NAVIGATING PF
How Serious Is Pulmonary Fibrosis?
PF is a condition that typically gets worse over time. The scarring that
happens cannot be reversed or repaired. Because the cause is unknown
in IPF, it cannot be prevented either. In other forms of PF when a
cause can be identified, removal of certain exposures or treatment of
autoimmune disease may result in improvement. Unfortunately, there
is no known way to reverse PF.
Unlike other chronic diseases, such as diabetes or high blood pressure, PF is relatively
rare, especially in younger patients. IPF affects about 132,000 people in the United
States. Almost 50,000 new cases are diagnosed every year. The symptoms of PF are
similar to those of other more common lung diseases, including pneumonia, asthma
and chronic obstructive pulmonary disease (COPD). Because of that, patients have a
delay in the correct diagnosis up to 50% of the time.
If the main symptom is shortness of breath, heart disease may be suspected first
rather than a lung disorder. The earlier a correct diagnosis is made, the better a
treatment plan can be put in place, including possible prescriptions for medications
that may help slow down the disease.
Some patients with IPF may not have much worsening of their symptoms over
time or may never need to use supplemental oxygen. However, most patients will
gradually lose lung function over time and will likely suffer from flare-ups (also called
exacerbations). Acute exacerbations may cause rapid worsening of the disease and
require hospitalization. Sadly, some patients experience rapid loss of lung function,
which may lead to respiratory failure and death. Their only option is a lung transplant,
if they are eligible.
Patients with PF that is not due to IPF may respond to certain medications. Their lung
disease may stabilize or even improve with the right treatments.
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