The basic genetic defect which is responsible for cystic fibrosis remains unknown, but it is known that this defect causes excessively thick and sticky secretions of mucus. This thick mucus brings on an almost unbelievable variety of problems, the most serious being chronic and progressive lung infections, and inefficient digestion resulting from clogged digestive ducts. Mucus is produced by the lungs as their primary means of fighting infection (which is why people get congested when they have a cold), but CF patients cannot properly expel the thick secretions they produce and the mucus remains trapped in the lungs, where it fosters the growth of bacteria — which in turn promotes the secretion of more mucus, which then provides a home for more bacteria. It is this circular pattern of lung infections which eventually proves fatal.
In the ’50s a CF child could not be expected to reach school age. By the mid-’60s the average life expectancy had grown to age 11. Now, with better treatment and earlier detection, the national Cystic Fibrosis Foundation projects that half of all CF patients born today will live past the age of 21. But increased longevity has in many cases become something of a mixed blessing for longer-lived patients who have become grown-ups burdened with a disease perceived almost exclusively — both by the general medical community and the public — as an affliction of children only.
The executive director of the Cystic Fibrosis Foundation’s local chapter is Winnie Burke, an energetic and outspoken woman in her mid-40s who directs the chapter’s fund-raising and public-education activities from her office on Fourth Avenue in Hillcrest. “When I first took this job,” she says, “the first time I got close to a kid who died, I sat here in my office and I cried and cried. The other woman who was working in the office at that time came in and she said, ‘If you’re going to react like that every time one of these kids dies, I don’t think you’re going to be suited for this job.’ I looked at her and I said, ‘If I don’t react this way, I don’t think I’m going to be suited for this job.’ ” In the intervening ten years, Burke has had no shortage of opportunities to adjudge her job fitness, since this series of events repeats itself in her office with distressing regularity.
Like virtually anyone whose life has been touched by cystic fibrosis, Winnie Burke learned its lessons quickly. Doctors have called it “the great masquerader.” It can be subtle, whimsical, merciless, and above all, arbitrary. For a CF patient, life is a misshapen path of sharp turns and detours about which nothing is predictable except that it always leads downhill, sometimes gently, sometimes steeply. The disease is maddeningly erratic; what happens to one person is no indication of what will happen to another. Within a single family one child might die at a few years of age while another makes it into his 30s to become one of CF’s “old folks.” But such reprieves are understood to be only temporary. Today’s apparent good health, the brisk seven-mile run, the 75-mile bike ride, the workout at the gym, all too frequently give way to next week’s ten-day hospital stay. One minute’s laughter becomes the next minute’s wrenching cough, which, sometimes, leads to the burst artery and coughed-up blood of hemoptysis. And if it doesn’t, there is always the fear.
If these are extremes — the more common story of CF being one of frail, barrel-chested children, of frequently missed school and canceled family vacations — they are not atypical; it is a disease which defies neat description. It is a disease Camus or Sartre would have understood, for it teaches the meaning of existentialism as few things can. It is a disease which causes five- and six-year-olds to think deeply about sorrow and joy, the unfairness of fate, and why people are born to die.
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In 1967 cystic fibrosis became part of the life of an attractive, composed Coronado woman named Judy Longfellow. Her son, Mark, was 14 months old at the time and she recognized, she says, “that something was wrong. He had diarrhea an awful lot and was eating me out of house and home — 18 pancakes at one sitting, that sort of thing; it was incredible. It was some time before we came up with the diagnosis, because after we had tried eliminating different foods [to check for allergies], we did a sweat test at Mercy Hospital which came back negative. But our doctor was very suspicious that Mark had CF, so we took him up to Children’s Hospital for more work. They did several tests and they all came back positive.
“There was a panel of doctors, there were like three, and we went into this room — Ralph [her husband] wasn’t with me. It was a very dark dismal kind of room, a room that would hold maybe 30 people, and we sat in this little nucleus and they basically told me Mark had CF, and they didn’t really elaborate much at all except to say that it was a very harsh disease. Then they sent me on my way.”
But a few days later Mrs. Longfellow and her husband were provided with a few details by their specialist at Children’s.
“He came into the room and he said, ‘Well, you might as well know right now that if your son coughs, that’s just about the end; he will probably not last three months. He’ll never be able to go to school.’ The picture he painted for us was very, very black. So we were left with absolutely no hope that this child would live to be two years old.… His manner was very matter-of-fact, he was in the room maybe three minutes; he went through all this stuff and then he was gone. That angered me considerably.”
Mark first exhibited the deep, thick cough of CF when he got a lung infection about six months after this. The doctor’s three-month time limit proved wrong, however, and Mark did go to school — where he excelled. He was tested at the age of six and found capable of doing college-level math. “He loved math,” said his mother, “just loved it.” He was also an avid baseball player and one year he became a Little League all-star. Not until last spring, when Mark was 14 years old, did he die.