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
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
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.
American Association For Respiratory Care
9425 N MacArthur Blvd, Suite 100 , Irving , TX 75063
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
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
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.