Encyclopedia of Health and Medicine

cystic fibrosis and the lungs 201
easing the flow of air in and out of the lungs. COR-
TICOSTEROID MEDICATIONS reduce inflammation and
in some people may also help open the airways.
When infection is present, the doctor may prescribe
ANTIBIOTIC MEDICATIONS. However, people
who have COPD often have extensive bacterial
flora, making it difficult for the doctor to determine
whether there is an actual infection present.
People who have COPD should receive annual
INFLUENZA immunizations and PNEUMONIA vaccination
every five years. As with all lung diseases, it is
important to minimize as much as possible other
triggers: SINUSITIS, GASTROESOPHAGEAL REFLUX DISOR-
DER (GERD), and exposure to known ALLERGENS.
For some people surgery to remove the upper
lobes of the lungs, called lung volume reduction
surgery (LVRS), relieves tension within the thoracic
cavity and improves pulmonary function and
overall lung capacity. LUNG TRANSPLANTATION may
be a viable treatment option for some people,
replacing one of the diseased lungs with a donor
lung. The criteria for these procedures are stringent
and take into account numerous health and
lifestyle factors.
Nutritional support is essential for people with
advanced COPD, who typically lose significant
body weight as the effort to breathe requires
intense work from numerous muscles. Regular
physical exercise is also important. Though breathing
with exertion may severely limit the duration of
activity, maintaining physical STRENGTH allows the
body to make the most of the available oxygen the
lungs can diffuse into the bloodstream. Many hospitals
have pulmonary rehabilitation programs
with specialists who can teach targeted exercises to
improve AEROBIC FITNESS and MUSCLE strength. For
many people pulmonary rehabilitation is as effective
as any surgical alternatives. Walking remains
one of the most effective activities.
Complications of COPD are common, particularly
in the later stages. Typical complications
include HEART FAILURE, PULMONARY HYPERTENSION and
RESPIRATORY FAILURE. Doctors sometimes refer to the
combination of right-heart failure and pulmonary
hypertension as cor pulmonale. People who have
COPD are particularly vulnerable to viral infections
such as COLDS and influenza, and often
develop secondary bacterial infections such as
pneumonia and acute bronchitis.
Risk Factors and Preventive Measures
The leading risk factor for COPD is cigarette smoking.
The most effective preventive measure is
never to smoke and to avoid exposure to secondhand
smoke (ENVIRONMENTAL CIGARETTE SMOKE).
Smoking cessation can improve lung capacity and
function, though cannot undo damage that has
already occurred. Prompt and appropriate treatment
of other pulmonary conditions, such as
asthma, helps minimize permanent damage that
could set the stage for COPD to subsequently
develop. Though COPD occurs primarily in people
over age 40, this is a consequence of cumulative
damage to the lungs over time rather than aging.
See also BRONCHITIS; LIVING WITH CHRONIC PUL-
MONARY CONDITIONS; PNEUMOTHORAX; SMOKING AND
PULMONARY DISEASE.
collapsed lung
See ATELECTASIS.
cystic fibrosis and the lungs An inherited
genetic disorder affecting mucus production and
clearance, CYSTIC FIBROSIS alters the functioning of
exocrine glands throughout the body and affects
nearly all of the body’s systems. In the LUNGS, cystic
fibrosis causes changes in the consistency and
composition of the mucus the lungs secrete. The
mucus accumulates along the inner walls of the
bronchi, causing irritation and INFLAMMATION that
eventually thickens the walls of the bronchi. The
mucus becomes thick, creating obstructions in the
bronchi that reduce air flow and eventually produce
regions of ATELECTASIS (collapsed bronchial
segments). The plugs of thickened mucus also
attract BACTERIA, resulting in recurrent INFECTION
that manifest as BRONCHITIS and PNEUMONIA.
Health experts estimate that about 3 percent of
the population in the United States carries the
recessive GENE MUTATION for cystic fibrosis. The disorder
is 5 to 10 times more common among
whites than among other racial populations. Even
one generation ago, cystic fibrosis typically caused
death by late ADOLESCENCE. Current treatment
methods and early diagnosis has extended life
expectancy into the 30s for most people who have
cystic fibrosis, and many live longer. Cystic fibrosis
nearly always affects the lungs and requires continuous
therapy to maintain adequate BREATHING
and oxygenation.