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Ivacaftor: Drug information

Ivacaftor: Drug information
(For additional information see "Ivacaftor: Pediatric drug information" and see "Ivacaftor: Patient drug information")

For abbreviations, symbols, and age group definitions used in Lexicomp (show table)
Brand Names: US
  • Kalydeco
Brand Names: Canada
  • Kalydeco
Pharmacologic Category
  • Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
Dosing: Adult
Cystic fibrosis

Cystic fibrosis: Oral: Tablet: 150 mg every 12 hours

Missed dose: If dose is missed within 6 hours of the usual time it is taken, take the dose as soon as possible; otherwise, skip the missed dose and resume the normal dosing schedule.

Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.

Dosing: Kidney Impairment: Adult

CrCl >30 mL/minute: No dosage adjustment necessary (has not been studied).

CrCl ≤30 mL/minute: There are no dosage adjustments provided in the manufacturer's labeling (has not been studied); use with caution.

End-stage renal disease (ESRD): There are no dosage adjustments provided in the manufacturer's labeling (has not been studied); use with caution.

Dosing: Hepatic Impairment: Adult

Mild impairment (Child-Pugh class A): No dosage adjustment necessary.

Moderate impairment (Child-Pugh class B): 150 mg once daily

Severe impairment (Child-Pugh class C): Has not been studied; use with caution: 150 mg once daily or less frequently

Dosing: Pediatric

(For additional information see "Ivacaftor: Pediatric drug information")

Cystic fibrosis

Cystic fibrosis: Oral:

Infants 4 to <6 months weighing ≥5 kg: Oral granules: 25 mg granule packet every 12 hours.

Infants ≥6 months and Children <6 years: Oral granules:

5 to <7 kg: 25 mg granule packet every 12 hours.

7 to <14 kg: 50 mg granule packet every 12 hours.

≥14 kg: 75 mg granule packet every 12 hours.

Children ≥6 years and Adolescents: Oral tablet: 150 mg every 12 hours.

Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.

Dosing: Kidney Impairment: Pediatric

Infants ≥4 months, Children, and Adolescents:

CrCl >30 mL/minute: No dosage adjustment necessary.

CrCl ≤30 mL/minute: There are no dosage adjustments provided in manufacturer's labeling (not studied); use with caution.

End-stage renal disease (ESRD): There are no dosage adjustments provided in manufacturer's labeling (not studied); use with caution.

Dosing: Hepatic Impairment: Pediatric

Infants 4 to <6 months: Any degree of hepatic impairment: Use is not recommended.

Infants ≥6 months, Children, and Adolescents:

Baseline:

Mild impairment (Child-Pugh class A): No dosage adjustment necessary.

Moderate impairment (Child-Pugh class B): Oral: Administer usual dose once daily.

Severe impairment (Child-Pugh class C): Oral: Administer usual dose once daily or less frequently; use with caution.

Hepatotoxicity during therapy: If ALT or AST >5 times ULN: Hold ivacaftor; may resume if elevated transaminases resolved and after assessing benefits vs risks of continued treatment.

Dosing: Adjustment for Toxicity: Adult

ALT or AST >5 times ULN: Hold ivacaftor; may resume if elevated transaminases resolved and after assessing benefits vs risks of continued treatment.

Dosage Forms: US

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Packet, Oral:

Kalydeco: 25 mg (56 ea); 50 mg (56 ea); 75 mg (56 ea)

Tablet, Oral:

Kalydeco: 150 mg [contains fd&c blue #2 (indigotine)]

Generic Equivalent Available: US

No

Dosage Forms: Canada

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Packet, Oral:

Kalydeco: 25 mg (56 ea); 50 mg (56 ea); 75 mg (56 ea)

Tablet, Oral:

Kalydeco: 150 mg [contains fd&c blue #2 (indigo carm) aluminum lake]

Prescribing and Access Restrictions

Product is only available via authorized pharmacies and distributors. Further information is available at https://www.vrtx.com/authorized-distributors.

Administration: Adult

Oral: Administer before or after high-fat-containing foods (eg, butter, cheese pizza, eggs, peanut butter, whole-milk dairy products [eg, whole milk, cheese, yogurt]).

Granules: Mix entire packet of granules with 5 mL of soft food (eg, pureed fruits [excluding grapefruit] or vegetables, yogurt, applesauce) or liquid (eg, water, milk, juice [excluding grapefruit juice]); food or liquid should be at or below room temperature. Granule mixture should be completely consumed within 1 hour.

Administration: Pediatric

Oral: Granules, tablets: Administer before or after high-fat-containing foods (eg, butter, cheese pizza, eggs, peanut butter, whole milk dairy products [eg, whole milk, cheese, yogurt]). If a missed dose is identified within 6 hours, the patient should take the dose with fat-containing food. If more than 6 hours elapsed after the usual dosing time, the patient should skip that dose and resume the normal schedule for the following dose. A double dose should not be taken to make up for the forgotten dose.

Granules: Mix entire packet of granules with a teaspoon of soft food (eg, pureed fruits [excluding grapefruit or Seville oranges] or vegetables, yogurt, applesauce) or 5 mL of liquid (eg, water, milk, breast milk, infant formula, juice [excluding grapefruit or Seville oranges]); food or liquid should be at or below room temperature. Granule mixture should be completely consumed within 1 hour.

Use: Labeled Indications

Cystic fibrosis: Treatment of cystic fibrosis (CF) in patients ≥4 months of age who have one mutation in the CF transmembrane conductance regulator (CFTR) gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.

If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of a CFTR mutation followed by verification with bidirectional sequencing when recommended by the mutation test instructions for use.

Adverse Reactions

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.

>10%:

Central nervous system: Headache (24%)

Dermatologic: Skin rash (13%)

Gastrointestinal: Abdominal pain (16%), diarrhea (13%), nausea (12%)

Respiratory: Oropharyngeal pain (22%), upper respiratory tract infection (22%), nasal congestion (20%), nasopharyngitis (15%)

1% to 10%:

Central nervous system: Dizziness (9%)

Dermatologic: Acne vulgaris (4% to 7%)

Endocrine & metabolic: Increased serum glucose (4% to 7%)

Hepatic: Increased liver enzymes (4% to 7%), increased serum aspartate aminotransferase (4% to 7%)

Infection: Bacterial infection (sputum: 4% to 7%)

Neuromuscular & skeletal: Arthralgia (4% to 7%), musculoskeletal chest pain (4% to 7%), myalgia (4% to 7%)

Respiratory: Paranasal sinus congestion (4% to 7%), pharyngeal erythema (4% to 7%), pleuritic chest pain (4% to 7%), rhinitis (4% to 7%), sinus headache (4% to 7%), wheezing (4% to 7%)

Frequency not defined: Endocrine & metabolic: Hypoglycemia

<1%, postmarketing, and/or case reports: Cataract (children)

Contraindications

There are no contraindications listed in the manufacturer's labeling.

Canadian labeling: Hypersensitivity to ivacaftor or any component of the formulation.

Warnings/Precautions

Concerns related to adverse effects:

• Cataracts: Noncongenital lens opacities and cataracts have been reported in pediatric patients treated with ivacaftor; other risk factors were present in some cases (eg, corticosteroid use, exposure to radiation), but a possible risk related to ivacaftor cannot be excluded. Baseline and follow-up ophthalmological examinations are recommended in pediatric patients.

• CNS effects: May cause dizziness, which may impair physical or mental abilities; patients must be cautioned about performing tasks that require mental alertness (eg, operating machinery or driving).

• Hepatic effects: May increase hepatic transaminases. Monitor liver function; increased monitoring may be necessary in patients with a history of elevated hepatic transaminases. Temporarily discontinue treatment if ALT or AST >5 times ULN. Following resolution of transaminase elevations, consider the benefits and risks of resuming therapy.

Disease-related concerns:

• Hepatic impairment: Use with caution in patients with hepatic impairment; dosage adjustment recommended in patients with moderate to severe (Child-Pugh class B or C) impairment.

• Renal impairment: Use with caution in patients with severe renal impairment (CrCl ≤30 mL/minute) or ESRD.

Metabolism/Transport Effects

Substrate of CYP3A4 (major); Note: Assignment of Major/Minor substrate status based on clinically relevant drug interaction potential; Inhibits CYP3A4 (weak), P-glycoprotein/ABCB1

Drug Interactions

Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.

Afatinib: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Afatinib. Management: If combined, administer the P-gp inhibitor simultaneously with, or after, the dose of afatinib. Monitor closely for signs and symptoms of afatinib toxicity and if the combination is not tolerated, reduce the afatinib dose by 10 mg. Risk D: Consider therapy modification

Aliskiren: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Aliskiren. Risk C: Monitor therapy

ALPRAZolam: CYP3A4 Inhibitors (Weak) may increase the serum concentration of ALPRAZolam. Risk C: Monitor therapy

Berotralstat: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Berotralstat. Management: Decrease the berotralstat dose to 110 mg daily when combined with P-glycoprotein (P-gp) inhibitors. Risk D: Consider therapy modification

Bilastine: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Bilastine. Risk X: Avoid combination

Bitter Orange: May increase the serum concentration of Ivacaftor. Risk X: Avoid combination

Celiprolol: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Celiprolol. Risk C: Monitor therapy

Clofazimine: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Risk C: Monitor therapy

Colchicine: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Colchicine. Colchicine distribution into certain tissues (e.g., brain) may also be increased. Management: Colchicine is contraindicated in patients with impaired renal or hepatic function who are also receiving a P-gp inhibitor. In those with normal renal and hepatic function, reduce colchicine dose as directed. See interaction monograph for details. Risk D: Consider therapy modification

CycloSPORINE (Systemic): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of CycloSPORINE (Systemic). Risk C: Monitor therapy

CycloSPORINE (Systemic): CYP3A4 Inhibitors (Weak) may increase the serum concentration of CycloSPORINE (Systemic). Risk C: Monitor therapy

CYP3A4 Inducers (Moderate): May decrease the serum concentration of Ivacaftor. Risk C: Monitor therapy

CYP3A4 Inducers (Strong): May decrease the serum concentration of Ivacaftor. Risk X: Avoid combination

CYP3A4 Inhibitors (Moderate): May increase the serum concentration of Ivacaftor. Management: Ivacaftor dose reductions may be required; consult full drug interaction monograph content for age- and weight-specific dosage recommendations. Risk D: Consider therapy modification

CYP3A4 Inhibitors (Strong): May increase the serum concentration of Ivacaftor. Management: Ivacaftor dose reductions are required; consult full drug interaction monograph content for age- and weight-specific recommendations. Risk D: Consider therapy modification

Dabigatran Etexilate: P-glycoprotein/ABCB1 Inhibitors may increase serum concentrations of the active metabolite(s) of Dabigatran Etexilate. Risk C: Monitor therapy

Digoxin: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Digoxin. Management: Measure digoxin serum concentrations before initiating treatment with these P-glycoprotein (P-gp) inhibitors. Reduce digoxin concentrations by either reducing the digoxin dose by 15% to 30% or by modifying the dosing frequency. Risk D: Consider therapy modification

Dofetilide: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Dofetilide. Risk C: Monitor therapy

DOXOrubicin (Conventional): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of DOXOrubicin (Conventional). Risk X: Avoid combination

DOXOrubicin (Liposomal): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of DOXOrubicin (Liposomal). Risk C: Monitor therapy

Edoxaban: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Edoxaban. Risk C: Monitor therapy

Etoposide: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Etoposide. Risk C: Monitor therapy

Etoposide Phosphate: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Etoposide Phosphate. Risk C: Monitor therapy

Everolimus: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Everolimus. Risk C: Monitor therapy

Fexinidazole: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Risk X: Avoid combination

Finerenone: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Finerenone. Risk C: Monitor therapy

Flibanserin: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Flibanserin. Risk C: Monitor therapy

Fusidic Acid (Systemic): May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Risk X: Avoid combination

Glecaprevir and Pibrentasvir: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Glecaprevir and Pibrentasvir. Risk C: Monitor therapy

Grapefruit Juice: May increase the serum concentration of Ivacaftor. Risk X: Avoid combination

Ixabepilone: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Ixabepilone. Risk C: Monitor therapy

Lapatinib: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Lapatinib. Risk C: Monitor therapy

Larotrectinib: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Larotrectinib. Risk C: Monitor therapy

Lefamulin: May increase the serum concentration of Ivacaftor. Ivacaftor may increase the serum concentration of Lefamulin. Management: Consider alternatives to this combination when possible. If combined, monitor for increased lefamulin toxicities and decrease the ivacaftor dose. See full monograph for details. Risk D: Consider therapy modification

Lemborexant: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Lemborexant. Management: The maximum recommended dosage of lemborexant is 5 mg, no more than once per night, when coadministered with weak CYP3A4 inhibitors. Risk D: Consider therapy modification

Lomitapide: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Lomitapide. Management: Patients on lomitapide 5 mg/day may continue that dose. Patients taking lomitapide 10 mg/day or more should decrease the lomitapide dose by half. The lomitapide dose may then be titrated up to a max adult dose of 30 mg/day. Risk D: Consider therapy modification

Lonafarnib: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Lonafarnib. Management: Avoid concurrent use of lonafarnib with weak CYP3A4 inhibitors. If concurrent use is unavoidable, reduce the lonafarnib dose to or continue at a dose of 115 mg/square meter. Monitor for evidence of arrhythmia, syncope, palpitations, or similar effects. Risk D: Consider therapy modification

Midazolam: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Midazolam. Risk C: Monitor therapy

Morphine (Systemic): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Morphine (Systemic). Risk C: Monitor therapy

Nadolol: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Nadolol. Risk C: Monitor therapy

Naldemedine: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Naldemedine. Risk C: Monitor therapy

Naloxegol: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Naloxegol. Risk C: Monitor therapy

NiMODipine: CYP3A4 Inhibitors (Weak) may increase the serum concentration of NiMODipine. Risk C: Monitor therapy

PAZOPanib: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of PAZOPanib. Risk X: Avoid combination

Pimozide: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Pimozide. Risk X: Avoid combination

Ranolazine: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Ranolazine. Risk C: Monitor therapy

Relugolix: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Relugolix. Management: Avoid coadministration of relugolix with oral P-gp inhibitors whenever possible. If combined, take relugolix at least 6 hours prior to the P-gp inhibitor and monitor patients more frequently for adverse reactions. Risk D: Consider therapy modification

Relugolix, Estradiol, and Norethindrone: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Relugolix, Estradiol, and Norethindrone. Management: Avoid use of relugolix/estradiol/norethindrone with P-glycoprotein (P-gp) inhibitors. If concomitant use is unavoidable, relugolix/estradiol/norethindrone should be administered at least 6 hours before the P-gp inhibitor. Risk D: Consider therapy modification

Rifabutin: May decrease the serum concentration of Ivacaftor. Risk C: Monitor therapy

RifAXIMin: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of RifAXIMin. Risk C: Monitor therapy

Rimegepant: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Rimegepant. Management: Avoid administration of another dose of rimegepant within 48 hours if given concomitantly with a P-glycoprotein (P-gp) inhibitor. Risk D: Consider therapy modification

RisperiDONE: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of RisperiDONE. Risk C: Monitor therapy

RomiDEPsin: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of RomiDEPsin. Risk C: Monitor therapy

Silodosin: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Silodosin. Risk C: Monitor therapy

Simvastatin: CYP3A4 Inhibitors (Weak) may increase serum concentrations of the active metabolite(s) of Simvastatin. CYP3A4 Inhibitors (Weak) may increase the serum concentration of Simvastatin. Risk C: Monitor therapy

Sirolimus (Conventional): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Sirolimus (Conventional). Management: Avoid concurrent use of sirolimus with P-glycoprotein (P-gp) inhibitors when possible and alternative agents with lesser interaction potential with sirolimus should be considered. Monitor for increased sirolimus concentrations/toxicity if combined. Risk D: Consider therapy modification

Sirolimus (Protein Bound): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Sirolimus (Protein Bound). Risk X: Avoid combination

St John's Wort: May decrease the serum concentration of Ivacaftor. Risk X: Avoid combination

Tacrolimus (Systemic): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Tacrolimus (Systemic). Risk C: Monitor therapy

Tacrolimus (Systemic): CYP3A4 Inhibitors (Weak) may increase the serum concentration of Tacrolimus (Systemic). Risk C: Monitor therapy

Talazoparib: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Talazoparib. Risk C: Monitor therapy

Tegaserod: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Tegaserod. Risk C: Monitor therapy

Teniposide: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Teniposide. Risk C: Monitor therapy

Tenofovir Disoproxil Fumarate: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Tenofovir Disoproxil Fumarate. Risk C: Monitor therapy

Topotecan: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Topotecan. Risk X: Avoid combination

Triazolam: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Triazolam. Risk C: Monitor therapy

Ubrogepant: CYP3A4 Inhibitors (Weak) may increase the serum concentration of Ubrogepant. Management: In patients taking weak CYP3A4 inhibitors, the initial and second dose (given at least 2 hours later if needed) of ubrogepant should be limited to 50 mg. Risk D: Consider therapy modification

Ubrogepant: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Ubrogepant. Management: Use an initial ubrogepant dose of 50 mg and second dose (at least 2 hours later if needed) of 50 mg when used with a P-gp inhibitor. Risk D: Consider therapy modification

Venetoclax: P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of Venetoclax. Management: Reduce the venetoclax dose by at least 50% in patients requiring concomitant treatment with P-glycoprotein (P-gp) inhibitors. Resume the previous venetoclax dose 2 to 3 days after discontinuation of a P-gp inhibitor. Risk D: Consider therapy modification

VinCRIStine (Liposomal): P-glycoprotein/ABCB1 Inhibitors may increase the serum concentration of VinCRIStine (Liposomal). Risk X: Avoid combination

Food Interactions

Ivacaftor serum concentrations may be increased when taken with grapefruit. Management: Avoid concurrent use.

Pregnancy Considerations

Ivacaftor crosses the placenta (Trimble 2018).

In one case report, cord blood concentrations of ivacaftor at delivery were similar to maternal plasma concentrations following maternal use of ivacaftor/lumacaftor during pregnancy (Trimble 2018). Information related to ivacaftor use in pregnancy is limited (Heltshe 2017; Kaminski 2016; Ladores 2017; Marco 2015; Trimble 2018).

Breastfeeding Considerations

Ivacaftor is present in breast milk (Trimble 2018).

In one case report, ivacaftor was detectable in breast milk and infant plasma following maternal use of ivacaftor/lumacaftor during pregnancy and while breastfeeding (Trimble 2018).

According to the manufacturer, the decision to continue or discontinue breastfeeding during therapy should take into account the risk of infant exposure, the benefits of breastfeeding to the infant, and benefits of treatment to the mother.

Dietary Considerations

Avoid grapefruit.

Monitoring Parameters

CF mutation test (prior to therapy initiation if mutation status unknown); ALT/AST at baseline, every 3 months for 1 year, then annually thereafter or as clinically indicated (consider more frequent monitoring in patients with a history of elevated hepatic transaminases); FEV1; baseline and follow-up ophthalmological exams in pediatric patients.

Mechanism of Action

Potentiates epithelial cell chloride ion transport of defective cell-surface CFTR protein thereby improving the regulation of salt and water absorption and secretion in various tissues (eg, lung, GI tract).

Pharmacokinetics

Onset of action: FEV1 increased, sweat chloride decreased within ~2 weeks (Ramsey 2011)

Absorption: Variable; increased (by 2.5- to 4-fold) with fatty foods

Distribution: Vd: 353 L ± 122 L (fed state)

Protein binding: ~99%; primarily to alpha1 acid glycoprotein, albumin

Metabolism: Hepatic; extensive via CYP3A; forms 2 major metabolites (M1 [active; 1/6 potency] and M6 [inactive])

Half-life elimination: ~12 hours

Time to peak: Median: ~4 hours (range: 3 to 6 hours) (fed state)

Excretion: Feces (87.8%, ~65% of administered dose as metabolites); urine (minimal, as unchanged drug)

Pharmacokinetics: Additional Considerations

Hepatic function impairment: Adults with moderate hepatic impairment (Child-Pugh class B) had an ~2-fold increase in ivacaftor AUC0-∞; in adults with mild hepatic impairment (Child-Pugh class A), increase is expected to be <2-fold. The magnitude of increase in exposure in patients with severe hepatic impairment (Child-Pugh class C) is expected to be substantially higher than that observed in patients with moderate hepatic impairment.

Pricing: US

Pack (Kalydeco Oral)

25 mg (per each): $537.15

50 mg (per each): $537.15

75 mg (per each): $537.15

Tablets (Kalydeco Oral)

150 mg (per each): $537.15

Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.

Brand Names: International
  • Ivadeco (AR, CO, PE);
  • Kalydeco (AT, AU, BB, BE, CH, CZ, DE, DK, EE, ES, GB, HR, IE, IL, LT, LU, LV, MT, NL, NO, NZ, PL, PT, RO, SE, SK)


For country code abbreviations (show table)
  1. Accurso FJ, Rowe SM, Clancy JP, et al, “Effect of VX-770 in Persons With Cystic Fibrosis and the G551D-CFTR Mutation,” N Engl J Med, 2010, 363(21):1991-2003. [PubMed 21083385]
  2. Heltshe SL, Godfrey EM, Josephy T, Aitken ML, Taylor-Cousar JL. Pregnancy among cystic fibrosis women in the era of CFTR modulators. J Cyst Fibros. 2017;16(6):687-694. doi: 10.1016/j.jcf.2017.01.008. [PubMed 28190780]
  3. Kalydeco (ivacaftor) [prescribing information]. Boston, MA: Vertex Pharmaceuticals Inc; December 2020.
  4. Kalydeco (ivacaftor) [product monograph]. Toronto, Ontario, Canada: Vertex Pharmaceuticals (Canada) Inc; March 2022.
  5. Kaminski R, Nazareth D. A successful uncomplicated CF pregnancy while remaining on Ivacaftor. J Cyst Fibros. 2016;15(1):133-134. doi: 10.1016/j.jcf.2015.11.013. [PubMed 26698017]
  6. Ladores S, Kazmerski TM, Rowe SM. A case report of pregnancy during use of targeted therapeutics for cystic fibrosis. J Obstet Gynecol Neonatal Nurs. 2017;46(1):72-77. doi: 10.1016/j.jogn.2016.08.011. [PubMed 27875677]
  7. Marco A, Montales MT, Mittadodla P, et al. Tuberculosis reinfection in a pregnant cystic fibrosis patient. Respir Med Case Rep. 2015;16:57-59. doi: 10.1016/j.rmcr.2015.04.002. [PubMed 26744656]
  8. Ramsey BW, Davies J, McElvaney NG, et al, “A CFTR Potentiator in Patients With Cystic Fibrosis and the G551D Mutation,” N Engl J Med, 2011, 365(18):1663-72. [PubMed 22047557]
  9. Trimble A, McKinzie C, Terrell M, Stringer E, Esther CR Jr. Measured fetal and neonatal exposure to lumacaftor and ivacaftor during pregnancy and while breastfeeding. J Cyst Fibros. 2018;17(6):779-782. doi: 10.1016/j.jcf.2018.05.009. [PubMed 29866531]
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