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

Inclusion Criteria:

- Age 18 years or older.

- Documentation of CF diagnosis.

- Have a pre-bronchodilator FEV1 ≥ 40% of predicted at Visit 1 and have a Visit 2
pre-investigational product FEV1 that is ≥ 40% of predicted and within ± 15% of the
Visit 1 result.

- Deemed by the Investigator to be a suitable candidate for serial collection of
expectorated sputum.

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).