"The U.S. Food and Drug Administration yesterday approved Ruconest, the first recombinant C1-Esterase Inhibitor product for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).
KALYDECO is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator indicated for the treatment of cystic fibrosis (CF) in patients age 6 years and older who have one of the following mutations in the CFTR gene: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, or S549R. If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of a CFTR mutation followed by verification with bidirectional sequencing when recommended by the mutation test instructions for use.
Limitations of Use
KALYDECO is not effective in patients with CF who are homozygous for the F508del mutation in the CFTR gene.
DOSAGE AND ADMINISTRATION
Dosing Information In Adults And Children Ages 6 Years And Older
The recommended dose of KALYDECO for both adults and pediatric patients age 6 years and older is one 150 mg tablet taken orally every 12 hours (300 mg total daily dose) with fat-containing food. Examples of appropriate fat-containing foods include eggs, butter, peanut butter, cheese pizza, etc. [see CLINICAL PHARMACOLOGY and PATIENT INFORMATION].
Dosage Adjustment For Patients With Hepatic Impairment
The dose of KALYDECO should be reduced to 150 mg once daily for patients with moderate hepatic impairment (Child-Pugh Class B). KALYDECO should be used with caution in patients with severe hepatic impairment (Child-Pugh Class C) at a dose of 150 mg once daily or less frequently [see Use in Specific Populations, CLINICAL PHARMACOLOGY, and PATIENT INFORMATION].
Dosage Adjustment For Patients Taking Drugs That Are CYP3A Inhibitors
When KALYDECO is being co-administered with strong CYP3A inhibitors (e.g., ketoconazole), the dose should be reduced to 150 mg twice a week. The dose of KALYDECO should be reduced to 150 mg once daily when co-administered with moderate CYP3A inhibitors (e.g., fluconazole). Food containing grapefruit or Seville oranges should be avoided [see DRUG INTERACTIONS, CLINICAL PHARMACOLOGY, and PATIENT INFORMATION].
Dosage Forms And Strengths
150 mg tablets.
Storage And Handling
KALYDECO® (ivacaftor) is supplied as light blue, film-coated, capsule-shaped tablets containing 150 mg of ivacaftor. Each tablet is printed with the characters “V 150” on one side and plain on the other, and is packaged as follows:
56-count carton (contains 4 individual blister cards of 14 tablets per card) NDC 51167-200-01
60-count bottle NDC 51167-200-02
Store at 20-25°C (68-77°F); excursions permitted to 15-30°C (59-86°F) [see USP Controlled Room Temperature].
Manufactured for: Vertex Pharmaceuticals Incorporated 50 Northern Avenue Boston, MA 02210. Approved June 2014
Last reviewed on RxList: 7/15/2014
This monograph has been modified to include the generic and brand name in many instances.
Additional Kalydeco Information
Report Problems to the Food and Drug Administration
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.
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