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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 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 as die from Breast Cancer. 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
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Pulmonary Fibrosis…
it takes your breath away…
A Texas Non-Profit Organization
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Copyright 2009. PFAT All rights reserved.
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Pulmonary Fibrosis Association of Texas, Inc.
1442 Kingwood Drive #241
Kingwood, Texas 77339
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