Robert O'Rourke has idiopathic pulmonary fibrosis, and he wants the world to know about it. It bothers him that a disease that kills tens of thousands of people each year in this country is largely unknown, so he's using his public relations background to try to put IPF on the map.
O'Rourke was diagnosed in the fall of 2006, and at the time, he was in charge of public relations for Caltech, one of the top research institutions in the country -- a job he'd held for nearly a quarter-century.
His symptoms were shortness of breath and a dry cough -- not very specific. His pulmonologist wanted more details.
"I did a lot of breathing tests at the local hospital, and the clincher was the CT scan. It was very evident there's a lot of scarring going on," O'Rourke says.
Unlike normal lung tissue, scar tissue can't take oxygen from the air and get it into the blood. People can tolerate a little bit of scarring in their lungs, but O'Rourke's lungs are riddled with scar tissue.
I first met O'Rourke a year ago when I was in California on a fellowship. Last summer he seemed fine, and I had no idea he was sick. When I saw him last month, things had changed.
"April of this year, things went south rapidly," he says. "And since then I've been on oxygen 24/7. And that's the way this disease is -- you can ride along very nicely, and then it takes a dip."
Bob still looks like Bob, sounds like Bob, jokes like Bob. The difference is that he has a long plastic tube running from his nose to an oxygen tank.
He says IPF isn't a painful disease, at least not in the usual sense.
"But it does have pain because of the breathing," he says. "You're always working at getting your breath."
'A Death Sentence'
Since his diagnosis, he's met a lot of people with the disease. He says newly diagnosed patients don't always know how bad the disease they have is.
"This is a very serious disease -- that there is no cure [for]," he says. "And I don't know whether the docs don't like telling you that news. I don't know. 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."
And right now, the only way to delay that is a lung transplant. O'Rourke's doctors put that option on the table back in April.
"They said, 'We want to start looking at you as a transplant candidate,' " he says. "So my pulmonologist said, '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 coming 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 one 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."
O'Rourke convinced them he was worth keeping around -- he's on the list. Now he has to wait.
Mary Armanios, a clinician and researcher at Johns Hopkins University School of Medicine, would like to find a better option than a transplant for people with O'Rourke's disease. She thinks the scarring may be a natural process that occurs as we get older.
"If you look at folks who live over 100 years of age, invariably they have a very, very early stage of idiopathic pulmonary fibrosis," she says. "That's why we've started to think about this as aging in the lung. And in some people it shows up more quickly or more prematurely than in other folks."
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.
"At least 10 percent of them have defects in telomerase," she says.
Armanios has been working with Carol Greider, who won a Nobel Prize this year for discovering telomerase. They believe a variety of diseases are caused by defects in telomerase, bone marrow disease and liver diseases, as well as lung diseases. Armanios says this insight should help researchers find new treatments.
"Without having a clue about cause, we don't have a shot. But now, with genetics, we can really begin to move forward," she says.
And nothing would please Bob O'Rourke more.