HALIFAX—Jerry Craig answers the door to his Dartmouth, N.S. home with a beaming smile despite the 50 feet of bright orange tubing trailing behind him.

For the last two months, he has been tethered to oxygen 24-7. But he doesn’t let that dampen his positivity. Craig is a fierce advocate for increasing awareness about Idiopathic Pulmonary Fibrosis (IPF), a disease he knew nothing about until his own diagnosis in 2014.

The chronic condition causes irreversible scarring (fibrosis) of the lungs. As the disease progresses the scarring worsens, making it challenging to breathe.

Last month Craig, 73, underwent intensive testing at Toronto General Hospital, where Atlantic Canadians are sent for lung transplants. A double lung transplant is the only treatment currently available to him as his condition deteriorates. He expects to hear within the next two months if he’ll be likely to get one.

“My Christmas wish is lungs,” Craig laughed. “The whole thing with the IPF, or any disease that is terminal, basically is that you have to look at every opportunity you can and every method to either minimize the damage of it, to make your life last longer, or find a solution, which is the transplant, and just make the best of it. Because otherwise you’re going to die.”

More than 14,000 Canadians currently live with the condition, equal to about the number of men (14,900) and women (14,500) diagnosed with lung cancer every year in this country.

A report released last month reveals gaps in quality of care for Canadians living with IPF. The report, titled The Burden of Idiopathic Pulmonary Fibrosis in Canada, showed disparities across the country when it comes to accessing treatments, resources and support services.

“While much progress has been made in the diagnosis and management of IPF, there are still ways to improve patient-centric care of this devastating disease in Canada,” the report’s conclusion notes. “Data shows that access to health services differ across the country, which could contribute to variation in care, treatment and ultimately health outcomes.”

Developed in collaboration with the Canadian Pulmonary Fibrosis Foundation (CPFF) and Hoffmann-La Roche Limited with input and guidance from a committee of IPF experts, the report concludes that “immediate action must be taken to make meaningful changes that will help alleviate the considerable burdens associated with the condition.”

“The key issue is probably access to specialists … There are not a large number of specialists available, and of course if the referring physicians don’t refer people to the specialists, then there will be a delay in getting proper diagnosis and treatment,” Dr. Martin Kolb, a professor of medicine in the respirology division at McMaster University and ones of the report’s contributors, explained in an interview.

“It was really dismal six or seven years ago with lag times (before diagnosis). That got better, but it is still not optimal.”

Kolb said of the almost 15,000 Canadians living with IPF today, about 7,000 of them will be dead within three years.

“When I talk to politicians or other people, I say that people with this disease are typically are not the people who would go out and rally. They just don’t have the breath,” Kolb said.

“They often suffer in a contained environment at home. They just don’t go out. That’s why you don’t see them. A lot of the suffering for patients with IPF is done behind closed doors.”

Delayed diagnosis is an issue facing many IPF sufferers, with some not receiving a correct diagnosis for five years. Craig was one of them. A non-smoker, he was healthy and very active before becoming afflicted with breathing difficulties.

Craig believes he was misdiagnosed for at least five years, receiving treatment for conditions ranging from pneumonia and asthma to postnasal drip. He was even tested for tuberculosis until he was finally referred in 2014 to a respirologist by his new and “astute” family physician.

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That was when he heard the three letters that would become an intimate part of his life — IPF.

“It’s idiopathic because they don’t know what the heck causes it. I was a dental hygienist in the military for 12 years and for 26 years after that in civilian life … and before that a dental assistant,” Craig said.

“So I was working on teeth all the time and in the days when barriers weren’t used. We didn’t use barriers up until about ‘80 or ‘81, so I could’ve breathed in a lot of crap in the meantime.”

In 2017, Craig co-founded a CPFF Halifax support group for IPF sufferers and their families. They meet the third Wednesday of every month and have anywhere from 20 to 28 members. He said they do their best to increase awareness about the disease not only for members of the general public, but among physicians too.

“General practitioners generally listen for the upper part of the chest instead of the lower, and there’s a description of the breathing that you hear (in IPF), it’s the tissue, the hardened tissue crinkling, that sounds like Velcro tearing,” he explained.

“They’re trying to educate doctors. I educate every physician I talk to now about it and say look for this because I would say the majority of people that are in our support group here have been misdiagnosed.”

Last week, Craig’s son set up a GoFundMe online fundraising page to help his parents weather the financial burden of living in Toronto for months before, during and after a lung transplant.

“I never thought that I would turn 73 and have only two choices to make — either let my lung disease kill me or get a life-changing lung transplant,” Craig is quoted as saying. “It’s come down to that and I know which my choice is.”

An avid and continuing volunteer for ElderDog, Craig has given many hours to numerous organizations and also served as a volunteer firefighter. He said the support he’s received from friends, neighbours and his family has made things much easier.

He beams proudly when citing the accomplishments of his wife Vickie, his son James, and grandchildren, Owen, 24, and Emily, 20.

“There are lots of reasons why it’s nice to be able to stick around after and get some extra life out of it and there are all kinds of things I still want to do,” Craig said.

“I love woodworking. I have a big shed out back here where I woodwork … I want to do model railroading still. I like to volunteer with ElderDog, things like that. Basically it’s just having more life.”

Yvette d’Entremont is a Halifax-based reporter focusing on health. Follow her on Twitter: @ydentremont

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