It's a disease that kills its own spokespeople.
Pulmonary fibrosis is incurable, typically progresses towards a rapid death and is expected to become more common as the population ages. But so far the lung disease that killed Evel Knievel and affects about 30,000 Canadians has flown under the public radar.
Robert Davidson, given a new lease on life with a double lung-transplant, is out to change that. After his own diagnosis with the disease, he started the Canadian Pulmonary Fibrosis Foundation, a registered charity that donated about $50,000 last year for study of the disease.
"We saw two needs, support for families and raising money for research," said the retired chartered accountant, 63, who is running this weekend at the Scotiabank Blue Nose Marathon in Halifax.
Mr. Davidson is planning to do the 10-kilometre event on Sunday to raise money and awareness. The distance may require some walking, which will be frustrating for the once-very-fit soccer referee, but he's still a far cry from his pre-transplant worst.
"I couldn't walk more than four or five paces before having to sit down," he said from his home in Markham, near Toronto.
Pulmonary fibrosis involves fibrous growth, commonly referred to as scarring, in the lungs. It can be connected to breathing harmful material, but it's not always clear what causes the condition; it tends more often to affect older people. The scarring worsens until people struggle to breathe. Patients are susceptible to infection and may come to rely on supplemental oxygen.
The only solution is a lung transplant, a procedure with a long waiting list that leaves recipients with a relatively short extended life expectancy. Without a transplant, average life expectancy for those with pulmonary fibrosis is about five years from the onset of symptoms.
"It's shocking how little media attention this disease gets," said Shane Shapera, a respirologist with the interstitial lung disease centre at Toronto's University Health Network.
He suspects that's partly because pulmonary fibrosis tends to progress too quickly for spokespeople to gain profile as the public face of the disease. And he noted that some who do survive end up becoming advocates for lung transplants instead of the disease that necessitated them.
"If we could learn more, understand more about pulmonary fibrosis itself, we could maybe find a cure and not need transplants," Dr. Shapera said.
Money raised by Mr. Davidson's foundation will be used at UNH to explore the fact that the vast majority of people with idiopathic pulmonary fibrosis suffer acid reflux. There is the intriguing possibility that the scarring of their lungs is caused by stomach acid.
"If we treat them for heartburn or acid reflux, can we try to prevent the progression of the disease?" Dr. Shapera wondered. "Theoretically we could have a very simple, easy-to-take treatment."
Mr. Davidson, who expects the foundation to continue to make grants of about $50,000 annually in pursuit of a cure, will be joined Sunday by a group of people touched by the disease. Among them will be Peter Kennedy, a resident of Sydney, N.S., who was diagnosed 3 1/2 years ago.
"You sort of create a bucket list," Mr. Kennedy said. "I said, okay, I want to do a half-marathon. I did that last year. I certainly wouldn't be able to do that again. But this year I'm going to do the 10K walk."