BEGIN Novel ImagiNG Biomarkers

Prior treatment

• Individuals currently on ivacaftor therapy (including Kalydeco, Orkambi, and Symdeko) and with at least one gating mutation. Gating mutations include G551D, G178R, S549N, S549R, G551S, G970R, G1244E, S1251N, S1255P, or G1349D.
• Acute respiratory infection, defined as increased cough, wheezing or respiratory rate in the 28 days prior to enrollment.

Other illness

• Chronic lung disease not related to CF
• Chronic liver disease not related to CF
• Chronic pancreatic disease not related to CF.
• Any other condition that, in the opinion of the Site Investigator/designee, would preclude informed consent or assent, make study participation unsafe, complicate interpretation of study outcome data, or otherwise interfere with achieving the study objectives.

Other

• Acute respiratory symptoms (e.g. wheezing) at the time of the MRI.
• Acute pancreatitis, defined by clinical criteria (45).
• Physical findings that would compromise the safety of the subject or the quality of the study data as determined at the discretion of the site investigator.

• Written informed consent (and assent where appropriate) obtained from the subject or subject's legal representative.
• Willingness to adhere to the study-visit schedule and other protocol requirements.
• Ages 6-8 years old at baseline MRI visit (may be enrolled up to 60 days before 6th birthday).
• Documentation of CF diagnosis as evidenced by one or more clinical features consistent with the CF phenotype and one or more of the following criteria:
  • Sweat chloride equal to or greater than 60 mEq/liter by quantitative pilocarpine iontophoresis test
  • Two well-characterized mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene
• Physician intent to prescribe triple-combination therapy
• Clinically-stable with no respiratory tract infection at the time of enrollment.
• No change in chronic maintenance therapies in the 28 days prior to enrollment.
• Ability to cooperate with MRI procedures