Vaso-occlusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease.
Vaso-occlusion results in recurrent painful episodes (previously called sickle cell crisis) and a variety of serious organ system complications that can lead to life-long disabilities and even death.
Hemolysis of red blood cells (RBC) causes chronic anemia and pigment gallstones.
There are multiple components to the management of SCD, including the prevention and treatment of the complications of SCD, as well as the potential cure for this illness:
Initial prevention of complications includes the use of antibiotic prophylaxis started in the newborn period, appropriate immunizations, and blood transfusions for those at risk for stroke.
Therapies to prevent pain episodes in SCD including hydroxyurea, L-glutamine.
"Hydroxyurea use in sickle cell disease" and "Disease-modifying therapies to prevent pain and other complications of sickle cell disease".
Gene therapy trials are ongoing and show promise.