Sickle cell disease is an inherited blood disorder that affects hemoglobin, the protein that carries oxygen through the body. In sickle cell disease, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a “sickle” . Normal red blood cells are round and flexible, which allows them to move easily through small blood vessels in the body to deliver oxygen to organs and tissues. However, in sickle cell disease, the abnormal hemoglobin changes the shape of the red blood cell to a crescent shape, causing the cells to become rigid, lack flexibility, and stick together. This can block blood flow, preventing oxygen from getting to vital organs and tissues throughout the body, leading to serious complications including pain, infections, and organ damage and failure.
Signs and symptoms of sickle cell disease usually appear around 6 months of age and vary from person to person. They may include anemia, fatigue, jaundice, and episodes of pain, among others. There is no cure for most people with sickle cell disease, but treatments can relieve pain and help prevent complications associated with the disease.
