Sickle Cell Disease is an inheritable disorder, from the disorder called anemia. It causes the red blood cells to break down, which damages their shape. Since the shape is altered, the sickle cells die earlier. Also, because the shape is changed, there is often a blockage within the blood flow which causes crisis. Since there is not enough healthy RBC, but rather sickle cells, there is an inadequate amount of oxygen supplied within the body.
The causes of this disease occurs in the mutation of the formation of red blood cells. The hemoglobin molecule, which is altered with sickle cell disease patients, causes the RBC to become rigid and changes their shape.
The inheritance of this disorder is by an autosomal recessive inheritance.
Complications of this disorder include:
A blood test can diagnose a patient suffering from sickle cell disease, by the hemoglobin S molecule. In a hospital when a child is born, this test is mandatory. Prior to the birth of a child, a mother can have the amniotic fluid surrounding the child to look for the sickle cell trait.
Treatment include a bone marrow transplant. Normally this treatment occurs in children under 16 years. However, since sickle cell is chronic the treatment include avoiding crises, relieving symptoms and preventing complications.
-Antibiotics: penicillin (2mon-5y) to prevent against infection like pneumonia.
-Hydroxyurea: reduces crisis and decreases the need for many blood transfusions.