A Study to Evaluate the Safety and Efficacy of VX-371 in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation

Exclusion Criteria

• •History of any comorbidity, which 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.
• •Any clinically significant laboratory abnormalities at the Screening Visit that would interfere with the study assessments or pose an undue risk for the subject.
• •An acute upper or lower respiratory infection, pulmonary exacerbation, or changes in therapy (including antibiotics) for pulmonary disease within 28 days before Day 1 (first dose of study drug).
• •A 12 lead ECG demonstrating QTcF \>450 msec at the Screening Visit.
• •History of solid organ or hematological transplantation.
• •Used diuretics or renin-angiotensin aldosterone system antihypertensive drugs in the 28 days prior to Screening or an anticipated need for any of these medications during the study.
• •Ongoing or prior participation in an investigational drug study within 30 days of the Screening Visit.
• •Inability to withhold short-acting, long-acting, or once-daily, long-acting bronchodilator use for 4, 12, or 24 hours prior to clinic visit, respectively.
• •History of significant intolerance to inhaled saline, or intolerance to the single dose of saline at Screening
• •Known hypersensitivity or history of intolerance to Orkambi.
• •Pregnant and nursing females.
• •Subjects who have participated in Parion Sciences Study NCT02343445.