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PHM702 > Exam 2 - Cystic Fibrosis > Flashcards

Exam 2 - Cystic Fibrosis Flashcards

1
Q

Which class of CFTR mutation is most common?

A

Class II

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2
Q

What is the name of most common CFTR mutation?

A

F508del

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3
Q

What was the initial identified mutation for cystic fibrosis?

A

G551D

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4
Q

What is the age indication for ivacaftor (Kalydeco)?

A

1 month or more

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5
Q

What is the age indication for lumacaftor/ivacaftor (Orkambi)?

A

1 year or more

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6
Q

What is the age indication for tezacaftor/ivacaftor (Symdeko)?

A

6 years or more

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7
Q

What is the age indication for elexacaftor/tezacaftor/ivacaftor (Trikafta)?

A

2 years or more

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8
Q

What genotype must patients have to qualify for ivacaftor (Kalydeco)?

A

heterozygous

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9
Q

What genotype must patients have to qualify for lumacaftor/ivacaftor (Orkambi)?

A

homozygous

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10
Q

What genotype must patients have to qualify for tezacaftor/ivacaftor (Symdeko)?

A

homo or heterozygous

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11
Q

What genotype must patients have to qualify for elexacaftor/tezacaftor/ivacaftor (Trikafta)?

A

homo or heterozygous

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12
Q

What is the MOA of the ivacaftor component?

A

facilitates the opening of the chloride channel (potentiator)

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13
Q

What is the MOA of the lumacaftor, tezacaftor, and elexacaftor components?

A

chaperone that aids in trafficking of CFTR protein to cell membrane (corrector)

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14
Q

What are the AEs of CFTRs? (3)

A

GI, rash, increased ALT/AST

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15
Q

What are counseling points for CFTR modulators? (3)

A

take w/fat-containing meal, avoid grapefruit juice, dose reduction in hepatic dysfunction

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16
Q

Which drugs cause CYP3A4 inhibition? (4)

A

erythromycin, clarithromycin, fluconzaole, itraconazole

17
Q

Which drugs cause CYP3A4 induction? (5)

A

rifampin, carbamazepine, phenobarbital, phenytoin, St. John’s wort

18
Q

What are the physiotherapy treatments for cystic fibrosis? (4)

A

high frequency chest wall oscillation (“vest” therapy), postural drainage, positive expiratory pressure, exercise

19
Q

What are the traditional pharmacotherapeutic treatments for cystic fibrosis? (4)

A

bronchodilators (SABAs), topical mucolyics (dornase alfa), hydrating agents (hypertonic saline), anti-inflammatory agents (azithromycin)

20
Q

What are the AEs of dornase alfa? (2)

A

voice hoarseness, rash

21
Q

What is the dosing frequency for azithromycin in cystic fibrosis?

A

tiw

22
Q

What are the treatments for pseudomonas infections in cystic fibrosis? (2)

A

tobramycin (TOBI as nebulized solution or dry powder device), aztreonam (inhaled)

23
Q

What is the dosing frequency for tobramycin in cystic fibrosis?

A

28 days on and 28 days off

24
Q

What are the AEs of tobramycin? (2)

A

voice alteration, tinnitus

25
Q

What are the AEs of aztreonam?

A

bronchospasm (pre-treat with SABA)

26
Q

What is the treatment for an acute exacerbation in cystic fibrosis with MSSA and no PA?

A

anti-staphylococcal penicillin or cephalosporin

27
Q

What is the treatment for an acute exacerbation in cystic fibrosis with MSSA and PA?

A

cefepime plus aminoglycoside

28
Q

What is the treatment for an acute exacerbation in cystic fibrosis with MRSA and no PA?

A

vancomycin or linezolid

29
Q

What is the treatment for an acute exacerbation in cystic fibrosis with MRSA and PA?

A

vancomycin or linezolid AND aminoglycoside plus beta-lactam (ceftazidime)

30
Q

What type of dosing is recommended for antibiotics in acute exacerbations in cystic fibrosis?

A

once-daily (extended-interval)

31
Q

What is the dosing duration for antibiotics in acute exacerbations in cystic fibrosis?

A

10-14 days

32
Q

What are monitoring parameters for antibiotics used in acute exacerbations of cystic fibrosis? (4)

A

symptom persistence/resolution, pulmonary function tests (FEV1, FVC), sputum culture and susceptibilities, BUN/SCr

33
Q

What are the goals for peak and trough in traditional dosing of aminoglycosides?

A

peak = 10-12, trough = <1.5 mcg/mL

34
Q

What are the goals for peak and trough in extended-interval dosing of aminoglycosides?

A

peak = 22.5-27.5, trough = <0.1 mcg/mL

35
Q

What are the goals for trough and AUC/MIC ratio for vancomycin?

A

trough = 10-20 mcg/mL, AUC/MIC = >/=400

36
Q

What is the age indication for azithromycin and or tobramycin treatment in cystic fibrosis?

A

6 plus

PHM702 (19 decks)

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