Sickle cell anemia


Disease Overview

Sickle cell anemia is a disease in which the body produces abnormally shaped red blood cells that have a crescent or sickle shape. These cells do not last as long as normal, round, red blood cells, which leads to anemia (low number of red blood cells). The sickle cells also get stuck in blood vessels, blocking blood flow.[2768] Signs and symptoms of sickle cell disease usually begin in early childhood and may include anemia, repeated infections, and periodic episodes of pain (called crises). This condition is caused by mutations in the HBB gene and is inherited in an autosomal recessive pattern.[2767] Treatment typically focuses on controlling symptoms and may include pain medicines during crises; hydroxyurea to reduce the number of pain episodes; antibiotics and vaccines to prevent bacterial infections; and blood transfusions.[2768] On July 7, 2017, the FDA in the United States approved the use of Endari (prescription grade L-glutamine) to reduce the number of sickle cell crisis. Endari is the first FDA approved treatment that is also available for children with sickle cell disease five years of age and older.[13585]


  • HbS disease
  • Hemoglobin S Disease
  • Sickling disorder due to hemoglobin S

For more information, visit GARD.

National Organization for Rare Disorders