My uncle has idiopathic Pulmonary fibrosis, please suggest treatment if any of you know anyone survived?

Dear friends

My uncle has been diagnoised with idiopathic Pulmonary fibrosis 6 months now. He has been told that he would survive for only 4 years. If any one you know survived from this, please suggest what treatment would be good for this.He has dry cough and during cough he has little breathing trouble. No chest pain or so. Please advise.Thank you

I also have idiopathic pulmonary fibrosis. I was diagnosed in 98, the doctors cannot give an accurate prognosis for this disease. It varies from person to person. I'm on 2.5 to 4 liters of oxygen depending on my level of activity, I am not taking any medications now. The shortness of breath and coughing are a normal part of the disease. I'm doing OK. Your uncle must do what's right for him. One thing, eat small (6 meals a day is what my doctor has me eating) healthy balanced meals to keep chest discomfort down and his weight up. Keep a good outlook, a great sense of humor, and don't fret this disease, learn to coexist with it. I think that's what has kept me going. Say hi to your uncle for me, he's not alone.

What is Pulmonary Fibrosis?

Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue鈥檚 ability to transfer oxygen into the bloodstream.

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

Cigarette smoking

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.

There are a number of new trials testing drugs to treat Pulmonary Fibrosis. For more information contact us at:
Pulmonary Fibrosis Foundation
1332 North Halsted Street Suite 201
Chicago, Illinois 60622
(312) 587-9272 fax (312) 587- 9273