Sickle-cell anemia is a genetic blood disorder that affects the hemoglobin protein in red blood cells. Hemoglobin is responsible for carrying oxygen from the lungs to the body’s tissues. In sickle-cell anemia, the abnormal hemoglobin causes red blood cells to become rigid and form a crescent or sickle shape, which can cause the cells to get stuck in blood vessels and block blood flow.
Sickle-cell anemia is caused by a mutation in the HBB gene, which provides instructions for making the beta-globin subunit of hemoglobin. The mutation causes the production of an abnormal form of hemoglobin, known as hemoglobin S, which can cause red blood cells to become sickle-shaped.
Symptoms of sickle-cell anemia can include pain, anemia, fatigue, jaundice, and an increased risk of infections. Complications of the disease can include stroke, acute chest syndrome, and damage to the organs, including the lungs, kidneys, and liver.
Treatment for sickle-cell anemia focuses on managing symptoms and preventing complications. This may include medication to manage pain, antibiotics to prevent infections, blood transfusions, and bone marrow transplants for severe cases. Patients with sickle-cell anemia may also benefit from lifestyle changes, such as staying hydrated, avoiding extreme temperatures, and getting regular medical checkups.
The prognosis for sickle-cell anemia varies depending on the severity of the disease and how well it is managed. With appropriate treatment and lifestyle changes, many people with sickle-cell anemia can lead relatively normal lives, but the disease can still pose significant challenges and risks.