Sickle Cell Disease

Question 1

What vaccines do people with sickle cell disease get?

Answer 1

H influenza type B, the pneumonococcal series, the entire meningococcal series (menactra/menveo + serogroup B (bexsero or trumebba)

Question 2

What vaccines do the immunideficient get

Answer 2

the pneumonia series (acceperated), menoncococcal conjugate (menactra/menveo),hep a and B

Question 3

what vaccines do people with diabetes get

Answer 3

pneumovax 23, hep b

Question 4

what are the live vaccines

Answer 4

COZY IV RM

Cholera, oral typhoid (vivitod), Zoster, Yellow Fever, Influenzanasal, Varicella (varivax), rotavirus, MMR

Question 5

What are the pathogens of concern for sickle cell disease

Answer 5

S. pneumoniae, h. influenzae, nesseria meningitis

Question 6

what are the treatments for sickle cell

Answer 6

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

Question 7

How is prophylactic penicillin administered and what is it for

Answer 7

Initiated twice daily and treated until 5 years of age to prevent sepsis and meningitis

Question 8

what does hydroxyurea do?

Answer 8

Stimulates production of HgbF

Question 9

What is hydroxyurea indicated for?

Answer 9

Adults with > 3 moderate - severe pain crises in one year

Question 10

what are the three major warnings regarding hydroxyurea

Answer 10

myelosupressin, avoud live vaccines, and fetal toxcitity

Question 11

how should hydroxyurea be handled

Answer 11

with caution since hazerdous - wear gloves and wash hands

Question 12

what is required and what is reccomended during hydroxyurea thrapy

Answer 12

contraception reqyired, folic acid reccomended

Question 13

how is hyroxyurea monitored

Answer 13

via a cbc with differential

Question 14

what does voxelotor do

Answer 14

inhibits hemoglobin S polymerization

Question 15

What are the three iron chealating drugs

Answer 15

deferoxamine (antidote for iron toxicity, no longer used, not oral), deferasirox, deferiprone

Question 16

when do symptoms of SCD develop

Answer 16

2-3 months after birth

Question 17

how long do sickle cell RBCS last

Answer 17

10-20 days

Question 18

what happens in vaso occlusive crisis

Answer 18

when oxygen doesnt reach the tissues causing them to be ischemic -> if in chest can be called acute chest syndrome

Question 19

Major DDI with hydroxyurea

Answer 19

do not use with any other drugs that can cause myelosupression i.e. tacrolimus

Question 20

what age to give men group B

Answer 20

10

Question 21

What age to give pneymovax if recieved pcv13 as a child

Answer 21

age 2 then 5 years later

Question 22

if never recieved pcv 13 when to receive prevnar

Answer 22

age greater than 6

Question 23

ANC calculation formula

Answer 23

WBC x (%segs + %bands/100)

Question 24

ANC and platelet threshold for hydroxyurea

Answer 24

<2000 mm^3 for ANC and <80,000 MM^3