VX-770 for the Treatment of Chronic Bronchitis

The study is an open label study of orally-administered ivacaftor in subjects with chronic
bronchitis and/or bronchiectasis. Subjects will be administered the study drug ivacaftor 150
mg twice daily (BID). The study drug is commercially available and will be purchased by the
participant.

Inclusion Criteria:

- Male or Female age 18 years

- A Clinical diagnosis of Bronchiectasis and/or Chronic Bronchitis in the investigators
opinion

- Exhibit symptoms of chronic bronchitis as defined by the Medical Research Council

- FEV1 percent predicted greater than 40 percent Post Bronchodilator

- Clinically stable in the last 4 weeks with no evidence of exacerbation

- Weight of 40 kg to120 kg

- Willingness to use at least one form of acceptable birth control including abstinence,
condom with spermicide, or hormonal contraceptives

- Willing to monitor blood glucose if known history of diabetes mellitus requiring
insulin or medical therapy

- Element of CFTR Dysfunction, as defined by Sweat Chloride

Exclusion

- Daytime use of Oxygen Therapy

- Documented history of drug abuse within the last year

- Subjects should not have a pulmonary exacerbation or changes in therapy for pulmonary
disease within 28 days before receiving the first dose of study drug.

- Cirrhosis or elevated liver transaminases > 3X ULN

- GFR < 50 estimated by Cockroft-Gault

- Any illness or abnormal lab finding 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.

- Pregnant or Breastfeeding

- Subjects taking any inhibitors or inducers of CYP3A4, including certain herbal
medications and grapefruit juice. (Excluded medications and foods including the drugs
and foods are provided in the appendix document)

- Uncontrolled Diabetes

- Clinically significant arrhythmias or conduction abnormalities that in the opinion of
the investigator that affect patient safety