Breathing New Life Into IPF Treatments

t 60 years old, Robert Davidson considered himself a lucky man. But in May of 2007, after completing a fitness test to referee at high-level soccer games, he found himself short of breath, and wondering if his luck had run out.

“That was enough to get me down to the doctor,” Davidson says. His family doctor recognized the signs of what could be a serious lung disorder and sent him to a respirologist for further testing. By October of that year, he received a diagnosis that would change his life: he had idiopathic pulmonary fibrosis (IPF).

“IPF is a condition where patients get progressive scar formations in the lungs,” explains Toronto respirologist Dr. Shane Shapera. “We don’t know why it happens, but over time the scar formation becomes more severe and eventually it causes problems with the exchange of oxygen from the air into the lungs.”

The correct diagnosis

Symptoms of IPF — such as shortness of breath, chronic dry cough, and a bluish tinge to the lips — are often misinterpreted as signs of other lung conditions. There are an estimated 14,000 Canadians living with IPF, and according to the Canadian Pulmonary Fibrosis Foundation, getting the correct diagnosis can take up to two years.

“My story is different from many others in that I was fortunate to immediately get two very good doctors who recognized the disease,” says Davidson, adding that since the disease is typically seen in patients over the age of 50, he’s heard of some professionals disregarding the symptoms as simply “old age.”

There is no cure for IPF and once diagnosed, patients are often given between two and five years to live. “Almost everyone gets worse over time, and eventually most people with IPF die from lung failure,” says Dr. Shapera.
However, when Davidson was diagnosed and faced with this timeline, he refused to give in. Since staying fit can help manage symptoms of the disease, he continued to referee soccer, and he made sure to get regular flu and pneumonia shots, as both illnesses can worsen IPF symptoms.

“I didn’t give up,” says Davidson, who founded the Canadian Pulmonary Fibrosis Foundation in 2009 to help others dealing with IPF and to fund research for treatments.

Life with IPF

Unfortunately, Davidson’s IPF progressed rapidly. Two years after his diagnosis, he required supplemental oxygen and was in a wheelchair, due to exacerbations.

Exacerbations are an unexplained sudden worsening of IPF. Having one is detrimental to an IPF patient and can have a negative impact on a patient’s prognosis. Indeed, mortality approaches approximately 50 percent in IPF patients following just one exacerbation.

“I went from an active person to really quite sedentary,” he says. Getting around was so exhausting that the Markham, ON man had to have chairs lined up to help him get to the washroom.

Davidson says he was close to death when he received news he was eligible for a double lung transplant — a procedure that is rarely an option for IPF patients. He successfully received new lungs in January of 2010.

New treatment, new hope 

In the nearly 10 years since his diagnosis, Davidson has seen remarkable advancements in IPF treatment including two new drugs that are able to slow the progression of the disease. Having lived with this debilitating condition, Davidson encourages all adults who notice IPF-like symptoms to see a respirologist to either confirm or rule out the disease. 

“Because there are now treatments that can slow the progression of the disease, there’s an emerging thinking that if we can find it early, we can consider starting patients on therapy when it’s appropriate,” says Dr. Shapera.
Davidson is living and breathing freely these days, and despite enduring IPF, he still considers himself lucky. 

“I have two grandchildren and another granddaughter on the way. These are people I never would’ve met if I hadn’t gotten a transplant,” he says. “Every day is a bonus.”

For more information about the Canadian Pulmonary Fibrosis Foundation please visit www.cpff.ca