CF- Ivacaftor DI Flashcards
Ivacaftor brand name
Kalydeco
Ivacaftor MoA
Facilitates opening of the chloride channel
Is ivacaftor a CFTR potentiator or corrector?
Potentiator
What class CF mutations does ivacaftor work on?
Class III and Class IV
Ivacaftor indication
Patients ≥4 months with ≥1 of 97 ivacaftor-responsive CFTR mutations
CF Class III mutation
CFTR protein is created and gets to the cell surface, but the channel doesn’t open
CF Class IV mutation
Opening in the CFTR protein ion is faulty
Ivacaftor, along with all other CFTR modulators, must be taken with what?
High-fat meal
When to dose adjust for ivacaftor (and all CFTR modulators)
Moderate-severe hepatic dysfunction
Benefits of CFTR modulators in CF
Improve QoL, decrease APEs, decrease morbidity and mortality