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Sickle cell anemia
Sickle cell anemia is an inherited disease
in which the red blood cells, normally disc-shaped, become
crescent shaped. As a result, they function abnormally and
cause small blood clots. These clots give rise to recurrent
painful episodes called 'sickle cell crises'.
Sickle cell anemia is caused by an abnormal
type of hemoglobin (oxygen carrying molecule) called hemoglobin
S. It is inherited as an autosomal recessive trait -- that
is, it occurs in someone who has inherited hemoglobin S from
both parents.
Someone who inherits hemoglobin S from
one parent and normal hemoglobin (A) from the other parent
will have sickle cell trait. Approximately 8%-10% of African
Americans have sickle cell trait.
Someone with sickle cell trait or these
forms of sickle cell disease will usually have no symptoms
or only mild ones. However, some of these conditions can cause
symptoms similar to sickle cell anemia.
Sickle cell disease is much more common
in certain ethnic groups, affecting approximately one out
of every 500 African Americans. Because people with sickle
trait were more likely to survive malaria outbreaks in Africa
than those with normal hemoglobin, it is believed that this
genetically aberrant hemoglobin evolved as a protection against
malaria.
Although sickle cell disease is inherited
and present at birth, symptoms usually don't occur until after
4 months of age because infants have hemoglobin F. The life
of Red Blood Cells are 120 days.
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Sickle cell anemia may become life-threatening
when damaged red blood cells break down (hemolytic crisis),
when the spleen enlarges and traps the blood cells (splenic
sequestration crisis), or when a certain type of infection
causes the bone marrow to stop producing red blood cells (aplastic
crisis). Repeated crises can cause damage to the kidneys,
lungs, bones, eyes, and central nervous system.
Blocked blood vessels and damaged organs
can cause acute painful episodes. These painful crises, which
occur in almost all patients at some point in their lives,
can last hours to days, affecting the bones of the back, the
long bones, and the chest.
Some patients have one episode every few
years, while others have many episodes per year. The crises
can be severe enough to require admission to the hospital
for pain control and intravenous fluids.
Many manifestations of this disease are
a result of the fragility and inflexibility of the sickle
red blood cells. When patients experience dehydration, infection,
and low oxygen supply, these fragile red blood cells assume
a crescent shape, causing red blood cell destruction and poor
flow of these blood cells through blood vessels, resulting
in a lack of oxygen to the body's tissues.
Sign and Symtoms
Possible
Treatment
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