Lumacaftor and ivacaftor

Generic name: Lumacaftor And Ivacaftor
Drug class: CFTR combinations

Usage of Lumacaftor and ivacaftor

Lumacaftor and ivacaftor combination is used to treat cystic fibrosis (CF) in patients 1 year of age and older. It is only used for patients who have two copies of F508del mutation in their CF transmembrane conductance regulator (CFTR) gene. Your doctor will use a test to check for the mutation before you receive the medicine.

This medicine is available only with your doctor's prescription.

Lumacaftor and ivacaftor side effects

Along with its needed effects, a medicine 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:

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

difficulty swallowing

discomfort in the upper right stomach area

dizziness

fast heartbeat

general feeling of tiredness or weakness

hives, itching, skin rash

large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs

light-colored stools

loss of appetite

nausea

nervousness

pounding in the ears

puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue

slow or fast heartbeat

stomach pain, continuing

vomiting

yellow eyes or skin

Some side effects 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

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Before taking Lumacaftor and ivacaftor

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

Allergies

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Pediatric

Appropriate studies have not been performed on the relationship of age to the effects of lumacaftor and ivacaftor combination in children younger than 1 year of age. Safety and efficacy have not been established.

Geriatric

Appropriate studies on the relationship of age to the effects of lumacaftor and ivacaftor combination have not been performed in the geriatric population.

Breast Feeding

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Interactions with Medicines

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. When you are taking this medicine, it is especially important that your healthcare professional know if you are taking any of the medicines listed below. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following medicines is not recommended. Your doctor may decide not to treat you with this medication or change some of the other medicines you take.

Doravirine

Fostemsavir

Isavuconazonium

Lenacapavir

Levoketoconazole

Lorlatinib

Mavacamten

Nirmatrelvir

Pacritinib

Ritonavir

Using this medicine with any of the following medicines is usually not recommended, but may be required in some cases. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

Abemaciclib

Abiraterone Acetate

Acalabrutinib

Adagrasib

Alfentanil

Alpelisib

Alprazolam

Amiodarone

Amprenavir

Apixaban

Aprepitant

Aripiprazole

Atazanavir

Atogepant

Avacopan

Avapritinib

Axitinib

Belumosudil

Belzutifan

Bentazepam

Benzhydrocodone

Bictegravir

Boceprevir

Bortezomib

Bosutinib

Brigatinib

Bromazepam

Ceritinib

Chlordiazepoxide

Cimetidine

Ciprofloxacin

Clarithromycin

Clobazam

Clofazimine

Clonazepam

Clorazepate

Cloxazolam

Clozapine

Cobicistat

Codeine

Conivaptan

Copanlisib

Crizotinib

Cyclosporine

Dabrafenib

Daridorexant

Darifenacin

Darunavir

Dasatinib

Deflazacort

Delorazepam

Desogestrel

Dexamethasone

Diazepam

Dienogest

Digoxin

Dihydrocodeine

Dihydroergotamine

Diltiazem

Dofetilide

Dronedarone

Drospirenone

Duvelisib

Elacestrant

Eletriptan

Elexacaftor

Eliglustat

Encorafenib

Entrectinib

Enzalutamide

Eplerenone

Eravacycline

Erdafitinib

Ergotamine

Erlotinib

Erythromycin

Estazolam

Estetrol

Estradiol

Ethinyl Estradiol

Ethynodiol

Etonogestrel

Everolimus

Fedratinib

Felodipine

Fenfluramine

Fentanyl

Fexinidazole

Finerenone

Flibanserin

Fluconazole

Flunitrazepam

Flurazepam

Fluticasone

Fluvoxamine

Fosamprenavir

Fosaprepitant

Fosnetupitant

Fosphenytoin

Fostamatinib

Gepirone

Gestodene

Glasdegib

Glimepiride

Glipizide

Halazepam

Hemin

Hydrocodone

Ibrexafungerp

Ibrutinib

Ibuprofen

Idelalisib

Imatinib

Indinavir

Infigratinib

Istradefylline

Itraconazole

Ivabradine

Ivacaftor

Ivosidenib

Ixabepilone

Josamycin

Ketazolam

Ketoconazole

Larotrectinib

Lefamulin

Lemborexant

Lenacapavir

Leniolisib

Letermovir

Levoketoconazole

Levonorgestrel

Linagliptin

Lonafarnib

Lopinavir

Loprazolam

Lorazepam

Lorlatinib

Lormetazepam

Lovastatin

Lumacaftor

Lumateperone

Lurasidone

Lurbinectedin

Macimorelin

Macitentan

Maraviroc

Maribavir

Mavacamten

Medazepam

Medroxyprogesterone

Meperidine

Mestranol

Metaclazepam

Methadone

Mibefradil

Midazolam

Midostaurin

Mifepristone

Mirtazapine

Mitapivat

Mitotane

Mobocertinib

Morphine

Morphine Sulfate Liposome

Naloxegol

Nefazodone

Nelfinavir

Neratinib

Netupitant

Nilotinib

Nitrazepam

Nomegestrol

Nordazepam

Norelgestromin

Norethindrone

Norgestimate

Norgestrel

Olanzapine

Olaparib

Olutasidenib

Omaveloxolone

Osimertinib

Oxazepam

Oxcarbazepine

Oxycodone

Pacritinib

Paliperidone

Palovarotene

Pemigatinib

Pentazocine

Perampanel

Pexidartinib

Phenobarbital

Phenytoin

Pimavanserin

Pimozide

Pinazepam

Pirtobrutinib

Pitolisant

Ponatinib

Posaconazole

Pralsetinib

Prazepam

Pretomanid

Primidone

Quazepam

Quetiapine

Quinidine

Quizartinib

Regorafenib

Remimazolam

Ribociclib

Rifabutin

Rifampin

Rifapentine

Rimegepant

Ripretinib

Risperidone

Ritlecitinib

Rivaroxaban

Romidepsin

Ruxolitinib

Samidorphan

Saquinavir

Schisandra sphenanthera

Segesterone

Selpercatinib

Selumetinib

Sildenafil

Simvastatin

Sirolimus

Sirolimus Protein-Bound

Sotorasib

Sparsentan

Stiripentol

St John's Wort

Sufentanil

Sunitinib

Tacrolimus

Tamoxifen

Tasimelteon

Tazemetostat

Telaprevir

Telithromycin

Temazepam

Temsirolimus

Tetrazepam

Tezacaftor

Thiotepa

Ticagrelor

Tipranavir

Tivozanib

Tocilizumab

Tofacitinib

Tolvaptan

Tramadol

Treosulfan

Triazolam

Trofinetide

Troleandomycin

Tucatinib

Ubrogepant

Ulipristal

Upadacitinib

Valbenazine

Vardenafil

Velpatasvir

Vemurafenib

Verapamil

Vilazodone

Vincristine

Vincristine Sulfate Liposome

Voclosporin

Vonoprazan

Voriconazole

Vortioxetine

Voxelotor

Voxilaprevir

Warfarin

Zanubrutinib

Zolpidem

Zuranolone

Using this medicine with any of the following medicines may cause an increased risk of certain side effects, but using both drugs may be the best treatment for you. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

Aripiprazole Lauroxil

Interactions with Food/Tobacco/Alcohol

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following is usually not recommended, but may be unavoidable in some cases. If used together, your doctor may change the dose or how often you use this medicine, or give you special instructions about the use of food, alcohol, or tobacco.

Grapefruit Juice

Relate drugs

How to use Lumacaftor and ivacaftor

Take this medicine exactly as directed by your doctor. Do not take more of it, do not take it more often, and do not take it for a longer time than your doctor ordered. To do so may increase the chance of side effects.

This medicine comes with a patient information insert. Read and follow the instructions carefully. Ask your doctor if you have any questions.

Take this medicine with fat-containing foods including avocados, butter, cheese pizza, eggs, nuts, peanut butter, breast milk, baby formula, or whole-milk dairy products (eg, whole milk, cheese, yogurt).

If you are using the granules, mix all the content of each packet with 1 teaspoon (5 milliliters [mL]) of cool to room temperature soft food (eg, puréed fruits, flavored yogurt, pudding, applesauce) or liquid (eg, water, milk, breast milk, baby formula, or juice). Take this mixture within 1 hour after mixing.

Tell your doctor if you stopped taking this medicine for more than 1 week. Your doctor may need to change your dose of Orkambi® or other medicines you take.

Dosing

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

For oral dosage form (granules, tablets):

For cystic fibrosis:

Adults and children 12 years of age and older—2 tablets every 12 hours, taken with fat-containing food. Each tablet contains 200 milligrams (mg) lumacaftor and 125 mg ivacaftor.

Children 6 to 11 years of age—2 tablets every 12 hours, taken with fat-containing food. Each tablet contains 100 mg lumacaftor and 125 mg ivacaftor.

Children 2 to 5 years of age—Dose is based on your body weight and must be determined by your doctor.

Weighing 14 kilograms (kg) or more—1 packet of granules every 12 hours, taken with fat-containing food. Each packet contains 150 mg lumacaftor and 188 mg ivacaftor.

Weighing less than 14 kg—1 packet of granules every 12 hours, taken with fat-containing food. Each packet contains 100 mg lumacaftor and 125 mg ivacaftor.

Children 1 to 2 years of age—Dose is based on your body weight and must be determined by your doctor.

Weighing 14 kilograms (kg) or more—1 packet of granules every 12 hours, taken with fat-containing food. Each packet contains 150 milligrams (mg) lumacaftor and 188 mg ivacaftor.

Weighing 9 to less than 14 kg—1 packet of granules every 12 hours, taken with fat-containing food. Each packet contains 100 mg lumacaftor and 125 mg ivacaftor.

Weighing 7 to less than 9 kg—1 packet of granules every 12 hours, taken with fat-containing food. Each packet contains 75 mg lumacaftor and 94 mg ivacaftor.

Children younger than 1 year of age—Use and dose must be determined by your doctor.

Missed Dose

If you miss a dose of this medicine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

If you miss a dose and it is 6 hours or less from the time you normally take your medicine, take a dose as soon as you can. If it is more than 6 hours, wait until then to use the medicine and skip the missed dose. Do not take extra medicine to make up for a missed dose.

Storage

Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.

Warnings

It is very important that your doctor check your or your child's progress at regular visits to make sure that this medicine is working properly. Blood tests will be needed to check for unwanted effects. You may also need to monitor your blood pressure at home. If you notice any changes to your normal blood pressure, call your doctor right away.

Birth control pills and other forms of hormone-based contraceptives (eg, patches, rings, implants, and injections) may not work while you are using this medicine. To keep from getting pregnant, use another form of birth control (eg, condoms, diaphragm, or a contraceptive foam or jelly) while using Orkambi®.

Liver problems may be more likely to occur if you already have an existing liver problem (eg, cirrhosis). Check with your doctor right away if you or your child has pain or tenderness in the upper stomach, pale stools, dark urine, loss of appetite, nausea, vomiting, or yellow eyes or skin.

This medicine may cause serious allergic reactions, including anaphylaxis and angioedema, which can be life-threatening and require immediate medical attention. Check with your doctor right away if you have chest tightness, cough, difficulty swallowing, dizziness, fast heartbeat, hives, itching, skin rash, large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs, puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue, trouble breathing, or unusual tiredness or weakness.

Check with your doctor right away if you or your child has cough, trouble breathing, chest tightness, or any type of breathing problem when starting treatment with this medicine.

This medicine may cause eye problem, including cataracts. Check with your doctor right away if you or your child has blindness, blurred vision, or decreased vision.

Do not take other medicines unless they have been discussed with your doctor. This includes prescription or nonprescription (over-the-counter [OTC]) medicines and herbal (eg, St. John's wort) or vitamin supplements.

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