Breathing New Life Into Lung Disease Awareness For patients with idiopathic pulmonary fibrosis, breathing becomes difficult as scar tissue builds in the lungs. No cause is known and no treatment is yet available, and one patient with IPF is using his public relations background to put the disease on the map.

Breathing New Life Into Lung Disease Awareness

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Idiopathic pulmonary fibrosis is a dreadful disease. If you get it, your lungs stop working and you die. It's rare but not that rare. By some estimates around 100,000 people in this country have the disease.

NPR's Joe Palca has this story about a man with pulmonary fibrosis and some research that may point the way to a treatment.

JOE PALCA: It bothers Robert O'Rourke that so few people have heard of idiopathic pulmonary fibrosis. He wants to change that, so he's telling his story.

Mr. ROBERT O'ROURKE: I got diagnosed in the fall of 2006.

PALCA: At the time, Bob was in charge of public relations for CalTech, one of the top research institutions in the country. His symptoms were shortness of breath and a dry cough; not very specific. His pulmonologist wanted more details.

Mr. O'ROURKE: Did a lot of breathing tests at the local hospital, and then clincher was the CT scan. And thats very evident that there's a lot of scarring going on.

PALCA: Bob's lung is full of scar tissue. Thats what happens with this disease. And scar tissue can't take oxygen from the air and get it into the blood like normal lung tissue can.

I first met Bob a year ago when I was in California on a fellowship. I had no idea he was sick. When I saw him last month, things had changed.

Mr. O'ROURKE: April of this year, things went south rapidly. And since then, I've been on oxygen 24/7. And that's the way this disease is: You can ride along very nicely, but then it takes a dip.

PALCA: Bob still looks like Bob, sounds like Bob, jokes like Bob. The difference is he's got a long plastic tube running from his nose to an oxygen tank. He says idiopathic pulmonary fibrosis isn't a painful disease, at least not in the usual sense.

Mr. O'ROURKE: But it does have pain because of the breathing. You're always working at getting your breath.

PALCA: Since his diagnosis, he's met other people with the disease. He says newly diagnosed patients don't always know what they're up against.

Mr. O'ROURKE: This is a very serious disease that there is no cure. And I don't know whether the docs don't like telling you that news, or I don't know. But it's something that - the reality of it, you've got to learn how are you going to live with this? How's your spouse going to live with this? How about your kids, your grandkids? It is a death sentence. There's no other way to put it.

PALCA: And right now, the only way to delay that death sentence is a lung transplant. Bob's doctors put that option on the table back in April.

Mr. O'ROURKE: They said, we want to start looking at you as a potential transplant candidate. So my pulmonologist said, now, you have got to put on your PR face, of all the times you've been doing this, because when they come around, there'll be a whole team come through to interview you. If you look like a dumpy old 71-year-old man, you're not going to get a transplant.

So the first guy that came to see me the first one that came to see me was a psychiatrist. She looked like she was 12. Then came the head of surgery for transplant. All kinds of doctors coming through at UCLA to check me out. And they had to each write a report about this Irish kid in bed 12.

(Soundbite of laughter)

PALCA: I guess Bob convinced them he was worth keeping around: He's on the list. Now he has to wait for the call.

The word idiopathic in idiopathic pulmonary fibrosis means no one knows what causes the disease. That's one reason why it's been hard to find a treatment. But Mary Armanios has some ideas. She's a clinician and researcher at Johns Hopkins Medical School. She thinks the scarring may be a natural process of aging, it just hit Bob early.

Ms. MARY ARMANIOS (Clinician, Researcher, Johns Hopkins Medical School): If you look at folks who live over 100 years of age, invariably they have a very, very early stage of idiopathic pulmonary fibrosis. So that's why we've kind of started to think about this as aging in the lung. And in some people, it happens more quickly or more prematurely than in other folks.

PALCA: Armanios thinks this aging process is related to an enzyme called telomerase. Telomerase is in every cell in the body and is critical for preventing premature aging. So she looked at this enzyme in people with pulmonary fibrosis.

Ms. ARMANIOS: At least 10 percent of them have defects in telomerase.

PALCA: Armanios has been working with Carol Greider, who won the Nobel Prize last year for discovering telomerase. Theyre beginning to think that the defects in telomerase may be the cause of a whole host of diseases, not just lung disease.

Ms. ARMANIOS: Without having a clue about cause, we don't have a shot. But now, with genetics, we can really begin to move forward.

PALCA: Move forward and find treatments for pulmonary fibrosis and other diseases. I'm guess nothing would please Bob O'Rourke more.

Joe Palca, NPR News.

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