Cystic Fibrosis

Question 1

3 primary immunization recommendations for CF

Answer 1

flu, covid, pneumo

Question 2

how should CF meds be taken

Answer 2

BID with fat containing meal
no GFJ

Question 3

what condition may require dose adjustment for CF meds

Answer 3

hepatic dysfunction mod to sev

Question 4

main AEs of CF meds

Answer 4

inc AST, ALT, bili
cataract risk

Question 5

what should be monitored while on CF meds

Answer 5

AST, ALT, bili, eye exams

Question 6

CF meds are substrates of

Answer 6

CYP3A

Question 7

2 moderate CYP3A inhibitors?

how does dose need to be adjusted for CF meds?

Answer 7

erythromycin, fluconazole

IVA once daily
TEZ & ELEX alternate QD dosing between combo and I

Question 8

2 strong CYP3A inhibitors?

how does dose need to be adjustment for CF meds?

Answer 8

clarithromycin, itraconazole

IVA twice weekly dosing
TEZ & ELEX twice weekly dosing

Question 9

which 5 meds are CYP3A inducers?

how do CF med doses need to be adjusted?

Answer 9

rifampin, carbamazepine, phenobarbital, phenytoin, SJW

avoid concomitant use with I T or E

Question 10

ivacaftor age indication

Answer 10

1 month or older

Question 11

ivacaftor brand name

Answer 11

kalydeco

Question 12

lumacaftor + ivacaftor brand name

Answer 12

orkambi

Question 13

lumacaftor + ivacaftor age indication

Answer 13

1 year or older

Question 14

brand name of tezacaftor + ivacaftor

Answer 14

symdeko

Question 15

age indication for tezacaftor + ivacaftor

Answer 15

6 years and older

Question 16

brand name of elexacaftor + tezacaftor + ivacaftor

Answer 16

trikafta

Question 17

age indication for elex + teza + iva

Answer 17

2 years and older

Question 18

mutation indication for ivacaftor

Answer 18

IVA responsive mutation

Question 19

LUMA + IVA mutation indication

Answer 19

2 copies of F508del (homozygous)

Question 20

TEZA + IVA mutation indication

Answer 20

2 copies F408del
OR
1 or more TEZ/IVA responsive mutation

Question 21

mutation indication for ELEX-TEZ-IVA

Answer 21

1 or more copy of F508 del
OR
1 or more E/T/I responsive mutation

Question 22

what is the order of inhaled CF med admin

Answer 22

B2 agonist > hypertonic saline > dornase alfa > airway CL techniques > inhaled ABX

Question 23

what are the 2 mucociliary clearance agents

Answer 23

dornase alfa
hypertonic saline

Question 24

common AE of hypertonic saline?
how is it avoided?

Answer 24

bronchospasm
pretreat with albuterol

Question 25

which CF meds can be trialed off if have mild disease?

Answer 25

dornase alfa OR hypertonic saline

Question 26

2 inhaled antibiotics used in CF

Answer 26

tobramycin aztreonam

Question 27

what to do for early pseudomonas eradication? if fails?

Answer 27

tobra BID x 28 days fail- repeat course

Question 28

what to do for early PA eradication if fail 1st 2 tobra courses?

Answer 28

IV abx x 14days (IV tobra + B-lactam) then repeat tobra course

Question 29

what to do for early MRSA eradication

Answer 29

Bactrim + rifampin x14 days nasal/skin/oral decontam environmental decontam

Question 30

what is used for chronic infection in CF

Answer 30

inhaled tobramycin

Question 31

what antiinflammatory is given in CF if chronically infected with pseudomonas

Answer 31

azithromycin

Question 32

when does azithromycin get held?

Answer 32

positive for mycobacterium avium

Question 33

which vitamins may need to be supplemented?

Answer 33

ADEK

Question 34

what is used for exocrine deficiency

Answer 34

pancreatic enzyme replacement therapy

Question 35

what kind of diet should be followed in CF

Answer 35

moderate fat, high protein, high calorie

Question 36

severity of pulm exacerbation if modest symptom change and/or FEV1 decline < 10%

Answer 36

mild

Question 37

severity of pulm exacerbation if substantial symptom change and/or FEV1 decline >10%

Answer 37

moderate/severe

Question 38

do CF patients need higher or lower doses of antibiotics

Answer 38

higher doses

Question 39

what is the impact of having multiple antibiotic courses over a lifespan

Answer 39

high rate abx allergies & AEs higher rate MDR organisms

Question 40

2 most common pathogens in pulm exacerbation

Answer 40

S. aureus P. aeruginosa

Question 41

what is given for MILD with PA

Answer 41

PO cipro or levoflox +/- inhaled abx

Question 42

what is given for MILD with MSSA

Answer 42

PO Bactrim, doxycycline, or amox/clav

Question 43

what is given for MILD with MRSA

Answer 43

PO Bactrim, doxycycline, Linezolid

Question 44

what is used for MOD/SEV with PA

Answer 44

IV beta lactam + IV tobramycin

Question 45

what is used for MOD/SEV with MSSA

Answer 45

IV beta-lactam

Question 46

what is used for MOD/SEV MRSA

Answer 46

IV/PO linezolid IV vanco IV ceftaroline

Question 47

what is used for MILD with PA + MSSA

Answer 47

PO cipro or levo +/- inhaled abx and PO Bactrim, doxy, amox/clav

Question 48

what is used for MILD with PA + MRSA

Answer 48

PO cipro or levo +/- inhaled abx and PO Bactrim, doxy, Linezolid

Question 49

what is used for MOD/SEV if PA + MSSA

Answer 49

IV/PO fluoroquinolone or IV tobra AND IV beta-lactam (pip/tazo, cefepime, meropenem)

Question 50

what is used for MOD/SEV with PA + MRSA

Answer 50

IV/PO fluoroquinolone or IV tobra AND IV vanco or IV/PO linezolid + IV/PO fluoroquinolone or IV tobra

Question 51

how long is antibiotic treatment duration for APE

Answer 51

10-14 days