Cedars-Sinai Blog

Beating the Odds

Idiopathic pulmonary fibrosis (IPF) patient Paul Giordano standing next to his classic car.

Paul Giordano felt like he'd fallen down a very deep rabbit hole when he learned 4 years ago that he has a rare, incurable lung disease with an average life expectancy of 3 to 5 years.

"I thought I might as well sell everything I own and make sure my wife is taken care of because this is it," the 73-year-old Yorba Linda retiree recalls.

But instead of selling, he ended up buying. He and his wife, Suszann, who have been married 51 years, acquired a small vacation home in Palm Desert after Giordano began a groundbreaking treatment regimen prescribed by a leading specialist at Cedars-Sinai.

Today Giordano can say, "I'm optimistic that I'm going to outlive this disease."

Strokes of luck

Despite the grim diagnosis he received when a pulmonologist identified his condition as idiopathic pulmonary fibrosis (IPF), a form of Interstitial Lung Disease (ILD). Giordano feels luck was on his side.

"Pulmonary fibrosis slowly robs patients of breath and, finally, life. We are working to change that."

The first sign of that was the vigilance of his primary care physician in Anaheim, which led to an early diagnosis. She detected a problem during a routine checkup before he had any symptoms, and referred him to a pulmonologist. IPF causes progressive lung scarring that makes breathing increasingly difficult and eventually leads to lung and heart failure. Although the pulmonologist offered little hope, he played a vital role in helping Giordano beat the odds by telling him about a renowned IPF expert at Cedars-Sinai.

Cedars-Sinai's Director of the Women's Guild Lung Institute, Paul W. Noble, MD.

Paul W. Noble, MD

Soon, Giordano was in the office of Paul W. Noble, MD, director of the Women's Guild Lung Institute and the Vera and Paul Guerin Family Distinguished Chair. The timing couldn't have been better. The FDA had just approved the first two medications to fight the progression of IPF—another stroke of luck for Giordano. Noble was the driving force behind a series of clinical trials that brought these new, game-changing therapies to the clinic.

"Dr. Noble was so kind, compassionate and reassuring," Giordano says. "His first words to me were, 'This is a journey, and I'll be there with you all the way.'"

Giordano and his wife embraced outside Noble's office that day. "Instead of being a voice of doom, he gave us hope. We couldn't believe it," Giordano says.

Paul Giordano

Optimistic about his disease, one of Paul Giordano's greatest joys these days is taking out his silver 1966 Mustang GT convertible for a drive with the top down.

Getting his life back

Giordano has benefited from both of the medications approved by the FDA. He took pirfenidone for a couple of years, and when side effects began to affect his digestive system, he found he was able to better tolerate nintedanib.

"These drugs slow the disease process down," Noble explains. "Increasingly, we're able to keep patients stable, or a lung transplant may be an option for those whose symptoms have progressed."

Knowing a transplant might be a possibility down the road, if necessary, gives Giordano peace of mind. Cedars-Sinai is one of the few medical centers in Southern California with Medicare certification for lung transplants, and the expertise of the Women's Guild Lung Institute enables older and sicker patients to be accepted for care.

Noble and his research team are making progress toward understanding the role of genetic predispositions in causing IPF in certain individuals and identifying how the disease functions at a cellular level. Their work could lead to the development of new medications to manage the disease and perhaps even to a cure.

"Pulmonary fibrosis slowly robs patients of breath and, finally, life," Noble says. "We are working to change that."

Giordano is profoundly grateful to be receiving the most advanced care, which has enabled him to continue to lead an active life. In addition to enjoying time with his wife, he walks his Tibetan terrier, Lilly, every day, rides his bike, plays guitar and takes good care of his silver 1966 Mustang GT convertible. He's proud of his 5 classic car trophies, and one of his greatest joys is taking drives with the top down.

"Dr. Noble gave me my life back," he says.