It is the inheritance of the sickle genes that causes red blood cell (RBC) abnormality. All complications of Sickle Cell Disease can he traced to changes in the make-up of the RBC. Normal RBC’s are smooth surfaced, enabling them to change their shape to flow through small blood vessels. Under certain conditions (i.e., acidosis, dehydration, infection, and low oxygen. etc.) RBC’s containing Sickle Hemoglobin become rigid, elongated, and sickle shaped. Some RBCs sickle immediately, while others remain normal for hours before sickling. Most RBCs containing Sickle Hemoglobin can sickle and then unsick-le. After repeated cycles of sickling and unsickling, the RBC’s become irreversibly sickled.

In Sickle C Disease, some RBCs are sickle cells. The C Hemoglobin forms slightly misshapened RBCs but they are of normal size and color. The C Hemoglobin tends to reduce the complications caused by the sickled calls.

In Sickle Beta Plus or Zero ThaIassemia, a portion of the RBCs are sickle cells. The Thalassemia cells are paler than usual and too small. There is no Hemoglobin A present in Sickle Beta Zero Thalassemia. There is a small amount of Hemoglobin A present in Sickle Beta Plus Thalassemia which tends to minimize the complications caused by the sickled cells.

Sickled RBC's can become trapped within the blood vessels and thus interfere with normal blood flow. This obstruction can lead to sudden pain anywhere in the body as well as cause damage to body tissues and organs over time.

Origin and Distribution of Sickle Cell Disease, Genetics, Sickle Red Blood Cells, Infections ,Splenic Sequestration, Pain, Chest Syndrome, Aplastic Crisis, Strokes, Gall Stones and Jaundice, Growth and Development, Retinopathy, Priapism