How does this medication work? What will it do for me?
This is a combination product that contains two medications: ivacaftor and lumacaftor. It is used to treat cystic fibrosis (CF) in adults and children over 2 years old who have one of several possible mutations (a change) in their CFTR gene, the F508del mutation.
Ivacaftor belongs to the class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) potentiators. It increases the movement of chloride into and out of cells. Lumacaftor belongs to the class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) correctors. It is believed to work by correcting the shape of the mutated CFTR gene and thereby making more protein that works correctly at the cell wall.
In CF, an abnormal protein called CFTR is produced. This protein changes the way chloride (a component of salt, which is also called sodium chloride) moves in and out of cells. This affects the balance between salt and water in the body, making the mucus that lines the lungs, pancreas, and other organs thicker and stickier. Lumacaftor helps get more CFTR proteins to the cell surface and ivacaftor helps the CFTR proteins at the cell surface work better.
This medication may be available under multiple brand names and/or in several different forms. Any specific brand name of this medication may not be available in all of the forms or approved for all of the conditions discussed here. As well, some forms of this medication may not be used for all of the conditions discussed here.
Your doctor may have suggested this medication for conditions other than those listed in these drug information articles. If you have not discussed this with your doctor or are not sure why you are taking this medication, speak to your doctor. Do not stop taking this medication without consulting your doctor.
Do not give this medication to anyone else, even if they have the same symptoms as you do. It can be harmful for people to take this medication if their doctor has not prescribed it.
What form(s) does this medication come in?
100 mg/125 mg
Each pink, oval-shaped tablet printed with "1V125" in black ink on one side contains 100 mg of lumacaftor and 125 mg of ivacaftor. Nonmedicinal ingredients: microcrystalline cellulose, croscarmellose sodium, hypromellose acetate succinate, magnesium stearate, povidone, and sodium lauryl sulfate; tablet film coat: carmine, FD&C Blue No. 1, FD&C Blue No. 2, polyethylene glycol, polyvinyl alcohol, talc, and titanium dioxide; printing ink: ammonium hydroxide, iron oxide black, propylene glycol, and shellac.
200 mg/125 mg
Each pink, oval-shaped tablet printed with "2V125" in black ink on one side contains 200 mg of lumacaftor and 125 mg of ivacaftor. Nonmedicinal ingredients: microcrystalline cellulose, croscarmellose sodium, hypromellose acetate succinate, magnesium stearate, povidone, and sodium lauryl sulfate; tablet film coat: carmine, FD&C Blue No. 1, FD&C Blue No. 2, polyethylene glycol, polyvinyl alcohol, talc, and titanium dioxide; printing ink: ammonium hydroxide, iron oxide black, propylene glycol, and shellac.
100 mg/125 mg
Each packet of white-to-off-white granules for oral administration, contains 100 mg of lumacaftor and 125 mg of ivacaftor. Nonmedicinal ingredients: microcrystalline cellulose, croscarmellose sodium, hypromellose acetate succinate, povidone, and sodium lauryl sulfate.
150 mg/188 mg
Each packet of white-to-off-white granules for oral administration, contains 150 mg of lumacaftor and 188 mg of ivacaftor. Nonmedicinal ingredients: microcrystalline cellulose, croscarmellose sodium, hypromellose acetate succinate, povidone, and sodium lauryl sulfate.
How should I use this medication?
The usual dose of this medication for children aged 2 to 5 years old, who weigh less than 14 kg is the contents of 1 packet of granules containing 100 mg of lumacaftor and 125 mg of ivacaftor, taken by mouth every 12 hours, with fat-containing food. The total daily dose is 200 mg of lumacaftor and 250 mg of ivacaftor.
The usual dose of this medication for children aged 2 to 5 years old, who weigh more than 14 kg is the contents of 1 packet of granules, containing 150 mg of lumacaftor and 188 mg of ivacaftor, taken by mouth every 12 hours, with fat-containing food. The total daily dose is 300 mg of lumacaftor and 376 mg of ivacaftor.
The entire packet of granules should be dissolved in a teaspoonful of soft food or liquid and consumed completely within one hour of mixing. Do not pre-mix the medication for a future dose. Suitable soft foods include pureed fruits or vegetables, flavoured yogurt or pudding, applesauce, milk, or juice (except grapefruit juice).
The usual dose of this medication for children aged 6 to 11 years old is 2 tablets (each containing 100 mg of lumacaftor and 125 mg of ivacaftor) taken by mouth every 12 hours, with fat-containing food. The total daily dose is 400 mg of lumacaftor and 500 mg of ivacaftor.
The usual dose of this medication for adults and children aged 12 years and older is 2 tablets (each containing 200 mg of lumacaftor and 125 mg of ivacaftor), taken by mouth every 12 hours, with fat-containing food. The total daily dose is 800 mg of lumacaftor and 500 mg of ivacaftor.
Fat-containing food helps get the right amount of medication into your body. Examples include butter or oils, eggs, cheeses, nuts, whole milk, or meats. Meals and snacks recommended in CF guidelines contain the right amounts of fat.
The tablets should be swallowed whole. Do not chew, crush, or break the tablets.
Many things can affect the dose of medication that a person needs, such as body weight, other medical conditions, and other medications. If your doctor has recommended a dose different from the ones listed here, do not change the way that you are taking the medication without consulting your doctor.
It is important to take this medication exactly as prescribed by your doctor. If you miss a dose and it is less than 6 hours since the missed dose, take it as soon as possible with fat-containing food and continue with your regular schedule. If it is less than 6 hours until your next dose, skip the missed dose and continue with your regular dosing schedule. Do not take a double dose to make up for a missed one. If you are not sure what to do after missing a dose, contact your doctor or pharmacist for advice.
Store this medication at room temperature, protect it from light and moisture, and keep it out of the reach of children.
Do not dispose of medications in wastewater (e.g. down the sink or in the toilet) or in household garbage. Ask your pharmacist how to dispose of medications that are no longer needed or have expired.
Who should NOT take this medication?
Do not take this medication if you are allergic to ivacaftor-lumacaftor or any ingredients of the medication.
What side effects are possible with this medication?
Many medications can cause side effects. A side effect is an unwanted response to a medication when it is taken in normal doses. Side effects can be mild or severe, temporary or permanent.
The side effects listed below are not experienced by everyone who takes this medication. If you are concerned about side effects, discuss the risks and benefits of this medication with your doctor.
The following side effects have been reported by at least 1% of people taking this medication. Many of these side effects can be managed, and some may go away on their own over time.
Contact your doctor if you experience these side effects and they are severe or bothersome. Your pharmacist may be able to advise you on managing side effects.
- flu-like symptoms (sudden lack of energy, fever, cough, sore throat)
- runny or stuffy nose
- stomach pain
Although most of the side effects listed below don't happen very often, they could lead to serious problems if you do not seek medical attention.
Check with your doctor as soon as possible if any of the following side effects occur:
- coughing up blood
- decreased heart rate
- increased blood pressure
- irregular menstrual periods (e.g., increased bleeding, pain, other changes)
- shortness of breath
- signs of liver problems (e.g., nausea, vomiting, diarrhea, loss of appetite, weight loss, yellowing of the skin or whites of the eyes, dark urine, pale stools)
- signs of pneumonia (e.g., fever, chills, shortness of breath, cough)
- symptoms of muscle break-down in the body (e.g., muscle pain, dark-coloured urine)
Stop taking the medication and seek immediate medical attention if any of the following occur:
- signs of a serious allergic reaction (e.g., abdominal cramps, difficulty breathing, nausea and vomiting, or swelling of the face and throat)
Some people may experience side effects other than those listed. Check with your doctor if you notice any symptom that worries you while you are taking this medication.
Are there any other precautions or warnings for this medication?
Before you begin using a medication, be sure to inform your doctor of any medical conditions or allergies you may have, any medications you are taking, whether you are pregnant or breast-feeding, and any other significant facts about your health. These factors may affect how you should use this medication.
Blood pressure and heart rate: This medication may cause an increase in blood pressure and a decrease in heart rate. If you have increased blood pressure (hypertension) or a low heart rate, discuss with your doctor how this medication may affect your medical condition, how your medical condition may affect the dosing and effectiveness of this medication, and whether any special monitoring is needed.
Breathing problems: Increased shortness of breath, chest discomfort, and difficulty breathing have been reported by people when they first start to take ivacaftor-lumacaftor. This may be more likely to occur if you have poor lung function. Tell your doctor if your breathing worsens.
Cataracts: Cataracts have been reported in children treated with this medication. Your doctor may recommend eye examinations before and during treatment with this medication.
Kidney function: The safety and effectiveness of ivacaftor-lumacaftor have not been established for people with decreased kidney function or end-stage kidney disease. If you have severely decreased kidney function or kidney disease, discuss with your doctor how this medication may affect your medical condition, how your medical condition may affect the dosing and effectiveness of this medication, and whether any special monitoring is needed.
Liver function: People taking ivacaftor-lumacaftor may have changes in liver function that produce abnormal liver test results and may result in liver damage. Your doctor may recommend regular liver tests while you are taking this medication. If you have severe changes in liver function, your doctor may recommend that you take a lower dose of this medication or stop taking it altogether.
Liver disease or reduced liver function may cause this medication to build up in the body, causing side effects. If you have liver problems, discuss with your doctor how this medication may affect your medical condition and how your medical condition may affect the dosing and effectiveness of this medication. Your doctor will perform regular tests to check your liver function while you are taking this medication.
Pregnancy: The safety and effectiveness of ivacaftor-lumacaftor have not been established for women who are pregnant. This medication should not be used during pregnancy unless the benefits outweigh the risks. Talk to your doctor if you become pregnant while taking this medication.
Breast-feeding: It is not known whether ivacaftor or lumacaftor pass into breast milk. If you are a breast-feeding mother and are taking this medication, it may affect your baby. Talk to your doctor about whether you should continue breast-feeding.
Children: The safety and effectiveness of ivacaftor-lumacaftor have not been established for children less than 2 years old.
Seniors: The safety and effectiveness of ivacaftor-lumacaftor have not been established for seniors 65 years of age and older.
What other drugs could interact with this medication?
There may be an interaction between ivacaftor - lumacaftor and any of the following:
- abiraterone acetate
- alpha-blockers (e.g., alfuzosin, doxazosin, silodosin, tamsulosin)
- anticancer medications (e.g., cabazitaxel, docetaxel, doxorubicin, etoposide, ifosfamide, irinotecan, vincristine)
- antipsychotics (e.g., aripiprazole, clozapine, haloperidol, quetiapine)
- "azole" antifungals (e.g., itraconazole, ketoconazole, voriconazole)
- benzodiazepines (e.g., chlordiazepoxide, clonazepam, diazepam, lorazepam)
- birth control pills (estrogens, progestins)
- calcium channel blockers (e.g., amlodipine, diltiazem, nifedipine, verapamil)
- corticosteroids (e.g., dexamethasone, hydrocortisone, prednisone)
- estrogens (e.g., conjugated estrogen, estradiol, ethinyl estradiol)
- "gliptin" diabetes medications (e.g., linagliptin, saxagliptin, sitagliptin)
- grapefruit juice
- hepatitis C antivirals (e.g., daclatasvir, dasabuvir, ledipasvir, paritaprevir, ombitasvir, sofosbuvir)
- HIV non-nucleoside reverse transcriptase inhibitors (NNRTIs; e.g., delavirdine, efavirenz, etravirine, nevirapine)
- HIV protease inhibitors (e.g., atazanavir, indinavir, ritonavir, saquinavir)
- macrolide antibiotics (e.g., clarithromycin, erythromycin)
- narcotic pain relievers (e.g., codeine, fentanyl, morphine, oxycodone)
- nitrates (e.g., isosorbide dinitrate, isosorbide mononitrate)
- progestins (e.g., dienogest, levonorgestrel, medroxyprogesterone, norethindrone)
- protein kinase inhibitors (e.g., bosutinib, dabrafenib, imatinib, nilotinib)
- proton pump inhibitors (e.g., lansoprazole, omeprazole)
- quinolone antibiotics (e.g., ciprofloxacin, norfloxacin)
- St. John's wort
- selective serotonin reuptake inhibitors (SSRIs; e.g., citalopram, fluoxetine, paroxetine, sertraline)
- "statin" anti-cholesterol medications (e.g., atorvastatin, lovastatin, simvastatin)
- sulfonamide antibiotics ("sulfas"; e.g., sulfisoxazole, sulfamethoxazole)
- sulfonylureas (e.g., glyburide, gliclazide)
- tricyclic antidepressants (e.g., amitriptyline, clomipramine, desipramine, trimipramine)
If you are taking any of these medications, speak with your doctor or pharmacist. Depending on your specific circumstances, your doctor may want you to:
- stop taking one of the medications,
- change one of the medications to another,
- change how you are taking one or both of the medications, or
- leave everything as is.
An interaction between two medications does not always mean that you must stop taking one of them. Speak to your doctor about how any drug interactions are being managed or should be managed.
Medications other than those listed above may interact with this medication. Tell your doctor or prescriber about all prescription, over-the-counter (non-prescription), and herbal medications you are taking. Also tell them about any supplements you take. Since caffeine, alcohol, the nicotine from cigarettes, or street drugs can affect the action of many medications, you should let your prescriber know if you use them.
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