— Despite claiming an estimated 40,000 lives annually —
roughly the same number as breast cancer or prostate cancer
— pulmonary fibrosis remains one of the least known covert
killers in the country.
of breast cancer come together in droves to raise
awareness," said Teresa Barnes, vice president of patient
outreach and program support for the Coalition for Pulmonary
Fibrosis, based in Culver City, Calif. "Pulmonary
fibrosis leaves no survivors," except a relative handful
who receive lung transplants.
fibrosis causes progressive lung scarring and eventually
on the horizon. The first treatment options available,
InterMune’s pirfenidone and Boehringer Ingelheim’s
nintedanib, are being evaluated by the Food and Drug
Administration and may become available as early as this fall.
disease has an annual fundraising walk/run in the summer in
Pittsburgh organized by Tami Rippy, whose mother died of the
disease in March 2009. "Knowing she was suffering, and
literally suffocating, that there was no treatment, no idea
where it came from or why it happened makes it
devastating," said Rippy. "People assume my mom got
it because she did this or that or smoked, but she was healthy
all these years."
terminal diagnosis often disguises itself in generic symptoms,
such as dry cough and fatigue. It typically affects people
over 50 but does not discriminate, debilitating runners and
smokers alike. The overwhelming majority of cases are
idiopathic, meaning no known cause is ever identified and the
condition is known as idiopathic pulmonary fibrosis or IPF.
to the coalition, pulmonary fibrosis patients may have an
exaggerated or uncontrolled healing response that, over time,
produces excessive fibrous scar tissue — or fibrosis — in
the lungs. It’s not known what sets this abnormal
tissue-repair process in motion.
is worse than a cancer diagnosis," said Kevin Gibson,
clinical director of a center at the University of Pittsburgh
Medical center that specializes in lung disease. "With
cancer you’re offered treatment, but with IPF there is no
treatment apart from transplant." Fewer than 1 percent of
these patients receive them.
drugs are considered breakthrough therapies, meaning the FDA
will expedite the process of their development and review. The
drugs work by trying to reduce the onset and progression of
the disorder, which also destroys blood vessels, diminishing
the ability to circulate oxygen in the body.
they’re approved it’s great, but the treatment’s effects
are modest at best," said Dr. Gibson. "It is still a
long way until a cure."
the condition has been recognized for the past century,
relatively little is known about pulmonary fibrosis, the
"disease course being highly variable and
unpredictable," he said.
being five times as prevalent as cystic fibrosis, which
receives about $85 million in federal funding annually, the
condition receives only $18 million a year, according to the
Coalition for Pulmonary Fibrosis.