About
Sickle Cell Disease
WHAT IS SICKLE CELL DISEASE?
Sickle Cell Disease is an inherited disease of red blood cells that
can cause attacks of pain and damage to vital organs and can lead
to early death. Sickle Cell Disease affects a protein inside the
red blood cells called hemoglobin. The disease occurs when a person
inherits two sickle cell genes.
Hemoglobin carries oxygen from the lungs and takes it to every part
of the body. Normal red blood cells are round and flexible when
oxygen is released from the hemoglobin. But when oxygen is released
from the red blood cells in people with Sickle Cell Disease, the
red blood cells become irregular in shape. These irregularly shaped
cells can cause traffic jams throughout the artery highway within
the body. When these traffic jams occur there is severe pain in
the bones and joints.
When there is a build up of irregularly shaped red blood cells they
can become trapped and destroyed, leaving the body with a shortage
of red blood cells. This shortage can lead to anemia. In severe
cases of anemia a person can appear to be pale, may have shortness
of breath and/or have little energy.
There are several forms of Sickle Cell Disease. The most common
forms are referred to as SS (the child inherits two sickle cell
genes), SC (the child inherits one sickle cell gene and one gene
for another type of hemoglobin called C), and SB0 thalassemia (the
child inherits one sickle cell gene and one gene for a type of thalassemia,
another inherited anemia).
The effects of sickle cell disease vary greatly from one person
to the next. Some people affected with Sickle Cell Disease rarely
become ill while other are frequently hospitalized.
DID YOU KNOW?
- Sickle cell disease is a group of related disorders that affect
red blood cells.
- Sickle cell disease is an inherited genetic disorder, which
means that it is passed from mothers and fathers to their children.
- Two parents with sickle cell trait have 1 in 4 chances of having
a child with sickle cell disease with each pregnancy.
- One in 10 African Americans has Sickle Cell Trait.
- Sickle cell trait is not a disease.Individuals with trait are
carriers and possess the potential to pass on the abnormal sickle
gene to their children.
- Approximately one in 400 African Americans is born with sickle
cell disease.
- Approximately 70,000 people in the USA have sickle cell disease.
- Genetic disorders are found in all races of people.
- Children of any race may be born with sickle cell disease. However,
in the USA it is more common among African Americans.
- A simple blood test called hemoglobin electrophoresis can detect
the presence of sickle and other abnormal hemoglobins.
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