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Ivacaftor / lumacaftor Side Effects

Medically reviewed by Last updated on Nov 22, 2022.


Commonly reported side effects of ivacaftor/lumacaftor include: upper respiratory tract infection, dyspnea, chest discomfort, nausea, and skin rash. Other side effects include: influenza, and flatulence. Continue reading for a comprehensive list of adverse effects.

Applies to ivacaftor / lumacaftor: oral granule, oral tablet.

Serious side effects

Along with its needed effects, ivacaftor/lumacaftor may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor immediately if any of the following side effects occur while taking ivacaftor / lumacaftor:

More common

  • Body aches or pain
  • chest tightness
  • chills
  • cough
  • difficult or labored breathing
  • ear congestion
  • fever
  • headache
  • loss of voice
  • muscle aches
  • sneezing
  • sore throat
  • stuffy or runny nose
  • unusual tiredness or weakness
  • upper abdominal or stomach pain

Incidence not known

  • Blurred vision
  • dark urine
  • discomfort in the upper right stomach area
  • dizziness
  • general feeling of tiredness or weakness
  • light-colored stools
  • loss of appetite
  • nausea
  • nervousness
  • pounding in the ears
  • slow or fast heartbeat
  • stomach pain, continuing
  • vomiting
  • yellow eyes or skin

Other side effects

Some side effects of ivacaftor / lumacaftor may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects.

Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

  • Absent, missed, or irregular menstrual periods
  • bloated feeling
  • cough producing mucus
  • cramps
  • diarrhea
  • excess air or gas in the stomach or bowels
  • feeling of fullness
  • heavy bleeding
  • increased sputum production
  • longer or heavier menstrual periods
  • loss of voice
  • passing gas
  • rash
  • stopping of menstrual bleeding

For Healthcare Professionals

Applies to ivacaftor / lumacaftor: oral granule for reconstitution, oral tablet.


The most frequently reported side effects were dyspnea, nasopharyngitis, nausea, diarrhea, upper respiratory tract infection, fatigue, respiration abnormal, blood creatine phosphokinase increased, rash, flatulence, rhinorrhea, and influenza.[Ref]


Very common (10% or more): Infective pulmonary exacerbation of cystic fibrosis (37.5%), cough (30.5%), sputum increased (14.8 %), dyspnea (up to 14%), hemoptysis (13.8%), nasopharyngitis (up to 13%), upper respiratory tract infection (up to 10%)

Common (1% to 10%): Rhinitis, pharyngeal erythema, sinus congestion, respiration abnormal, rhinorrhea, nasal congestion


Very common (10% or more): Nasopharyngitis, nasal congestion

Common (1% to 10%): Sinus congestion, pharyngeal erythema[Ref]


Very common (10% or more): Nausea (up to 13%), diarrhea (up to 12%), abdominal pain

Common (1% to 10%): Flatulence, oropharyngeal pain, vomiting


Very common (10% or more): Abdominal pain[Ref]


Common (1% to 10%): Rash[Ref]

Nervous system

Very common (10% or more): Headache (up to 15.7%), dizziness


Very common (10% or more): Dizziness, headache[Ref]


Very common (10% or more): Bacteria in sputum

Common (1% to 10%): Blood creatine phosphokinase increased, fatigue, influenza, ear discomfort, ear pain, tinnitus, tympanic membrane hyperemia, vestibular disorder, chest discomfort

Uncommon (0.1% to 1%): Ear congestion


Very common (10% or more): Bacteria in sputum

Common (1% to 10%): Ear discomfort, ear pain, tinnitus, tympanic membrane hyperemia, vestibular disorder

Uncommon (0.1% to 1%): Ear congestion[Ref]


Very common (10% or more): Breast mass

Uncommon (0.1% to 1%): Breast inflammation, gynecomastia, nipple disorder, nipple pain


Common (1% to 10%): Breast mass

Uncommon (0.1% to 1%): Breast inflammation, gynecomastia, nipple disorder, nipple pain[Ref]


Common (1% to 10%): Blood pressure increased/hypertension[Ref]


Common (1% to 10%): AST increased, ALT increased

Uncommon (0.1% to 1%): Hepatic encephalopathy, cholestatic hepatitis

Frequency not reported: Total bilirubin increased

Postmarketing reports: Liver failure leading to death, liver function decompensation[Ref]


Common (1% to 10%): Menstruation abnormal/irregular, dysmenorrhea, metrorrhagia, amenorrhea, menorrhagia

Uncommon (0.1% to 1%): Oligomenorrhea, polymenorrhea[Ref]


Frequency not reported: Non-congenital lens opacities[Ref]

Frequently asked questions


1. Cerner Multum, Inc. "UK Summary of Product Characteristics." O 0

2. Cerner Multum, Inc. "Australian Product Information." O 0

3. "Product Information. Orkambi (ivacaftor-lumacaftor)." Vertex Pharmaceuticals (2015):

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

Some side effects may not be reported. You may report them to the FDA.