Roger Lemoyne/ Denise Grant/ Maclean's archives
Anyone driving along Highway 401 west to Toronto from Montreal on Oct. 6 would never have known Caroline Donelle had turned her red Honda Civic into a hospital room on wheels. In the trunk were five oxygen tanks and stashes of painkillers. Donelle kept thinking, please don’t let anyone rear-end me.
In the backseat was her daughter, Ashley, then 24, shrouded in a thick comforter, pillows and tubes, wafting in and out of consciousness. Diagnosed with cystic fibrosis as a baby, Ashley had defied expectations—reaching adulthood, attending university, travelling, even having a daughter, Leila.
Now though, her lungs were clogged and damaged with thick mucous, and her lung function was at an all-time low of 11 per cent. “I knew, I could feel it,” recalled Ashley recently from her apartment just outside of Montreal, “I was dying. I wasn’t coming out of it.”
A double-lung transplant is the only hope for CF patients who get as sick as Ashley was, and that procedure comes with a whole new set of complications and risks, including death. Ashley, who had been in and out of hospital for months as doctors tried unsuccessfully to get her disease under control, had been approved to have the surgery in Toronto, where wait times are shorter.
But just before she was supposed to leave Montreal for a preoperative assessment, Ashley took a serious turn for the worse. Doctors urged mother and daughter to re-book the appointment. They refused. Instead, Donelle packed up her car and, against medical advice, had Ashley released from the hospital. “I didn’t want her to miss this chance to get saved,” Donelle says, sitting across from Ashley at the kitchen table. “We had nothing to lose and everything to gain.”
Today, several months after her double-lung transplant, Ashley barely recalls the six-hour trip to Toronto, never mind the weeks before and after surgery. “I think it’s because I was oxygen-deprived, ” she half-jokes. Her mother, on the other hand, can’t shake the memory. “I remember that drive well,” Donelle says, “because I was scared. I’d occasionally reach back to feel if she was still warm.”
No one could have foreseen this future when Ashley appeared on the cover of Maclean’s in mid-July 1991 as a six-year-old astride her bike, smiling. “A genetic revolution” was written in bright-white type. “Why Ashley Dyer hopes to survive cystic fibrosis. The promise of new ways to treat fatal diseases.” Researchers had identified the gene responsible for CF, and the scientific community was ecstatic, certain even, that soon the disease would be annihilated.
Unfortunately, 19 years later, there is still no cure for CF. “There never was a genetic revolution,” says Donelle matter-of-factly. “Despite all the medical advances that have been made, there’s still no fix,” adds Ashley. “Even the transplant is only going to buy me time. I’m happy to have the time. If I live another 20 years, I’d be thrilled. If in two years I need another transplant, we’ll cross that bridge when we get there. But the fact that this illness is still there, it’s just an awful thing.”
That CF hasn’t been cured since Ashley became the de facto cover girl for the disease is not for lack of trying. Early on, scientists believed that gene therapy would solve the problem. The basic idea was to inject a good copy of the CF gene into a virus, which would then transfer it to the lungs. “Then presto, you have a cure,” says Dr. Francis Collins, one of the co-discoverers of the CF gene. Now, as the director of the National Institutes of Health, the federal biomedical research agency in the U.S., Collins looks back and says, “We were pretty naive.”
