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Cystic Fibrosis Flashcards

1
Q

3 primary immunization recommendations for CF

A

flu, covid, pneumo

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2
Q

how should CF meds be taken

A

BID with fat containing meal
no GFJ

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3
Q

what condition may require dose adjustment for CF meds

A

hepatic dysfunction mod to sev

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4
Q

main AEs of CF meds

A

inc AST, ALT, bili
cataract risk

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5
Q

what should be monitored while on CF meds

A

AST, ALT, bili, eye exams

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6
Q

CF meds are substrates of

A

CYP3A

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7
Q

2 moderate CYP3A inhibitors?

how does dose need to be adjusted for CF meds?

A

erythromycin, fluconazole

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

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8
Q

2 strong CYP3A inhibitors?

how does dose need to be adjustment for CF meds?

A

clarithromycin, itraconazole

IVA twice weekly dosing
TEZ & ELEX twice weekly dosing

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9
Q

which 5 meds are CYP3A inducers?

how do CF med doses need to be adjusted?

A

rifampin, carbamazepine, phenobarbital, phenytoin, SJW

avoid concomitant use with I T or E

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10
Q

ivacaftor age indication

A

1 month or older

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11
Q

ivacaftor brand name

A

kalydeco

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12
Q

lumacaftor + ivacaftor brand name

A

orkambi

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13
Q

lumacaftor + ivacaftor age indication

A

1 year or older

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14
Q

brand name of tezacaftor + ivacaftor

A

symdeko

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15
Q

age indication for tezacaftor + ivacaftor

A

6 years and older

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16
Q

brand name of elexacaftor + tezacaftor + ivacaftor

A

trikafta

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17
Q

age indication for elex + teza + iva

A

2 years and older

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18
Q

mutation indication for ivacaftor

A

IVA responsive mutation

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19
Q

LUMA + IVA mutation indication

A

2 copies of F508del (homozygous)

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20
Q

TEZA + IVA mutation indication

A

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

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21
Q

mutation indication for ELEX-TEZ-IVA

A

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

22
Q

what is the order of inhaled CF med admin

A

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

23
Q

what are the 2 mucociliary clearance agents

A

dornase alfa
hypertonic saline

24
Q

common AE of hypertonic saline?
how is it avoided?

A

bronchospasm
pretreat with albuterol

25
Q

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

A

dornase alfa OR hypertonic saline

26
Q

2 inhaled antibiotics used in CF

A

tobramycin
aztreonam

27
Q

what to do for early pseudomonas eradication? if fails?

A

tobra BID x 28 days
fail- repeat course

28
Q

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

A

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

29
Q

what to do for early MRSA eradication

A

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

30
Q

what is used for chronic infection in CF

A

inhaled tobramycin

31
Q

what antiinflammatory is given in CF if chronically infected with pseudomonas

A

azithromycin

32
Q

when does azithromycin get held?

A

positive for mycobacterium avium

33
Q

which vitamins may need to be supplemented?

34
Q

what is used for exocrine deficiency

A

pancreatic enzyme replacement therapy

35
Q

what kind of diet should be followed in CF

A

moderate fat, high protein, high calorie

36
Q

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

37
Q

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

A

moderate/severe

38
Q

do CF patients need higher or lower doses of antibiotics

A

higher doses

39
Q

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

A

high rate abx allergies & AEs
higher rate MDR organisms

40
Q

2 most common pathogens in pulm exacerbation

A

S. aureus
P. aeruginosa

41
Q

what is given for MILD with PA

A

PO cipro or levoflox
+/- inhaled abx

42
Q

what is given for MILD with MSSA

A

PO Bactrim, doxycycline, or amox/clav

43
Q

what is given for MILD with MRSA

A

PO Bactrim, doxycycline, Linezolid

44
Q

what is used for MOD/SEV with PA

A

IV beta lactam + IV tobramycin

45
Q

what is used for MOD/SEV with MSSA

A

IV beta-lactam

46
Q

what is used for MOD/SEV MRSA

A

IV/PO linezolid
IV vanco
IV ceftaroline

47
Q

what is used for MILD with PA + MSSA

A

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

48
Q

what is used for MILD with PA + MRSA

A

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

49
Q

what is used for MOD/SEV if PA + MSSA

A

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

50
Q

what is used for MOD/SEV with PA + MRSA

A

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

51
Q

how long is antibiotic treatment duration for APE

A

10-14 days

Therapeutics (32 decks)

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