Sickle-Cell Disease (Scd), Also Known As Sickle-Cell Anemia (Sca),

NURSING SICKLE CELL SICKLE CELL DISEASE Sickle-cell disease (SCD), also known as sickle-cell anemia (SCA), is a hereditary blood disorder, caused by an abnormality in the oxygen-carrying protein hemoglobin found in red blood cells. This leads to a propensity for the cells to assume an abnormal, rigid, sickle-like shape under certain circumstances. 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. These changes also produce the sickle shape. Less the pliability needed to traverse small capillaries leading to altered sticky membranes that are abnormally adherent to the endothelium of small venules (This venoocclusive component usually dominates the clinical course) Episodes of microvascular vasoocclusion Premature RBC destruction (hemolytic anemia) because the spleen destroys the abnormal RBC. The rigid adherent cells clog small capillaries and venules, causing Tissue ischemia, Acute pain, and Gradual end-organ damage. Prominent manifestations include Episodes of ischemic pain (i.e., painful crises) Ischemic malfunction or frank infarction in the Spleen, Central nervous