Root Cause of Sickle Cell

Root Cause of Sickle Cell

What Happens in Sickle Cell

The root cause of sickle cell starts with a process called hemoglobin polymerization. This process changes the shape of red blood cells and impacts their ability to carry oxygen throughout the body.

 

Why do red blood cells form a sickle shape?

Red blood cells contain a protein called hemoglobin. Hemoglobin carries the oxygen that blood delivers throughout the body.

  • When the hemoglobin molecules are carrying oxygen, they move around the blood cell freely
  • In sickle cell, after hemoglobin has delivered Circulatory system illustrationoxygen to tissues, the red blood cell does not keep its round shape
  • When hemoglobin lose their oxygen, they start to stick together and form long, stiff chains (called polymers) inside the cell
  • Because these chains of hemoglobin keep building, they can get longer than the red blood cell itself and curve its shape into the sickle or crescent shape.

 

Hemoglobin Polymerization

Illustrated diagram comparing the difference between hemoglobin inside healthy cells and hemoglobin inside sickled cells

When you have sickle cell disease, red blood cells are sickling and continue to cause damage and injury even when you do not feel any pain. The silent damage of sickle cell is caused by these three things:

  • Hemolysis – The fast breakdown of red blood cells
  • Anemia – Having too few healthy red blood cells to carry oxygen to the body’s tissues
  • Vaso-occlusion – Blockage of blood vessels by sickled red blood cells, which can lead to pain crises and organ damage

Recently, researchers have learned a lot more about these changes and what causes them.