Sickle Cell

SICKLE CELL
Sickle cell anemia is one of a group of inherited disorders known as sickle cell
disease. It affects the shape of red blood cells, which carry oxygen to all parts of
the body.
Red blood cells are usually round and flexible, so they move easily through blood
vessels. In sickle cell anemia, some red blood cells are shaped like sickles or
crescent moons. These sickle cells also become rigid and sticky, which can slow or
block blood flow.
There's no cure for most people with sickle cell anemia. Treatments can relieve
pain and help prevent complications associated with the disease.
Sickle cell anemia

Red blood cells are usually round and flexible. In sickle cell anemia, some red
blood cells look like sickles used to cut wheat. These unusually shaped cells give
the disease its name
Signs and Symptoms
Signs and symptoms of sickle cell anemia usually appear around 6 months of age.
They vary from person to person and may change over time. Signs and symptoms
can include:
 One-sided paralysis or weakness in the face, arms or legs

 Confusion
 Difficulty walking or talking
 Sudden vision changes
 Unexplained numbness
 Severe headache
 Anemia. Sickle cells break apart easily and die. Red blood cells usually live
for about 120 days before they need to be replaced. But sickle cells typically
die in 10 to 20 days, leaving a shortage of red blood cells (anemia). Without
enough red blood cells, the body can't get enough oxygen and this causes
fatigue.
 Episodes of pain. Periodic episodes of extreme pain, called pain crises, are
a major symptom of sickle cell anemia. Pain develops when sickle-shaped
red blood cells block blood flow through tiny blood vessels to your chest,
abdomen and joints.
The pain varies in intensity and can last for a few hours to a few days. Some
people have only a few pain crises a year. Others have a dozen or more a
year. A severe pain crisis requires a hospital stay.
Some adolescents and adults with sickle cell anemia also have chronic pain,
which can result from bone and joint damage, ulcers, and other causes.
 Swelling of hands and feet. The swelling is caused by sickle-shaped red

blood cells blocking blood circulation in the hands and feet.
 Frequent infections. Sickle cells can damage the spleen, increasing
vulnerability to infections. Infants and children with sickle cell anemia
commonly receive vaccinations and antibiotics to prevent potentially life-
threatening infections, such as pneumonia.
 Delayed growth or puberty. Red blood cells provide the body with the
oxygen and nutrients needed for growth. A shortage of healthy red blood
cells can slow growth in infants and children and delay puberty in teenagers.
 Vision problems. Tiny blood vessels that supply the eyes can become
plugged with sickle cells. This can damage the retina — the portion of the
eye that processes visual images — and lead to vision problems.
Causes
Sickle cell anemia is caused by a change in the gene that tells the body to make the
iron-rich compound in red blood cells called hemoglobin. Hemoglobin enables red
blood cells to carry oxygen from the lungs throughout the body. The hemoglobin
associated with sickle cell anemia causes red blood cells to become rigid, sticky
and misshapen.
For a child to be affected, both mother and father must carry one copy of the sickle
cell gene — also known as sickle cell trait — and pass both copies of the altered
form to the child.
If only one parent passes the sickle cell gene to the child, that child will have the
sickle cell trait. With one typical hemoglobin gene and one altered form of the
gene, people with the sickle cell trait make both typical hemoglobin and sickle cell
hemoglobin.
Their blood might contain some sickle cells, but they generally don't have
symptoms. They're carriers of the disease, however, which means they can pass the
gene to their children.
Risk factors
For a baby to be born with sickle cell anemia, both parents must carry a sickle cell
gene.
Complications
Sickle cell anemia can lead to a host of complications, including:
 Stroke. Sickle cells can block blood flow to an area of the brain. Signs of
stroke include seizures, weakness or numbness of the arms and legs, sudden
speech difficulties, and loss of consciousness. If your child has any of these
signs and symptoms, seek medical treatment immediately. A stroke can be
fatal.
 Acute chest syndrome. A lung infection or sickle cells blocking blood
vessels in the lungs can cause this life-threatening complication, resulting in
chest pain, fever and difficulty breathing. It might require emergency
medical treatment.
 Pulmonary hypertension. People with sickle cell anemia can develop high
blood pressure in their lungs. This complication usually affects adults.
Shortness of breath and fatigue are common symptoms of this condition,
which can be fatal.
 Organ damage. Sickle cells that block blood flow to organs deprive the
affected organs of blood and oxygen. In sickle cell anemia, blood is also
chronically low in oxygen. This lack of oxygen-rich blood can damage
nerves and organs, including kidneys, liver and spleen, and can be fatal.
 Splenic sequestration. A large number of sickle cells can get trapped in the
spleen, causing it to enlarge and possibly causing belly pain on the left side
of the body. This can be life-threatening. Parents of children with sickle cell
anemia should learn to regularly feel their child's spleen for enlargement.
 Blindness. Sickle cells can block tiny blood vessels that supply the eyes.
Over time, this can lead to blindness.
 Leg ulcers. Sickle cell anemia can cause painful open sores on the legs.
 Gallstones. The breakdown of red blood cells produces a substance called
bilirubin. A high level of bilirubin in the body can lead to gallstones.
 Priapism. In this condition, men with sickle cell anemia can have painful,
long-lasting erections. Sickle cells can block the blood vessels in the penis,
which can lead to impotence over time.
 Deep vein thrombosis. Sickling of red cells can cause blood clots,
increasing the risk of a clot lodging in a deep vein (deep vein thrombosis) or
a lung (pulmonary embolism). Either can cause serious illness or even death.
 Pregnancy complications. Sickle cell anemia can increase the risk of high
blood pressure and blood clots during pregnancy. It can also increase the risk
of miscarriage, premature birth and having low birth weight babies.
Prevention
If you carry the sickle cell trait, seeing a genetic counselor before trying to
conceive can help you understand your risk of having a child with sickle cell
anemia. A genetic counselor can also explain possible treatments, preventive
measures and reproductive options.

Sickle Cell

Uploaded by

Copyright:

Available Formats

Sickle Cell

Uploaded by

Document Information

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Sickle Cell

Uploaded by

Copyright:

Available Formats

SICKLE CELL

Sickle cell anemia

Signs and Symptoms

 One-sided paralysis or weakness in the face, arms or legs

 Swelling of hands and feet. The swelling is caused by sickle-shaped red

Sickle cell anemia can lead to a host of complications, including:

You might also like