WILTIMS #67: Ickle Sickle Pickle

In a refreshing reminder that we are adults (and occasionally treated accordingly), we had homework due today that we were simply trusted to have completed. We then had an intelligent student led discussion of the answers, with the people who researched the most complete responses to questions supplementing the answers of others. A smidge of my faith in my fellow classmates has been restored.

TIL: Sickle cell anemia is caused by the substitution of one amino acid (the building blocks of proteins) for another in the beta globin subunit of hemoglobin (the protein in red blood cells that carries oxygen from your lungs to your tissues). The mutant hemoglobin can bind with other hemoglobin proteins, resulting in polymerization. These aggregations of hemoglobin cause the red blood cells (RBCs) to become deformed (hence the sickle cell name) and get stuck in the narrow capillary blood vessels of the body. The tissues become deoxygenated due to the RBCs piling up causing damage and pain.

The body also quickly attacks these sickle cells in the spleen, the body's quality control center for blood cells resulting in anemia and the bone marrow can't replenish RBCs fast enough. The spleen often gets infected due to damage and overwork from this process.

The only cure is a bone marrow transplant, which replaces the stem cells that create the mutant RBCs. Other treatment options include blood transfusions (to swap out bad cells for good), antibiotics (to fight infection in the spleen), erythropoietin (to spur RBC production to combat the anemia), and the administration of the anti-tumor drug hydroxyurea which promotes the production of fetal hemoglobin (which cannot aggregate like the mutant adult hemoglobin).