Orkambi® granules

This product is manufactured by Vertex Pharmaceuticals and is a combination of two ingredients, ivacaftor and lumacaftor.

This product is taken orally.


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orally

Why is it prescribed?

Orkambi® is indicated for the treatment of cystic fibrosis (CF) in patients 1 year of age and older who are homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.  

  • Each dose should be taken just before or just after fat-containing food.
  • The entire contents of each packet of granules should be mixed with 5 mL (one teaspoon) of age-appropriate soft food (e.g., puréed fruits or vegetables, flavored yogurt or pudding, applesauce) or liquid (e.g., water, breastmilk, infant formula, juice [except grapefruit]) and the mixture completely consumed.
  • Food should be at 5 to 25ºC.
  • Once mixed, the product needs to be ingested within one hour and not stored for future use.

Take regularly even if feel well.

  • Take doses 12 hours apart (morning and evening).
  • If a dose is missed and it is within 6 hours of when it's usually taken, take the dose as soon as possible.
  • If a dose is missed and it is more than 6 hours after the time it's usually taken, skip that dose only and take the next dose at usual time.
  • Do not take 2 doses at the same time to make up for a missed dose.

Store at room temperature.


Alternatives

Other products that have the same ingredients as Orkambi® granules are •Orkambi® tablets ••Orkambi® tablets •

See other products used in the treatment of •cystic fibrosis •

Dosage:

  • 1 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 through 5 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

The CFTR protein helps bring chloride ions out of the cells in many organs. People who have two copies of the F508del mutation in the CFTR gene have a lower amount of CFTR protein at the surface of their cells.

Orkambi® is a “cystic fibrosis transmembrane conductance regulator corrector and potentiator.”  The two ingredients in this medication help bring more chloride ions out of the cells in many organs. Lumacaftor, a CFTR corrector, increases the amount of the CFTR proteins at the cell surface.  Ivacaftor, a CFTR potentiator, makes the CFTR protein at the cell surface work better


Possible side effects - contact physician if serious or 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

Take this medication with caution if you:

  • have liver disease
  • have catarats
  • 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
  • Antibiotics such as telithromycin, clarithromycin, rifabutin, rifampicin, levofloxacin, erythromycin
  • Anticonvulsant medicines such as phenobarbital, carbamazepine, phenytoin
  • Ranitidine
  • St. John’s wort
  • Benzodiazepines such as midazolam, triazolam
  • Antidepressants such as citalopram, escitalopram, sertraline
  • Anti-allergics such as montelukast

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.