Strength and Muscle Related Outcomes for Nutrition and Lung Function in CF (STRONG-CF)
There are currently two main ways of measuring nutrition in the Cystic Fibrosis (CF) population: body mass index (BMI) and laboratory values. This study plans to look at more ways to measure nutrition, and body composition, like the percentages of fat, bone and muscle in your body. One of the ways we will measure these items is by using dual energy X-ray absorptiometry (DXA) scan, which is a type of x-ray. This study hopes to provide researchers with more detailed information about nutrition and body composition in adults with CF.
• diagnosed with Cystic Fibrosis
• clinically stable with no significant changes in health status within the 14 days prior to the first study visit
• no prior solid organ transplantation
• no initiation of an investigation drug within 28 days before
• no initiation of new chronic therapy (e.g., ibuprofen, azithromycin, inhaled tobramycin, Cayston, CFTR modulator) within 28 days
• no acute use of antibiotics (oral, inhaled or IV) or acute use of systemic corticosteroids for respiratory tract symptoms within 14 days
Other: 12-month Questionnaire, Diagnostic Test: Accelerometry to assess physical activity, Diagnostic Test: Anthropometric Measurements, Diagnostic Test: BIA Sub-study, Diagnostic Test: BMI and lean mass index from DXA, Diagnostic Test: Chest CT scans (When available within the past 6 months in medical records), Device: Continuous glucose monitoring (CGM), Other: Gastrointestinal (GI) and nutrition questionnaires:, Diagnostic Test: Hand-grip strength, Diagnostic Test: Hologic Dual X-Ray Absorptiometry (DXA), Other: Oral glucose tolerance testing (OGTT), Other: Psychosocial questionnaire: Additional Health Questionnaire, Other: Psychosocial questionnaire: Body Esteem Scale for Adolescents and Adults (BESAA), Other: Psychosocial questionnaire: CF Fatalism Scale, Other: Psychosocial questionnaire: GAD-7, Diagnostic Test: Psychosocial questionnaire: Hunger Vital Sign questionnaire, Other: Psychosocial questionnaire: PHQ-8, Other: Psychosocial questionnaire: The Treatment Self-Regulation Questionnaire (TSRQ), Other: Respiratory symptom questionnaire: CRISS, Diagnostic Test: Short physical performance battery (SPPB), Diagnostic Test: Sit-to-Stand Test, Diagnostic Test: Six-minute walk Test, Diagnostic Test: Spirometry, Diagnostic Test: Ultrasound Sub-study of assessment of appendage muscles using ultrasound
Respiratory System, Rare Diseases, Rare Diseases
cystic fibrosis