BEGIN Novel ImagiNG Biomarkers
To determine the treatment effect of triple-combination therapy in 6-8 year olds after presumed FDA approval, using rapid structural and functional pulmonary and abdominal MRI (UTE and 129Xe).
• 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