Utilizing Exhaled Breathe Condensate Collection to Study Ion Regulation in Cystic Fibrosis

Modifying Genes in Cystic Fibrosis: The Beta-2 Adrenergic Receptors and Epithelial Na+ Channels

Our aims were to determine if exhaled breath condensate (EBC) could detect differences in ion regulation between cystic fibrosis (CF) and healthy and measure the effect of the albuterol on EBC ions in these populations. We hypothesized EBC chloride and sodium would be lower in CF patients at baseline and that albuterol would decrease EBC sodium and increase EBC chloride.

Inclusion Criteria:

Healthy subjects:

• no cardiovascular abnormalities
• not overweight BMI>25
• 18-55 years of age

CF subjects:

• mild to moderate CF (FEV1>40% predicted)
• clinically diagnosed with positive sweat test (sweat Cl->60mmol/L)
• 10-55 years of age
• clinically stable

Exclusion Criteria:

Healthy subjects will be excluded if:

• If unable to consent for him/herself (cognitive impairment)
• Have a history or evidence of cardiovascular and/or pulmonary abnormalities.
• Have an abnormal 12-lead EKG
• Have an abnormal pulmonary function test
• Have a history of asthma
• Have a history of renal disease or estimated creatinine clearance < 55ml/min
• Women who are pregnant or planning to become pregnant during the study

CF subjects:

• If unable to consent for him/herself (cognitive impairment)
• Physically unable to perform exercise or breathing tests
• Have a history of renal disease or estimated creatinine clearance < 55ml/min
• Women who are pregnant or planning to become pregnant during the study.
• Have an abnormal 12-lead EKG
• Cystic Fibrosis related diabetes is uncontrolled
• Forced Expiratory Volume after 1 second (FEV1) is less than 40% predicted
• Have a history of joint disease
• Have history of pulmonary exacerbation within the last two weeks
• Experienced pulmonary hemorrhage within 6 months resulting in greater than 50cc of blood in the sputum
• not currently enrolled in any other research study