Indications for KALYDECO:

Treatment of cystic fibrosis (CF) in patients ≥6yrs who have one of the following mutations in the CFTR gene: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, or S549R. Limitations of use: not effective in patients with CF who are homozygous for the F508del mutation in the CFTR gene.

Adults and Children:

<6yrs: not established. Take with fat-containing food (eg, eggs, butter, peanut butter, cheese pizza). ≥6yrs: 150mg every 12 hours. Concomitant strong CYP3A inhibitors: 150mg twice weekly. Concomitant moderate CYP3A inhibitors: 150mg once daily. Moderate hepatic impairment: 150mg once daily. Severe hepatic impairment: 150mg once daily or less frequently.

Pharmacological Class:

Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator.

Warnings/Precautions:

If genotype is unknown, use an FDA-cleared CF mutation test to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test instructions. Assess ALT/AST levels prior to initiating therapy, every 3 months during the first year of treatment, and annually thereafter. If increased ALT/AST levels develop, monitor closely until resolved. Interrupt dosing if ALT/AST is >5×ULN; after resolution, consider restarting. Hepatic impairment. Severe renal impairment or ESRD. Pregnancy (Cat.B). Nursing mothers.

Interactions:

Concomitant strong CYP3A inducers (eg, rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, St. John’s Wort): not recommended. Potentiated by strong CYP3A inhibitors (eg, ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, clarithromycin) and moderate CYP3A inhibitors (eg, fluconazole, erythromycin). May be potentiated by grapefruit and Seville oranges; avoid. Potentiates CYP3A and/or P-gp substrates (eg, digoxin, cyclosporine, tacrolimus).

Adverse Reactions:

Headache, oropharyngeal pain, upper respiratory tract infection, nasal congestion, abdominal pain, nasopharyngitis, diarrhea, rash, nausea, dizziness.

How Supplied:

Tabs—60