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$Unique_ID{PAR00149}
$Pretitle{}
$Title{Medical Advice: Sickle Cell Anemia}
$Subtitle{}
$Author{
Editors of Consumer Guide
Chasnoff, Ira J}
$Subject{Sickle Cell Anemia trait disease Weakness Fatigue swollen joint
Swelling joints Pain African descent black Americans frequent infections
illness illnesses fever surgery dental work blood disease hemoglobin red blood
cells cell protein carry oxygen carrying bloodstream sickle-shaped hemoglobin
S India Greece Italy Middle East genes tiredness sickle cell crisis blood
vessel vessels sickle prep liver kidneys heart lungs nervous system eyes
antibiotics Blood transfusion transfusions pneumococcal infection vaccination
vaccinations vaccine vaccines}
$Log{
Normal and Sickle Blood Cells*0014901.tif}
Your Child: A Medical Guide
Sickle Cell Anemia
Quick Reference
SYMPTOMS
- Weakness
- Fatigue
- Swelling of the joints
- Pain
HOME CARE
- See your doctor for instructions about home care.
PRECAUTIONS
- Sickle cell trait and sickle cell disease occur most often in persons
of African descent, and are found in many black Americans. Children of
black parents and children with a family history of sickle cell disease
should be tested before the age of one year.
- A child with sickle cell disease may get frequent infections and
illnesses. Treat all infections immediately.
- If a child with sickle cell disease runs a high fever, call the doctor.
- A child with sickle cell disease should have frequent checkups.
- A child with the disease may need special treatment before surgery or
dental work.
- No special treatment is needed if the child has sickle cell trait.
Sickle cell anemia, also called sickle cell disease, is an inherited
blood disease. It is caused by an abnormality in the hemoglobin, which is a
special protein that helps the red blood cells to carry oxygen through the
bloodstream. The abnormal hemoglobin makes the red blood cells become rigid
and sickle-shaped (hence the name hemoglobin S). The deformed blood cells
have difficulty passing through the blood vessels. Blood cells are constantly
being destroyed and replaced by the body, but these abnormal, deformed cells
are destroyed more quickly than normal ones. The replacement process cannot
keep up with the destruction of the sickled cells, which causes recurrent
anemia.
Sickle cell trait and sickle cell disease are most common among persons
of African descent. The disease is also found in persons from certain areas
of India, Greece, Italy, and the Middle East.
A child may be born with sickle cell trait, rather than sickle cell
disease itself. The red blood cells of a child with sickle cell trait have
about 40 percent hemoglobin S and 60 percent normal hemoglobin. The child
will function normally and will show no signs of sickle cell disease.
However, the child carries the trait in his or her genes, and either the trait
or the disease may appear in the next generation. If the child eventually
marries someone who also has the trait, the chances of their child having the
disease are greatly increased.
SIGNS AND SYMPTOMS
In a child who has sickle cell disease, almost all the hemoglobin is
hemoglobin S. The child may often show symptoms of anemia, which include
weakness and constant tiredness. In certain circumstances, such as when the
child has an infection, a sickle cell crisis may occur. Crises may also occur
for no apparent reason, or as a result of flying in an unpressurized airplane
or traveling to altitudes over 6,000 feet. Other stresses on the body, such
as injuries or surgery, can also cause a sickle cell crisis.
In a sickle cell crisis, the abnormal red blood cells are destroyed
rapidly, causing severe anemia. At the same time, sickled cells may lodge in
the blood vessels, causing swelling of the joints and severe pain. Sickle
cell crises can damage body organs, and such damage can eventually cause
death.
To find out if a child has this disease, a blood test called a "sickle
prep" is first performed to look for sickled cells in the blood. If abnormal
cells are found, a more complicated test is done to identify the types of
hemoglobin in the blood. The amount of hemoglobin S relative to the amount of
normal hemoglobin indicates whether the child has the trait or the disease.
HOME CARE
Sickle cell anemia requires medical treatment.
PRECAUTIONS
- All black parents, as well as other parents who know that there have been
cases of sickle cell disease in their families, should have their
children tested for the disease before they are a year old.
- All infections should be treated immediately, and high fevers should also
be reported to the doctor right away.
- A child with this disease should have frequent checkups and may need
special treatment before having dental work or surgery.
- A child who has sickle cell trait needs no special treatment.
MEDICAL TREATMENT
Periodically, the doctor will carefully examine the child's liver,
kidneys, heart, lungs, nervous system, and eyes. The doctor will probably
prescribe antibiotics immediately if the child gets any infection. Blood
transfusions may be needed to prevent anemia, and a vaccination against
pneumococcal infection may be given as a precaution.
In a sickle cell crisis, the child should be hospitalized. Intravenous
fluids and pain medications will be given until the crisis passes.
RELATED TOPIC: Anemia