CF- Pulmonary Manifestations and Treatments

Question 1

Physiotherapy options for CF

Answer 1

Vest therapy, postural damage (PD), positive expiratory pressure (PEP)/oscillatory PEP, exercise

Question 2

Annual vaccination for CF patients

Answer 2

Yearly flu vaccine starting at 6 months

Question 3

CF pulmonary meds: bronchodilators

Answer 3

SABAs (albuterol) preferred over LABAs (salmeterol)

Question 4

CF pulmonary meds: topical mucolytic/hydratting agents

Answer 4

Dornase alfa, hypertonic saline

Question 5

Dornase alfa dosing

Answer 5

2.5mg INH BID

Question 6

Hypertonic saline dosing

Answer 6

7% INH BID

Question 7

Dornase alfa ADEs

Answer 7

May have voice hoarseness, rash

Question 8

Hypertonic saline ADEs

Answer 8

May have bronchospasm

Question 9

CF pulmonary med: anti-inflammatory med

Answer 9

Azithromycin

Question 10

Azithromycin dosing

Answer 10

250mg PO MWF for patients <40kg
500mg PO MWF for patients ≥40kg

Question 11

Azithromycin ADEs

Answer 11

Nausea, diarrhea, wheezing, possible ABX resistance

Question 12

CF pulmonary meds: inhaled ABX

Answer 12

Tobramycin, aztreonam

Question 13

Tobramycin dosing

Answer 13

TOBI 300mg INH BID 28 days on, then 28 days off administered over 15 minutes

TOBI PodHaler 112mg (4, 28mg capsules) INH BID, 28 days on, 28 days off administered over 2-7 minutes

Question 14

Tobramycin ADEs

Answer 14

May have voice alteration, tinnitus

Question 15

Aztreonam dosing

Answer 15

75mg INH BID 28 days on, 28 days off administered over 2-3 minutes

Question 16

Aztreonam ADEs

Answer 16

May have bronchospasm, pre-treat with albuterol