Sickle Cell Flashcards
What is a genetic disorder that’s most common in African American’s where people inherit an abnormal/defective hgb molecule known as HB S trait in their RBC’s from BOTH parents?
Sickle Cell Disease
What if people only inherit the hgb S trait from ONE parent?
Have sickle cell trait
What is a normal hemoglobin?
HB A
The shape of a normal HB A is circular, what’s the shape of HB S?
Sickled shape
What is a chronic hemolytic anemia and is the most common type of sickle cell disease?
Sickle Cell Anemia
In sickle cell disease the person will produce normal ____, but when _____ attaches to the RBC the cell _____ during a time of _____.
RBC’s, Hgb, sickles, stress
How long is the lifespan of a normal RBC? What about a sickled RBC?
120days; 20days
RBC’s get destroyed faster than normal RBC’s if they are sickled, so when they break apart bilirubin is sent out which can’t get rid of fast enough so can cause what?
Jaundice
How many grams of hgb per mL of blood does a normal RBC have? A sickled RBC?
12-14g; 6-9g
What can be done to put more normal RBC’s in the body of someone w/ sickle cell disease?
Blood transfusion
The membrane of a normal RBC is smooth and round, what’s the shape of HB S that cause it to get stuck and occludes the blood vessel?
Hard and rigid
What causes the RBC to sickle?
Episodes of hypoxemia causing HB S to deoxygenate
Dehydration, fever, physical/emotional stress, increase/decrease in environmental temps, high altitudes, poorly pressurized airplanes, and smoking are all what?
Causes of hypoxemia
If a pt. wasn’t getting good O2 causing their RBC’s to sickle can their RBC’s revert back to normal shape after they start getting good O2?
Yes
What is painful periods resulting from ischemia where RBC’s get stuck everywhere and are not reverted?
Sickle cell crisis (Vaso-occlusive crisis)
What can occur from a sickle cell crisis?
Thrombosis or infarction
Dehydration, cold, acidosis, localized hypoxemia, and severe ischemia in limb are all what?
Precipitations of sickle cell crisis