Ivacaftor (Kalydeco) and Insulin in Cystic Fibrosis (CF)

Exclusion Criteria

• •established diagnosis of non-CF related diabetes (ie., Type I diabetes)
• •history of clinically symptomatic pancreatitis in past year
• •prior lung or liver transplant
• •severe CF liver disease
• •fundoplication-related dumping syndrome
• •medical co-morbidities that are not CF-related or are unstable per the Investigator opinion
• •acute CF pulmonary exacerbation within 4 weeks prior to study procedures
• •treatment with oral or intravenous corticosteroids within 4 weeks of study
• •hemoglobin \<10g/dL within 90 days of GPA test or at Screening
• •abnormal renal function within 90 days of GPA test or at Screening
• •long-standing CFRD with fasting hyperglycemia, elevated HbA1C (\>8) beyond time surrounding diagnosis of CFRD, significant basal insulin requirement
• •inability to perform study specific procedures (MMTT, GPA).