The effects of sickle cell disease can be devastating with symptoms consisting of severe pain, anemia, infection and stroke. Sickle cell disease affects approximately 100,000 people in the United States.
While it affects mainly African Americans and people of Hispanic heritage, it is also prevalent in persons of South or Central American, Caribbean, Mediterranean (countries such as Turkey, Greece, and Italy), Indian, and Saudi Arabian descent. Sickle cell disease or trait also affects people of Caucasian descent.
Through a protein called hemoglobin, red blood cells carry oxygen throughout the body. Red bloods cells are normally flexible and round. However, SCD causes your hemoglobin to form into hard, sticky, and crescent or sickle shaped rods in the red blood cells.
Sickle-shaped cells make it difficult for hemoglobin to flow through the blood vessels, thus blocking or slowing blood flow to various organs in the body. When this blockage of blood cells happens, oxygen cannot get to those organs, which can cause intense pain, known as pain crisis, pain episode, or vaso-occlusive (VOC) pain crisis.
Additionally, sickled cells break apart easily and die quicker than normal red cells. When your body cannot produce enough healthy red blood cells to replace the ones lost, this condition is called anemia.
Sickle cell genes are inherited, one from each parent. The figure below shows how each of the two inherited genes determine the type of sickle cell you have.
For example, in order to have sickle cell anemia, you must inherit a sickle "S" gene from both mother and father - HbSS. For those with sickle cell trait, you must inherit the sickle "S" gene and a normal "A" gene - HbSA.
A = normal hemoglobin
S = sickle hemoglobin
TYPES OF SICKLE CELL:
*other rare forms
A blood test is used to diagnose sickle cell disease. Newborn babies delivered in the U.S. are routinely screened for sickle cell at birth. If you are unsure of your status, please contact our office to make an appointment for testing.
Sickle cell disease affects each person differently. Some of the more common complications include:
For a more detailed list, visit the National Heart, Lung, and Blood Institute.
A bone marrow or stem cell transplant is the only cure for sickle cell disease. However, there are other methods used to manage symptoms and complications associated with the disease. Those options include:
Augusta University is currently part of a large Bone Marrow Transplant (BMT) study funded by the National Institutes of Health. This study will help clinicians and researchers determine if bone marrow transplantations should be considered standard of care for people with sickle cell disease. For more information, please visit the Center for Blood Disorders research page or contact our research team at 706-721-2171.