September is Pulmonary Fibrosis Awareness Month
The incidence and seriousness of the lung disease pulmonary fibrosis.
Balintfy: Pulmonary fibrosis is a rare but serious lung condition that affects approximately 150,000 people in the United States. Dr. James Kiley at the NIH adds, while there are about 20- to 50,000 new cases each year, there may be more cases that are not diagnosed.
Kiley: And what's important about pulmonary fibrosis, September being pulmonary fibrosis month, is that this is an attempt to raise awareness about a very serious and important lung condition
Balintfy: Dr. Kiley explains that pulmonary fibrosis is a disease in which tissue deep in the lungs over time becomes thick and stiff, or scarred.
Kiley: That scarred tissue is what's called fibrosis. So when you have pulmonary fibrosis, you have lung impairment due to scarring of the lung tissue, which makes it very difficult to breathe and it then becomes a very debilitating disease.
Balintfy: Sometimes doctors can find out what's causing fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis or IPF. Dr. Kiley explains the symptoms.
Kiley: With many of the lung diseases, you will often experience a shortness of breath, difficulty breathing. You may be fatigued. You may have some aching muscles. You may actually have some unintended weight loss that you really can’t explain. There’s sometimes a very dry cough that goes on. So these are some of the signs and symptoms, the biggest of all are the shortness of breath, the difficulty breathing in doing just normal activities and that's one that you would want to be certainly aware of and discuss with your physician.
Balintfy: IPF has no cure yet. Therapies that are available include supplemental oxygen, pulmonary rehabilitation or possibly a lung transplant. Dr. Kiley is optimistic that new therapies or medications may become available through an NIH-funded research network.
Kiley: Right now, it's sponsoring two clinical trials, one of them looking at current ways to manage IPF through pharmacotherapy, another looking at carbon monoxide as a treatment for IPF. So there are a number of trials going on that are addressing the problem. We also know that the pharmaceutical industry is very interested in this disease and they’re conducting a couple of trials of some very novel agents that may be prospects for the future.
Balintfy: For now, Dr. Kiley stresses the seriousness of the disease.
Kiley: It's one that gradually develops and it's one that has a poor prognosis: three to five years after diagnosis tends to be the current prognostic development of the disease and that's sad and it's not good. So we're very actively engaged in research to try to look for new therapies and look for ways to best manage this disease.
Balintfy: For more information on pulmonary fibrosis and IPF visit www.nhlbi.nih.gov. And to hear more from Dr. Kiley on this topic, listen to episode 168 of the NIH Research Radio podcast. For NIH Radio, this is Joe Balintfy — NIH...Turning Discovery Into Health®