An open label non-randomized study enrolling up to 20 healthy participants and up to 30 participants with cystic fibrosis to establish a healthy versus disease comparison. Each participant will receive a mixture of inert gas (perfluoropropane (PFP)) in a ratio of 79% PFP to 21% oxygen as a contrast agent to enhance visualization of the airway and alveolar spaces using magnetic resonance imaging of inert gas/oxygen mixtures. The study consists of a screening visit followed by up to 2 study visits.

The goal of this study is to evaluate the ability of conventional 'thermally' polarized perfluorinated gases (19F) mixed with oxygen to detect changes in ventilation using magnetic resonance imaging (MRI). A secondary goal is to assess the repeatability and the within-subject variability of these findings in CF lung disease. This is an open label pilot study expanding on work by other investigators currently using this technique in human subjects. Projection images using controlled breathing techniques will be obtained using 19F MRI, correlating spirometric variables with regional distribution of the gases.

Inclusion Criteria for Healthy Participants:

• Non-smokers (<10 pack-year history, and no active smoking in last 1 year);
• No evidence of prior lung disease or lung injury by medical history, physical exam, and/or spirometry testing;
• Evidence of a personally signed and dated informed consent document indicating that the subject has been informed of all pertinent aspects of the trial.
• Participants must be willing and able to comply with scheduled visits and other trial procedures.

Exclusion Criteria for Healthy Participants:

• Active or past smokers with less than 1 years since quitting or >10 pack-year smoking history

• Unable to undergo a 3.0-Tesla MRI exam of the lungs and chest because of contraindications including

  1. Occupation (past or present) of machiner, welder, grinder;
  2. Injury to the eye involving a metallic object
  3. Injury to the body by a metallic object (bullet, BB, shrapnel)
  4. Presence of a cardiac pacemaker or defibrillator
  5. Presence of aneurysm clips
  6. Presence of carotid artery vascular clamp
  7. Presence of neurostimulator
  8. Presence of insulin or infusion pump
  9. Presence of implanted drug infusion device that is not known to be MRI compatible (i.e., was placed outside of UNCH or is older than 10 years)
  10. Bone growth or fusion simulator
  11. Presence of cochlear, otologic or ear implant
  12. Any type of prosthesis (eye, penile, etc.)
  13. Artificial limb or joint
  14. Non-removable electrodes (on body, head or brain)
  15. Intravascular stents, filters or coils
  16. Shunt (spinal or intraventricular)
  17. Swan-ganz catheter
  18. Any implant held in place by a magnet
  19. Transdermal delivery system (e.g. Nitro)
  20. IUD or diaphragm
  21. Tattooed makeup (eyeliner, lips, etc.) or tattoos covering >25% of body surface area
  22. Body piercings (MUST BE REMOVED BEFORE MRI)
  23. Any metal fragments
  24. Internal pacing wires
  25. Metal or wire mesh implants
  26. Hearing aid (REMOVE BEFORE MRI) aa. Dentures (REMOVE BEFORE MRI) bb. Claustrophobia

• Unable to tolerate inhalation of gas mixture

• Participation in a clinical trial with a study drug that may impact lung function in the past 14 days

• Other severe acute or chronic medical or psychiatric condition or clinical laboratory abnormality that may increase the risk associated with trial participation or may interfere with the interpretation of trial results and, in the judgment of the investigator, would make the participant inappropriate for entry into this trial.

• Pregnancy: women of childbearing potential must have a confirmed negative urine pregnancy test on the day of the MR scan, prior to the MRI scan.

Inclusion Criteria for CF Participants:

• Non-smokers (<10 pack year history and no active smoking in the past year;
• Diagnosis of cystic fibrosis as via standard sweat chloride/phenotypic features/genotyping
• Stable lung disease as evidenced by no change in respiratory medications or change in FEV1 of >15% from baseline over the preceding 4 weeks prior to enrollment
• Baseline FEV1 >30% of predicted. Roughly equivalent numbers of CF subjects with mild FEV1 >80% of predicted), moderate (FEV1 50-80% of predicted) and severe (FEV1 <50% of predicted) will be targeted for enrollment.
• No use of supplemental oxygen
• Evidence of a personally signed and dated informed consent document indicating that the subject has been informed of all pertinent aspects of the trial.
• Participants must be willing and able to comply with scheduled visits and other trial procedures.

Exclusion Criteria for CF Participants:

• Active or past smokers with less than 1 years since quitting or >10 pack-year smoking history

• Active asthma flare, as perceived by the study physician or unstable asthma characterized by advancement of asthma therapy in the last month or two courses of oral steroids in the past six months.

• Unable to undergo a 3.0-Tesla MRI exam of the lungs and chest because of contraindications including

  1. Occupation (past or present) of machiner, welder, grinder;
  2. Injury to the eye involving a metallic object
  3. Injury to the body by a metallic object (bullet, BB, shrapnel)
  4. Presence of a cardiac pacemaker or defibrillator
  5. Presence of aneurysm clips
  6. Presence of carotid artery vascular clamp
  7. Presence of neurostimulator
  8. Presence of insulin or infusion pump
  9. Presence of implanted drug infusion device that is not known to be MRI compatible (i.e., was placed outside of UNCH or is older than 10 years)
  10. Bone growth or fusion simulator
  11. Presence of cochlear, otologic or ear implant
  12. Any type of prosthesis (eye, penile, etc.)
  13. Artificial limb or joint
  14. Non-removable electrodes (on body, head or brain)
  15. Intravascular stents, filters or coils
  16. Shunt (spinal or intraventricular)
  17. Swan-ganz catheter
  18. Any implant held in place by a magnet
  19. Transdermal delivery system (e.g. Nitro)
  20. IUD or diaphragm
  21. Tattooed makeup (eyeliner, lips, etc.) or tattoos covering >25% of body surface area
  22. Body piercings (MUST BE REMOVED BEFORE MRI)
  23. Any metal fragments
  24. Internal pacing wires
  25. Metal or wire mesh implants
  26. Hearing aid (REMOVE BEFORE MRI) aa. Dentures (REMOVE BEFORE MRI) bb. Claustrophobia

• Unable to tolerate inhalation of gas mixture

• Participation in a clinical trial with a study drug that may impact lung function in the past 14 days

• Other severe acute or chronic medical or psychiatric condition or laboratory abnormality that may increase the risk associated with trial participation or may interfere with the interpretation of trial results and, in the judgment of the investigator, would make the participant inappropriate for entry into this trial.

• Pregnancy; women of childbearing potential must have a confirmed negative urine pregnancy test on the day of the MR scan, prior to the MRI scan.