Cystic Fibrosis Flashcards
dx, s/sx, complications, tx, infections
what is the order of therapy initiation in CF
inhaled bronchodilators
hypertonic saline
dornase alfa
chest physiotherapy
inhaled ABX
hypertonic saline MOA in CF
mobilizes mucous and increases airway clearence
dornase alfa MOA in CF
decreases mucus viscosity
what pathogens typically cause infxn in CF
pseud!
s aureus
h inf
how is dosing of ABX different in pseud? why?
doses are usually higher to account for altered kinetics in CF patients, penetrate lung tissue and account for resistance
what ABX are common in CF exacerbations
tobramycin
aztreonam
azithromycin
bronchodilators and mucolytics should be given before or after ABX in CF?
before!
tobramycin dosing
TOBI (soln for neb) = 300mg neb Q12H
TOBI Podhaler (cap for neb) = 112mg (4x28mg caps) Q12H
28d on and 28d off cycle
side effects of tobramycin to warn patients about
tinnitus
ototoxicity
voice alteration
dizziness
mouth and throat pain
if a patient does not want to wake up in the night to take a second dose of tobramycin, what is the soonest they can take the second dose?
6 hours apart
tobramycin is for use in _____ yo
6+ yo
aztreonam dosing CF
75mg neb TID
28d on and 28d off cycle
if a patient does not want to wake up in the night to take an aztreonam 75mg dose via neb, when is the soonest they can take it after the previous dose?
4 hours apart
aztreonam is for use in ____ yo
7+
aztreonam is stable at room temp for ____
28 days
side effects to warn patients about with aztreonam use
serious allergic reaction, bronchospasm, fever, wheezing, cough, chest discomfort
azithromycin dosing in CF
<40kg: 250mg TIW
40+kg: 500mg TIW
what is the function of azithromycin in CF
has no bacteriocidal activity in CF
disrupts biofilm formation by bacteria which can improve lung function and decrease exacerbations
patient is a 6 yo male (22.7kg, 50lb) with a new CF dx. What should the patient be started on?
CF is heterozygous for the F508del mutation
albuterol prn
candidate for Trikafta
should initiate pancreatic enzymes (patient is over 4 yo so should receive 500U lipase/kg/meal and 50% of that as snacks)
mealtime dose = 11,350 U lipase
snack dose = 5,675 U lipase
round depending on brand used
if a patient is already on albuterol and hypertonic saline, what is the next recommended addition to therapy
dornase alfa
dornase alfa
brand
dosing
MOA
Pulmozyme
2.5mg daily w neb
decreases mucus viscocity
pancreatic enzyme dosing
<4 yo: 1,000 U lipase/kg/meal
4+ yo: 500 U lipase/kg/meal
what is the max dose of lipase per meal? per day?
max 2,500 U/kg/meal
max 10,000 U/kg/day
pearls for pancreatic enzyme admin
can sprinkle cap
keep good fluid intake
brands are not interchangeable
no dairy
CFTR modulators for homozygous F508del
Orkambi (lmacaftor/ivacaftor)
Syndeko (tezacaftor/ivacaftor)
CFTR modulator for heterozygous F508del
Trikafta (elexacaftor/tezacaftor/ivacaftor)
what are the diet concerns and or recommendations in CF
high fat and high calorie diet
ADEK supplements needed
may require insulin for CF-related DM
