Sickle cell disease (SCD) is a group of inherited blood disorders that affects the shape and function of red blood cells. Normally, red blood cells are round and flexible, allowing them to move easily through blood vessels, delivering oxygen to different parts of the body. However, in SCD, the red blood cells become crescent or sickle-shaped, rigid and sticky. This causes blockage blood flow in the very tiny blood vessels (capillaries) of the blood vessels outside of the heart and prevents the normal flow of nutrition and oxygen.
While signs and symptoms vary from person to person, common symptoms associated with SCD include excruciating pain known as sickle cell crises which can last days (acute) or long-term (chronic), severe infections, low levels of circulating red blood cells (anemia) and yellowing of the skin (jaundice). The blocked blood flow can also cause severe organ damage including stroke and other long-term health problems.
SCD is an inherited condition, and people who receive the sickle mutation from each parent have the disease. However, some people with SCD only get the sickle cell mutation from one parent, but have a different mutation from the other parent, which can lead to other forms of the disease. There are several forms of sickle cell disease, with the most common ones being the sickle cell anemia (SS), sickle hemoglobin disease C (SC), sickle beta-plus thalassemia (Sβ+) and sickle beta-zero thalassemia (Sβ0). Sickle cell anemia is the most severe and common type.
The treatment for SCD aims to manage symptoms, prevent complications and improve the quality of life for individuals affected by the condition. The specific treatment plan may vary based on the type and severity of SCD, as well as the individual's age and overall health.