Sickle cell disease (SCD) is a group of inherited blood disorders that affect hemoglobin, the protein in red blood cells responsible for carrying oxygen throughout the body. In people with SCD, the hemoglobin is abnormal, causing red blood cells to become hard, sticky, and shaped like a crescent or sickle, rather than their normal round and flexible shape
. These sickle-shaped cells can clump together and block small blood vessels, which reduces or stops blood flow and oxygen delivery to tissues and organs. This can cause episodes of severe pain (called sickle cell crises), anemia (due to the rapid breakdown of sickle cells), increased risk of infections, organ damage, and other serious complications such as stroke
. SCD is inherited when a person receives two abnormal copies of the gene that codes for hemoglobin, one from each parent. People with only one abnormal gene copy usually do not have symptoms and are carriers (sickle cell trait)
. The most severe form of SCD is sickle cell anemia, characterized by a predominance of sickle-shaped red blood cells and severe anemia
. SCD is a lifelong condition, but treatments such as pain management, hydration, blood transfusions, medications like hydroxyurea, and recently approved gene therapies can help manage symptoms and improve life expectancy
. Sickle cell disease is especially common in people of African or Caribbean descent but also occurs in other populations worldwide
. In summary, sickle cell disease is an inherited disorder causing abnormal, sickle-shaped red blood cells that block blood flow and lead to pain, anemia, and organ damage. It requires ongoing medical care to manage its complications
