Safety and Tolerability Trial of Inhaled Alpha1-Proteinase Inhibitor (Human), Hydrophobic Chromatography Process (Alpha-1 HC) in Subjects With Cystic Fibrosis

Exclusion Criteria

• •Had a pulmonary exacerbation during the 4 weeks before screening (Visit 1) which required the initiation of new antibiotic treatment
• •Have a pulmonary exacerbation during the screening period (between Visit 1 and Visit 2) which requires the initiation of new antibiotic treatment
• •FEV1 \< 0.59 liters at the screening visit
• •Respiratory insufficiency with continuous supplemental oxygen therapy, or carbon dioxide retention
• •Elevated aspartate transaminase (AST) or alanine aminotransferase (ALT) that is ≥ 3 times the upper limit of normal for age and gender
• •Smoking during the past 6 months
• •Lung surgery during the past 2 years
• •Positive culture for Burkholderia cepacia or mycobacterium during the past two years.
• •Active allergic bronchopulmonary aspergillosis
• •Pre-treatment sputum collection at Visit 1 or Visit 2 (Randomization) characterized by problems such as inadequate sputum volume or quality.
• •Known selective Immunoglobulin A (IgA) deficiency with known antibody against IgA (anti-IgA antibody).
• •History of anaphylaxis or severe systemic response to any plasma-derived alpha1-proteinase inhibitor preparation or other blood product(s), or to polysorbates.
• •Use of chronic oral steroids during the study. Note: Inhaled corticosteroids that had been administered for at least 4 weeks prior to Visit 1 were permissible during the study.
• •Use of chronic, high dose ibuprofen therapy within 3 weeks of screening and at anytime during the study.
• •Chronic maintenance therapy with systemic antibiotics within 3 weeks of screening and through last dose of investigational product.
• •Use of leukotriene synthesis inhibitor (zileuton) or leukotriene receptor antagonists (montelukast, zafirlukast) within 3 weeks of screening and at anytime during the study.
• •Use of roflumilast within 3 weeks of screening and at any time during the study.
• •Initiation of a new chronic medication or dosage change of a chronic medication for treatment of cystic fibrosis (example: Kalydeco™ \[ivacaftor\]) within 3 weeks of screening (Visit 1).