September is Pulmonary Fibrosis Awareness month, but what exactly is this serious lung disease? To break it down phonetically: pulmonary means lung and fibrosis means scar tissue, so basically the name pulmonary fibrosis translates to: scarring in the lungs. Beyond the problems scars present, pulmonary fibrosis impacts how you breathe and get oxygen into the blood. We sat down with Dr. Albert Rizzo, Chief Medical Spokesperson for the American Lung Association, and asked him what you need to know about pulmonary fibrosis.

1. What is pulmonary fibrosis?

In technical terms, fibrosis means thickening or scarring of the tissue. In this case, the normally thin, lacy walls of the air sacs in the lungs are no longer thin and lacy, but get thick, stiff and scarred, also called becoming fibrotic. With this scarring, the architecture of the lung gets stiffer and is less efficient at delivering oxygen into the blood stream. In addition, the stiffness or fibrosis of the tissue makes it more difficult to expand the lungs and breathe. It takes more effort to breathe and this additional demand of energy leads to shortness of breath.

2. What causes pulmonary fibrosis?

There are a number of known causes of pulmonary fibrosis, but there are also unknown causes called idiopathic. Exposure to toxins like asbestos, coal dust or silica (including workers in the coal mining and sandblasting industry) can lead to pulmonary fibrosis. Certain medications (amiodarone, bleomycin, nitrofurantoin, to name a few) list pulmonary fibrosis as a side-effect. A more common cause of pulmonary fibrosis is that it is diagnosed in conjunction with a group of diseases known as collagen vascular diseases. This would include systemic lupus, scleroderma, rheumatoid arthritis and Sjogren's syndrome. Pulmonary fibrosis can be hereditary as well. And as mentioned earlier, there are many cases of pulmonary fibrosis which cannot currently identify a cause and is therefore called "idiopathic" pulmonary fibrosis.

3. What is life like for those living with pulmonary fibrosis?

Early symptoms consist of chronic dry cough, and as it progresses, shortness of breath with exertion becomes evident. This can lead to a decrease in exercise tolerance, and this prolonged state of muscles throughout the body going unused can make the patient more easily fatigued. Every person who is diagnosed with pulmonary fibrosis has a unique experience with the disease. Some people stay in stable condition for years, while others may experience rapidly-worsening symptoms and overall health. Although no one can predict your prognosis for certain, you can help yourself by maintaining a healthy lifestyle and working closely with your healthcare team.

4. How common is pulmonary fibrosis?

It is hard to put a figure on the incidence of pulmonary fibrosis in general because it can co-exist with other conditions such as the collagen vascular diseases mentioned above and is also due to occupational exposures and reactions to certain medications. Idiopathic pulmonary fibrosis, or IPF, is more common than we once thought, with up to 132,000 people affected in the United States. About 50,000 new cases are diagnosed each year. It is more common in men than women, and mostly affects people over 50 years of age.

5. Are there resources available for patients and caregivers?

The Lung Association recommends patients join the Living with Pulmonary Fibrosis and caregivers join the Caring for Pulmonary Fibrosis online support communities to connect with others facing this disease. You can also call the Lung Association's Lung HelpLine at 1-800-LUNGUSA to talk to a trained respiratory professional or registered nurse who can answer your questions and connect you with support.

6. Are clinical trials an option?

It is always important to discuss treatment options with your physician. This may lead to secondary opinions or the possibility of being in a clinical trial. There are a number of clinical trials across the country that can be found at clinicaltrials.gov where new drugs are being tested or current drugs are being evaluated in different ways. If you live near a major academic center, it is worth reviewing their website for pulmonary fibrosis clinical trials for which you might be a candidate.

7. What is the most important thing everyone should know about pulmonary fibrosis?

There is no cure for pulmonary fibrosis. Current treatments are aimed at preventing more lung scarring, relieving symptoms and helping you stay active and healthy. Treatment cannot fix lung scarring that has already occurred. It is important to be a good communicator with your doctor and making sure that symptoms you have such as cough, shortness of breath are heard and evaluated earlier rather than later.

To learn more, visit the pulmonary fibrosis section of our website

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