Orkambi Oral Granules

— THERAPEUTIC CATEGORIES —
  • Inborn errors of metabolism
PAGE CONTENTS
  • Jump to Section
  • Generic Name & Formulations
  • Indications
  • Dosage and Administration
  • Contraindications
  • Boxed Warnings
  • Warnings/Precautions
  • Pharmacokinetics
  • Interactions
  • Adverse Reactions
  • Clinical Trials
  • Note
  • Patient Counseling

    • Orkambi Oral Granules Generic Name & Formulations

    Legal Class

    Rx

    General Description

    Lumacaftor, ivacaftor; 75mg/94mg, 100mg/125mg, 150mg/188mg; per pkt.

    Pharmacological Class

    Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator.

    See Also

    How Supplied

    Tabs—112; Oral granules—56

    Manufacturer

    Vertex Pharmaceuticals

    Generic Availability

    NO

    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

    Efficacy and safety not established in patients with CF other than those homozygous for the F508del mutation.

    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). 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 >5XULN or if ALT/AST is >3XULN with bilirubin elevations >2XULN; after resolution, consider restarting. 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

    See Literature

    Orkambi Oral Granules Interactions

    Interactions

    Ivacaftor potentiated by strong CYP3A inhibitors (eg, itraconazole, ketoconazole, posaconazole, voriconazole, telithromycin, clarithromycin); see Adults, Children. Concomitant strong CYP3A inducers (eg, rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, St. John’s wort): not recommended. Concomitant sensitive CYP3A substrates or those with a narrow therapeutic index (eg, midazolam, triazolam, cyclosporine, everolimus, sirolimus, tacrolimus): not recommended. May affect CYP2B6, CYP2C8, CYP2C9, CYP2C19, P-gp substrates. Monitor digoxin. May antagonize montelukast, systemic corticosteroids (eg, prednisone, methylprednisolone), ibuprofen, citalopram, escitalopram, sertraline, omeprazole, esomeprazole, lansoprazole, ranitidine; dose adjustment may be needed. May antagonize clarithromycin, erythromycin, telithromycin; consider alternatives (eg, ciprofloxacin, azithromycin, levofloxacin). Concomitant antifungals (eg, itraconazole, ketoconazole, posaconazole, voriconazole): not recommended; if necessary, monitor closely or consider alternatives (eg, fluconazole). May antagonize repaglinide or affect sulfonylureas; dose adjustment may be needed. Concomitant warfarin; monitor INR. May reduce effectiveness of hormonal contraceptives and increase menstruation abnormality events; avoid.

    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.

    Orkambi Oral Granules Clinical Trials

    See Literature

    Orkambi Oral Granules Note

    Not Applicable

    Orkambi Oral Granules Patient Counseling

    See Literature

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