What is Sickle Cell Disease ?
Sickle Cell Disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and sickle shape. When these hard and pointed red cells go through the small blood tube, they clog blood flow and break apart. This can cause pain, tissue damage and a low blood count, or anemia.
What makes the red cell sickle?
There is a substance in the red blood cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red blood cell when it gives away oxygen. These rigid rods change the red bloods cells into a sickle shape.
How do you get sickle cell anemia?
You inherit the abnormal hemoglobin from parents if both are carriers of the sickle cell trait. The abnormal hemoglobin can also be inherited if one of your parents has sickle cell disease. Sickle cell disease is not contagious. You are born with the sickle cell hemoglobin and it is present for life.
How can I be Tested?
A simple blood test called the hemoglobin electrophoresis can be done by your doctor or local sickle cell foundation. This test will tell if you are a carrier of the sickle cell trait or if you have the disease. New born babies can also be screened for sickle cell disease.
What is sickle cell trait?
A person with sickle cell trait carries one sickle hemoglobin producing gene and one normal hemoglobin gene. Normal hemoglobin is called type A. Sickle hemoglobin called S. Sickle cell trait is the presence of hemoglobin AS on the hemoglobin electrophoresis. This will NOT cause sickle cell disease. Other hemoglobin traits are AC and AE traits.
What are the other forms of sickle cell disease?
There are three common types of sickle cell disease.
- Hemoglobin SS or or sickle cell anemia
- Hemoglobin SC disease
- Hemoglobin sickle beta-thalassemia
Each of these can cause sickle pain episodes and complications, but some are more common than others. An increase in fetal hemoglobin can protect the red blood cells from sickling in all of these forms of sickle cell disease. The medication hydroxyurea increases fetal hemoglobin.
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