What is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease where the lung tissue and the air sacs in the lungs become scarred and stiff.  This scarring (called fibrosis) makes it difficult for a person to breathe because the oxygen cannot pass through the walls of the air sac into the bloodstream.

About 140,000 Americans have been diagnosed with pulmonary fibrosis and it is most common in people between the ages of 50 and 75.  It belongs to a family of related diseases called interstitial lung diseases.  Because several of these diseases are similar, doctors and patients may refer to pulmonary fibrosis by a number of names.

Researchers suspect that pulmonary fibrosis changes the lung’s normal healing process.  Patients may have an exaggerated healing response that produces excessive scar tissue which causes the lung’s air sacs to thicken and stiffen. This leaves the lungs less able to function and provide the body with the oxygen it needs.

The cause of pulmonary fibrosis can sometimes be identified, but in many cases remains unknown.  If the cause is unknown, it is called idiopathic pulmonary fibrosis.  Some of the factors that may increase the risk of pulmonary fibrosis include cigarette smoking, some infections, exposure to pollutants, certain medicines, genetics, and gastroesophageal reflux disease.

Pulmonary fibrosis can develop quickly or slowly over time.  In some people, the disease progresses slowly, leaving the patient in a similar situation for years.  However, in most cases, the person has increased difficulty breathing over time and may become short of breath even when resting.

Adding to the seriousness of this disease is that it tends to lead to other lung problems including collapsed lung, infections, blood clots, and cancer.  As the disease progresses, it can also lead to respiratory failure, pulmonary hypertension, and heart failure.

There is no known cure for pulmonary fibrosis.  The life expectancy can vary greatly depending on the seriousness of the diagnosis, but many people live only 3-5 years after being diagnosed.

However, with proper treatment and careful supervision by a doctor, patients with pulmonary fibrosis can extend their both the length and the quality of their life.  Our next blog post will discuss in more detail the symptoms and treatment of pulmonary fibrosis.

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