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What is the order of physiotherapy and inhaled therapies for CF?
inhaled bronchodilator (albuterol) - open airways
hypertonic saline - mobilize mucus
dornase alfa - thin mucus
chest physiotherapy - mobilize to increase clearance
inhaled antibiotics - decrease infection
hypertonic saline
HyperSal, PulmoSal
dornase alfa
Pulmozyme
tobramycin
TOBI, TOBI Podhaler
aztreonam
Cayston
azithromycin
Zithromax
pancrelipase
Creon, Viokace, Zenpep
ivacaftor
Malydeco
CFTR modulator. increases time that channels are open
NOT approved for F508del mutation
lumacaftor/ivacaftor
Okambi
corrects CFTR folding. increases CFTR at cell surface. also increases channel open time
approved for F508del mutation
tezacaftor/ivacaftor
Symdeko
corrects CFTR folding. increases CFTR at cell surface. also increases channel open time
approved for F508del mutation
elexacaftor/tezacaftor/ivacaftor
Trikafta
corrects CFTR folding. increases CFTR at cell surface. also increases channel open time
approved for F508del mutation
vanzacaftor/tezacaftor/deutivacaftor
Alyftrek
corrects CFTR folding. increases CFTR at cell surface. also increases channel open time
approved for F508del mutation