Orkambi (Lumacaftor And Ivacaftor)

Complete patient guide for uses, dosage, side effects, and safety information

Prescription Required

Quick Facts

Generic Name
lumacaftor and ivacaftor
Drug Class
cystic fibrosis transmembrane conductance regulator (CFTR) modulators
Used For
treatment of cystic fibrosis in patients aged 1 year or older with the F508del mutation
How Taken
oral granules or tablets taken every 12 hours with fat-containing food

What Is Orkambi Used For?

Orkambi is a medication used to treat cystic fibrosis, specifically in patients who have the F508del mutation in the CFTR gene.

Primary Uses

Orkambi is primarily used to manage cystic fibrosis in individuals aged 1 year and older who are homozygous for the F508del mutation.

How It Works

Orkambi works by improving the flow of chloride and water in the lungs, which helps to reduce mucus buildup and improve lung function.

Good to know: Orkambi is not addictive.

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How to Take Orkambi

It is important to take Orkambi exactly as prescribed by your doctor, with fat-containing food to ensure proper absorption.

Dosage Instructions

Dosage varies by age and weight: For patients aged 1 to less than 2 years: 75 mg/94 mg granules for those weighing 7-9 kg, 100 mg/125 mg for 9-14 kg, and 150 mg/188 mg for those ≤14 kg, taken every 12 hours. For ages 2-5 years: 100 mg/125 mg or 150 mg/188 mg granules every 12 hours based on weight. For ages 6-11 years: 200 mg/125 mg tablets every 12 hours. For ages 12 years and older: 400 mg/500 mg tablets every 12 hours.

What If You Miss a Dose?

If you miss a dose and it's less than 6 hours late, take it as soon as possible. If more than 6 hours have passed, skip the missed dose and resume your regular schedule. Do not double up on doses.

Important: Never take a double dose to make up for a missed dose.

Available Tablet Strengths

75 mg/94 mg
75 mg/94 mg Tablet White to off-white, Granules • sachet
100 mg/125 mg
100 mg/125 mg Tablet White to off-white, Granules • sachet
150 mg/188 mg
150 mg/188 mg Tablet White to off-white, Granules • sachet
100 mg/125 mg
100 mg/125 mg Tablet Pink, Oval • 1V125
200 mg/125 mg
200 mg/125 mg Tablet Pink, Oval • 2V125

Side Effects

Like all medications, this medicine can cause side effects. Most are mild and temporary, but some require medical attention.

Common Side Effects

These side effects occur relatively frequently and usually don’t require emergency care:

  • shortness of breath
  • diarrhea
  • nausea
  • chest tightness
  • sore throat
  • upper respiratory tract infection
  • gas
  • rash
  • stuffy or runny nose
  • vomiting
  • irregular periods

Seek immediate medical attention if you experience:

  • significant abdominal pain
  • yellowing of skin or eyes
  • loss of appetite
  • nausea or vomiting
  • dark urine
  • confusion
  • rash or hives
  • tightness in chest or throat
  • difficulty breathing
  • swelling of face, lips, or tongue
  • light-headedness or dizziness

Impulse Control Issues

There is no specific mention of impulse control issues related to Orkambi.

Important Warnings

There are several important warnings to consider before taking Orkambi.

Who Should Not Take Orkambi

Do not take Orkambi if you have had an allergic reaction to it or any of its ingredients.

Medical Conditions to Discuss

Discuss any liver or kidney disease with your doctor, as Orkambi may worsen these conditions.

Pregnancy and Breastfeeding

Consult your doctor if you are pregnant, plan to become pregnant, or are breastfeeding, as it is unknown if Orkambi is excreted in breast milk.

Elderly Patients With Dementia: Special caution is advised for elderly patients, especially those with dementia.

Drug Interactions

Certain medications can interact with Orkambi, affecting its efficacy.

Grapefruit interaction: Avoid grapefruit products as they may affect how Orkambi works.

  • montelukast
  • fexofenadine
  • ketoconazole
  • itraconazole
  • posaconazole
  • voriconazole
  • fluconazole
  • telithromycin
  • clarithromycin
  • erythromycin
  • rifampicin
  • rifabutin
  • rifapentine
  • phenobarbital
  • carbamazepine
  • phenytoin
  • ibuprofen
  • St. John's wort
  • midazolam
  • triazolam
  • ciclosporin
  • everolimus
  • sirolimus
  • tacrolimus
  • digoxin
  • warfarin
  • dabigatran
  • oral contraceptives
  • prednisone
  • methylprednisolone
  • citalopram
  • escitalopram
  • sertraline
  • bupropion
  • repaglinide
  • omeprazole
  • esomeprazole
  • lansoprazole
  • ranitidine

Alcohol & Driving

Be cautious with alcohol and driving while taking Orkambi.

Alcohol

Consult your doctor regarding alcohol consumption while on Orkambi.

Driving and Operating Machinery

Be careful when driving or using machinery until you know how Orkambi affects you.

Temperature Regulation

No specific temperature regulation warnings were mentioned.

Storage

Store Orkambi below 30°C in a dry place away from moisture, heat, and sunlight. Keep out of reach of children.

Overdose Information

If you suspect an overdose of Orkambi, seek immediate medical attention. Contact the Poisons Information Centre at 13 11 26 or go to the nearest hospital.

Frequently Asked Questions

Orkambi may take some time to show improvements in lung function and overall health.

Yes, Orkambi should be taken with fat-containing food to ensure proper absorption.

No, Orkambi is not addictive.

Do not stop taking Orkambi without consulting your doctor, as this may affect your health.

Consult your doctor regarding alcohol consumption while on Orkambi.

There is no specific mention of weight gain as a side effect of Orkambi.

Elderly patients should consult their doctor before taking Orkambi, especially if they have dementia.

If you miss a dose, take it as soon as possible if it's less than 6 hours late; otherwise, skip it and continue with your regular schedule.

Important Disclaimer

This information is intended for general educational purposes only and should not be considered medical advice. Always consult your healthcare provider or pharmacist before starting, stopping, or changing any medication.

If you think you may have a medical emergency, call your doctor or emergency services immediately.

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