An Unexpected Reprieve

When 6-month-old Tiffany was diagnosed with cystic fibrosis in 1972, her doctor warned her mother not to let her play with dolls. The girl would die before her 5th birthday, he said; why stir up maternal instincts she could not hope to fulfill? But by the time Tiffany reached 5, new treatments had arrived, and the doctors promised her a few years longer. It was to be the first of many reprieves as medical advances kept barely a step ahead of the growing girl. At 10, doctors said Tiffany would die in adolescence; at 18, she abandoned her dream of going to college because she did not expect to live to graduate. "I can't remember a time when I didn't know I was supposed to die," says Tiffany, now 33, who lives in Bradenton, Fla., with her husband, John Reid, and their three children. "But I'm still proving them wrong."

When cystic fibrosis was first diagnosed in the 1930s, 80 percent of its victims died before their 1st birthday as their bodies' mucus thickened, clogging their lungs and digestive tracts. But in the last two decades, new treatments have extended patients' life spans from months to years, and from years to decades. Cystic fibrosis is still the most lethal genetic disorder in America, affecting 30,000 people, but most sufferers now do not succumb until their mid-30s; a lucky few reach old age. With 40 percent of patients now older than 18, a new generation is living to face the challenges--both medical and emotional--of an adulthood nobody thought they would see.

The gift of life has come in installments. The extended life span of today's CF patients stems not from a single breakthrough but from a stream of minor innovations. Patients now stave off infection with a battery of different treatments: aerosols deliver increasingly potent antibiotics directly to their lungs; vibrating vests loosen their phlegm; fistfuls of enzyme supplements maintain their failing digestive tracts. "It's been an incredible success story, but we still have a lot of ground to cover," says Dr. Bruce Marshall, VP for clinical affairs at the Cystic Fibrosis Foundation. And despite new therapies targeting the deadly double-CF gene (carried in harmless single form by 10 million Americans), researchers say no decisive victory is imminent.

Doctors have sometimes been slow to realize the implications of their success. Until recently, patients in their 30s were still treated in pediatric wards, sitting in the same Winnie the Pooh chairs they had used as children. Dozens of adult centers have now opened, but the years in limbo left a mark, says Dr. Mike Knowles, codirector of the University of North Carolina's adult CF center. "Patients were sort of lost," he says. "They were not treated as if they were going to have a future, so they were not given the opportunity or responsibility to grow into mature young adults."

David Trester, a 32-year-old machine operator from Winona, Minn., blames cystic fibrosis for a youth spent drinking, fighting and racking up credit-card debt he never believed he would have to pay. "I was living in the fast lane," he says. "I didn't expect to live, so I figured, why not go out and enjoy every minute I can?" He reformed only when doctors told him potent new drugs meant he might live for decades--if his spiral into alcoholism and taste for Marlboros didn't kill him first. "As I got into my late 20s I realized, hey, I'm going to live," says Trester, who is now alcohol-free, married and expecting his first child. "I figured it was time to clean up my act."

As they embrace adult life, more and more CF patients are starting families, but the path to parenthood can be tough. Though few male CF patients can father children without expensive surgery, many young men were never told they were infertile. Female patients, in contrast, can conceive relatively easily, despite the predictions of some doctors that the disease would leave them sterile. There was initial confusion, too, over whether patients' children would inherit CF; doctors now know that transmission is impossible unless both partners carry a defective gene, and screening has become widely available only in the last decade.

And while genetic tests bring some peace of mind, prospective parents still face an agonizing dilemma. Despite medical advances, cystic fibrosis remains a fatal disease, and doctors often have to remind patients that they may not survive to raise their children. Pregnancy and sleepless nights strain patients' health, and exposure to schoolyard sniffles can trigger life-threatening infections in a parent's CF-ravaged lungs. "The easy part is getting pregnant," says Dr. Michael Boyle, director of the John Hopkins adult CF program. "Our job is to get patients to think more than nine months at a time."

For those who decide to have children, parenting can be both deeply fulfilling and bittersweet. Stacy Danko, a 40-year-old Baltimore mother of three, says she never regrets her decision; still, it's difficult watching her children, ages 14, 10 and 7, struggle to accept that their mother will die before her time. "I see their pain and it kills me," Danko says. "Your children are going to suffer as much as you do." And learning to live with that is the hardest thing of all.

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