A Phase 2 Study to Evaluate Effects of VX-661/Ivacaftor on Lung and Extrapulmonary Systems in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation
A Phase 2 Study to Evaluate Effects of VX-661/Ivacaftor on Lung and Extrapulmonary Systems in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation
To evaluate the clinical mechanisms of action in lung and extrapulmonary systems of VX-661
 (tezacaftor; TEZ) in combination with ivacaftor (IVA) (TEZ/IVA) in participants with cystic
 fibrosis (CF) who are homozygous for the F508del mutation on the CF transmembrane conductance
 regulator (CFTR) gene.
Inclusion Criteria:

 -  Male and female participants, homozygous for the F508del CFTR mutation

 -  Confirmed diagnosis of CF by sweat chloride testing

 -  Forced Expiratory Volume in 1 Second (FEV1) ≥40% and ≤90% of predicted normal for age,
 sex, and height at Screening Visit

 -  Stable CF disease as judged by the investigator.

 Exclusion Criteria:

 -  History of any comorbidity that, in the opinion of the investigator, might confound
 the results of the study or pose an additional risk in administering study drug to the
 subject.

 -  An acute upper or lower respiratory infection, pulmonary exacerbation, or changes in
 therapy for pulmonary disease within 28 days before Day 1

 -  History or evidence of clinically significant findings on ophthalmologic examination
 during the Screening Period.

 -  History of solid organ or hematological transplantation

 -  Pregnant or nursing females

 -  Participants who have had radiation exposure within 1 year before the first
 mucociliary clearance (MCC) procedure that would cause them to exceed federal
 regulations by participating in this study

 -  In the opinion of the investigator, unable to adequately perform inhalation maneuvers
 during the MCC procedures