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What is “Idiopathic Pulmonary Fibrosis”? 

For Immediate Release

IRVING , TX (July 1, 2005) – Most people think of asthma or emphysema when they think of conditions affecting the lungs. But there's another lung disease that deserves our attention as well: idiopathic pulmonary fibrosis, or “IPF.” While not as well known as other lung diseases, respiratory therapists (RTs) from the American Association for Respiratory Care (AARC) say over 200 diseases result in pulmonary fibrosis and affect more than five million people worldwide.

“Idiopathic pulmonary fibrosis is a chronic lung disease that causes inflammation, scarring, and thickening of the lung's tissues,” explains AARC member Trish Blakely, RRT, from Palmetto Respiratory Inc., in Columbia , SC. “As the thickening increases, the ability of the lung tissue to transfer oxygen to the bloodstream is compromised.”

Blakely says IPF is most common in people between the ages of 50 and 70, who are often diagnosed after suffering from symptoms like shortness of breath, a dry, hacking cough, and fatigue and weakness. The disease can also cause discomfort in the chest, loss of appetite, and rapid weight loss.

Doctors aren't sure what causes IPF, but Blakely says researchers speculate it may be related to microscopic injury to the lung. “Associations have also been made with inhaled environmental and occupational pollutants, certain medications, or exposure to therapeutic radiation.” Some people may have a genetic predisposition to the disease as well.

How is IPF diagnosed? “Tests that help to diagnose IPF include chest x-ray, CT scan of the chest, pulmonary function tests, bronchoscopy with lung biopsy, measurement of blood oxygen levels at rest or during exercise, and tests for connective tissue diseases such as sarcoidosis, lupus, or systemic scleroderma,” says the registered respiratory therapist.

Once diagnosed, people are usually treated with medications that help suppress inflammation in the lungs, but Blakely says the most helpful treatment is usually supplemental oxygen, which can be provided to patients at home by a home care company and respiratory therapists. “Most IPF patients are followed regularly by their doctor and home care RT to monitor their lung function and to help identify acute changes in breathing and blood oxygen levels,” says Blakely. “Patients who use oxygen on a regular basis should be monitored regularly by their home care RT to help reduce the severity of symptoms and to improve their quality of life.” 

Is there anything people can do to prevent IPF? Since the cause of the disease remains unknown, Blakely says it's difficult to offer specific advice. But at the least, people can avoid exposure to environmental or occupational pollutants and – most specifically – cigarette smoke and smoking. Indeed, those are good recommendations for just about anyone, regardless of whether they are susceptible to IPF or not.

Idiopathic pulmonary fibrosis isn't an easy disease to live with, concedes Blakely, but help is available. “Support groups can often help people with IPF through sharing common experiences and problems and as a mechanism for reducing stress.”

Respiratory Therapists (RTs) are specially trained and licensed respiratory health care professionals assisting physicians in diagnosis, treatment, and management of respiratory diseases. RTs provide care in hospitals, outpatient centers, physicians' offices, skilled nursing facilities, and patients' homes.

The American Association for Respiratory Care (AARC) is a not-for-profit, professional organization, consisting of 36,000 respiratory therapists, physicians, and other health care professionals. AARC is dedicated to assisting persons with respiratory diseases receive safe and effective respiratory care.


Contact:  Beth Binkley
American Association For Respiratory Care
9425 N MacArthur Blvd, Suite 100 , Irving , TX 75063
972-406-4657, 972-243-2272


Facts About Idiopathic Pulmonary Fibrosis

The American Association for Respiratory Care offers the following facts about pulmonary fibrosis:

  • More than 5 million people worldwide suffer from the disease; 200,000 in the U.S. alone.

  • There is no known cause for the condition.

  • Key symptoms include shortness of breath, dry cough, fatigue and weakness, chest discomfort, loss of appetite, and rapid weight loss.

  • Diagnostic tests include chest x-ray, CT scans, pulmonary function tests, bronchoscopy with lung biopsy, measurement of blood oxygen levels, and tests for connective tissue diseases.

  • Treatment includes anti-inflammatory medications and supplemental oxygen; in severe cases, patients may undergo a lung transplant.

  • Average survival time is five-to-seven years, but survival times vary widely between patients.

  • Support groups are available across the country to help patients cope with the condition.

For more information about idiopathic pulmonary fibrosis, ask your doctor or respiratory therapist. If you're a idiopathic pulmonary fibrosis patient, your doctor or RT can also help you find a support group in your area.