What Is Pulmonary Fibrosis (PF)?

Pulmonary Fibrosis is a sub-category of Interstitial Lung Disease (ILD) that sometimes is also known as “interstitial pulmonary fibrosis.” The terms interstitial lung disease, pulmonary fibrosis and interstitial pulmonary fibrosis are often used to describe the same condition.

The course of these diseases is unpredictable. If they progress, the lung tissue thickens and becomes stiff. The work of breathing then becomes more difficult and demanding. Some of the diseases improve with medication if treated when inflammation occurs. Some people may need oxygen therapy as part of their treatment.

The diseases may run a gradual course or a rapid course. People with ILD may notice variations in symptoms — from very mild to moderate to very severe. Their condition may remain the same for long periods of time or it may change quickly. It’s important to report any changes in symptoms. You and your doctor can work together to manage ILD.

What are the symptoms?

  • Shortness of breath, particularly with exertion
  • Chronic dry, hacking cough
  • Fatigue and weakness
  • Discomfort in the chest
  • Loss of appetite
  • Rapid weight loss

What Treatments are Available?

  • There are drugs that your doctor will suggest to try to reduce inflammation, slow the disease down, and help relieve your symptoms.
  • You may need continuous oxygen therapy at some point during the disease. At first it may be prescribed for use while you sleep or on exertion only. Do not be afraid or embarrassed to use your oxygen.
  • Treatment by a Pulmonologist, possibly at a local teaching hospital, that specializes in Pulmonary Fibrosis is always highly recommended. Following your treatment plan is very important.

What Do I Do Now?
You should:

  • Take your medicine as your doctor prescribes
  • Make any changes in diet or exercise that your doctor recommends
  • Keep all medical appointments
  • Be as active as you can be
  • Stay positive and hopeful

What is the prevalence of Pulmonary Fibrosis?
There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same number of people that die from Breast Cancer annually. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.

What are the causes?
Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:

  • Inhaled environmental and occupational pollutants
  • Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis
  • Certain medications
  • Therapeutic radiation

How is it treated?
There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress. Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.

What PF Has Taught Me

  • Join a Support Group
  • Get the BEST medical care possible from a Physician who specializes in PF
  • Learn good breathing techniques
  • Continue to exercise
  • Do not give up emotionally and conserve your energy

Pulmonary Fibrosis… it takes your breath away… A Texas Non-Profit Organization