What is Sickle Cell Disease?

Sickle Cell is an inherited blood disorder which can cause bouts of pain, damage of vital organs, and even death in childhood and early adulthood. Its effects vary greatly from one person to another and from one time to another in the same person. Most people with sickle cell anemia enjoy reasonably good health.

Oxygen-carrying red blood cells are normally round and flexible; however, under certain cinditions, the red blood cells of a person with sickle cell anemia may change into a crescent or sickle shape within their blood vessels. Sickled cells tend to become trapper in the spleen and elsewhere and are destroyed, resulting in a shortage of red blood cells. This shortage can cause the patient to be pale, short of breath, and easily tired. Patients may be prone to certain infections which can worsen their condition and also cause physical growth and development to be slower than normal in children. 

Who is affected by sickle cell?

In the United States, most cases of Sickle Cell Anemia occur among Black and Hispanic ethnicities. About one in every 400-600 people with black ethnicity and one in every 1000-1500 people with hispanic ethnicity inherit sickle cell disease. The disease also affects some people of Arabian, Greek, Maltese, Sicilian, Sardinian, Turkish, and South Asian ancestry. 

How do we test for sickle cell diease and trait? 

Sickle Cell Disease and trait may be detected by different methods. The current preferred routine method is by cellulose acetate electrophoresis together with the solubility (or turbidity) test on blood samples.