sickle cell anemia Flashcards
what is sickle cell anemia
chronic hemolytic anemia with sickle shaped RBCs that clog capillaries causing organ ischemia in hypoxia or dehydrated conditions
does acidosis increase sickling?
yes
percentage of African Americans that carry this trait
8%
what type of hereditary disorder is sickle cell anemia
autosomal recessive
carrier trait of sickle cell anemia offers protection agaisnt
malaria
average life expectancy for pt with sickle cell anemia in US
40-50 years
symptoms of sickle cell anemia
anemia jaundice cholelithiasis increased cardiac output- murmur delayed growth
vaso occlusion leads to ischemic organ damage, especially splenic infarction. ischemia can also cause _____ of fingers and toes
gangrene
common triggers for vasoocclusive crisis
cold temperatures
dehydration
infection
what tests are used to diagnose sickle cell anemia
peripheral blood smear and electropheresis
what will peripheral blood smear shoe for pt with sickle cell anemia
sickled cells, Howell jolly bodies, nucleated RBCs and target cells
possible increased WBC and platelets
possible indirect bilirubin elevation
what does Hgb electrophoresis show
Hbgs in RBC, reticulocytes present, normochromic
treatment for sickle cell
conservative management: vaccines folate supplementation prophylactic antibiotics hydroxyurea hydration NSAIDS opiates transfusion fluids regular eye exams to check for retinopathy bone marrow transplant can be helpful
surgical:
cholecystechtomy
splenectomy
dialysis
avoid dehydration
