sickle cell disease

Question 1

Hgb SS

Answer 1

sickled cells

Question 2

alpha 2 gamma 2

Answer 2

fetal hgb (hgb F)

Question 3

hydrops fetalis

Answer 3

when no alpha chains, not compatable with life

Question 4

HgbSS under stress

Answer 4

will sickle
rigid, cannot deform
cannot go through capillaries

= ischemia distally
= hemolysis of sickel cells –> hemolytic anemia

Question 5

increased RBC lysis from sickle damage causes …

Answer 5

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

Question 6

baseline numbers to know for sickle pts

Answer 6

baseline bilirubin (1-2)
baseline Hgb (7-9) 
baseline reticulocyte

Question 7

sickle cell anemia leads to…..

Answer 7

• kidney low blood
• ramp up EPO
• BM ramp up RBC formation

= elevated reticulocyte ct

Question 8

caution with sickle cell transfusions

Answer 8

high risk for iron overload

..reverse with chelator deferoxamine

Question 9

spleen what happens?

Answer 9

autoinfarct

most sickle cell asplenic .. but increase risk infxn encapsulated

Question 10

asplenic sickle cell kids regimen

Answer 10

PCN till age 5 and pneumococcal vax

Question 11

osteomyelitis

Answer 11

higher risk sickle cell, staph MC, also salmonella

Question 12

orthopedic injury at risk for

Answer 12

• osteomyelitis

- AVN of femur

Question 13

AVN of hip tx

Answer 13

conservative NWB 4-6 mo, NSAID.. then surg

Question 14

acute risks for sickle cell

Answer 14

• stroke / AMS
• priapism
• CP, SOB, Pulmonary edema = acute chest

Question 15

two indications exchange transfusions

Answer 15

• stroke / ams
• acute chest
• both emergencies

Question 16

chronic pain typical?

Answer 16

yes

Question 17

t/f hgbss on prenatal screen?

Answer 17

yes

Question 18

confirm dx with

Answer 18

Hgb electrophoresis

Question 19

acute pain crisis tx

Answer 19

vasooclusive crisis that is not a indication for a exchange transfusion is treated by:
IVF, O2, PAIN CONTROL

Question 20

what three labs evaluate in sickle?

Answer 20

• is Hgb lower?
• is reticulocyte count higher?
• is bilirubin higher?

than their baseline? this is acute crisis

Question 21

in acute crisis but no labs to show this how tx?

Answer 21

f/u with psychosocial stressors

may not be pain medications… may just need help with something going on at home

Question 22

prophylaxis how does hydroxyurea work

Answer 22

increases production of Hgb F which cannot sickle = less crisis

Question 23

Hgb SS 
Hgb SC 
Trait 
HgbSB+ 
HgbSB0

Answer 23

SS: sickle cell dz 
SC: low Hgb, no dz 
trait: counsel pregnant, carriers 
B+: mild 
B0: most severe cases

Question 24

common chronic sickle med regimen

Answer 24

• chronic opioids
• stool softener
• gut motility agent
• PCN as kid
• hydroxyurea
• vaccinations!