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Orkambi Oral Granules Generic Name & Formulations
Legal Class
General Description
Pharmacological Class
See Also
How Supplied
Manufacturer
Generic Availability
Mechanism of Action
Orkambi Oral Granules Indications
Indications
Treatment of cystic fibrosis (CF) in patients ≥1yr who are homozygous for the F508del mutation in the CFTR gene.
Limitations of Use
Orkambi Oral Granules Dosage and Administration
Adult
Take with fat-containing food (eg, eggs, avocados, nuts, butter, peanut butter, cheese pizza, whole-milk dairy products). ≥12yrs: 2 tabs (200mg/125mg) every 12hrs. Currently taking strong CYP3A inhibitors: initially 1 tab (200mg/125mg) once daily for 1st week then continue with recommended daily dose. Hepatic impairment (moderate): 2 tabs in the AM and 1 tab in the PM; (severe): max 1 tab in the AM and 1 tab in the PM, or less; use with caution.
Children
<1yr: not established. Take with fat-containing food (eg, eggs, avocados, nuts, butter, peanut butter, cheese pizza, whole-milk dairy products). Mix oral granules in 5mL soft food or liquid (eg, pureed fruits or vegetables, yogurt, pudding, applesauce, water, milk, breast milk, infant formula, juice). 1–2yrs (7–<9kg): 1pkt (75mg/94mg) every 12hrs; (9–<14kg): 1pkt (100mg/125mg) every 12hrs; (≥14kg): 1 pkt (150mg/188mg) every 12hrs. 2–5yrs (<14kg): 1 pkt (100mg/125mg) every 12hrs; (≥14kg): 1 pkt (150mg/188mg) every 12hrs. 6–11yrs: 2 tabs (100mg/125mg) every 12hrs. Currently taking strong CYP3A inhibitors: initially 1 pkt every other day (1–5yrs) or 1 tab once daily (≥6yrs) for 1st week then continue with recommended daily dose. Hepatic impairment: 1–5yrs (moderate): 1 pkt in the AM and 1 pkt every other day in the PM; (severe): max 1 pkt in the AM, or less; ≥6yrs (moderate): 2 tabs in the AM and 1 tab in the PM; (severe): max 1 tab in the AM and 1 tab in the PM, or less; all: use with caution.
Orkambi Oral Granules Contraindications
Not Applicable
Orkambi Oral Granules Boxed Warnings
Not Applicable
Orkambi Oral Granules Warnings/Precautions
Warnings/Precautions
If genotype is unknown, use an FDA cleared CF mutation test to detect the presence of the F508del mutation on both alleles of the CFTR gene. Advanced liver disease: monitor closely after initiation and reduce dose. Assess ALT/AST and bilirubin levels prior to initiating therapy, every 3 months during the first year of treatment, and annually thereafter. If ALT/AST or bilirubin levels increased, monitor closely until resolved. Interrupt dosing if ALT/AST is >5×ULN or if ALT/AST is >3×ULN with bilirubin elevations >2×ULN; after resolution, consider restarting. Discontinue if serious hypersensitivity reactions develop. Monitor BP periodically and for respiratory events during treatment initiation in patients with ppFEV1 <40. Perform baseline and follow-up eye exams. Transplanted patients: not recommended. Severe renal impairment (CrCl ≤30mL/min) or ESRD. Pregnancy. Nursing mothers.
Orkambi Oral Granules Pharmacokinetics
Absorption
Median (range) Tmax: ~4.0 hours (2.0; 9.0) in the fed state for lumacaftor; ~4.0 hours (2.0; 6.0) in the fed state for ivacaftor.
Distribution
Plasma protein bound: ~99%.
Elimination
Lumacaftor: fecal (51%), renal (minimal); Ivacaftor: fecal (87.8%), renal (minimal).
Half-life: 25.2 hours (lumacaftor), 9.34 hours (ivacaftor).
Orkambi Oral Granules Interactions
Interactions
Orkambi Oral Granules Adverse Reactions
Adverse Reactions
Dyspnea, nasopharyngitis, nausea, diarrhea, upper respiratory tract infection, fatigue, abnormal respiration, increased blood creatinine phosphokinase, rash, flatulence, rhinorrhea, influenza; elevated transaminases, respiratory events, hypersensitivity reactions (eg, angioedema, anaphylaxis).
Orkambi Oral Granules Clinical Trials
See Literature
Orkambi Oral Granules Note
Not Applicable
Orkambi Oral Granules Patient Counseling
See Literature
