NAC Phase IIB: A Multi-Center, Phase IIB, Randomized, Placebo-controlled, Double-Blind Study Of The Effects Of N-Acetylcysteine On Redox Changes and Lung Inflammation In Cystic Fibrosis Patients

Inclusion criteria

• Male or female 7 years of age or older
• Diagnosis of CF based upon the following criteria:

• One or more clinical features characteristic of CF AND (b or c)
• Positive sweat test > 60 mEq/L by quantitative pilocarpine iontophoresis
• A genotype with two identifiable mutations consistent with CF
• Written informed consent (and assent when applicable) obtained from subject or subject's legal representative
• Clinically stable with no evidence of acute upper or lower respiratory tract infection within 4 weeks prior to enrollment
• Stable mild or moderately severe lung disease defined by an FEV1 > or = 40% and < or = 85% predicted for age based on the Wang (males < 18 years, females < 16 years) or Hankinson (males > or = 18 years, females > or = 16 years) standardized equations
• Able to tolerate sputum induction with 3% hypertonic saline and to expectorate
• Able to perform repeatable, consistent efforts in pulmonary function testing
• Weight > or = 25 kg at time of enrollment
• Females of child bearing potential must be willing to use birth control (IUD, oral, transdermal, or parenteral contraceptives; abstinence)

Exclusion criteria

• Clinically significant liver enzymes (AST, ALT or GGT) > 2.5 times the upper limit of normal at screening
• History of ABPA, unless have evidence of a stable IgE (< 5% increase compared to previous test) for 6 months prior to enrollment
• Current or history of rheumatic or collagen vascular disorders
• Use of NSAIDS other than for chronic therapy within 1 week prior to enrollment
• Initiation of chronic therapy with ibuprofen, azithromycin, TOBI® or Aztreonam within 6 weeks prior to enrollment
• Consumption or inhalation of antioxidants (including NAC, GSH, Immunocal, Nacystelyn, pentoxyfilline) within 6 weeks prior to enrollment
• Use of oral or IV corticosteroids within 4 weeks prior to enrollment
• Use of acetaminophen within 3 days prior to enrollment
• Unable to forego during the study:

• Vitamin E: more than 400 IU/day for subjects < or = 12 years of age and 800 IU/day for subjects > 12 years of age
• Vitamin C: more than 0.5 gm/day
• More than two alcoholic drinks per day
• Known hypersensitivity to oral PharmaNAC®
• Current cigarette consumption
• Pregnant or breastfeeding
• Subject unlikely to complete the study as determined by the Investigator
• Any condition that the Investigator believes would interfere with the intent of this study or would make participation not in the best interest of the subject
• Participation in trials for other anti-inflammatory or therapeutic investigational drugs within 6 weeks prior to enrollment