Understanding Pulmonary Fibrosis
Randy was diagnosed with idiopathic pulmonary fibrosis (IPF) on Christmas Eve 2008 — a day he will never forget. If you, or someone you love, has been diagnosed with pulmonary fibrosis, there are steps you can take to make living with this disease easier. Dr. Jane Dematte and Randy share more information about living and managing this rare lung disease.
Produced with support from Three Lakes Partners.
See these little coffee stirrers? You ever tried to breathe through one? That's exactly what having pulmonary fibrosis is like. No matter how hard you try, you can't get enough air.
Christmas Eve 2008, I got a call from my doctor. At work I was a firefighter, and we had to do a breathing test every year. Well, it came back that I flunked. I went in and had my lungs tested to determine what was wrong with them. The doctor called and told me, "You have idiopathic pulmonary fibrosis."
I was scared. First thing I thought was, the things I'd be missing. My wife, Marlene. My kids. Grandkids. Everything. I knew I wanted to live my life to the fullest, the amount of time that I had left. So Marlene and I did a lot of research, found a doctor we wanted to go see, and made an appointment.
Pulmonary fibrosis, in the literal sense, is scarring in the lung. When the soft tissue of the lung becomes scarred, it becomes much more difficult for oxygen to pass from the air sacs into the bloodstream. There are many causes of pulmonary fibrosis, and after close evaluation, if no cause is found, we then refer to it as idiopathic pulmonary fibrosis, or IPF.
If you've been diagnosed with pulmonary fibrosis, there are steps you can take to make living with this disease easier. Oxygen therapy may be prescribed. Pulmonary rehabilitation will help keep you active and maintain your quality of life. You may be eligible for clinical trials. And for some patients, pulmonary lung transplantation is an option.
If you've been diagnosed with idiopathic pulmonary fibrosis, while there is no cure for this disease, there are approved medications which help slow its progression. The most important thing you can do is speak with your physician about what treatment options are best for you.
After consulting my doctor, came up with the decision that I needed oxygen. And also, it was best for me to stay active. Being active, it showed I was fighting. I went for walks. I love to cook. I did whatever I could. And the oxygen therapy, it helped me keep up my energy and my strength.
My wife, Marlene, was my rock. She stood right through it, and still does, to this day. She was an advocate for research, where we needed to go, what doctors we needed to see. Without my wife, I probably wouldn't be here today. Together we kept hope that we could find a way forward. And we did.
After three years of living with IPF, and fighting for breath every day, I got the phone call that they had a donor, and I was going to get a double lung transplant. I'm one of the most blessed people in the world. I'm so lucky, it's unbelievable.
If you've been diagnosed with pulmonary fibrosis, you're not alone. Education and support are the keys to living with this disease. Visit lung.org/pf to learn more.
Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel. Last reviewed February 5, 2018.