People who have the disease usually receive the
diagnosis as infants. The disease causes red blood cells to change from
healthy, round red blood cells to sickly and crescent-shaped ones. The
disorder causes anemia and pain, among other problems. Sickle cell anemia is one form of
sickle cell disease - a category of blood disorders caused by defective
hemoglobin. Hemoglobin is a substance in all red blood cells that
enables them to carry oxygen from your lungs, through your bloodstream,
to all parts of your body.
Sickle cell anemia affects mainly
blacks, though people of South American, Southern European or Middle
Eastern descent also are at risk. A baby born with
sickle cell anemia inherits a gene for the disorder from each parent.
Some people inherit only one gene for the disease. This is referred to
as having the sickle cell trait. People who have the sickle cell trait
don't develop the disease, but they can pass the gene on to their
children. Almost 10 percent of black Americans carry the sickle cell
There's no cure for sickle cell
although gene therapy may someday provide the answer. However,
treatments can relieve your symptoms and prolong your life. With proper
treatment, many people with sickle cell anemia lead productive lives and
enjoy reasonably good health into their 40s and beyond.
Signs and symptoms
Signs and symptoms of sickle cell anemia vary from person to person.
People with the sickle cell trait ordinarily have no symptoms or
problems. Some people have mild symptoms. Other people have severe
symptoms and need frequent hospitalization.
Signs and symptoms of the disease include:
In this state, your blood is low on red blood cells. Sickle cells
are fragile. They break apart easily and die, leaving you
chronically short on red blood cells to carry oxygen to your
tissues. Without enough red blood cells in circulation, your body
can't get the oxygen it needs to feel energized. That's why anemia
Periodic episodes of pain, called crises, are a major symptom of
sickle cell anemia. Pain develops when sickle-shaped red blood cells
block blood flow through tiny blood vessels. Usually blood vessels
in your chest, abdomen and joints are affected. Pain also occurs
sometimes in bones. The pain may vary in intensity and can last for
a few hours to a few weeks. Some people experience only a few
episodes of pain. Others experience a dozen or more crises a year.
If a crisis is severe enough, you may need hospitalization for
painkillers to be injected into your veins (intravenously).
Swollen hands and feet are often the first signs of sickle cell
anemia in babies. The swelling is caused by sickle-shaped red blood
cells blocking blood flow out of the hands and feet. The condition —
hand-foot syndrome — is often accompanied by pain and fever.
Jaundice is a yellowing of the skin and eyes that occurs because of
liver damage or dysfunction. Occasionally, people who have sickle
cell anemia have some degree of jaundice because the liver, which
filters harmful substances from the blood, is overwhelmed by the
rapid breakdown of red blood cells. In people with dark skin,
jaundice is visible mostly as yellowing of the eyes.
Sickle cell anemia may make you more vulnerable to infections. The
risk of infection increases because sickle cells damage your spleen,
an organ that filters out germs from your blood and makes certain
proteins (antibodies) that counteract foreign substances in your
body. Doctors commonly give infants and children with sickle cell
anemia antibiotics to prevent potentially life-threatening
infections, such as pneumonia.
Red blood cells provide your body with the oxygen and nutrients you
need for growth. A shortage of healthy red blood cells in sickle
cell anemia can slow growth in infants and children and delay
puberty in teenagers.
Some people with sickle cell anemia experience vision problems. Tiny
blood vessels that feed your eyes may become plugged with sickle
cells. This can damage the retina — the portion of each eye that
processes visual images.