Sickle Cell Disease Flashcards
What vaccines do people with sickle cell disease get?
H influenza type B, the pneumonococcal series, the entire meningococcal series (menactra/menveo + serogroup B (bexsero or trumebba)
What vaccines do the immunideficient get
the pneumonia series (acceperated), menoncococcal conjugate (menactra/menveo),hep a and B
what vaccines do people with diabetes get
pneumovax 23, hep b
what are the live vaccines
COZY IV RM
Cholera, oral typhoid (vivitod), Zoster, Yellow Fever, Influenzanasal, Varicella (varivax), rotavirus, MMR
What are the pathogens of concern for sickle cell disease
S. pneumoniae, h. influenzae, nesseria meningitis
what are the treatments for sickle cell
1) chelation to remove extra iron, 2) BMT as a cure, 3) Analgensics for pain 4) hydroxyrea to reduce frequency of acute and chronic complications
How is prophylactic penicillin administered and what is it for
Initiated twice daily and treated until 5 years of age to prevent sepsis and meningitis
what does hydroxyurea do?
Stimulates production of HgbF
What is hydroxyurea indicated for?
Adults with > 3 moderate - severe pain crises in one year
what are the three major warnings regarding hydroxyurea
myelosupressin, avoud live vaccines, and fetal toxcitity
how should hydroxyurea be handled
with caution since hazerdous - wear gloves and wash hands
what is required and what is reccomended during hydroxyurea thrapy
contraception reqyired, folic acid reccomended
how is hyroxyurea monitored
via a cbc with differential
what does voxelotor do
inhibits hemoglobin S polymerization
What are the three iron chealating drugs
deferoxamine (antidote for iron toxicity, no longer used, not oral), deferasirox, deferiprone
when do symptoms of SCD develop
2-3 months after birth
how long do sickle cell RBCS last
10-20 days
what happens in vaso occlusive crisis
when oxygen doesnt reach the tissues causing them to be ischemic -> if in chest can be called acute chest syndrome
Major DDI with hydroxyurea
do not use with any other drugs that can cause myelosupression i.e. tacrolimus
what age to give men group B
10
What age to give pneymovax if recieved pcv13 as a child
age 2 then 5 years later
if never recieved pcv 13 when to receive prevnar
age greater than 6
ANC calculation formula
WBC x (%segs + %bands/100)
ANC and platelet threshold for hydroxyurea
<2000 mm^3 for ANC and <80,000 MM^3
