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PHM 702: Pulm and ToXXX > Cystic Fibrooooo > Flashcards

Cystic Fibrooooo Flashcards

1
Q

How old do you have to be to use ivacaftor?

A

older than 1 month

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2
Q

How old do you have to be to use Lumacaftor-Ivacaftor

A

Older than 1 year

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3
Q

How old do you have to be to use tezacaftor/ivacaftor

A

older than 6 years

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4
Q

How old do you have to be to use elexacaftor/tezacaftor/ivacaftor

A

Older than 2 years

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5
Q

Mutation that can use ivacaftor

A

ivacaftor-responsive CFTR mutation (G551D) - I think we get this chart on the exam??

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6
Q

Mutation that can use lumacaftor/ivacaftor

A

2 copies of the F508 del CFTR mutation (homozygous)

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7
Q

Mutation that can use tezacaftor/ivacaftor

A

2 copies of the F508 del CFTR mutation (homozygous)

OR

> 1 CFTR mutation that is reponsive to T-I

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8
Q

Mutation that can use elexacaftor/tezacaftor/ivacaftor

A

Everyone :)

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9
Q

ivacaftor MOA

A
  • Facilitate opening of the Cl channel (CFTR potentiator)
  • Does NOT create more protein, helps existing proteins work better
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10
Q

lumacaftor MOA

A

fixes defective CFTR protein so that it can move to the proper place on the airway cell surface (CFTR corrector)

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11
Q

tezacaftor MOA

A

fixes defective CFTR protein so that it can move to the proper place on the airway cell surface (CFTR corrector)

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12
Q

CFTR modulator agents

A

-caftor

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13
Q

CFTR modulator monitoring

A
  • AST/ALT Q3mo. for the first year and then annualy after
    • Need dose adjustment if liver impairment
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14
Q

CFTR admin

A
  • Give with fat containing meal to boost absoprtion
    • NO GRAPEFRUIT
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15
Q

Dornase alfa in CF

A
  • Breaks up secretions
  • Improves pulmonary function and reduce acute pulmonary exacerbations
  • Well tolerated
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16
Q

Hypertonic salinein CF

A
  • Doesn’t improve lung function but does reduce times that pateint gets sick
  • Well tolerated, but some bronchospasm reported as AE
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17
Q

Bronchodilator use in CF

A
  • Really only used because some of the other meds can cause bronchospasms (e.g. hypertonic saline
  • Albuterol is the most used agent
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18
Q

anti-inflammatory agent in CF

A

Azithromycin - not being used as an ABX
- Dosed MWF
- If pt <25kg: 10mg/kg
- If pt <40kg: 250mg
- If pt >40kg: 500mg

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19
Q

Azithromycin indication in CF?

A

most clear indication for patients w PA colonization
Can be used w/o PA if hx of frequent APEs

20
Q

CF non-pharm and supplements

A
  • Flu vaccine
  • Physiotherapy
  • Vitamins: A, D, E, K
  • Pancreatic enzyme replacement (PERT) - microencapsulated products
    - dose dependent on lipase component
  • Fe
  • Zinc if poor weight gain
21
Q

CF inhaled ABX

A
  • Tobramycin
    • Nebulizer admined over 15min
    • DPI admined over 2-7 min
  • Aztreonam
    • Nebulizer admined over 2-3 min
  • Use ABX alternating 28 days ON and 28 days OFF → reduce resistnace
22
Q

S/S of acute CF pulmonary exacerbation

A
  • Cough, Increased sputum production
  • SOB
  • Chest pain
  • Loss of appetite, Weight loss
  • Lung function decline
23
Q

CF exacerbation admission: what to do with homes meds

A

basically double frequency
- Increase vest treatment frequency
- Increase dornase alfa frequency
- Infrease hypertonic saline frequency
- Increase bronchodilator frequency

doubleitandgiveittothenextperson

24
Q

CF exacerbation admission: ABX monitoring

A
  • S/S persistence and/or resolution
  • Pulmonary function tests: FEV1 and FVC
  • Sputum culture and susceptibilities
  • BUN/SCr
  • ABX serum [ ]
    if no improvement in 5-7 days, re-culture and/or adjust ABX (
25
Q

CF exacerbation: MSSA infection ABX options

A

anti-staph penicillin OR cephalosporin 10-14D

26
Q

CF exacerbation: MSSA and PA infection ABX options

A

Aminoglycoside AND cefepime 10-14D

27
Q

CF exacerbation: MRSA infection ABX options

A

Vanco or linezolid 10-14D

28
Q

CF exacerbation: MRSA and PA infection ABX options

A
  • vanco or linezolid +
  • aminoglycoside +
  • beta-lactam (ceftazidime)
    for 10-14D
29
Q

Aminoglycoside goal [ ]

A
  • Traditional
    • Goal peak: 10-12 mcg/ml
    • Goal trough: <1.5 mcg/ml
  • Extended interval
    • Goal peak: 22.5-27.5 mcg/ml
    • Goal 18hr: <1mcg/ml
    • AUC 80-100 mcg/ml*hr
30
Q

Vanco goal [ ]

A
  • Goal trough: 10-20 mcg/mL (→ AUC/MIC >400)
  • Serum concentrations drawn initially and then every 3 – 7d
31
Q

CFTR modulator AE

A

Generally well tolerated, but….
- abdominal pain
- diarrhea
- rash
- rhinorrhea
- headache
- elevated ALT/AST

32
Q

How is ivacaftor dosed?

A
  • BID
  • strength dependent on age and weight
33
Q

How is lumacaftor/ivacaftor dosed

A
  • combo tab BID
  • strength dependent on age and weight
34
Q

How is tezacaftor/ivacaftor dosed

A
  • combo tab in AM, ivacaftor tab in PM
  • strength dependent on age and weight
35
Q

How is elexacaftor/tezacaftor/ivacaftor dosed?

A
  • combo tab in AM and ivacaftor in PM
  • strength dependent on age and weight
36
Q

CFTR modulator DDI with moderate CYP3A4 inhibitors

A

erythromycin and fluconazole
- iva: decrease to QD
- teza or tri: combo and iva taken on alternating days

37
Q

CFTR modulator DDI with strong CYP3A4 inhibitors

A

clarithromycin and itraconazole
- iva: decrease to twice weekly
- teza or tri: decrease to twice weekly
- luma: decrease dose to 1 tab QD for 1 week then return back to BID (only if luma is being added to strong CYP3A4, not if CYP3A4 being added to luma

38
Q

CFTR modulator DDI with CYP3A4 inducers

A

rifampi, CBZ, phenobarbital, phenytoin, St. John
- avoid CFTR use

39
Q

When to use ETI

A

First line, whenever applicable in whoever can

40
Q

When to use TI

A

In patients who have the appropriate mutation, can’t use ETI and are over 6

41
Q

When to use iva

A

if pt < 2 years but greater than 1 month old and has appropriate mutation; switch to ETI when they turn 2

42
Q

When to use LI

A

if pt < 2 years but greater than 1 year and is F508delta homo
* switch to ETI when they turn 2

43
Q

Important equations for CF ABX

there’s 3: Ke, C, and T1/2

A
  • Ke = ln(C1/C2) / ∆t
  • C = C0 (e^-kt)
  • T1/2 = 0.693/ Ke

AGS peak is 1 hr post infusion

44
Q

The patient develops an erythematous, pruritic rash on her face, neck, and upper torso, during
vancomycin infusion. What is it?

A

AnaphyLACTOID reaction - vanco flushing syndrome (mast cell mediated but not hypersensitivity)

45
Q

How to treat vanco anaphylactoid reaction?

A

Step 1: Stop vancomycin infusion
Step 2: Order diphenhydramine +/- H2RA +/- IV fluids (if patient hypotensive)
Most episodes will resolve within 20 minutes, and the vancomycin may be restarted at 50% of the original rate (mild cases)

46
Q

What do you do if Scr increases 50% while on abx therapy for APE?

A

Check tobramycin 18-hour level and vancomycin trough prior to next dose.
Hold doses pending level assessment.

PHM 702: Pulm and ToXXX (14 decks)

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