Sickle Cell Anemia Flashcards
Why is RBC count usually lower in patients with sickle cell
the body can’t keep up with the death of RBCs
What is the cause of sickle cell anemia
a mutation in one of the genes that directs the synthesis of hemoglobin beta polypeptide chains (v is substituted for ga)
Why is hemoglobin inefficient at carrying O2
due to the hemoglobin S shape
Abnormal RBC lifespan
short life and are quickly broken down in the spleen
Why does hemolytic anemia occur
the bone marrow cannot keep up with RBC production compared with lost RBCs
What does the blockage of capillaries by misshapen RBCs cause
ischemia and vaso-occlusive crisis
Vaso-occlusive crisis
sickled cells become trapped, blocking blood flow and creating ischemia and tissue hypoxia leading to organ damage and infarction
When is vaso-occlusive crisis more likely
illness, stress, heat/cold, dehydration, high altitude
What is the main clinical manifestation of sickle cell anemia (especially during vaso-occlusive crisis)
pain
What body areas are most effected by vaso-occlusive crisis
chest, abdomen, long bones, and joints
Blood test diagnostics for sickle cell
CBC, blood smear, reticulocytosis
When is screening done
newborn age
Sickle cell treatment during crisis
opioids, oxygen, hydration, and prophylactic antibiotics
Sickle cells treatment for managing and prevention of crisis
hydroxyurea, blood transfusions, iron chelating agents, bone marrow transplants, immunizations
Splenic sequestration crisis
ischemia and occlusion of vessels in the spleen
