Sickle Cell Anemia
What is it?
Sickle cell anemia is an inherited blood
disorder that causes red blood cells to be
abnormally shaped. The condition can cause anemia,
painful events and various complications.
Who gets it?
Sickle cell anemia primarily affects people of
African, Mediterranean, Middle Eastern, and Asian
Indian ancestry. In the United States, the disease
occurs in one out of every 600 African-Americans.
What causes it?
Sickle cell anemia is cause by a genetic change
in hemoglobin, the oxygen-carrying protein inside
the red blood cells. This causes the cells to take
on a sickle-shape and have a shorter life span,
which can cause anemia. Sickle cells are also less
flexible and stickier than normal red blood cells,
and can become trapped in small blood vessels
preventing blood flow and starving tissues and
organs. This compromises the delivery of oxygen,
which may result in damage to associated tissues
and organs. The disease is often inherited.
What are the symptoms?
Symptoms of sickle cell anemia include paleness
of skin as the anemia worsens, especially on the
insides of eyelids, under fingernails and in the
creases of the palm of the hand. The skin may also
turn yellow (jaundiced). If the anemia is severe,
the shortage of normal red blood cells may cause
weakness, shortness of breath, or even heart
failure. The symptoms of shock caused by heart
failure are low blood pressure, rapid pulse, and
decreasing consciousness.
How is it diagnosed?
Sickle cell anemia may be diagnosed through a
test called hemoglobin electrophoresis. This blood
test detects the sickle cell hemoglobin S. It can
also be used to find carriers of the sickle cell
trait. In addition, the doctor may order a
complete blood count to confirm the effects or
complications of sickle cell anemia. During this
test, red blood cells are counted under a
microscope and checked for abnormal shapes.
What is the treatment?
There is no cure for sickle cell anemia, so
treatment focuses on lessening a patient’s
symptoms and prolonging their lives. Recent
studies have shown that the drug hydroxyurea may
reduce pain attacks in people with sickle cell
anemia. It also has been shown to prolong the
lives of patients diagnosed with sickle cell
anemia. Patients with sickle cell anemia are more
likely to have serious infections such as the flu
or pneumonia. Prevention of serious infections is
an important part of treatment. The doctor may
recommend immunizations are kept up-to-date, get a
flu shot each year, and take antibiotics to
prevent infections.
Blood transfusions are not usually given on a
regular basis but may be used to treat patients
who experience frequent and severe painful events,
severe anemia, and other emergencies.
Self care tips
Couples who have sickle cell anemia or who are
carriers of the disease may consider not having
children to prevent the spread of the disease.
Screening tests can be used to find carriers.
Couples who find that they are carriers should
seek genetic counseling.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
|
