Lightning Learning: Sickle Cell Disease
Sickle Cell disease is a genetic disease that causes red blood cells to become sickle-shaped. The sickle-shaped cells are more rigid and stick together causing obstruction to blood flow.
The obstruction stops oxygen reaching the tissues which is called a vaso-occlusive crisis. A crisis causes sudden severe pain often requiring strong analgesia.
Causes: infection or sepsis, hypoxia, cold weather and dehydration
Treatment: high flow oxygen, strong analgesia and fluids
80% of admissions related to sickle cell disease are related to vaso-occlusive crises. Can be life-threatening and shortens life expectancy. Contact haematology and get advice regarding transfusion.
Other crisis presentations: chest syndrome, splenic sequestration and aplastic crisis.
Complications: stroke, priapism, renal failure and can be more prone to infections due to splenic damage.