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Orkambi Generic Name & Formulations
Legal Class
General Description
Pharmacological Class
See Also
How Supplied
Manufacturer
Generic Availability
Mechanism of Action
Orkambi 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 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 Contraindications
Not Applicable
Orkambi Boxed Warnings
Not Applicable
Orkambi 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 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
Orkambi Interactions
Interactions
Orkambi 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 Clinical Trials
See Literature
Orkambi Note
Not Applicable
Orkambi Patient Counseling
See Literature
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