sickle cell disease Flashcards
Hgb SS
sickled cells
alpha 2 gamma 2
fetal hgb (hgb F)
hydrops fetalis
when no alpha chains, not compatable with life
HgbSS under stress
will sickle
rigid, cannot deform
cannot go through capillaries
= ischemia distally
= hemolysis of sickel cells –> hemolytic anemia
increased RBC lysis from sickle damage causes …
increased uncon.bili
- always be slightly jaundiced
- bilirubin always elevated 1-2 instead of <1 like ppl w/o sickle cell dz
- higher risk pigmented GB stones
baseline numbers to know for sickle pts
baseline bilirubin (1-2) baseline Hgb (7-9) baseline reticulocyte
sickle cell anemia leads to…..
- kidney low blood
- ramp up EPO
- BM ramp up RBC formation
= elevated reticulocyte ct
caution with sickle cell transfusions
high risk for iron overload
..reverse with chelator deferoxamine
spleen what happens?
autoinfarct
most sickle cell asplenic .. but increase risk infxn encapsulated
asplenic sickle cell kids regimen
PCN till age 5 and pneumococcal vax
osteomyelitis
higher risk sickle cell, staph MC, also salmonella
orthopedic injury at risk for
- osteomyelitis
- AVN of femur
AVN of hip tx
conservative NWB 4-6 mo, NSAID.. then surg
acute risks for sickle cell
- stroke / AMS
- priapism
- CP, SOB, Pulmonary edema = acute chest
two indications exchange transfusions
- stroke / ams
- acute chest
- both emergencies
chronic pain typical?
yes
t/f hgbss on prenatal screen?
yes
confirm dx with
Hgb electrophoresis
acute pain crisis tx
vasooclusive crisis that is not a indication for a exchange transfusion is treated by:
IVF, O2, PAIN CONTROL
what three labs evaluate in sickle?
- is Hgb lower?
- is reticulocyte count higher?
- is bilirubin higher?
than their baseline? this is acute crisis
in acute crisis but no labs to show this how tx?
f/u with psychosocial stressors
may not be pain medications… may just need help with something going on at home
prophylaxis how does hydroxyurea work
increases production of Hgb F which cannot sickle = less crisis
Hgb SS Hgb SC Trait HgbSB+ HgbSB0
SS: sickle cell dz SC: low Hgb, no dz trait: counsel pregnant, carriers B+: mild B0: most severe cases
common chronic sickle med regimen
- chronic opioids
- stool softener
- gut motility agent
- PCN as kid
- hydroxyurea
- vaccinations!
