Sickle Cell Disease (SCD) – Basic Notes-and latest MOH GUIDELINES
Sickle Cell Disease (SCD) – Basic Notes Definition Sickle Cell Disease (SCD) is an inherited blood disorder caused by abnormal hemoglobin called Hemoglobin S (HbS). Red blood cells become sickle-shaped leading to blockage of blood vessels and destruction of red blood cells. Genetics / Inheritance It is an autosomal recessive disorder. A person develops SCD when they inherit one abnormal gene from each parent. Genotypes HbAA – Normal HbAS – Sickle cell trait HbSS – Sickle cell disease HbSC – Variant form of SCD Pathophysiology HbS polymerizes during low oxygen states causing red blood cells to become rigid and sickle-shaped. These cells block blood vessels leading to ischemia, pain, organ damage, and hemolytic anemia. Triggers of Sickling Infection, dehydration, cold exposure, stress, hypoxia, and acidosis. Clinical Features Recurrent body pains, easy fatigability, pallor, jaundice, fever, delayed growth, anemia, yellow eyes, splenomegaly, leg ulcers, an...