Lametra Scott, PharmD, CCHP, CEO of Breaking The Sickle Cell Cycle Foundation Inc, discussed the pathophysiology of sickle cell disease (SCD) the dangers of leaving it untreated.
Lametra Scott, PharmD, CCHP, chief executive officer of Breaking The Sickle Cell Cycle Foundation Inc, discussed the pathophysiology of sickle cell disease (SCD) and the dangers of insufficient treatment.
What is the pathophysiology of sickle cell disease (SCD) and what are the consequences that may occur if not properly treated?
SCD is very complex in regards to how it affects the body. But in terms of what begins those changes is very simple. It starts with a DNA substitution down at the cellular level, which creates a defective form of hemoglobin. That defective form of hemoglobin does not carry oxygen well, and those red blood cells that contain that defective hemoglobin become very fragile.
So, in that, these cells hemolyze very quickly, which causes anemia and secondary inflammation in the body. That secondary inflammation causes basal occlusion, where those blood cells stick together. Those cells that stick together also form blood clots and those blood clots actually block blood flow. Blocking blood flow is what causes tissue necrosis, organ death, stroke, and even death.
So, that's what's happened along in the hemolysis phase of it. And SCD is also compounded with changing the characteristics of the red blood cells in itself, meaning that those red blood cells in addition to hemolyzing, they also become sticky. They also become a distorted shape. They become hard and rigid. So, these hard rigid sails with a distorted shape that takes on the form of a sickle, hence the name sickle cell, also causes basal occlusion because now that these cells stick to the vasculature, other cells, and each other, they cause basal occlusion.
So, hemolysis and basal occlusion are the primary pathophysiologic concepts associated with SCD that causes all the sequela from the disease. And it is not just one, it's a total body effect that can happen because of sickle cell.