Therapeutics - Cystic Fibrosis Part 2 Flashcards
(23 cards)
name the 4 CFTR modulators
ivacaftor
lumacaftor/ivacaftor
tezacaftor/ivacaftor
elexacaftor/texacaftor/ivacaftor
corrector vs potentiator vs amplifiere
corrector - improves the QUALITY of CFTR protein
potentiator - improves the OPENING OF THE CHANNEL
amplifier - increase AMOUNT of CFTR mRNA and protein in the cell
some patients may have MORE THAN ONE mutation in CFTR
what does this mean for disease modifying therapy
may need combo therapy
ivacaftor is a……
potentiator, corrector, or amplifier
how does it work
potentiator
enhances the activity of the protein at the plasma membrane
ivacaftor is approved in patients how young
1 month and older (very young!)
true or false
the disease modifiers actually decrease mortality
true
*in general for ALL MODULATORS – how should they ben taken? when should dose be reduced?
TAKE WITH HIGH FAT FOODS
reduce dose when giving with a strong CYP3A4 Inhibitor or in patients with HEPATIC IMPAIRMENT
once mixed with food, how long is ivacaftor stable
only like 1 hr - try to eat right away
if a dose of ivacaftor is missed, skip if it has been longer than….
6 hours
name some AE of ivacaftor
dizzy
skin issues
ND
hypo/hyperglycemia
increased LFT ((HEPATIC)
cataracs
explain how lumacaftor + ivacaftor work together
lumacaftor is a corrector - chaperone that corrects the misfolding. however, the gate still isn’t open long enough
giving ifacaftor too (potentiator) augments the gating function and increases ion conductance
correct misfolding + increase channel opening to allow chloride to get out
F508 delta mutation ON 2 ALLELES uses this drug….
lumacaftor + ivacafator
or
tezacaftor + ivacaftor
how young is lumacaftor + ivacaftor approved
1 year and up
(just ivacaftor was 1 month and up_
name some AE of lumacaftor + ivacaftor
chest tightness
increased LFT
monitor!
advantage of tezacaftor + ivacaftor over lumacaftor + ivacaftor
less chest tightness and less DDI
texacaftor is a ——-
corrector
state what each is:
elexacaftor + tezacaftor + ivacaftor
elexacaftor and tezacaftor are correctors
ivacatftor is a potentiator
why are elexacaftor and tezacaftor used together even tho both are correctors
they bind different sites
indication for trikafta (elexacaftor + tezacaftor + ivacaftor)
2 years and older with at least 1 copy of the 508del
how often is trikafta administered vs alyftrek
trikafta - BID
alyftrek - QD
noninferior to each other, by alyftrek has an advnatage in that its QD
indication for alyftrek (vanzacaftor + tezacaftor + deutivacaftor)
6 years and older
F508del mutation
true or false
CFTR therapy is continues for life
TRUE
if you stop - abnormalities return
doesnt permanently fix
