Effects of an Antioxidant-Enriched Multivitamin Supplement on Inflammation and Oxidative Stress in Cystic Fibrosis

Inclusion Criteria:

- Male or female ≥10 years of age

- Documentation of a Cystic Fibrosis (CF) diagnosis as evidenced by 1 or more clinical
features consistent with the CF phenotype and 1 or more of the following criteria:

- Sweat chloride equal to or greater than 60 milliequivalent (mEq/L) by
quantitative pilocarpine iontophoresis test (QPIT)

- 2 well-characterized mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene

- Pancreatic insufficiency documented by having a spot fecal elastase-1 (FE-1) ≤
100μg/g in a stool sample done either historically or at the screening visit

- Clinically stable with no significant changes in health status within 2 weeks prior
to randomization

- FEV1 ≥ 40 and ≤ 100% of predicted for age based on the Wang (males < 18 years,females
< 16 years) or Hankinson (males ≥ 18 years, females ≥ 16 years) standardized
equations at the screening visit

- Weight ≥ 30 kg at the screening visit

- Able to perform repeatable, consistent efforts in pulmonary function testing

- Able to tolerate sputum induction with 3% hypertonic saline and to expectorate with
induction

- Written informed consent (and assent when applicable) obtained from subject or
subject's legal representative

- Ability to swallow softgel capsules

Exclusion Criteria:

- Subjects being treated with ivacaftor (Kalydeco™)

- Liver enzymes aspartate aminotransferase (AST), alanine aminotransferase (ALT), or
gamma-glutamyl transferase (GGT) > 3 times the upper limits of normal at the
screening visit

- Use of antibiotics (oral, iv, and/or inhaled) for acute respiratory symptoms within 2
weeks prior to randomization

- Active treatment for allergic bronchopulmonary aspergillosis (ABPA)

- Current use of oral corticosteroids in doses exceeding the equivalent of 10 mg
prednisone/day or 20 mg prednisone every other day

- Active treatment for nontuberculous mycobacterial (NTM) infection

- Initiation of any new chronic therapy (e.g., ibuprofen, Pulmozyme®, hypertonic
saline,azithromycin,Tobramycin Inhalation solution (TOBI®), Cayston® within 8 weeks
prior to randomization

- Unwilling to discontinue current oral vitamin and antioxidant supplementation
(e.g.,AquADEKs®, another source of β-carotene, vitamin A, vitamin E or
tocopherols,vitamins D or K, n-acetylcysteine, glutathione, CoQ10, other
over-the-counter antioxidant) for the duration of the study

- Use of vitamins (other than control vitamin) or antioxidants within 4 weeks prior to
randomization

- Daily use of > 2 cans of Boost or Pulmocare dietary supplement formulas

- Known hypersensitivity to oral AquADEKs®

- For women of child bearing potential:

1. positive pregnancy test at Visit 1 or at Visit 2, or

2. lactating or

3. unwilling to practice a medically acceptable form of contraception (acceptable
forms of contraception: abstinence, hormonal birth control, intrauterine device,
or barrier method plus a spermicidal agent)

- 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

- Use of investigational therapies within 4 weeks prior to randomization

- Current tobacco smoker

- Current use of anticoagulant medications

- Severe malnutrition based either on having a BMI less than the 5th percentile for
subjects < 18 years of age or a BMI less than 18 kg/m2 for subjects > 18 years of
age.

- Subjects with poorly controlled CF-related diabetes on active insulin therapy,
defined as having a Glycosylated Hemoglobin (HgbA1c) ≥ 7.5% at the most recent
historic evaluation of HgbA1c