Source: Wake Forest University Baptist Medical Center
Date: 2004-07-01
Surprising Finding Could Lead To New Treatment For Cystic
Fibrosis WINSTON-SALEM, N.C. – The surprising finding that
people with cystic fibrosis
(CF) produce too little airway mucus – rather than too
much, as it commonly believed – could lead to more
effective treatments for the genetic disorder, say
researchers at Wake Forest Baptist Medical Center.
"It has always been thought, but never proven, that CF
causes the body to produce too much abnormally thick mucus
that accumulates in the lungs and intestines," said Bruce
Rubin, M.D., professor of pediatrics. "However, we have now
shown that these patients actually have very little mucus in
their airways. This finding could change the way we think
about CF treatment."
The research is reported online today in the American
Journal of Respiratory Cell and Molecular Biology.
CF is a genetic disease that affects about 40,000 children
and adults in the United States. The disease is
characterized by frequent respiratory infections, breathing
difficulties, and eventually, permanent lung damage.
Physicians have always believed that the airways fill with
mucus, which normally lubricates and protects the
respiratory system. Because people with CF have chronic
cough and infection it has long been assumed that the
airways were full of mucus.
Rubin and colleagues, however, have shown otherwise. They
collected sputum from 12 patients with CF and 11
participants without lung disease and analyzed the contents.
Participants with CF had significantly less (70 percent and
93 percent) of two proteins that form mucus than
participants with healthy lungs.
"This showed unequivocally there is much less mucus in the
CF airway," said Rubin, a pediatric pulmonologist at Wake
Forest Baptist's Brenner Children's Hospital.
The research was conducted by Markus Henke, M.D., while he
was completing a fellowship at Wake Forest Baptist in
Rubin's laboratory. He is now at Philipps-University in
Marburg, Germany. Henke has since analyzed the sputum from
35 CF patients and said the results are consistent with the
earlier findings.
The researchers have shown that the substance clogging the
lungs of CF patients is actually pus. They suspect that the
airway in CF patients is chronically infected and that it
fills with pus. They also suspect that mucus may actually
protect the airway from infection. To test their theory,
they will conduct a study in animals to determine if mucus
can effectively "soak up" the bacteria that they believe is
reproducing in the airway of CF patients.
"If it turns out that mucus is protective against the
bacteria, we may have a treatment for CF," said Rubin. "We
believe that by increasing the mucus in the airway early on,
it may help prevent the infection. This certainly wouldn't
be a cure for CF, but it would make a wonderful difference
in quality of life while a cure is being sought."
Henke stressed that the finding applies to patients who are
stable, and not having a flare-up of their disease that
requires hospitalization.
Rubin said that if the animal research proves
effective, treatment in humans might be available in
the next five years.
"There are ways to increase mucus production in normal
airways, we just need to show that they are also effective
in CF airways," he said.
The research was funded by the Cystic Fibrosis Foundation.
Rubin is the author of "Therapy for Mucus Clearance
Disorders," published by Dekker/NIH as part of a series on
the biology of the lungs.
###
About Wake Forest University Baptist Medical Center: Wake
Forest Baptist is an academic health system comprised of
North Carolina Baptist Hospital and Wake Forest University
Health Sciences, which operates the university's School of
Medicine. The system comprises 1,282 acute care,
psychiatric, rehabilitation and long-term care beds and is
consistently ranked as one of "America's Best Hospitals" by
U.S. News & World Report.
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This story has been adapted from a news release issued by
Wake Forest University Baptist Medical Center.
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