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Kalydeco
ivacaftor
Adult Dosing .
Dosage forms: TAB: 150 mg
cystic fibrosis
- [150 mg PO q12h]
- Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
renal dosing
- [see below]
- CrCl >31: no adjustment; CrCl <31: not defined, caution advised
- HD/PD: not defined, caution advised
hepatic dosing
- [adjust dose frequency]
- Child-Pugh Class B: give usual dose q24h; Child-Pugh Class C: give usual dose q24h or less frequently
Peds Dosing .
- Dosage forms: TAB: 150 mg; GRANULE: 5.8 mg per packet, 13.4 mg per packet, 25 mg per packet, 50 mg per packet, 75 mg per packet
cystic fibrosis
- [1-2 mo, >3 kg]
- Dose: 5.8 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
- [2-3 mo, >3 kg]
- Dose: 13.4 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
- [4-5 mo, >5 kg]
- Dose: 25 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
- [6 mo-5 yo, 5-6.9 kg]
- Dose: 25 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
- [6 mo-5 yo, 7-13.9 kg]
- Dose: 50 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
- [6 mo-5 yo, >14 kg]
- Dose: 75 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
- [6 yo and older]
- Dose: 150 mg PO q12h; Info: for patients with ivacaftor-responsive CFTR gene mutations; give with fat-containing food
renal dosing
- [see below]
- CrCl >31: no adjustment; CrCl <31: not defined, caution advised
- HD/PD: not defined, caution advised
hepatic dosing
- [<6 mo]
- hepatic impairment: avoid use
- [6 mo and older]
- Child-Pugh Class B: give usual dose q24h; Child-Pugh Class C: give usual dose q24h or less frequently
Contraindications / Cautions .
- hepatic impairment (patients <6 mo)
- caution: hepatic impairment (patients 6 mo and older)
- caution: CrCl <31
- caution: elevated LFTs history
- caution: hypersensitivity to drug or ingredient
- caution: hypervitaminosis A
Drug Interactions .
Overview
ivacaftor
CFTR potentiator
- CYP3A4 substrate
- CYP2C9 inhibitor, weak
- CYP3A4 inhibitor, weak
- P-gp inhibitor, weak
- drug interactions apply only to Kalydeco (ivacaftor); see other ivacaftor products for potentially relevant interactions
Contraindicated
- cisapride
- colchicine
- eliglustat
Avoid/Use Alternative
- adagrasib
- alprazolam
- apalutamide
- apixaban
- aprepitant
- atazanavir
- avacopan
- berotralstat
- bosentan
- butalbital
- carbamazepine
- cenobamate
- ceritinib
- chloramphenicol
- clarithromycin
- clofazimine
- cobicistat
- codeine
- conivaptan
- crizotinib
- cyclosporine
- dabigatran
- dabrafenib
- danazol
- darunavir
- diltiazem
- dronedarone
- duvelisib
- efavirenz
- encorafenib
- ensartinib
- enzalutamide
- erythromycin
- etravirine
- fedratinib
- fexinidazole
- fluconazole
- fosamprenavir
- fosphenytoin
- grapefruit
- hydrocodone
- idelalisib
- imatinib
- isavuconazonium
- itraconazole
- ivosidenib
- ketoconazole
- lefamulin
- lenacapavir
- letermovir
- levoketoconazole
- lonafarnib
- lopinavir/ritonavir
- lorlatinib
- lumacaftor/ivacaftor
- mavacamten
- meperidine
- methadone
- mifepristone
- mitapivat
- mitotane
- modafinil
- nafcillin
- nefazodone
- nelfinavir
- neratinib
- netupitant
- nilotinib
- nirogacestat
- oxycodone
- pacritinib
- pentobarbital
- pexidartinib
- phenobarbital
- phenytoin
- pimozide
- posaconazole
- pralsetinib
- primidone
- propafenone
- relugolix
- repotrectinib
- ribociclib
- rifabutin
- rifampin
- rifapentine
- rilzabrutinib
- ritonavir
- rivaroxaban
- simvastatin
- sirolimus albumin-bound
- sotorasib
- St. John's wort
- tipranavir
- topotecan
- tucatinib
- venetoclax
- verapamil
- voriconazole
Monitor/Modify Tx
- abemaciclib
- acalabrutinib
- afatinib
- aficamten
- alfentanil
- amiodarone
- atorvastatin
- avanafil
- avapritinib
- brigatinib
- capivasertib
- celecoxib
- clozapine
- deuruxolitinib
- diclofenac
- digoxin
- disopyramide
- docetaxel
- dofetilide
- domperidone
- edoxaban
- enfortumab vedotin
- entrectinib
- eplerenone
- everolimus
- felodipine
- fentanyl
- finerenone
- flibanserin
- fluvastatin
- gepirone
- glimepiride
- glipizide
- ibrutinib
- ivabradine
- ixabepilone
- lemborexant
- lomitapide
- lovastatin
- lumateperone
- lurasidone
- lurbinectedin
- mavorixafor
- midazolam
- mobocertinib
- morphine
- nifedipine
- nimodipine
- nisoldipine
- oliceridine
- pemigatinib
- quinidine (antiarrhythmic)
- ranolazine
- red yeast rice
- selpercatinib
- sirolimus
- sufentanil
- tacrolimus
- tadalafil
- talazoparib
- taletrectinib
- temsirolimus
- tenofovir alafenamide
- tenofovir disoproxil
- tolvaptan
- torsemide
- triazolam
- ubrogepant
- vardenafil
- vinblastine
- vincristine
- vinorelbine
- warfarin
- zanubrutinib
Caution Advised
- armodafinil
- artemether/lumefantrine
- belzutifan
- bexarotene
- cariprazine
- clobazam
- cobimetinib
- cyclophosphamide
- danshen
- daridorexant
- darolutamide
- dexamethasone
- dicloxacillin
- dihydroergotamine
- echinacea
- elafibranor
- elagolix
- elinzanetant
- enasidenib
- ergotamine
- eslicarbazepine acetate
- felbamate
- garlic
- ginkgo
- ginseng, Asian
- glecaprevir
- glycerol phenylbutyrate
- griseofulvin
- meropenem
- methylergonovine
- naldemedine
- nevirapine
- odevixibat
- olutasidenib
- omaveloxolone
- oxcarbazepine
- palovarotene
- perampanel
- pibrentasvir
- pioglitazone
- prednisone
- roflumilast
- roflumilast topical
- rufinamide
- sarilumab
- sirolimus topical
- stiripentol
- sunvozertinib
- suzetrigine
- tazemetostat
- tecovirimat
- telotristat ethyl
- tocilizumab
- topiramate
- tovorafenib
- vaborbactam
- vamorolone
- vemurafenib
- vorasidenib
Adverse Reactions .
Serious Reactions
- hypersensitivity reaction
- anaphylaxis
- hepatic impairment
- intracranial HTN
- cataracts (peds patients)
Common Reactions
- headache
- oropharyngeal pain
- URI
- nasal congestion
- abdominal pain
- diarrhea
- rash
- nausea
- dizziness
- LFTs elevated
- glucose incr.
- arthralgia
- myalgia
- musculoskeletal chest pain
- pharyngeal erythema
- pleuritic pain
- wheezing
- acne
Safety/Monitoring .
Monitoring Parameters
LFTs before tx start, then q3mo x12mo, then q12mo, or if elevated ALT or AST history then consider monitoring more frequently; ophthalmic exam at baseline, then during tx if peds patient
Pregnancy/Lactation .
Pregnancy
Clinical Summary
weigh risk/benefit during pregnancy; inadequate human data available; no known risk of teratogenicity based on animal data at 5x and 11x MRHD
Lactation
Clinical Summary
weigh risk/benefit while breastfeeding; no human data available to assess risk of infant harm or effects on milk production
Pharmacology .
Metabolism: for ivacaftor: liver extensively; CYP450: 3A4/5 substrate; Info: active metabolite
Excretion: for ivacaftor: feces 87.8% (35% unchanged); Half-life: 12h
Subclass: Cystic Fibrosis
Mechanism of Action
for ivacaftor: potentiates mutant CFTR protein, increasing chloride channel open probability and chloride transport
Formulary .
No Formulary Selected
Manufacturer/Pricing .
Manufacturer: Vertex Pharmaceuticals, Inc.
DEA/FDA: Rx
Retail Price
Estimated prices only. For current pricing, visit GoodRx.
oral granule for reconstitution:
- 25 mg (1 carton, 56 packets): $24,384.00
- 50 mg (1 carton, 56 packets): $28,194.00
- 75 mg (1 carton, 56 packets): $28,677.00
oral tablet:
- 150 mg (1 carton, 56 tablets): $27,369.00
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