Q: I was recently diagnosed with pulmonary fibrosis. I realize I won’t be cured. But what might I do to help prevent it from getting worse?
A: Pulmonary fibrosis is a condition in which there is significant or widespread scarring in the lungs.
The disease has many causes or triggers. These include medications, immune diseases and exposure to inhaled toxins such as asbestos. Sometimes no trigger is ever found.
Scar tissue in the lungs is usually permanent, just as it is in other parts of the body. There is currently no known way to cure pulmonary fibrosis. However, there are treatments that can reduce symptoms and slow the progression of the disease. In addition, avoiding pulmonary toxins (such as air pollution) and not smoking are essential.
Treatments for pulmonary fibrosis may include:
–oxygen
–pulmonary rehabilitation (exercise, chest physical therapy, and education)
–medications, such as pirfenidone, nintedanib and sildenafil
–lung transplantation
The choice of treatment depends on the severity and complications of disease and potential side effects of treatment. For example, sildenafil may be considered when the disease is advanced and causing strain on the heart. Nintedanib tends to cause diarrhea, while pirfenidone is more likely to cause nausea. But these two medications have been shown to slow worsening of pulmonary fibrosis.
Research is exploring therapies that can slow or reverse lung scarring. So, it’s worth asking your doctor about clinical trials of promising experimental treatments or you go online to Clinicaltrials.gov. You may be able to enroll in a study in your area.
Life expectancy with pulmonary fibrosis varies. Minor amounts of scarring in the lung may have little or no impact on lifespan. People with more severe or progressive disease may have a five-year survival as low as 20 percent. However, for any individual, prognosis is difficult to determine with high accuracy.
(Robert H. Shmerling, M.D., is Associate Professor of Medicine at Harvard Medical School and Clinical Chief of Rheumatology at Beth Israel Deaconess Medical Center in Boston. For additional consumer health information, please visit www.health.harvard.edu.)
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