Sickle Cell Disease Flashcards
Race most affected by sickle cell disease
African Americans
Pathophysiology of sickle cell disease
RBC’s are not shaped correctly; cannot effectively carry oxygen
RBCs hemolyze after 10-20 days
leading cause of death in SCD
Acute chest syndrome
sickle cell crisis - pain in the chest and signs of infection
Why are sickle cell pts at incr risk for infection?
Pts spleen loses function early in life
spleen plays a vital role in immune function
Non-drug treatment for sickle cell
blood transfusions - protects against many life threatening complications
goal Hgb ~ 10
Acute complications of sickle cell
Anemia Cholecystitis infection pain crisis stroke priapism
Preferred oral contraception for sickle cell pts
progestin only contraception
IUDs
bc pts are at increased risk of stroke
What antibiotic is given as prophylaxis in children and infants?
penicllin
Organisms that are more likely to cause infections in pts with functional asplenia
S. pneimoniae
H influenzae
N meningitidis
Routine childhood vaccines needed for kids with sickle cell
HIB
PCV13 (Prevnar)
Vaccines recommended due to functional asplenia
Meningococcal conjugate
Meningococcal serogroup B (>/= 10 y/o)
PPSV23 (> 2 y/o, then 5 years later, then > 65)
Droxia
Hydrea
Hydroxyurea
Role of Hydroxyurea
stimulates production of HgbF
Reduces frequency of pain crises, need for blood transfusions
Indicated for adults with 3 or more pain crises in 1 year and all kids > 9 months old
Hydroxyura boxed warning
Myelosuppression
Hydroxyurea side effects
N/v/d
incr LFTs, uric acid, BUN , SCr
Hydroxyurea starting dose
15 mg/kg/day as a single dose
20 for kids and children
Hydroxyurea monitoring
absolute Neutrophil count
goal ANC: 2000-4000
stop therapy if ANC < 2000 and/or PLT < 80,000
How long does it take to see a clinical response from hydroyurea?
3-6 months
can hydroyurea be used in pregnancy?
NOOOO
Contraception is required for all pts taking and females must use contraception for 6 months after d/c (12 mons for males)
Endari
L-glutamine oral powder
Role of Endari
reduce acute complications of SCD (like hydroxyurea)
Endari dosing
<30 kg: 5 g BID
30-65 kg: 10 mg BID
> 65 kg: 15 mg BID
Endari administration
mix with 8 oz of liquid (water, milk, etc)
or 4-6 oz of food (applesauce, yogurt)
What is the concern of giving multiple blood transfusions?
Iron overload; can damage organs
