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■ HopkinsLung.com
Idiopathic pulmonary fibrosis (IPF) body with the oxygen it needs. doctors still may prescribe the
is a disease of mysteries. It has no The main symptom of pulmon- combination therapy for three to six
known cause, no cure, and reports ary fibrosis is shortness of breath, months to see if it has any effect.
of it are on the rise, although re- which may first appear during ex- Lung transplantation. This pro-
searchers aren’t quite sure why. ercise. As the disease gets worse, cedure is an option for some peo-
Today 200,000 Americans—most breathlessness can occur even ple with advanced IPF who are
over age 50—suffer from IPF, and when walking on level ground. Dry younger than age 65 and have no
it will claim the lives of nearly two cough is also a common symptom. other major health problems. How-
thirds of them within five years of Often, a person with IPF will as- ever, lung rejection is a common
diagnosis. Unfortunately, current sume their shortness of breath is problem, often leading to signifi-
treatments cannot be counted on simply a sign of aging or weight cant illness or death—only about
to stop the progressive lung scar- gain and, therefore, won’t seek half of IPF patients who have a
ring (fibrosis) that is the hallmark of medical help until the problem lung transplant are alive after five
IPF—and only one treatment, lung becomes severe and the lungs are years. Those who do survive must,
transplantation, has been shown to seriously scarred. Symptoms of for the rest of their lives, take drugs
improve survival. IPF may also be mistaken for other that suppress the immune system
Fortunately, the National Heart, lung diseases, such as asthma, in order to prevent rejection and
Lung, and Blood Institute (NHLBI) further delaying diagnosis and undergo frequent bronchoscopic
is spearheading a massive effort to treatment. As the disease pro- testing to look for infectious and
find an effective treatment for this gresses, however, other serious, inflammatory complications. Still,
devastating disease. Several new possibly life-threatening condi- lung transplantation offers the best
therapies are being studied around tions, including pulmonary arterial chances for survival.
the country, many of them at 12 hypertension (high blood pressure
medical centers that are part of the in the arteries of the lungs) and Supportive Care
NHLBI’s Idiopathic Pulmonary heart failure, may develop. The goals of supportive care are to
Fibrosis Network (IPFnet). Under relieve symptoms, keep patients as
the guidance of the NHLBI, these Limited Treatment Options active as possible, and improve
centers are conducting clinical tri- The goal of current treatments for quality of life.
als designed to identify better ther- IPF is to prevent more lung scar- Oxygen therapy. Supplemental
apies for IPF, in both its early and ring. What today’s treatments can’t oxygen therapy has been shown to
advanced stages. do, however, is reverse scarring improve quality of life and the abili-
that has already occurred. ty to exercise in people with chron-
What Is IPF? Steroids. The most common ic obstructive pulmonary disease
For years, researchers suspected treatment is the corticosteroid (COPD) and other lung diseases
that inflammation triggered the prednisone, which is used to re- and is often given to people with
lung scarring that occurs in people duce inflammation. It is often com- IPF who have low blood oxygen
with IPF. But more recent research bined with azathioprine (Imuran) or levels. But few studies have been
suggests that IPF’s chronic cycle cyclophosphamide (Cytoxan), conducted to evaluate its effective-
of injury leads to an exaggerated or drugs that suppress the body's ness in IPF.
uncontrolled healing response that, immune response. But because Pulmonary rehabilitation. Peo-
over time, produces fibrous scar researchers now know that inflam- ple with IPF may benefit from pul-
tissue. This scarring, in turn, caus- mation is not at the root of IPF, it’s monary rehabilitation. This is a pro-
es the walls of the tiny air sacs not surprising that this treatment gram of education and exercise
(alveoli), and the tissue between option is effective in only a small classes that teach you about your
them to thicken, making them less number of people. Nonetheless, in lungs, how to exercise and do
able to function and provide the the absence of good alternatives, activities with less shortness of
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