Improving P. Aeruginosa Detection With Breath-based Diagnostics (IMPACT-Breath)

Inclusion Criteria:

• Aim 1, Cross-Sectional IInclusion Criteria:

1. Male or female, ages 8 years and older
2. Diagnosis of CF based on sweat chloride ≥ 60 mmol/L and/or 2 known CF mutations
3. @ BC Children's Hospital only- Non-expectorators are eligible if having a clinicallyindicated OP swab.
4. FEV1 ≥ 30% predicted for spontaneous expectorators or FEV1 ≥ 40% predicted forsubjects undergoing sputum induction
5. Either P. aeruginosa (Pa) negative or chronically infected with P. aeruginosa (Papositive) as defined below: a. P. aeruginosa negative: must meet one of the following criteria: i: No growth of Pain previous 12 months and ≥ 4 consecutive Pa-negative cultures, OR ii: No history ofPa positive airway cultures (sputum, OP, Bronchoalveolar lavage) b. P. aeruginosapositive i: Over 50% of cultures positive and at least 2 cultures positive for Pa inprevious 18 months
6. Willing to participate in and comply with the study procedures and willingness of thesubject, parent or legally authorized representative to provide written informedconsent.

Exclusion criteria:

1. Age < 8 years
2. Intermittently infected with Pa
3. FEV1 < 30%
4. History of lung transplant
5. Presence of a condition or abnormality that in the opinion of the PrincipalInvestigator would compromise the safety of the subject or the quality of the data. Aim 2, Longitudinal Inclusion Criteria, Expectorating Cohort (n=48):
6. Male or female, ages 8-16 years
7. Diagnosis of CF based on sweat chloride ≥ 60 mmol/L and/or 2 known CF mutations
8. @ BC Children's Hospital only- Non-expectorators are eligible if having a clinicallyindicated OP swab.
9. FEV1 ≥ 30% predicted for spontaneous expectorators or FEV1 ≥ 40% predicted forsubjects undergoing sputum induction
10. P. aeruginosa negative, based on one of the following criteria:
11. No growth of Pa in previous 12 months and ≥ 4 consecutive Pa-negative cultures
12. No history of Pa positive airway cultures (sputum, OP, bronchoalveolar lavage)
13. Willing to participate in and comply with the study procedures and willingness of thesubject, parent or legally authorized representative to provide written informedconsent. Exclusion criteria:
14. Age < 8 years
15. Intermittently or chronically infected with Pa
16. Unable to expectorate sputum or undergo sputum induction
17. FEV1 < 30%
18. History of lung transplant
19. Presence of a condition or abnormality that in the opinion of the PrincipalInvestigator would compromise the safety of the subject or the quality of the data. Inclusion Criteria, Non-Expectorating Cohort (n=10):
20. Male or female, ages 3-8 years
21. Diagnosis of CF based on sweat chloride ≥ 60 mmol/L and/or 2 known CF mutations 3.FEV1 ≥ 30%
22. Unable to expectorate sputum 5. P. aeruginosa negative, based on one of the followingcriteria:
23. No growth of Pa in previous 12 months and ≥ 4 consecutive Pa-negative cultures
24. No history of Pa positive airway cultures (sputum, OP, bronchoalveolar lavage) 6.Willing to participate in and comply with the study procedures and willingness of thesubject, parent or legally authorized representative to provide written informedconsent. Exclusion criteria:
25. Age < 3 years
26. Intermittently or chronically infected with Pa
27. FEV1 < 30%
28. History of lung transplant
29. Presence of a condition or abnormality that in the opinion of the PrincipalInvestigator would compromise the safety of the subject or the quality of the data.