Dosage:

• 1 year to <2 years: 
  • 7 kg to <9 kg: lumacaftor 75 mg/ivacaftor 94 mg granules every 12 hours
  • 9 kg to <14 kg: lumacaftor 100 mg/ivacaftor 125 mg granules every 12 hours
  • ≥14 kg: lumacaftor 150 mg/ivacaftor 188 mg granules every 12 hours
• 2 years to < 6 years:
  • < 14 kg: lumacaftor 100 mg/ivacaftor 125 mg granules every 12 hours
  • ≥ 14 kg: lumacaftor 150 mg/ivacaftor 188 mg granules every 12 hours
• 6 years and older
  • See Orkambi® tablets

Various mutations of the cystic fibrosis gene block the "gates" that allow the flow of salt across the cell membranes. This causes mucus to become thick and clog the lungs. Orkambi® helps "open the gates" more often allowing more salt to pass through.  The thick mucus is reduced and cystic fibrosis symptoms lessen.

Important potential side effects. These are not common but are possible:

• increased blood pressure, decreased heart rate
• cataracts
• shortness of breath, especially if you already have lung problems
• liver problems
• pneumonia
• allergic reactions

Contact your doctor if any of these side effects persists or are bothersome:

• stuffy or runny nose
• headache
• stomach pain
• increased cough and/or sputum
• sore throat
• shortness of breath and/or chest tightness
• increase in blood pressure
• decrease in heart rate
• rash
• fatigue
• flu or flu-like symptoms
• increase in muscle enzyme levels
• irregular, abnormal, or painful periods and increase in the amount of menstrual bleeding

• Drive and use machinery with caution as you may feel dizzy.
• Do not take this medication if you are allergic to any part of it.

Take this medication with caution if you:

• have liver disease
• have had an organ transplant
• have high blood pressure, a low heart rate, or other heart conditions
• have kidney disease.
• are using birth control (hormonal contraceptives including oral, injectable, transdermal, or implantable forms). Hormonal contraceptives should not be used as a method of birth control when taking Orkambi®.

The following medications may interact with Orkambi®:

• antifungal medicines such as ketoconazole, itraconazole, posaconazole, voriconazole, fluconazole
• some antibiotics such as telithromycin, clarithromycin, rifabutin, rifampicin, levofloxacin, erythromycin
• some anti-seizure medications such as phenobarbital, carbamazepine, phenytoin
• ranitidine
• St. John’s wort
• some medications used to treat anxiety, insomnia, and other conditions such as midazolam, triazolam
• some antidepressant medications such as citalopram, escitalopram, sertraline
• some anti-allergic/anti-asthma medications such as montelukast
• some medications used to reduce pain, inflammation, and fever such as ibuprofen
• some medications used after an organ transplantation such as cyclosporine, everolimus, sirolimus, and tacrolimus
• digoxin
• warfarin
• hormonal contraceptives (used to prevent pregnancy) including oral, injectable, skin patches (transdermal), and implantable products
  • these should not be relied upon as an effective method of birth control when given with Orkambi®
• some medications used to treat inflammation such as methylprednisolone, and prednisone
• some medications used to treat acid reflux disease and ulcers such as omeprazole, esomeprazole, and lansoprazole

Use in pregnancy and breastfeeding: Little is known about the safety of this medication when used in pregnancy and breastfeeding. Consult your doctor or pharmacist before use.