June 19 is World Sickle Cell Day. Sickle cell disease (also called "sickle cell anemia") is a genetic blood disorder that affects millions of children and adults worldwide. People with this disease may spend their days battling exhaustion, and often miss school and sports activities for medical appointments. Adults also have to plan their lives around monthly transfusions, as these keep them in the hospital for an entire day.
What is sickle cell disease?
Sickle cell disease is a common genetic disorder affecting the blood: a mutation in beta-globin creates abnormal hemoglobin, which usually looks like a crescent or a sickle. The mutant configuration of these cells makes red blood cells rigid, sticky, and deformed. Because of this shape, they can block blood vessels, causing intense pain, high blood pressure, and swelling. Moreover, the cells are not fully capable of carrying oxygen or traveling in the blood, which makes people with sickle cell disease feel tired most of the time.
Sickle cell disease is usually diagnosed in childhood. Children inherit the recessive trait if both parents are carriers of the mutated gene. People who have the recessive gene sometimes have sickle cell hemoglobin in their bloodstream, but not enough to cause problems or symptoms. They will never develop sickle cell disease, but there is a chance they can pass it on to their children: this is called sickle cell trait.
