Sickle Cell Disease Flashcards
What do red blood cells contain?
adult hemoglobin
What is the lifespan of a RBC?
90-120 days
What is sickle cell disease?
a group of inherited RBC disorders resulting from a genetic mutation in the genes that encode hemoglobin
Patients with the homozygous inheritance of the sickle cell gene have RBCs that contain abnormal hemoglobin, called
hemoglobin S (HgbS or sickle hemoglobin)
Sickled RBCs burst after how many days?
10-20 days, which causes anemia and fatigue
When do symptoms of SCD develop?
2-3 months after birth
Why does SCD present a few months after birth?
because a fetus and young infants have RBCs with fetal hemoglobin which blocks the sickling of RBCs
What does vascular occlusion mean?
prevents oxygen from reaching the tissues, causing them to become ischemic
What is vase-occlusive crisis?
aka acute pain crisis
most commonly occurs in the lower back, legs, hips, abdomen and chest, can last for days or weeks
What is it called when the pain is in the chest and there is evidence of pulmonary infection?
acute chest syndrome
