Strength and Muscle Related Outcomes for Nutrition and Lung Function in CF

The goal of the study is to examine multiple markers of anthropometrics, body composition, sarcopenia and frailty and compare them to dual energy X-ray absorptiometry (DXA) output, which is considered the current clinical gold-standard tool to measure body composition. The result of this study will provide detailed data regarding the nutrition and body composition within this Cystic Fibrosis population and also provide a baseline evaluation for use of these biomarkers in the future studies including evaluation of nutritional intervention. Further, the study will also include psychosocial and other patient-reported outcomes and medical contributors to understand their contributions to the nutritional failure in the adult advanced lung disease population. Finally, the study will evaluate both established and emerging nutritional and body composition parameters and link them to clinical outcomes in adults with CF across the spectrum of pulmonary function.

Inclusion Criteria:Cohort 1: Patients are eligible if their percentage of predicated forced expiratory volume in1 second (FEV1) is 60% or lower at screening.Cohort 2: Patients are eligible if their percentage of predicted forced expiratory volume in 1 second (FEV1) is 60% or greater at screening.Both cohorts match by age, gender, race and CFTR genotype severity.Exclusion Criteria:No prior whole organ transplantationNo initiation of an investigation drug within 28 days prior to and including Visit 1No initiation of new chronic therapy (e.g., ibuprofen, azithromycin, inhaled tobramycin, Cayston, CFTR modulator) within 28 days prior to and including Visit 1.No acute use of antibiotics (oral, inhaled or IV) or acute use of systemic corticosteroids for respiratory tract sympoms within 14 days prior to and including Visit 1.For the BIA substudy - Individuals with an implanted pacemaker will be excluded.