home in Stoneleigh as a gathering point. This is where we usually saw Leslie, who because she was dependent on Mom, never lived more than a few miles away.
But on this occasion, I made a point of taking my family for a long visit to her apartment. She showed it off proudly, and we spent hours watching our dogs play and laughing as if nothing was wrong. My wife, Tracy, a physical therapist, performed the therapeutic pounding for Leslie, who was exhausted by the holiday demands and too tired to do it herself.
On the way back to Mom's house, recalling Leslie's tubes, her lack of color and her face made puffy by the drug Prednisone, Tracy and I wondered if this would be the last time we would see my sister.
With the end of hope, Leslie's spirits drooped. She had longer and more frequent periods of depression. To combat this, Duke and their friends hatched a plan. The B-52's, a favorite band of Duke and Leslie's because of its upbeat style, would be in concert at Georgetown University's Smith Center on Jan. 16, 1993.
"As soon as I heard they were on tour I ordered tickets," Duke says. "Then I worried Leslie wouldn't want to go; she didn't want to get too far from the hospital or her mother. She was self-conscious in public carrying the oxygen. It was against her nature to have those feelings because she still loved people and parties, but she felt isolated.
"I wanted to make sure she felt comfortable so I called the Smith Center, got ahold of the production manager there, and made some special arrangements so we could be seated without a lot of walking; she wasn't strong enough to do all that maneuvering. They were very accommodating. He made sure we had a special entrance, he made sure we were escorted by the shortest route possible to our seats. He had reserved a spot for her on the floor, and made sure we didn't have to deal with any steps.
"As Leslie put it, it was just like the old times. We hadn't really been out to any event, out on a date, in a long time. We just really had the best time. I was so happy to see her forget about things for awhile. She forgot about the oxygen, and was dancing."
My mother called, ecstatic, to tell me the news, that Leslie had actually gone dancing, an act for which she had been admonished half a life ago.
It was just nine weeks later that Tracy called me at work and said: "Leslie has died." The day was March 24, the 36th birthday of my sister Laurie.
Our family, spread out from the Gulf Coast to the Great Lakes, began assembling in Baltimore on March 26. The mood was surprisingly upbeat. Leslie had feared lingering in the hospital, tied down by tubes and pinned down by needles. She didn't want to suffer, and at the end, she didn't.
On her last morning, as Leslie was struggling to prepare for work, she began having trouble breathing. So she phoned Dr. Gresser's office to say she wouldn't be in.
"The day that she died, I heard from her that morning and knew she was supposed to go to the clinic," Dr. Gresser says. "Two hours later they called us to tell us she was dead. We couldn't believe it; we had just seen her. We thank God she lived that long. She appreciated the fact that she was one of the oldest living CF patients."
Leslie phoned Dr. Sandra Walden, head of adult CF patients at Johns Hopkins, and told her she was feeling worse than she ever had before. Dr. Walden told her to come right in. Our mother was in Florida; she had passed up many trips to stay near Leslie in those last years, but had allowed herself this pleasure. Leslie declined an ambulance ride so she could make the half-hour trip with Duke, who said later he assumed she just needed another tuneup.
Doctors at Johns Hopkins recognized Leslie was deteriorating quickly and asked permission to intervene aggressively. When Leslie, who had been preparing for this moment for years, refused, the physicians got Dr. Walden on an emergency-room phone and asked for her support. She told them they were to keep their hands off.
Dr. Walden then met with Leslie and told her she was dying. She accepted the news with a glance at Duke.
"I was with Leslie the whole way, they kicked me out for just a few minutes," he says. "I was holding hands with her the whole time, trying to encourage her to stay with us."
Dr. Rosenstein said blood gases often get out of whack for those with CF, causing them to get hazy, then comatose. This is what happened to my sister. Dr. Walden says Leslie was alert for about 15 minutes after arriving at the hospital, and then she just slipped away. She died about an hour after her arrival.
And so we gathered to celebrate her life instead of her death. At Second Presbyterian Church, where the funeral was held, photographs were spread out on the church piano for us to laugh over. My brother, Steve, in delivering the eulogy, demonstrated for the mourners the face that only he and Leslie were talented enough to make -- top lip left, bottom lip right so that they draw a diagonal line. We all laughed in recognition.
The gene that causes CF was discovered in 1989, but Dr. Rosenstein believes it will be at least seven more years before gene therapy can solve the pulmonary problems that killed Leslie. However, there are some CF sufferers for whom this cure may not be too late. And so we take part in the Great Strides walk, an annual fund-raiser for the CF Foundation; we donate money; we vow never to stop fighting this disease. Leslie never did.
FLETCHER DOYLE is a sportswriter for the Buffalo News.