Current Environment:

Summary

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.

Conditions

Cystic Fibrosis

Recruitment Status

RECRUITING

Eligibility Criteria

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 solid organ transplantation
* No initiation of an investigation drug within 28 days prior to and including Visit 1
* No 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 symptoms within 14 days prior to and including Visit 1.
* For the BIA sub-study - Individuals with an implanted pacemaker will be excluded.
* No initiation of a drug for weight loss (such as a GLP-1 receptor agonist) or bariatric surgery within 6-months prior to and including the Baseline visit.
* Patients with continued rapid change or extreme GI symptoms related to weight loss therapy should be excluded at the discretion of the study investigator.

Intervention

Intervention Type

Intervention Name

DIAGNOSTIC_TEST

BMI and lean mass index from DXA

DIAGNOSTIC_TEST

Anthropometric Measurements

DIAGNOSTIC_TEST

Hand-grip strength

DIAGNOSTIC_TEST

Six-minute walk Test

DIAGNOSTIC_TEST

Sit-to-Stand Test

DIAGNOSTIC_TEST

Short physical performance battery (SPPB)

DIAGNOSTIC_TEST

BIA Sub-study

DIAGNOSTIC_TEST

Accelerometry to assess physical activity

OTHER

Gastrointestinal (GI) and nutrition questionnaires:

OTHER

Psychosocial questionnaire: PHQ-8

OTHER

Psychosocial questionnaire: GAD-7

OTHER

Psychosocial questionnaire: The Treatment Self-Regulation Questionnaire (TSRQ)

OTHER

Psychosocial questionnaire: CF Fatalism Scale

OTHER

Psychosocial questionnaire: Body Esteem Scale for Adolescents and Adults (BESAA)

OTHER

12-month Questionnaire

OTHER

Oral glucose tolerance testing (OGTT)

DEVICE

Continuous glucose monitoring (CGM)

DIAGNOSTIC_TEST

Chest CT scans (When available within the past 6 months in medical records)

DIAGNOSTIC_TEST

Hologic Dual X-Ray Absorptiometry (DXA)

DIAGNOSTIC_TEST

Ultrasound Sub-study of assessment of appendage muscles using ultrasound

DIAGNOSTIC_TEST

Psychosocial questionnaire: Hunger Vital Sign questionnaire

OTHER

Respiratory symptom questionnaire: CRISS

DIAGNOSTIC_TEST

Spirometry

OTHER

Psychosocial questionnaire: Additional Health Questionnaire

Gender

ALL

Min Age

18 Years

Max Age

N/A

Download Date

2024-02-13

Principal Investigator

This field has been modified from ClinicalTrials.gov to show a contact specific to Boston Children's.

Primary Contact Information

Judy Sibayan

(813) 975-8690

jsibayan@jaeb.org

For more information on this trial, visit clinicaltrials.gov.

Contact

For more information and to contact the study team:

Strength and Muscle Related Outcomes for Nutrition and Lung Function in CF NCT05639556 Judy Sibayan (813) 975-8690 jsibayan@jaeb.org