Pulmonary Fibrosis is a serious lung disease, but what exactly is it? 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 problem scars present, pulmonary fibrosis impacts how you breathe and get oxygen into the blood. We sat down with Dr. Albert Rizzo, Chief Medical Officer and Spokesperson for the American Lung Association and asked him what you need to know about pulmonary fibrosis.

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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 makes it 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. It takes more effort to breathe, and this additional demand of energy or work leads to shortness of breath.

2. What causes pulmonary fibrosis?

There are a number of known causes of pulmonary fibrosis. 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. Pulmonary fibrosis can also be a result of chronic hypersensitivity pneumonitis as a result of exposure to things in the home or workplace such as molds. Another common cause of pulmonary fibrosis is a group of diseases known as autoimmune conditions. This would include systemic lupus, scleroderma, rheumatoid arthritis and Sjogren's syndrome and others. Pulmonary fibrosis can be hereditary as well. Lastly, there are many cases of pulmonary fibrosis in which a cause cannot be identified and are 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 promote a more sedentary lifestyle. This prolonged state of muscle inactivity leads to deconditioning, making the patient more easily fatigued and shortness of breath with similar exertion. 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 autoimmune diseases mentioned above, and is also due to occupational exposures and reactions to certain medications. Idiopathic pulmonary fibrosis, or IPF, is considered a rare disease but is more common than we once thought, with up to 207,000 people affected in the United States and about 58,000 new cases diagnosed each year. It is more common in men than women, and mostly affects people over 50 years of age.

5. Are resources available for patients and caregivers?

There are a variety of things you can do to live well with pulmonary fibrosis, including eating nutritious food, staying active, reducing stress and protecting your lungs. Patients and caregivers can join American Lung Association’s Patient & Caregiver Network, the Living with Pulmonary Fibrosis and the Caring for Pulmonary Fibrosis online support community 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. How is pulmonary fibrosis treated?

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. Your doctor may recommend medication, oxygen therapy, pulmonary rehabilitation, a lung transplant and/or lifestyle changes. Treatment cannot fix lung scarring that has already occurred. It is important to maintain good communication with your doctor, making sure to share new or worrisome symptoms you are experiencing such as an increase in coughing or shortness of breath, so that you can work together on the best treatment plan for you.

7. 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.

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