Ivacaftor / lumacaftor Disease Interactions

Cases of non- congenital lens opacities or cataracts have been reported in pediatric patients treated with ivacaftor, and although other risk factors could be present in some cases, a possible risk attributable to this medication cannot be excluded. Baseline and ophthalmological examinations are recommended in pediatric patients initiating treatment. Caution is advised in patients with ophthalmologic conditions.

Elevated transaminases have been reported in patients with cystic fibrosis receiving ivacaftor. It is recommended that liver function tests are assessed prior to treatment, every 3 months during the first year, and annually thereafter. Patients with increased levels should be closely monitored until the abnormalities resolve, and dosing should be interrupted in patients with ALT or AST of greater than 5 times the upper limit of normal (ULN). In patients with hepatic impairment, no dose adjustment is necessary in patients with mild impairment, but a reduced dose of ivacaftor is recommended in patients with moderate impairment (see prescribing information for dosing recommendations). Studies have not been conducted in patients with severe hepatic impairment, but exposure is expected to be higher than in patients with moderate impairment. Therefore, caution should be used on these patients, and a reduced dose is recommended after weighing the risks and benefits of treatment.

Ivacaftor has not been studied in patients with renal impairment. No dose adjustment is necessary for patients with mild or moderate renal impairment, however, caution is recommended in patients with severe renal impairment or end-stage renal disease.

The use of products containing lumacaftor and ivacaftor is associated with an increase in blood pressure. Close monitoring of blood pressure is recommended periodically in all patients, in particular in hypertensive patients.

The use of lumacaftor and ivacaftor has not been studied in patients with cystic fibrosis who have undergone organ transplantation. Use in transplanted patients is not recommended due to potential drug-drug interactions with Immunosuppressants.

The use of products containing lumacaftor and ivacaftor is associated with respiratory events (e.g., chest discomfort, dyspnea, and respiration abnormal). Close monitoring is recommended in patients with advanced lung disease (percent predicted FEV1 < 40).