Sickle cell disease is a group of inherited disorders that affect red blood cells. It affects about 100,000 people in the United States and is most commonly found in African-Americans and other ethnic and racial groups in Africa, South America, Saudi Arabia, Mediterranean countries and India.
How does sickle cell disease affect the body?
In a healthy person, red blood cells are round and move throughout the body, delivering oxygen. People with sickle cell disease have sickle-shaped red blood cells that are hard and sticky. These hard, pointed red blood cells clog blood tubes as they move throughout the body, causing pain, damage or anemia.
What are the symptoms associated with sickle cell?
Sickle cell disease is very painful. It can cause swelling of the hands and feet, damage to the spleen, retinal detachment, kidney problems and gallstones.
Sickle cell can also trigger an episode called an acute pain crisis. This is when extreme pain strikes one or more areas of the body, such as the chest, legs, hips or bones. While the intensity of the pain and length of the crisis varies, some sufferers may need to be hospitalized.
How do you acquire sickle cell?
Sickle cell disease is an inherited disorder, thus passed from parent to child.
Physicians say the identification of the disease begins with prenatal genetic counseling, in which the partners of patients with known sickle cell disease or trait are tested for the disease prior to conception. This is important so that expectations for the disease can be set, as well as early confirmatory testing following conception.
When both parents are carriers of the gene known as sickle cell trait, their children have a 25% chance of acquiring the disease.
As of 2008, universal screening for sickle cell disease in newborns is mandated in all 50 states and the District of Columbia.
How is it treated?
Treatment options for people suffering from sickle cell disease vary, but there are several methods depending on the symptoms or severity of the disease.
Pain or crisis episodes can be treated with pain medication, or a medicine called hydroxyurea can be taken to reduce the number of pain crises. Severe anemia can be treated by blood transfusions, and infections are treated with antibiotics, like penicillin.
Bone marrow transplants are being used to successfully cure sickle cell disease for patients who are under 16 years of age, offering hope for the future.
In places where comprehensive care is available, the disease has shifted from being a fatal pediatric illness to now being considered a chronic disease associated with deterioration past the fifth decade of life.
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