WHAT IS SICKLE CELL DISEASE?
Sickle Cell Disease is an inherited blood disorder where abnormal hemoglobin within the red blood cells causes the cells to become rigid, sticky, and shaped like a sickle. The sickling shape makes the blood cells fragile, impeding blood flow which over time can cause severe pain, and harm organs, muscles, and bones.
People with sickle cell disease often have anemia, caused by a shortage of red blood cells. Carriers of the sickle cell gene are said to have sickle cell trait. Sickle cell trait occurs when a person has one gene for sickle hemoglobin and one gene for normal hemoglobin. Unlike sickle cell disease, sickle cell trait does not cause medical problems, but the trait can be passed down. Sickle cell disease primarily affects those of African and Hispanic descents, Middle Eastern, Indian, Latin American, Native American and Mediterranean heritage.
Common Forms of Sickle Cell Disease
Sickle Cell Anemia
This is the most common form of sickle cell disease. Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. The red cells contain only hemoglobin S, and no normal hemoglobin A.
Sickle Cell-Hemoglobin C Disease
The red blood cells contain two abnormal hemoglobins, called hemoglobin S and hemoglobin C.
Sickle Cell-Hemoglobin E Disease
This variation is similar to sickle cell-SC disease, except that an element has been replaced in the hemoglobin molecule. This variation is often also seen in Southeast Asian populations. Some children with hemoglobin E disease are without symptoms. However, under certain conditions, such as exhaustion, hypoxia, severe infection, and/or iron deficiency, some mild to moderate anemia may occur.
This involves an inheritance of both the thalassemia and sickle cell genes. The disorder produces symptoms of moderate anemia and many of the same conditions associated with sickle cell disease.