Sickle
Cell Anemia
Sickle cell anemia is caused by a
mutation in the gene that tells your body to make hemoglobin — the red,
iron-rich compound that gives blood its red color. Hemoglobin allows red blood
cells to carry oxygen from your lungs to all parts of your body. In sickle cell
anemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky
and misshapen.
The sickle cell gene is passed from
generation to generation in a pattern of inheritance called autosomal recessive
inheritance. This means that both the mother and the father must pass on the
defective form of the gene for a child to be affected.
If only one parent passes the sickle
cell gene to the child, that child will have the sickle cell trait. With one
normal hemoglobin gene and one defective form of the gene, people with the
sickle cell trait make both normal hemoglobin and sickle cell hemoglobin. Their
blood may contain some sickle cells, but they generally don't experience
symptoms. However, they are carriers of the disease, which means they can pass
the defective gene on to their children.
With each pregnancy, two people with
sickle cell traits have:
- A 25 percent chance of having an unaffected child with normal hemoglobin
- A 50 percent chance of having a child who also is a carrier
- A 25 percent chance of having a child with sickle cell
Signs and symptoms
of sickle cell anemia usually show up after an infant is 4 months old and may
include:
- Episodes of pain. 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 to your chest, abdomen and joints. Pain can also occur in your 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 to be hospitalized.
- Hand-foot syndrome. Swollen hands and feet may be the first signs of sickle cell anemia in babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of their hands and feet.
- Frequent infections such as pneumonia.
- Delayed growth. Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
- Vision problems. Some people with sickle cell anemia experience vision problems. Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images.
Sickle cell anemia can lead to a host of complications,
including:
A stroke can occur
if sickle cells block blood flow to an area of your brain.
People with sickle
cell anemia can also develop high blood pressure in their lungs (pulmonary
hypertension). Shortness of breath and difficulty breathing are common symptoms
of this condition, which can be fatal.
Blindness.
Tiny blood vessels that supply your eyes can get blocked by sickle cells. Over
time, this can damage the retina — the portion of the eye that processes visual
images — and lead to blindness.
Skin ulcers. Sickle cell anemia can cause
open sores, called ulcers, on your legs.
By....Belinda Yeboah
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