The sickle-cell disease also known as sickle-cell anemia is a hereditary blood disorder caused by an abnormality in the oxygen-carrying protein hemoglobin found in red blood cells. Sickle-Cell disease leads to a propensity for the cells to assume an abnormal, rigid, sickle-like shape under certain circumstances. Sickle-cell disease is associated with some acute and chronic health problems, such as severe infections, attacks of severe pain (sickle-cell crisis), stroke, and an increased risk of death.
Sickle-cell disease occurs when a person inherits two abnormal copies of the hemoglobin gene, one from each parent. Several subtypes exist, depending on the exact mutation in each hemoglobin gene. A person with a single abnormal copy does not experience symptoms and is said to have sickle-cell trait. Such people are also referred to as carriers.
The complications of the sickle-cell disease can be prevented to a large extent with vaccination, preventive antibiotics, blood transfusion, and the drug hydroxyurea/hydroxycarbamide. A small proportion of patients requires a transplant of bone marrow cells.
Sickle cell anemia facts
- Sickle cell anemia is an inherited disorder of the hemoglobin in the blood.
- Sickle cell anemia requires the inheritance of two sickle cell genes.
- Sickle cell trait, which is the inheritance of one sickle gene, almost never causes problems.
- Virtually all of the major symptoms of sickle cell anemia are the direct result of the abnormally shaped sickled red blood cells blocking the flow of blood.
- The current treatment of sickle cell anemia is directed primarily toward managing the individual features of the illness as they occur.
What is Sickle Cell Trait?
Sickle Cell trait is an inherited condition in which both hemoglobin A and S are produced in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are healthy.
Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets one from each parent.