Toronto General helping patients cope with rare IPF lung disease

News Sep 18, 2014 by Justin Skinner City Centre Mirror

For years, Barbara Barr’s doctors chalked up her breathing troubles to “sensitive lungs.” When she finally received a proper diagnosis – idiopathic pulmonary fibrosis (IPF) – she learned just how serious her problems were.

Barr is one of thousands of Canadians affected by the disease, which is perhaps less well-known than it should be after claiming the lives of Marlon Brando, Robert Goulet and Evel Knievel.

With care from doctors such as respirologist Shane Shapera at the University Health Network’s Toronto General Hospital, she may not be cured but she at least has some comfort in knowing what she is facing and in receiving the proper treatments.

Shapera is one of a select few doctors who specialize in IPF.

“Idiopathic pulmonary fibrosis is a scarring condition in the lungs that happens for reasons we don’t understand,” he said. “One thing we do know is that in all patients, the scarring gets worse over time.”

Symptoms include coughing and breathlessness and they get worse as the illness progresses. From the time of diagnosis, Shapera said, most patients only live three to five years.

“A lot of people think it’s like COPD (chronic obstructive pulmonary disease) but there’s one great big difference and that’s that (IPF sufferers) tend to need a lot of oxygen when we’re under exertion,” Barr said.

Barr had struggled with her breathing for years. Prior to receiving support at the University Health Network, doctors spent years coming up with a variety of theories for her condition, which could have actually made matters worse.

“There are medications out there for other lung conditions that do more harm than good for IPF patients,” she said.

Barr was the only non-smoker in a family of smokers, and she has a family history of lung problems. Her mother died of lung cancer and her brother died of emphysema, which made her symptoms all the more frightening, even as doctors told her they could not detect anything seriously wrong.

“I had a lot of colds and shortness of breath,” she recalled. “Finally, I was walking 10 feet to the bathroom and I started coughing myself inside out. I said then, ‘I don’t have sensitive lungs. I have bad lungs.’”

While there is no cure for IPF, researchers are making headway and current treatments can at least improve the quality of life for those living with the illness.

“We try to optimize patients’ care and check for heart problems which can develop after pulmonary fibrosis, lung cancers and blood clots in the lungs,” Shapera said. “We recommend they get flu and pneumonia shots, give them oxygen when they require it and recommend they get in shape.”

Patients are sometimes even given antacids, as doctors believe the scarring could occur in part because of acid reflux, with acid coming up from the stomach and burning the lungs.

Earlier this year, results from a trio of studies – each of which were inconclusive on their own – helped doctors determine a new drug can help slow the disease.

The drug, perfenidone, is expected to come on the market in Canada and offers hope but not a cure.

“I accept that there’s no one silver bullet, no single drug or treatment that’s going to make it all better,” Barr said. “It’s all the little pieces that go together that help manage the disease.”

Shapera noted some patients may get access to the drug through Ontario’s Exceptional Access Program, provided they qualify to receive it.

“Not everyone will be able to get it, but we’re hopeful that we can get it out to as many patients as possible,” he said.

Barr noted any new treatment is better than the options she and her fellow IPF sufferers had until recently.

“Ultimately, the only treatment was a (lung) transplant,” she said. “Now, that’s a last resort, not the next resort.”

Even that option was open to few patients. Shapera said estimates suggest 3,000 to 5,000 Canadians suffer from IPF but only 50 patients receive life-saving lung transplants per year.

Toronto General Hospital is certainly a leading light in that regard as the site where the first successful single lung transplant and the first successful double lung transplant were performed.

While there is still no cure for her condition, Barr is thankful for the care she has received there.

“For people like me, down University Avenue is where you can not only find the right answer, it’s where you find hope,” she said, adding patients who do receive the kind of care she got “get told ‘there’s nothing we can do for you; go home and buy your coffin.’”

With September serving as Pulmonary Fibrosis Awareness Month, Shapera is hoping more people learn about IPF, particularly those who may be suffering from the disease but who have not been properly diagnosed.

For more information about the illness, visit the Canadian Pulmonary Fibrosis Foundation at www.cpff.ca

Toronto General helping patients cope with rare IPF lung disease

Patient Barbara Barr thankful lung transplant now last resort

News Sep 18, 2014 by Justin Skinner City Centre Mirror

For years, Barbara Barr’s doctors chalked up her breathing troubles to “sensitive lungs.” When she finally received a proper diagnosis – idiopathic pulmonary fibrosis (IPF) – she learned just how serious her problems were.

Barr is one of thousands of Canadians affected by the disease, which is perhaps less well-known than it should be after claiming the lives of Marlon Brando, Robert Goulet and Evel Knievel.

With care from doctors such as respirologist Shane Shapera at the University Health Network’s Toronto General Hospital, she may not be cured but she at least has some comfort in knowing what she is facing and in receiving the proper treatments.

Shapera is one of a select few doctors who specialize in IPF.

“Idiopathic pulmonary fibrosis is a scarring condition in the lungs that happens for reasons we don’t understand,” he said. “One thing we do know is that in all patients, the scarring gets worse over time.”

Symptoms include coughing and breathlessness and they get worse as the illness progresses. From the time of diagnosis, Shapera said, most patients only live three to five years.

“A lot of people think it’s like COPD (chronic obstructive pulmonary disease) but there’s one great big difference and that’s that (IPF sufferers) tend to need a lot of oxygen when we’re under exertion,” Barr said.

Barr had struggled with her breathing for years. Prior to receiving support at the University Health Network, doctors spent years coming up with a variety of theories for her condition, which could have actually made matters worse.

“There are medications out there for other lung conditions that do more harm than good for IPF patients,” she said.

Barr was the only non-smoker in a family of smokers, and she has a family history of lung problems. Her mother died of lung cancer and her brother died of emphysema, which made her symptoms all the more frightening, even as doctors told her they could not detect anything seriously wrong.

“I had a lot of colds and shortness of breath,” she recalled. “Finally, I was walking 10 feet to the bathroom and I started coughing myself inside out. I said then, ‘I don’t have sensitive lungs. I have bad lungs.’”

While there is no cure for IPF, researchers are making headway and current treatments can at least improve the quality of life for those living with the illness.

“We try to optimize patients’ care and check for heart problems which can develop after pulmonary fibrosis, lung cancers and blood clots in the lungs,” Shapera said. “We recommend they get flu and pneumonia shots, give them oxygen when they require it and recommend they get in shape.”

Patients are sometimes even given antacids, as doctors believe the scarring could occur in part because of acid reflux, with acid coming up from the stomach and burning the lungs.

Earlier this year, results from a trio of studies – each of which were inconclusive on their own – helped doctors determine a new drug can help slow the disease.

The drug, perfenidone, is expected to come on the market in Canada and offers hope but not a cure.

“I accept that there’s no one silver bullet, no single drug or treatment that’s going to make it all better,” Barr said. “It’s all the little pieces that go together that help manage the disease.”

Shapera noted some patients may get access to the drug through Ontario’s Exceptional Access Program, provided they qualify to receive it.

“Not everyone will be able to get it, but we’re hopeful that we can get it out to as many patients as possible,” he said.

Barr noted any new treatment is better than the options she and her fellow IPF sufferers had until recently.

“Ultimately, the only treatment was a (lung) transplant,” she said. “Now, that’s a last resort, not the next resort.”

Even that option was open to few patients. Shapera said estimates suggest 3,000 to 5,000 Canadians suffer from IPF but only 50 patients receive life-saving lung transplants per year.

Toronto General Hospital is certainly a leading light in that regard as the site where the first successful single lung transplant and the first successful double lung transplant were performed.

While there is still no cure for her condition, Barr is thankful for the care she has received there.

“For people like me, down University Avenue is where you can not only find the right answer, it’s where you find hope,” she said, adding patients who do receive the kind of care she got “get told ‘there’s nothing we can do for you; go home and buy your coffin.’”

With September serving as Pulmonary Fibrosis Awareness Month, Shapera is hoping more people learn about IPF, particularly those who may be suffering from the disease but who have not been properly diagnosed.

For more information about the illness, visit the Canadian Pulmonary Fibrosis Foundation at www.cpff.ca

Toronto General helping patients cope with rare IPF lung disease

Patient Barbara Barr thankful lung transplant now last resort

News Sep 18, 2014 by Justin Skinner City Centre Mirror

For years, Barbara Barr’s doctors chalked up her breathing troubles to “sensitive lungs.” When she finally received a proper diagnosis – idiopathic pulmonary fibrosis (IPF) – she learned just how serious her problems were.

Barr is one of thousands of Canadians affected by the disease, which is perhaps less well-known than it should be after claiming the lives of Marlon Brando, Robert Goulet and Evel Knievel.

With care from doctors such as respirologist Shane Shapera at the University Health Network’s Toronto General Hospital, she may not be cured but she at least has some comfort in knowing what she is facing and in receiving the proper treatments.

Shapera is one of a select few doctors who specialize in IPF.

“Idiopathic pulmonary fibrosis is a scarring condition in the lungs that happens for reasons we don’t understand,” he said. “One thing we do know is that in all patients, the scarring gets worse over time.”

Symptoms include coughing and breathlessness and they get worse as the illness progresses. From the time of diagnosis, Shapera said, most patients only live three to five years.

“A lot of people think it’s like COPD (chronic obstructive pulmonary disease) but there’s one great big difference and that’s that (IPF sufferers) tend to need a lot of oxygen when we’re under exertion,” Barr said.

Barr had struggled with her breathing for years. Prior to receiving support at the University Health Network, doctors spent years coming up with a variety of theories for her condition, which could have actually made matters worse.

“There are medications out there for other lung conditions that do more harm than good for IPF patients,” she said.

Barr was the only non-smoker in a family of smokers, and she has a family history of lung problems. Her mother died of lung cancer and her brother died of emphysema, which made her symptoms all the more frightening, even as doctors told her they could not detect anything seriously wrong.

“I had a lot of colds and shortness of breath,” she recalled. “Finally, I was walking 10 feet to the bathroom and I started coughing myself inside out. I said then, ‘I don’t have sensitive lungs. I have bad lungs.’”

While there is no cure for IPF, researchers are making headway and current treatments can at least improve the quality of life for those living with the illness.

“We try to optimize patients’ care and check for heart problems which can develop after pulmonary fibrosis, lung cancers and blood clots in the lungs,” Shapera said. “We recommend they get flu and pneumonia shots, give them oxygen when they require it and recommend they get in shape.”

Patients are sometimes even given antacids, as doctors believe the scarring could occur in part because of acid reflux, with acid coming up from the stomach and burning the lungs.

Earlier this year, results from a trio of studies – each of which were inconclusive on their own – helped doctors determine a new drug can help slow the disease.

The drug, perfenidone, is expected to come on the market in Canada and offers hope but not a cure.

“I accept that there’s no one silver bullet, no single drug or treatment that’s going to make it all better,” Barr said. “It’s all the little pieces that go together that help manage the disease.”

Shapera noted some patients may get access to the drug through Ontario’s Exceptional Access Program, provided they qualify to receive it.

“Not everyone will be able to get it, but we’re hopeful that we can get it out to as many patients as possible,” he said.

Barr noted any new treatment is better than the options she and her fellow IPF sufferers had until recently.

“Ultimately, the only treatment was a (lung) transplant,” she said. “Now, that’s a last resort, not the next resort.”

Even that option was open to few patients. Shapera said estimates suggest 3,000 to 5,000 Canadians suffer from IPF but only 50 patients receive life-saving lung transplants per year.

Toronto General Hospital is certainly a leading light in that regard as the site where the first successful single lung transplant and the first successful double lung transplant were performed.

While there is still no cure for her condition, Barr is thankful for the care she has received there.

“For people like me, down University Avenue is where you can not only find the right answer, it’s where you find hope,” she said, adding patients who do receive the kind of care she got “get told ‘there’s nothing we can do for you; go home and buy your coffin.’”

With September serving as Pulmonary Fibrosis Awareness Month, Shapera is hoping more people learn about IPF, particularly those who may be suffering from the disease but who have not been properly diagnosed.

For more information about the illness, visit the Canadian Pulmonary Fibrosis Foundation at www.cpff.ca