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Ivacaftor: Comprehensive Cystic Fibrosis Treatment & Dosage Guide
Summary of Key Points
Ivacaftor is a prescription medication primarily used to treat cystic fibrosis and other respiratory conditions. It works by improving the function of a specific protein in the body, which helps reduce symptoms. Ivacaftor is typically taken orally and is available in different strengths. It is essential to follow your healthcare provider’s instructions regarding dosage and administration. If you have any questions, our telemedicine providers are here to help.
Names of Medication
US Brand Name: Kalydeco (Ivacaftor)
The generic name refers to the active ingredient in the medication, listed in parentheses. This represents the chemical name of the drug, while the brand name is assigned by the manufacturer.
Pharmacologic Category
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator
Ivacaftor Dosage Guidelines
Ivacaftor is used to treat several conditions, primarily related to cystic fibrosis and other respiratory diseases. Below are the dosing guidelines for adults. Always consult your healthcare provider before making any changes to your medication regimen. If you need assistance, our telemedicine services are available.
Cystic Fibrosis Treatment
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: Long-term, as prescribed by your healthcare provider
Bronchiectasis
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: As determined by your healthcare provider
Pancreatic Insufficiency
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: As prescribed by your healthcare provider
Chronic Obstructive Pulmonary Disease (COPD)
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: Based on your doctor’s recommendation
Asthma
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: As directed by your healthcare provider
Respiratory Infections
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: As prescribed by your healthcare provider
Diabetes Mellitus
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: As determined by your healthcare provider
Liver Disease
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: Based on your doctor’s recommendation
Sinusitis
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: As directed by your healthcare provider
Allergic Rhinitis
Dosage: 150 mg taken orally every 12 hours
Length of Treatment: Based on your healthcare provider’s advice
Dosage Forms and Strengths
Ivacaftor is available in the following forms:
- Tablets: 150 mg
- Granules: 50 mg, 75 mg (for pediatric use)
Administration Instructions for Ivacaftor
Ivacaftor should be taken with a fat-containing meal, such as eggs, avocados, or nuts, to improve absorption. It is important to take the medication at the same time each day, approximately 12 hours apart. Do not crush or chew the tablets. If you miss a dose and it has been less than 6 hours, take it as soon as you remember. If more than 6 hours have passed, skip the missed dose and continue with your regular schedule. Never take two doses at once.
Contraindications for Ivacaftor
A contraindication is a specific situation where a medication should not be used because it may cause harm. If you have any of the following conditions, Ivacaftor may not be safe for you. Always consult your healthcare provider before starting any new medication. If you’re unsure, you can schedule a telemedicine appointment with one of our providers today.
Liver Disease: Ivacaftor is processed by the liver, and if you have moderate to severe liver disease, your body may not metabolize the medication properly. This can lead to higher levels of the drug in your system, increasing the risk of side effects.
Allergic Reaction to Ivacaftor: If you have had a previous allergic reaction to Ivacaftor or any of its ingredients, you should not take it. Allergic reactions can range from mild rashes to severe, life-threatening conditions like anaphylaxis.
Severe Renal (Kidney) Impairment: Although Ivacaftor is primarily processed by the liver, patients with severe kidney impairment may also have difficulty clearing the drug from their system, leading to potential toxicity.
Drug Interactions with Ivacaftor
Ivacaftor can interact with other medications, which may either increase the risk of side effects or reduce the drug’s effectiveness. Be sure to inform your healthcare provider about all medications you are currently taking, including over-the-counter drugs and supplements. Below are some common medications that may interact with Ivacaftor:
Rifampin (Rifadin): This antibiotic can significantly reduce the effectiveness of Ivacaftor by speeding up its breakdown in the liver.
Ketoconazole (Nizoral): This antifungal medication can increase the levels of Ivacaftor in your blood, raising the risk of side effects.
Carbamazepine (Tegretol): This medication, used to treat seizures, can reduce the effectiveness of Ivacaftor by increasing its metabolism in the liver.
Phenytoin (Dilantin): Another anti-seizure medication that can lower Ivacaftor levels in your body, making it less effective.
St. John’s Wort: This herbal supplement can also reduce Ivacaftor’s effectiveness by speeding up its breakdown.
Ivacaftor in Pregnancy
Is Ivacaftor safe to use during pregnancy? The answer remains uncertain due to limited data on its effects in pregnant women. While animal studies have not demonstrated harm to the fetus, there is a lack of comprehensive human studies. If you are pregnant or planning to become pregnant, it’s crucial to have a detailed discussion with your healthcare provider. Together, you can carefully weigh the potential benefits of continuing the medication against any possible risks to your baby. If you need guidance, consider scheduling a telemedicine consultation with one of our providers today.
Ivacaftor while Breastfeeding
Is Ivacaftor safe to take while breastfeeding? The safety of Ivacaftor during breastfeeding has not been well established. It is currently unknown whether Ivacaftor passes into breast milk or what effects it might have on a nursing infant. If you are breastfeeding or planning to breastfeed, it’s important to consult your healthcare provider to determine the best course of action. You can also schedule a telemedicine consultation with one of our providers to discuss your options in more detail.
Estimated Cost of Ivacaftor Medication
The cost of Ivacaftor can vary depending on your location and insurance coverage. Without insurance, a 30-day supply of Ivacaftor (150 mg tablets) is estimated to cost around $23,000 when using a GoodRX coupon. To find the most affordable option, check with your pharmacy or use a prescription discount service like GoodRX to compare prices for this cystic fibrosis medication.
Possible Alternatives to Ivacaftor
If Ivacaftor is not suitable for you, or if you’re exploring other treatment options, there are alternatives depending on the condition being treated. Always consult your healthcare provider before making any changes to your treatment plan. If you need further guidance, you can schedule a telemedicine appointment with one of our providers.
Alternative Treatments for Cystic Fibrosis
Cystic Fibrosis: Other CFTR modulators, such as Lumacaftor/Ivacaftor (Orkambi) or Tezacaftor/Ivacaftor (Symdeko), may be considered. Additionally, airway clearance techniques, inhaled medications, and nutritional support play a vital role in managing cystic fibrosis.
Alternative Treatments for Bronchiectasis
Bronchiectasis: Treatment options include antibiotics, bronchodilators, and chest physiotherapy to help clear mucus from the lungs.
Alternative Treatments for Pancreatic Insufficiency
Pancreatic Insufficiency: Pancreatic enzyme replacement therapy (PERT) is commonly used to aid digestion. Dietary adjustments, such as eating smaller, more frequent meals, can also be beneficial.
Alternative Treatments for Chronic Obstructive Pulmonary Disease (COPD)
Chronic Obstructive Pulmonary Disease (COPD): Inhalers, bronchodilators, and lifestyle changes, such as quitting smoking and participating in pulmonary rehabilitation, are effective ways to manage COPD.
Alternative Treatments for Asthma
Asthma: Common treatments include inhaled corticosteroids, bronchodilators, and avoiding known triggers.
Alternative Treatments for Respiratory Infections
Respiratory Infections: Depending on the cause, antibiotics or antiviral medications may be prescribed. Staying hydrated and getting plenty of rest are also important for recovery.
Alternative Treatments for Diabetes Mellitus
Diabetes Mellitus: Insulin therapy, oral medications, and lifestyle changes, such as diet and exercise, are key components of diabetes management.
Alternative Treatments for Liver Disease
Liver Disease: Treatment depends on the underlying cause and may include medications, lifestyle changes, or, in severe cases, liver transplantation.
Alternative Treatments for Sinusitis
Sinusitis: Nasal sprays, antihistamines, and sometimes antibiotics can help manage sinusitis. Avoiding allergens and using a humidifier may also provide relief.
Alternative Treatments for Allergic Rhinitis
Allergic Rhinitis: Antihistamines, nasal corticosteroids, and avoiding allergens are common treatments for allergic rhinitis.
Recent Updates on Ivacaftor Research
Recent studies on Ivacaftor have focused on expanding its use to a broader range of cystic fibrosis patients. Research indicates that Ivacaftor can improve lung function and reduce pulmonary exacerbations in patients with specific CFTR gene mutations. Ongoing clinical trials are investigating the long-term effects of Ivacaftor on lung health, quality of life, and life expectancy in cystic fibrosis patients. Additionally, researchers are exploring Ivacaftor’s potential use in other conditions involving CFTR mutations, such as bronchiectasis and certain liver diseases. If you’re interested in how new research may impact your treatment, schedule a telemedicine visit with one of our providers today.
