
Pulmonary fibrosis is the end stage of interstitial lung disease, a large group of disorders that cause progressive lung scarring. The current thinking is that pulmonary fibrosis begins with repeated injury to the lining of the alveoli, the small air sacs in your lungs. The damage eventually leads to scarring (fibrosis), which stiffens your lungs and makes breathing difficult.
No cure exists for pulmonary fibrosis, and current treatments often fail to slow the progress of the disease or relieve symptoms. A number of new therapies are in clinical trials, however, and researchers hope that better treatments will become available. In the meantime, a lung transplant may be an option for some people with pulmonary fibrosis.
Signs and Symptoms
The most common pulmonary fibrosis symptoms are shortness of breath (dyspnea), especially during or after physical activity, and a dry cough. Unfortunately, these often don’t appear until the disease is advanced, and irreversible lung damage has already occurred. Even then, you may downplay your symptoms, attributing them to aging, being out of shape or the lingering effects of a cold.
But breathing problems usually become progressively worse, and eventually you’re likely to get out of breath during routine activities — getting dressed, talking on the phone, even eating. At this point, symptoms are impossible to ignore.
You may also notice other signs and symptoms, including:
- Fatigue
- Unexplained weight loss
- Aching muscles and joints
Pulmonary fibrosis can vary considerably from person to person. Symptoms range from moderate to severe. Some people become ill very quickly, whereas others grow worse over a period of months or years.
Source: Mayo Clinic
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Is there a network blog of patients with pulmonary fibrosis who have taken pirfenidone (pirespa) for this condition. I would like to know their personal experience and which doctors are overseeing their treatment.
I’d like to share with other IPF patients. Have tons of questions. Need honest answers.