Cystic Fibrosis Flashcards
What is the minimum age for ivacaftor?
1 month
What is the minimum age for lumacaftor/ivacaftor?
1 year
What is the minimum age for tezacaftor/ivacaftor?
6 years
What is the minimum age for elexacaftor/tezacaftor/ivacaftor?
2 years
What is the main mutation that ivacaftor is indicated for?
G551D
T/F: Ivacaftor can be used to treat heterozygous F508del
FALSE: it can be used in these patients but it will not help the mutation
Which mutation is lumacaftor/ivacaftor approved for?
Homozygous F508del
Which mutation(s) is tezacaftor/ivacaftor approved for?
Homozygous F508del OR a CFTR mutation that is responsive to T-I
Which mutation(s) is elexacaftor/tezacaftor/izacaftor approved for?
At least 1 copy of F508del or a mutation that is responsive to T-I
What are possible side effects of CFTR modulators?
- Abdominal pain
- Diarrhea
- Rash
- Increased ALT/AST
- Increased blood creatine phosphokinase
- Rhinorrhea
- Headache
What should be monitored on CFTR modulator therapy?
- Baseline LFTs
- LFTS every 3 months for the first year, then annually
CFTR modulator pearls
- Take with fat containing meal
- Adjust dose with moderate-severe liver impairment
- Avoid grapefruit (CYP3A)
Which drugs have moderate CYP3A inhibition interactions with CFTR modulators?
Erythromycin, Fluconazole
How should ivacaftor dosing be changed with moderate CYP3A inhibition?
Once daily dosing
How should tezacaftor/elexacaftor dosing be changed with moderate CYP3A inhibition?
Take combination every other day alternating with ivacaftor alone
