Texas EMS Magazine, Volume 30, Number 6, November/December 2009 Page: 36
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Continuing Education
the sickling permanent. A permanently
sickled cell will eventually lodge
somewhere within the capillary system,
unless it can be destroyed by the body's
natural defenses.
Sickle Cell Disease Origin
Experts believe that sickle cell disease
originated in areas of the world with a
high incidence of malaria. In those regions
mosquitoes transmit a parasite that enters
human red blood cells. The parasite
completes its life cycle there and, in the
process, destroys the cell. As the parasites
destroy the red blood cells, the infected
patient becomes anemic and ultimately
dies.
However, the parasite cannot continue
its life cycle in red blood cells affected by
hemoglobin S. The presence of a single
mutated gene in the hemoglobin gene pair
provides the carrier with some resistance
to malaria, a clear survival advantage in a
malaria-prone region. The carrier will still
have some sickling, but no widespread
problems. Therefore, this individual is a
carrier of the sickle cell trait, but does not
have the disease.
Unfortunately, if both the mother and
father are carriers, there is a one-in-four
chance that the offspring will receive two
of the defective genes. When both of the
paired genes are defective, the child has
inherited sickle cell disease and may not
live to his or her reproductive years.
Complications of Sickle Cell
Disease
The spleen assists in maintaining
immunity against infection, but the organ
also plays a major role in removing
damaged and abnormal red blood cells
from circulation. The spleen, along with
other tissues, helps to destroy sickled
cells. In many cases however, the spleen
destroys the abnormal cells faster than thebody can create new ones, resulting in a
reduced oxygen-carrying capacity of the
blood, or anemia.
Because most of the vasculature in
the spleen is very narrow, sickled red
blood cells easily occlude the vessels and
splenic injury is common. In fact, in most
individuals with sickle cell disease, the
spleen has become nonfunctional by the
end of childhood, which then predisposes
them to infections.
If the body cannot remove the sickled
red blood cells from the circulatory system,
the abnormal cells lodge in blood vessels
throughout the body, which is sometimes
referred to as a vaso-occlusive event.
Blockages of cerebral vessels produce
strokes; blockages of peripheral vessels
produce extreme pain in the extremities;
blockages of vessels in the penis produce
a painful prolonged erection known as
a priapism. Eventually, vaso-occlusion
damages almost every major organ,
resulting in multiple-organ failure.
Acute chest syndrome is a sometimes
fatal complication of sickle cell disease.
Acute chest syndrome affects about 40
percent of all people with sickle cell
anemia (Steinberg 1999) and is more
common in children. Patients usually
present with pleuratic chest pain, fever,
referred abdominal pain, hypoxia and a
cough. X-ray examinations frequently
reveal infiltrates. With frequent episodes,
chronic respiratory insufficiency develops.
Assessment
Improving morbidity and mortality
in sickle cell disease patients requires
that medical personnel identify at-risk
patients early in order to provide timely
and effective care (Platt et al. 1994). In
any given year, 60 percent of individuals
with sickle cell disease will experience
an extremely painful crisis episode (Platt
et al. 1991). The duration of the vaso-36 Texas EMS Magazine November/December 2009
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Texas. Department of State Health Services. Texas EMS Magazine, Volume 30, Number 6, November/December 2009, periodical, December 2009; Austin, Texas. (https://texashistory.unt.edu/ark:/67531/metapth1507903/m1/36/?q=%222009%22: accessed July 16, 2024), University of North Texas Libraries, The Portal to Texas History, https://texashistory.unt.edu; crediting UNT Libraries Government Documents Department.