Sickle Cell Anemia

What is it?Sickle Cell Anemia is an inherited genetic

disorder caused by irregularly shaped oxygen-carrying proteins within the red blood cells. These red blood cells are fragile and burst easily. These cells can cause blood vessels to become blocked, which leads to internal organ and tissue damage as well as pain.

Where does it come from?Sickle Cell Anemia is passed on

from generation to generation (inherited) as a recessive non-sex linked gene, meaning the trait is not gender specific. This trait does not produce an effect unless both the mother and the father carry this characteristic. A single sickle cell trait does not cause this disorder.

Since the offspring’s mother and father both must have this trait in order for it to have an effect on them, only the child with the genetic make-up of “SS” (as shown in the diagram) will appear with the abnormal hemoglobin.

Do any pre-existing conditions contribute to this disorder?

Increases in the number of abnormal red blood cells can be associated with low oxygen levels, increased acidity or low volume of the blood. High metabolism rates in humans promote this disorder by extracting more oxygen from the blood. These conditions can cause severe organ damage to people with Sickle Cell Anemia.

How is this disorder diagnosed?Sickle Cell Anemia is tested for by a Medical Technologist in a lab most commonly in a hospital setting. A smear of blood prepared using a low-oxygen technique and is then placed under a microscope and the sickle cells are easily seen if present. Other tests include how well the blood solutions dissolve in different solutions. DNA testing on fetal cells before birth can also diagnose this disorder.

T r e a t m e n tS y m p t o m s &Symptoms include but are not limited to:• Pain• Fatigue• Swelling/inflammation

of hands and/or feet• Bacterial infections• Pooling of blood in

spleen• Liver congestion• Eye damage• Weakened bones• Leg Ulcers, or open

sores on legs

Treatment is provided according to the symptoms of the illness. Normally, this includes the management and prevention of red blood cells stacking together causing blocked vessels. Therapy keeps the blood moving but ultimately there is no fix to reverse this disorder.

The Facts

1 in 5,000 people (about 72,000) in the United States have this genetic disorder.

African Americans are more prone to this disease. Researchers found that the sickle cell gene is especially prevalent in areas of Africa hard-hit by malaria. The Sickle cell gene acted as a defense against malaria by carrying the parasite into the spinal cord through the deformed blood cells.

Two million African-Americans (1 in 12 people) have the sickle cell trait.

The dark purple shows the highest affected areas of malaria which is associated with sickle cell anemia.

The End