What is Kalydeco (ivacaftor) for?

Kalydeco (ivacaftor)* is indicated for individuals aged 4 months and above who have cystic fibrosis (CF) and possess at least one responsive mutation in their CF gene. This medication is conveniently obtainable in tablet formulation, with each tablet containing 150 mg of ivacaftor.

How does Kalydeco (ivacaftor) work?

The formation of thick mucus in the lungs, digestive tract, and other parts of the body characterizes Cystic Fibrosis (CF), a serious genetic disorder that leads to severe respiratory and digestive problems, infections, and diabetes. Mutations in the CFTR gene are responsible for this disease as it makes a protein called ‘cystic-fibrosis transmembrane conductance regulator’ (CFTR), which regulates the production of mucus and digestive juices. These mutations reduce the number of CFTR protein channels on the cell surface or affect their functioning, leading to abnormally thick and sticky mucus and digestive juices.

Kalydeco (ivacaftor)* enhances the transport of ions by boosting the activity of damaged CFTR protein, reducing disease symptoms by making mucus and digestive juice less thick.

How is Kalydeco (ivacaftor) taken?

For patients aged four months and above, the standard dosage for Kalydeco (ivacaftor)* is:
When used as a monotherapy:
– Taken orally twice daily
– One tablet every 12 hours with food containing fat
When used in combination with tezacaftor/ivacaftor for adults and adolescents aged six and above:
– Taken orally once daily
– One tezacaftor 100mg/ivacaftor 150 mg tablet orally in the morning and a Kalydeco (ivacaftor) tablet every evening approximately 12 hours apart with food containing fat
The dosage of Kalydeco (ivacaftor)* should be adjusted for patients with moderate or severe hepatic impairment, as well as for patients co-administered with strong or moderate CYP3A inhibitors. Dosing intervals should be adjusted based on clinical response and tolerability. For moderate hepatic impairment, the dosage is one tablet of ivacaftor 150mg once daily, while patients with severe hepatic impairment should take one tablet of ivacaftor 150mg every other day. When co-administered with strong CYP3A inhibitors, one tablet of Kalydeco (ivacaftor)* should be taken twice a week, while with moderate CYP3A inhibitors, one tablet should be taken once daily.
Further details on the administration and dosage of Kalydeco (ivacaftor)* are included in the official prescribing information, which can be found in our resources section.
Note: Please consult your treating physician for personalized dosing and any potential drug interactions.

Are there any known side effects of Kalydeco (ivacaftor)?

Yes, there are known side effects of Kalydeco (ivacaftor). The most commonly reported side effects include headache, upper respiratory tract infection, stomach pain, diarrhea, nausea, dizziness, and rash. Other less common side effects that have been reported include elevated liver enzymes, allergic reactions, and respiratory events. It is important to note that not all individuals will experience side effects, and some may experience different or no side effects. It is important to discuss any concerns about potential side effects with a healthcare provider.