Ivacaftor

Drug Status

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Government Approvals

US(FDA), UK(BNF)

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WHO Essential Medicine

NO

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Known Teratogen

NO

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Pharmaceutical Class

None

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Controlled Drug Substance

NO

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Drug Ingredient

Ivacaftor

Summary

  • Ivacaftor is used to treat cystic fibrosis in patients who have specific genetic mutations. It helps manage symptoms and improve quality of life but is not a cure.

  • Ivacaftor works by improving the function of a protein called CFTR. This helps reduce the buildup of thick mucus in the lungs, a common problem in cystic fibrosis.

  • Adults and children aged 6 years and older usually take 150 mg of Ivacaftor orally every 12 hours with food containing fat. For younger children, the dose is weight-based.

  • Common side effects of Ivacaftor include headache, upper respiratory tract infection, and abdominal pain. Serious adverse effects can include elevated liver enzymes and hypersensitivity reactions.

  • Ivacaftor can interact with certain medications and dietary supplements, so inform your doctor about everything you're taking. It's not recommended for people with severe liver impairment. Regular monitoring of liver enzymes is required.

Indications and Purpose

How does Ivacaftor work?

Ivacaftor works by improving the function of the CFTR protein, which regulates the flow of salt and fluids in and out of cells. Think of it like opening a gate that allows salt and water to move freely, helping to thin mucus in the lungs. This action improves lung function and reduces respiratory symptoms in cystic fibrosis patients with specific CFTR gene mutations.

Is Ivacaftor effective?

Ivacaftor is effective in treating cystic fibrosis in patients with specific CFTR gene mutations. It works by improving the function of the CFTR protein, which helps regulate the flow of salt and fluids in and out of cells. Clinical studies show Ivacaftor improves lung function, reduces respiratory symptoms, and decreases the frequency of pulmonary exacerbations. These results support its effectiveness in managing cystic fibrosis.

What is Ivacaftor?

Ivacaftor is a medication used to treat cystic fibrosis, a genetic disorder that affects the lungs and digestive system. It belongs to a class of drugs called CFTR modulators, which help improve the function of the CFTR protein. This protein regulates the flow of salt and fluids in and out of cells. Ivacaftor is used in patients with specific CFTR gene mutations to improve lung function and reduce respiratory symptoms.

Directions for Use

For how long do I take Ivacaftor?

Ivacaftor is usually a long-term medication for managing cystic fibrosis, a chronic condition. You'll typically take Ivacaftor every day as a lifelong treatment unless your doctor suggests otherwise. Stopping this medication without medical advice could cause your condition to worsen. How long you'll need this medication depends on your body's response, any side effects you experience, and changes in your overall health.

How do I dispose of Ivacaftor?

To dispose of Ivacaftor, bring unused medicines to a drug take-back program or collection site at a pharmacy or hospital. They will dispose of this medicine properly so it doesn't harm people or the environment. If you can't find a take-back program, you can throw most medicines in the trash at home. But first, take them out of their original containers, mix them with something undesirable like used coffee grounds, seal the mixture in a plastic bag, and throw it away.

How do I take Ivacaftor?

Take Ivacaftor as prescribed by your doctor, usually twice daily, 12 hours apart. It's important to take it with a fat-containing meal or snack to help your body absorb the medication better. Do not crush or chew the tablets. If you miss a dose, take it as soon as you remember unless it's within 6 hours of your next dose. In that case, skip the missed dose and continue your regular schedule. Avoid taking two doses at once.

How long does it take for Ivacaftor to start working?

Ivacaftor begins working in your body shortly after you take it, with improvements in lung function often noticeable within a few weeks. The full therapeutic effect may take several months to achieve. How quickly the medication works can depend on individual factors like your specific CFTR mutation and overall health. Take it exactly as prescribed for the best results.

How should I store Ivacaftor?

Store Ivacaftor at room temperature, away from moisture and light. Keep it in its original container with the lid tightly closed. Avoid storing it in humid places like bathrooms. Always keep Ivacaftor out of reach of children to prevent accidental ingestion. Check the expiration date regularly and properly dispose of any unused or expired medication.

What is the usual dose of Ivacaftor?

The usual dose of Ivacaftor for adults and children over 6 years old is 150 mg taken twice daily, 12 hours apart. It's important to take each dose with a fat-containing meal or snack. Your doctor may adjust the dose based on your specific health needs or if you have liver problems. Always follow your doctor's specific dosing instructions for your personal health needs.

Warnings and Precautions

Can I take Ivacaftor with other prescription drugs?

Ivacaftor can interact with certain medications, including strong CYP3A inhibitors like ketoconazole, which can increase Ivacaftor levels in the blood and lead to more side effects. It's important to inform your doctor about all medications you're taking to avoid interactions that could increase the risk of adverse effects or reduce the effectiveness of Ivacaftor. Your doctor can adjust your treatment plan to ensure safety.

Can Ivacaftor be taken safely while breastfeeding?

The safety of Ivacaftor while breastfeeding is not well-established due to limited evidence. It's unclear if Ivacaftor passes into human breast milk. If you're taking Ivacaftor and want to breastfeed, talk with your doctor about safer medication options that would allow you to nurse your baby safely. Your doctor can help you weigh the benefits and risks of continuing Ivacaftor while breastfeeding.

Can Ivacaftor be taken safely while pregnant?

The safety of Ivacaftor during pregnancy is not well-established due to limited evidence. Animal studies have shown some risks, but human data is lacking. If you're pregnant or planning to become pregnant, talk with your doctor about the safest way to manage your condition. Your doctor can help create a pregnancy-specific treatment plan that protects both you and your baby.

Does Ivacaftor have adverse effects?

Adverse effects are unwanted reactions to a medication. Common adverse effects of Ivacaftor include headache, dizziness, and stomach pain. These occur in a small percentage of users. Serious side effects can include liver problems and cataracts. If you notice symptoms like yellowing of the skin or eyes, or vision changes, contact your doctor immediately. Always inform your doctor about any new or worsening symptoms while taking Ivacaftor.

Does Ivacaftor have any safety warnings?

Ivacaftor has important safety warnings. It can affect liver function, so regular liver tests are recommended. If you experience symptoms like nausea, vomiting, or yellowing of the skin, contact your doctor immediately. Ivacaftor can also cause cataracts in children, so regular eye exams are advised. Not adhering to these warnings can lead to serious health issues. Always follow your doctor's advice and report any new symptoms.

Is it safe to drink alcohol while taking Ivacaftor?

It's best to avoid alcohol while taking Ivacaftor. Alcohol can affect liver function, and since Ivacaftor can also impact the liver, combining the two might increase the risk of liver problems. If you do choose to drink occasionally, limit how much alcohol you consume and watch for warning signs like nausea or yellowing of the skin. Talk with your doctor about alcohol use while taking Ivacaftor to get personalized advice.

Is it safe to exercise while taking Ivacaftor?

You can exercise while taking Ivacaftor, but keep a few things in mind. Ivacaftor can cause dizziness, which might affect your ability to exercise safely. If you feel dizzy or lightheaded during exercise, slow down or stop and rest. Drink plenty of water before, during, and after physical activity. Most people can maintain their regular exercise routine while taking Ivacaftor, but check with your doctor if you have concerns.

Is it safe to stop Ivacaftor?

Ivacaftor is usually taken long-term for managing cystic fibrosis. Stopping it suddenly can lead to a worsening of your condition. Always talk with your doctor before stopping Ivacaftor. They might suggest gradually reducing your dose or switching to a different medication to keep your condition under control. Your doctor will help you make any medication changes safely to protect your health.

Is Ivacaftor addictive?

Ivacaftor is not addictive or habit-forming. This medication doesn't cause dependency or withdrawal symptoms when you stop taking it. Ivacaftor works by affecting the CFTR protein to improve lung function in cystic fibrosis patients. This mechanism doesn't affect brain chemistry in ways that could lead to addiction. If you have concerns about medication dependence, you can feel confident that Ivacaftor doesn't carry this risk.

Is Ivacaftor safe for the elderly?

Elderly patients may be more vulnerable to the side effects of Ivacaftor, especially liver problems. It's important for older adults to have regular liver function tests while taking this medication. Specific risks or negative outcomes more frequently observed among elderly users include dizziness and liver issues. Always consult your doctor about the safety of Ivacaftor for elderly patients.

What are the most common side effects of Ivacaftor?

Side effects are unwanted reactions that can happen when taking a medication. Common side effects of Ivacaftor include headache, dizziness, and stomach pain. These effects vary from person to person. If you notice new symptoms after starting Ivacaftor, they might be temporary or unrelated to the medication. Talk with your doctor before stopping any medication.

Who should avoid taking Ivacaftor?

Don't take Ivacaftor if you're allergic to it or its ingredients. Serious allergic reactions, which cause rash, hives, or swelling that makes breathing difficult, require immediate medical help. Ivacaftor should be used with caution in people with liver problems, as it can affect liver function. Always consult your doctor about these concerns and follow their advice on using Ivacaftor safely.