Sickle cell disease
is an inherited disorder in which red blood
cells (RBCs) are abnormally shaped. This abnormality can result in painful episodes,
serious infections, chronic anemia,
and damage to body organs.
More to Know
Normally, RBCs have a round, doughnut shape and move easily through small blood
vessels. Sickle cell disease causes RBCs to form in the elongated shape of a sickle,
or the shape of the letter "C." These abnormal RBCs have a tendency to get stuck in
narrow blood vessels and block the flow of blood. This can cause episodes of pain
and lead to organ damage because the tissues aren't getting enough oxygen.
Sickle cells also have a shorter-than-normal life span, which leads to anemia
(low RBC count). People with sickle cell disease are also at increased risk for certain
bacterial infections and stroke.
Sickle cell disease is caused by a defect in the genes
that affect hemoglobin, a protein found in RBCs that transports oxygen throughout
the body. If a child inherits a sickle cell gene from each parent, he or she will
have sickle cell disease. If the child inherits one sickle cell gene and one normal
gene, he or she will have sickle cell trait. People with sickle cell trait usually
have no symptoms, but they can pass the sickle cell gene on to their children.
In most cases, treatment for sickle cell disease involves medicines to help manage
the pain, and immunizations and daily doses of penicillin (an antibiotic) to help
prevent infection. Severe anemia may be treated with blood transfusions.
Keep in Mind
Thanks to advancements in early diagnosis and treatment, most kids born with sickle
cell disease grow up to live relatively healthy and productive lives. The disease
is a constant presence, though, so seek immediate medical attention for any signs
of infection, severe anemia, chest pain, or seizures.
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