These highlights do not include all the information needed to use KALYDECO safely and effectively. See full prescribing information for KALYDECO.
KALYDECO � (ivacaftor) tablets, for oral use
KALYDECO � (ivacaftor) oral granules
Initial U.S. Approval: 2012
RECENT MAJOR CHANGES
Indications and Usage (1)
05/2017
INDICATIONS AND USAGE
KALYDECO is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator indicated for the treatment of cystic fibrosis (CF) in patients age 2 years and older who have one mutation in the CFTR gene that is responsive to ivacaftor based on clinical and/or in vitro assay data. (12.1, 14)
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 bi-directional sequencing when recommended by the mutation test instructions for use. (1)
DOSAGE AND ADMINISTRATION
Adults and pediatric patients age 6 years and older: one 150 mg tablet taken orally every 12 hours with fat-containing food. (2.2, 12.3)
Pediatric patients 2 to less than 6 years of age and less than 14 kg: one 50 mg packet mixed with 1 teaspoon (5 mL) of soft food or liquid and administered orally every 12 hours with fat-containing food. (2.3, 12.3)
Pediatric patients 2 to less than 6 years of age and 14 kg or greater: one 75 mg packet mixed with 1 teaspoon (5 mL) of soft food or liquid and administered orally every 12 hours with fat-containing food. (2.3, 12.3)
Pediatric patients less than 2 years of age: not recommended. (2.4, 8.4)
Reduce dose in patients with moderate and severe hepatic impairment. (2.5, 8.6, 12.3)
Reduce dose when co-administered with drugs that are moderate or strong CYP3A inhibitors. (2.6, 7.1, 12.3)
DOSAGE FORMS AND STRENGTHS
Tablets: 150 mg (3)
Oral granules: Unit-dose packets of 50 mg and 75 mg (3)
CONTRAINDICATIONS
None (4)
WARNINGS AND PRECAUTIONS
Elevated transaminases (ALT or AST): Transaminases (ALT and AST) should be assessed prior to initiating KALYDECO, every 3 months during the first year of treatment, and annually thereafter. In patients with a history of transaminase elevations, more frequent monitoring of liver function tests should be considered. Patients who develop increased transaminase levels should be closely monitored until the abnormalities resolve. Dosing should be interrupted in patients with ALT or AST of greater than 5 times the upper limit of normal (ULN). Following resolution of transaminase elevations, consider the benefits and risks of resuming KALYDECO dosing. (5.1, 6)
Use with CYP3A inducers: Concomitant use with strong CYP3A inducers (e.g., rifampin, St. John's wort) substantially decreases exposure of ivacaftor, which may diminish effectiveness. Therefore, co-administration is not recommended. (5.2, 7.2, 12.3)
Cataracts: Non-congenital lens opacities/cataracts have been reported in pediatric patients treated with KALYDECO. Baseline and follow-up examinations are recommended in pediatric patients initiating KALYDECO treatment. (5.3)
ADVERSE REACTIONS
The most common adverse drug reactions to KALYDECO (occurring in ?8% of patients with CF who have a G551D mutation in the CFTR gene) were headache, oropharyngeal pain, upper respiratory tract infection, nasal congestion, abdominal pain, nasopharyngitis, diarrhea, rash, nausea, and dizziness. (6.1)
To report SUSPECTED ADVERSE REACTIONS, contact Vertex Pharmaceuticals Incorporated at 1-877-634-8789 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
DRUG INTERACTIONS
CYP3A inhibitors: Reduce KALYDECO dose to one tablet or one packet of granules twice a week when co-administered with strong CYP3A inhibitors (e.g., ketoconazole). Reduce KALYDECO dose to one tablet or one packet of granules once daily when co-administered with moderate CYP3A inhibitors (e.g., fluconazole). Avoid food containing grapefruit or Seville oranges. (7.1, 12.3)
See 17 for PATIENT COUNSELING INFORMATION and FDA-approved patient labeling.
Revised: 7/2017
