Which of the following statements about the pathophysiology of SCD is correct?

Sickle Cell Disease (SCD) is characterized by the presence of sickled red blood cells that result from a genetic mutation. The correct statement highlights the critical pathophysiological component of SCD: sickled cells have a rigid, distorted shape, which causes them to become trapped in small blood vessels. This obstruction can lead to reduced or cut-off blood flow to various tissues and organs, resulting in ischemia. This ischemic event contributes to the pain crises commonly experienced by patients with SCD, known as vaso-occlusive crises.

In contrast, the other statements do not accurately reflect the underlying pathophysiology of SCD. Flexibility of red blood cells is decreased in SCD, as the abnormal hemoglobin (HbS) leads to the sickling of cells under low oxygen conditions. SCD is linked specifically to mutations in the beta globin gene rather than alpha globin. Lastly, while SCD does lead to increased red blood cell production in response to chronic anemia, this process is not indefinite and is influenced by various physiological factors, including the body’s oxygen needs and the functioning of the bone marrow. Understanding these dynamics helps reinforce the accurate picture of how SCD affects the body.