Waiting for ‘The Call’ means life on hold for cystic fibrosis patient

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Whenever Allison Watson's cellphone rings, her heart skips a beat and, understandably, she gasps for breath.

The 27-year-old needs a lung transplant and "The Call" could come any time. When it does, she will have to be at Toronto General Hospital prepping for surgery within a couple of hours.

As a result, Watson has had to move from her home in Springhill, N.S., to Toronto.

And wait.

Wait for tragedy to befall another family, and hope that the person who has died (a), has signed an organ donor card and (b), has viable lungs that are a match.

"The waiting is hard," she said. "It's especially hard because I had to move away from my family and friends." (However, her boyfriend has moved to Toronto with her.)

"But when you're dying, you do what you have to do." Watson is not alone. According to the Canadian Organ Replacement Registry, 329 patients were waiting for a lung transplant as of Dec. 31.

Last year, 194 transplants were performed – the highest per-capita rate in the world, but still well short of demand. The majority of those getting new lungs are, like Watson, suffering from cystic fibrosis; the balance are split almost evenly between patients with idiopathic pulmonary fibrosis (scarring of the lungs) and chronic obstructive pulmonary disease, an illness often caused by smoking.

CF is a genetic disease that affects principally the lungs and the digestive system. It afflicts about one in every 3,600 children. While there are treatments for the symptoms – enzymes to help with digestion, a regime of exercises to clear the lungs and, ultimately, transplant – there is no cure.

The progression of the illness is unpredictable. Watson, for example, did not have any serious health issues until she was a young adult in university. But she lived her life knowing the day would come when her lungs would essentially give out, a sword of Damocles hovering above her head.

Last fall, the sword fell. Her condition had deteriorated to the point where she had only 30-per-cent lung function. That is the unofficial "magic" number that triggers the need for a transplant.

Watson was placed on the waiting list for a double lung transplant in October, 2013, and since then she has been a patient at St. Michael's Hospital in Toronto, home to the country's largest CF clinic.

"Patients go on the list when they reach a tipping point – when the risks of doing a transplant are less than the risks of not doing one," said Dr. Elizabeth Tullis, head of the division of respirology at St. Mike's.

A patient's number comes up based on the severity of his or her condition but, on average, the wait is about nine months. "The challenge I have with patients is if I send them to the list too early, they wait a long time, and if I do it too late, they die," Tullis says.

The cruel math is as follows: When lung function falls below 30 per cent, a patient has a 50-50 chance of living another two years. With a double-lung transplant, recipients have a 50-50 chance of living eight years or more.

The catch is that the mortality rate after transplant is significant because of the risk of infection and, most of all, rejection. Then again, among the 1,441 Canadians who are living today with transplanted lungs, many have lived 20 years or more.

"Lung transplant is truly a miracle for CF patients," Tullis says. Consider that, in 1960, 50 per cent of people born with cystic fibrosis died before the age of four – in other words, the median survival for a child born with CF was four years. By 1980, thanks principally to a better ability to treat infections, median survival rose to almost 20, meaning barely half of CF patients reached adulthood. The first double-lung transplant for a CF patient was performed in 1988. Since then, the median age of survival soared to 49 and it's easing up steadily.

"It's a longer life, a better life, but it's still a hard life," Tullis says.

Allison Watson, for example, has a morning regime that lasts almost two hours and includes taking steroids wearing an aerosol mask and doing chest compressions to unclog her lungs; she uses a variety of puffers and requires handfuls of pills to help her digest food. She lives in constant fear of contracting a cold, flu or other respiratory illness, which could prove deadly.

In Canada, lung transplants are performed only in five hospitals: Vancouver General, the University of Alberta Hospital in Edmonton, Winnipeg Health Sciences Centre, Toronto General and Hôpital Notre-Dame in Montreal. There are no transplant centres in the Eastern provinces, which is why Watson moved.

Not every transplant program requires patients to move; some will instead fly patients in by medical transport when organs are available.

The transplant team in Toronto has found that outcomes are better when waiting patients undergo a rigorous three-times-a-week exercise regime while awaiting surgery. That's not easy when you struggle to breathe, and when you cough constantly but, for the most part, Watson is stoic: "My full-time job now is to get strong in preparation for the transplant." She acknowledges that the waiting and the knowledge of the risks of a transplant – and the greater risks if no lungs become available – can be emotionally draining.

"Every now and then, I have these pity parties where I'm all 'woe is me,'" she says with a laugh. "When you're young and you're dying you get a little angry sometimes. You can't help but wonder, why me?"

And you can't help checking your phone.