Cystic Fibrosis Pharmacology Flashcards
(51 cards)
What are the five types of drugs we use in CF patients?
-CFTR modulators
-antibiotics
-mucociliary clearance agents
-bronchodilators
-anti-inflammatory agents
Which drugs do we use as anti-inflammatory agents in CF?
-azithromycin
-high dose ibuprofen
Which drugs do we use as mucociliary clearance agents in CF?
-dornase alfa
-hypertonic saline
-mannitol (inhaled)
What are the advantages of inhaled medications in CF?
-direct delivery of med to target site
-rapid onset
-minimal systemic exposure
What are the disadvantages of inhaled medications in CF?
-variable lung deposition due to airway obstruction
What is the indication for Ivacaftor?
-age ≥ 1 month with ≥ 1 responsive mutation (class III/IV)
What is the indication for Lumacaftor/Ivacaftor?
-age ≥ 1 year with 2 copies of F508del mutation (homozygous)
What is the indication for Tezacaftor/Ivacaftor?
-age ≥ 6 years with 2 copies of F508del mutation or ≥ 1 responsive mutation
What is the indication for Elexacaftor/Tezacaftor/Ivacaftor?
-age ≥ 2 years with ≥ 1 copy of F508del mutation or ≥ 1 responsive mutation
What is the indication for Vanzacaftor/Tezacaftor/Deutivacaftor?
-age ≥ 6 years with ≥ 1 copy of F508del mutation or ≥ 1 responsive mutation
Which CFTR modulators are potentiors?
-ivacaftor
-deutivacaftor
Which CFTR modulators are correctors?
-lumacaftor
-tezacaftor
-elexacaftor
-vanzacaftor
Which of the following CFTR drugs cause a synergistic effect? SATA
A. Lumacaftor/Ivacaftor
B. Vanzacaftor/Tezacaftor/Deutivacaftor
C. Ivacaftor
D. Tezacaftor/Ivacaftor
A, B, D
How should patients be counseled to take CFTR modulators?
-take with fat-containing food (to increase absorption)
Which enzyme are CFTR modulators extensively metabolized by?
-CYP3A4/5 (so look out for DDIs!);
exception-Lumacaftor
True or False: Patients with hepatic impairment require dose adjustment of CFTR modulators
True
Which drugs are moderate CYP3A inhibitors?
-fluconazole
-erythromycin
-grapefruit juice
If a patient is taking grapefruit juice and Lumacaftor/Ivacaftor, does a dose adjustment need to be made, and if so would you increase or decrease the dose?
no change needed, AUC does not change
If a patient is taking grapefruit juice and Ivacaftor, does a dose adjustment need to be made, and if so would you increase or decrease the dose?
yes, decrease the dose
Which drugs are strong CYP3A inhibitors?
-azoles (keto, itra, posa, vori)
-clarithryomycin
Do we use CFTR modulators with CYP3A inducers?
NO WE DO NOT. AVOID CONCOMITANT USE
(except Lumacaftor/Ivacaftor)
What are some ADR of CFTR modulators?
-elevated AST/ALT and bilirubin
-risk for cataracts
Which CFTR modulator has a CI for moderate liver disease?
Alyfrtrek (vanzo/teza/deut)
How do you monitor patients after putting them on CFTR modulators?
- AST/ALT/bilirubin at baseline
- AST/ALT/bilirubin monthly (6 months)
- AST/ALT/bilirubin every 3 months for 1 year
- AST/ALT/bilirubin annually thereafter
- Opthalmological exams at baseline and during treatment
