Have you ever seen a sickle? It's a farm tool with a curved,
sharp edge for cutting wheat. Sickle cell anemia (say:
-mee-uh) is a disease of the blood. It gets its name because a
red blood cells
are shaped like sickles, or crescent moons, instead of their usual
round, disc shape.
Round is the healthiest shape for red blood cells (or RBCs)
because they can move easily through the body. RBCs carry oxygen
around your body, and every part of your body needs oxygen to work
Red blood cells are made inside the bones in the soft, spongy
area called the
-oh). Every time you take a breath, you breathe in oxygen and your
RBCs carry oxygen to every cell in your body. Any time a
person's body doesn't have enough red blood cells, it's
called anemia. When the cause is the sickle shape of the RBCs,
it's called sickle cell anemia.
When RBCs are shaped like sickles or crescent moons, they can
get stuck, especially inside smaller blood vessels. This keeps
blood from flowing properly in the body, which can cause a lot of
pain. Important organs like the brain, heart, and kidneys need
constant blood flow to work properly.
A person's body knows that the sickle cells aren't good,
so it destroys them more quickly. But the body can't make new
blood cells fast enough to replace the old ones.
Signs and Symptoms
Kids who have sickle cell anemia may feel pain in their chest,
stomach, or bones when blood vessels get clogged with sickle cells.
The pain can last a few minutes or several days, and it might hurt
a lot or just a little. When this happens, it's called a sickle
cell crisis (a crisis means a time of trouble).
Nobody knows exactly when sickle cells might get stuck or which
blood vessels might get clogged. Certain conditions, like if a
person gets too cold, gets sick, or doesn't drink enough fluid,
can lead to a sickle cell crisis. Doctors and nurses can help by
giving strong medicine to
relieve the pain
Because kids with sickle cell anemia don't have enough
normal RBCs, they get tired more easily. They also get infections
more often than other kids do. They may not grow as fast as their
Sometimes the whites of their eyes have a yellowish color, known
as jaundice (say:
-dus), and they may have to go to the bathroom a lot. In little
kids - usually those under age 2 - sickle cell anemia can cause
their hands and feet to swell and hurt. Sometimes they can have
more serious illnesses and have to stay in the hospital.
What Causes Sickle Cell Anemia?
Sickle cell anemia is an
-uh-ted) disease. That means you can't catch it like you can
catch a cold or the flu. Kids are born with the disease when both
parents pass along the sickle cell anemia gene to their
More African Americans have sickle cell anemia than any other
group of people. About 1 out of every 500 African Americans has the
disease. But some people whose ancestors came from countries around
the Mediterranean Sea - like Greece, Italy, and Saudi Arabia - have
sickle cell genes, too.
Some scientists think sickle cell anemia may be connected to
malaria (say: muh-
-ee-uh), a serious and sometimes deadly disease that was very
common in those countries. It is believed that people who carry the
gene for sickle cell anemia are less likely to catch malaria. So
more of these people survived and passed on the sickle cell gene to
How Does a Doctor Know a Kid Has It?
Special blood tests can tell a doctor if a kid has sickle cell
disease. The tests will show the doctor the type of hemoglobin
-muh-glow-bin) in the kid's red blood cells.
Hemoglobin is the part of the blood that carries oxygen to
different parts of the body in the RBCs. There are different types
of hemoglobin, including hemoglobin A and hemoglobin S. Normal RBCs
contain hemoglobin A, but people with sickle cell disease have
mostly hemoglobin S (the "S" stands for sickle) in their
red blood cells.
How Is Sickle Cell Anemia Treated?
Most young kids with sickle cell anemia take penicillin (say:
-lun), a drug that helps prevent infections. A doctor may also
prescribe a vitamin supplement called folic acid. Folic acid helps
the body make new RBCs.
Taking penicillin or vitamins does not cure sickle cell anemia,
but it can help keep a kid with sickle cell anemia from getting
sick. Pain medicine also helps kids with this disease. A kid with
sickle cell anemia may need to go to the hospital if he or she has
a lot of pain or a serious infection. At the hospital, the kid can
fluids, antibiotics, or other medicine.
Sometimes kids with sickle cell anemia need blood
-zyuns). That's a way to put healthy blood cells right into a
kid's body. A blood transfusion raises the amount of normal
hemoglobin in the blood. It also decreases the chances that
blockage, or crisis, will happen.
Can Sickle Cell Anemia Be Cured?
In some cases, a bone marrow transplant can cure sickle cell
anemia. Bone marrow transplants replace the sickle cells with
healthy cells from a donor. A donor is a person who gives healthy
bone marrow or other organs or body parts to someone else who needs
Not just any bone marrow will do. For the transplant to work,
the bone marrow must be a close match. Usually, the best donor is a
brother or sister.
What Can a Kid Do to Stay Well?
Taking penicillin every day helps prevent infections in kids who
have sickle cell anemia. It's important for them to stay as
healthy as possible. That means eating healthy foods and drinks and
getting plenty of sleep. Kids with the disease can play and
exercise, but they should not get too hot, too cold, or too
Another type of medicine, called
-uh), can help prevent infections and illnesses. This medicine must
be taken every day.
A kid with the disease who has a fever, pain, or any other
problems needs to tell an adult right away. Fast treatment is very
important, so the kid can get back to feeling good again soon.
Robin E. Miller, MD
Date reviewed: November 2008
Note: All information is for educational purposes only. For specific medical advice,
diagnoses, and treatment, consult your doctor.
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