Emphysema

Emphysema is a progressive lung disease that results in

shortness of breath and reduces your capacity for physical
activity. The cause of emphysema is damage to the small air
sacs and small airways in your lungs. This damage obstructs
airflow when you exhale.
When emphysema is advanced, you must work so hard to
expel air from your lungs that just the simple act of breathing
can consume a great deal of energy. Unfortunately, because
emphysema develops gradually over many years, you may not
experience symptoms such as shortness of breath until
irreversible damage has already occurred.
Treatments for emphysema focus on relieving symptoms and
avoiding complications.
The main emphysema symptoms are shortness of breath and
a reduced capacity for physical activity, both of which worsen
as the disease progresses. In time, you may have trouble
breathing even when lying down, and it may be especially hard
to breathe during and after respiratory infections, such as
colds or the flu.
When you inhale, air travels to your lungs through two major
airways off the windpipe (trachea) called bronchi. Inside your
lungs, the bronchi subdivide like the roots of a tree into a
million smaller airways (bronchioles) that finally end in
clusters of tiny air sacs (alveoli). You have about 300 million
air sacs in each lung.
Within the walls of the air sacs are tiny blood vessels
(capillaries) where oxygen is added to your blood and carbon
dioxide — a waste product of metabolism — is removed. The air
sac walls also contain elastic fibers that help the very small
airways leading to the air sacs expand like small balloons when
you breathe.
In emphysema, inflammation destroys these fragile walls of
the air sacs, causing them to lose their elasticity. As a result,
the bronchioles collapse, and air becomes trapped in the air
sacs, which overstretches them and interferes with your ability
to exhale (hyperinflation).
In time, this overstretching may cause several air sacs to
rupture, forming one larger air space instead of many small
ones. Because the larger, less elastic sacs aren't able to force
air completely out of your lungs when you exhale, you have to
breathe harder to take in enough oxygen and to eliminate
carbon dioxide.
The process works something like this: Normally, you exhale
in two ways, actively and passively. When you sit quietly, your
diaphragm contracts and your chest muscles expand to take
air in, but your muscles don't actively contract to let the air
out. Instead, the elastic tissue around your air sacs contracts
and your lungs passively shrink. On the other hand, when you
exert yourself and need more oxygen, your chest muscles
contract, forcing air out rapidly.
But if you have emphysema, many of these elastic fibers
have been destroyed, and you must consciously force air out of
your lungs. The forced exhalation compresses many of your
small airways, making expelling air even more difficult.
Cigarette smoke is by far the most common cause of
emphysema. The damage begins when tobacco smoke
temporarily paralyzes the microscopic hairs (cilia) that line
your bronchial tubes. Normally, these hairs sweep irritants and
germs out of your airways. But when smoke interferes with
this sweeping movement, irritants remain in your bronchial
tubes and infiltrate the alveoli, inflaming the tissue and
eventually breaking down elastic fibers.
In a small percentage of people, emphysema results from low
levels of a protein called alpha-1-antitrypsin (AAt), which
protects the elastic structures in your lungs from the
destructive effects of certain enzymes. A lack of AAt can lead
to progressive lung damage that eventually results in
emphysema. If you're a smoker with a lack of AAt, emphysema
can begin in your 30s and 40s.
AAt deficiency is a hereditary condition that occurs when you
inherit two defective genes, one from each parent. Although
severe AAt deficiency is rare, millions of people carry a single
defective AAt gene. Some of these people have mild to
moderate symptoms; others have no symptoms at all. Carriers
are at increased risk of lung and liver problems and can pass
the defect to their children.
People with two defective genes have a high likelihood of
developing emphysema, usually between the ages of 30 and
40. The progression and severity of the disease are greatly
exacerbated by smoking.
Experts recommend that people with early-onset emphysema
— especially those who don't smoke or who have other risk
factors for the disease or who have a family history of AAt
deficiency — be tested for the defective gene. People who are
found to have a genetic predisposition for AAt deficiency may
want to consider having close family members tested as well.
The single greatest risk factor for emphysema is smoking.
Emphysema is most likely to develop in cigarette smokers, but
cigar and pipe smokers also are susceptible, and the risk for all
types of smokers increases with the number of years and
amount of tobacco smoked. Men are affected more often than
women are, but this statistic is changing, as more women have
taken up smoking.