Inclusion Criteria:

Subjects must meet all of the following inclusion criteria to be eligible for participation in this study:

• ≥ 6 years of age

• Subject has CF as diagnosed by one of the following:

  • Documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis test
  • Two well characterized genetic mutations in the CFTR gene
  • Abnormal nasal potential difference with accompanying symptoms characteristic of CF

• At high risk for disease progression as defined by one of the following subject populations:

  • First Cohort Criteria:

    • Those who are wait listed or eligible for lung transplant based on FEV1 criteria. Patients who have a level of lung function impairment consistent with lung transplantation criteria, but who are ineligible for transplantation for other reasons, can enroll in this program; or
    • Completed participation in CP-AI-006 (through Visit 20). Subjects who withdraw from CP-AI-006 prior to completing all courses of AZLI and all study visits will not be eligible for this protocol.

  • Second Cohort Criteria:

    • Meets first cohort criteria
    • FEV1 ≤ 40% predicted at the time of consent

  • Third Cohort Criteria:

    • Meets first or second cohort criteria
    • FEV1 ≤ 50% predicted at the time of consent

Exclusion Criteria:

Subjects who meet any of the following exclusion criteria are not to be enrolled in this study:

• Subjects with any serious or active medical or psychiatric illness that, in the opinion of the investigator, would interfere with subject treatment, assessment or compliance with the protocol or dosing requirements
• Subjects with hypersensitivity to any of the components of the drug product
• Currently enrolled in another clinical trial
• Pregnant or lactating females