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Kalydeco Oral Granules Generic Name & Formulations
Legal Class
General Description
Pharmacological Class
See Also
How Supplied
Manufacturer
Generic Availability
Mechanism of Action
Kalydeco Oral Granules Indications
Indications
Treatment of cystic fibrosis (CF) in patients ≥1month of age who have at least one mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.
Kalydeco Oral Granules Dosage and Administration
Adults and Children
<1month or <6mos (with hepatic impairment and/or on concomitant moderate or strong CYP3A inhibitors): not recommended. 1–<6mos (born at a gestational age <37wks): not evaluated. Swallow tabs whole. Oral granules should be mixed with 1 tsp (5mL) of soft-food or liquid (eg, pureed fruits/vegetables, yogurt, applesauce, water, milk, juice, breast milk, or infant formula) and completely consumed within 1hr. Take with fat-containing food (eg, eggs, butter, peanut butter, cheese pizza, dairy products). 1–<2mos (≥3kg): 5.8mg packet every 12hrs; 2–<4mos (≥3kg): 13.4mg packet every 12hrs; 4–<6mos (≥5kg): 25mg packet every 12hrs. 6mos–<6yrs (5–<7kg): 25mg packet every 12hrs; (7–<14kg): 50mg packet every 12hrs; (≥14kg): 75mg packet every 12hrs. ≥6yrs: 150mg tab every 12hrs. Hepatic impairment, concomitant moderate or strong CYP3A inhibitors: reduce dosing frequency; see full labeling.
Kalydeco Oral Granules Contraindications
Not Applicable
Kalydeco Oral Granules Boxed Warnings
Not Applicable
Kalydeco Oral Granules Warnings/Precautions
Warnings/Precautions
If genotype is unknown, use an FDA-cleared CF mutation test to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test instructions. Assess ALT/AST levels prior to initiating therapy, every 3 months during the first year of treatment, and annually thereafter. History of ALT/AST elevations: monitor LFTs more frequently. If increased ALT/AST levels develop, monitor closely until abnormalities resolved. Interrupt dosing if ALT/AST is >5×ULN; after resolution, consider restarting. Discontinue if hypersensitivity reactions develop; treat appropriately. Perform baseline and follow-up eye exams. Hepatic impairment. Severe renal impairment or ESRD. Pregnancy. Nursing mothers.
Kalydeco Oral Granules Pharmacokinetics
Absorption
Peak plasma concentrations (Tmax): ~4 hours after a single 150mg dose.
Steady-state plasma concentrations were reached by days 3–5.
Exposure of ivacaftor is increased ~2.5- to 4-fold when given with food that contains fat.
Distribution
Plasma protein bound: ~99%.
Mean apparent volume of distribution: 353 (±122) L.
Elimination
Fecal (87.8%). Half-life: ~12 hours.
Kalydeco Oral Granules Interactions
Interactions
Potentiated by strong CYP3A inhibitors (eg, ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, clarithromycin) and moderate CYP3A inhibitors (eg, fluconazole, erythromycin); see Adults and Children. May be potentiated by grapefruit and Seville oranges; avoid. Antagonized by strong CYP3A inducers (eg, rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, St. John’s Wort); use not recommended. May potentiate warfarin; monitor INR. Potentiates CYP2C9 (eg, glimepiride, glipizide), CYP3A and/or P-gp substrates (eg, digoxin, cyclosporine, tacrolimus); monitor.
Kalydeco Oral Granules Adverse Reactions
Adverse Reactions
Headache, oropharyngeal pain, upper respiratory tract infection, nasal congestion, abdominal pain, nasopharyngitis, diarrhea, rash, nausea, dizziness; hypersensitivity reactions (eg, anaphylaxis), non-congenital lens opacities/cataracts.
Kalydeco Oral Granules Clinical Trials
See Literature
Kalydeco Oral Granules Note
Not Applicable
Kalydeco Oral Granules Patient Counseling
See Literature
