Struggling To Breathe With Pulmonary Fibrosis

Written by Cynthia Demos

(CBS4) There's a disease that strikes people who are otherwise perfectly healthy and slowly robs them of their ability to breathe.

"When you get diagnosed with this, that's a death sentence," said 71-year-old Bob O'Rourke who is fighting for his life.

O' Rourke has Pulmonary Fibrosis (PF), a lung ailment which is slowly robbing his ability to breathe. Every year, the disease claims approximately 40,000 lives.

Four years ago, O'Rourke was diagnosed with idiopathic pulmonary fibrosis - a disease that scars and deteriorates the lungs -- ultimately robbing its victims the ability to breathe.

"So the lungs get very hard and they don't expand, it's almost like someone describing breathing through a straw," explained O'Rourke.

Although anyone can get pulmonary fibrosis, it mainly affects those 50 and older. O'Rourke has been on oxygen for the last 3 months.

According to pulmonary specialist Dr. Joseph Lynch, over the past 8 years, pulmonary fibrosis has been on the rise as more symptoms are being discovered.

"Basically it's in people who are perfectly healthy, who out of the blue develop shortness of breath," explained Dr. Joseph Lynch, Assoc. Chief, UCLA Division of Pulmonary & Critical Care.

Dr. Lynch says the disease becomes progressively debilitating as evidenced in O'Rourke's CT scans.

"You can see these areas, these little holes and those holes are scar tissues and all these lines here, its scar tissue. What you see is the outermost portion of the lungs are very irregular."

Bob has been taking steroids to slow the disease but the only way to survive now is with a lung transplant.

"I deny everyday that I'm going to lose him so I feel we will be blessed," said Bob's wife Sandy.

Little is known about pulmonary fibrosis -- what causes it, who can get it, and its deadly progression. But what is known is that this fatal disease takes 40-thousand lives per year, the same number as breast cancer. Currently there is no cure.