Prednisone in Cystic Fibrosis Pulmonary Exacerbations

Inclusion criteria

• Diagnosis of CF by newborn screening or at least one clinical feature of CF, AND either (a) or (b) as follows:
• A documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis
• A genotype with two identifiable CF-causing mutations
• Age > 6 years old.
• Acute pulmonary exacerbation treated with IV antibiotics as previously defined 10% relative drop in FEV1 from baseline at the time of exacerbation
• Informed consent by patient or parent/legal guardian
• Ability to reproducibly perform pulmonary function testing
• Ability to comply with medication use including the ability to take capsules, study visits and study procedures as judged by the site investigator

Exclusion criteria

• A respiratory tract culture positive for Burkholderia cenocepacia in the 12 months prior to enrollment
• A respiratory tract culture positive for Mycobacterium abscessus in the 12 months prior to enrollment
• Treatment with IV or oral corticosteroids within 2 weeks of enrollment or from Day 0-Day 7 of the pulmonary exacerbation
• Active allergic bronchopulmonary aspergillosis (ABPA) at the time of enrollment as determined by treating physician
• Asthma related exacerbation at enrollment as defined by the treating physician based on clinically compatible symptoms (eg. wheeze)
• History of avascular necrosis or pathologic bone fracture
• Uncontrolled hypertension with end organ damage
• Active gastrointestinal bleeding
• Status post lung or other organ transplantation
• Pregnancy
• Lactose intolerance (contained in placebo)
• On Lumacaftor-Ivacaftor (Orkambi) at the time of exacerbation
• Investigational drug use within 30 days prior to enrollment visit
• Physical findings that would compromise the safety of the subject or the quality of the study data as determined by site investigator