Cystic Fibrosis
Conditions
Brief summary
Main Study Up to 100 subjects, both non-CF volunteers and Cystic Fibrosis (CF) patients, will participate in a single study visit that will include a DEXA scan, micro CT, and blood collection. Denosumab (Prolia) Sub study Approximately 10 adult subjects with CF who participated in the main study and have results indicating bone disease will receive treatment with Denosumab for up to 5 years. They will be asked to return for repeat DEXA scans, micro CT, blood collection, and documenting of any adverse events.
Interventions
DRUGDenosumab
treatment with denosumab every 6 months for up to 5 years
Sponsors
University of Texas Southwestern Medical Center
Study design
Allocation
NON_RANDOMIZED
Intervention model
SEQUENTIAL
Primary purpose
DIAGNOSTIC
Masking
TRIPLE (Subject, Caregiver, Investigator)
Eligibility
Sex/Gender
ALL
Age
18 Years to 64 Years
Healthy volunteers
Yes
Inclusion criteria
Cystic Fibrosis Main Study Inclusion Criteria: * Must have CF diagnosis confirmed by sweat test or genotype analysis * Subjects (and parents/legal guardians as applicable) must have the ability to read and write in English Sub-study
Exclusion criteria
* No CF diagnosis * Men or women without osteoporosis * Less than 18 years of age * Unwilling to return annually for study visits for up to 5 years * Unwilling and/or medically unable to take denosumab
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| DEXA results | Looking at single timepoints and well as changes for up to 5 years for those in the sub-study | Z and/or T scores |
| Micro CT results | Looking at single timepoints and well as changes for up to 5 years for those in the sub-study | Trabecular bone volume to tissue volume (BV/TV), cortical and trabecular thickness (Tb.Th, microns), trabecular spacing (Tb.Sp; microns), and structure model index (SMI; index of trabecular rod vs plate-like shape/ stoutness) will be assessed. Change in BV/TV and cortical thickness will be taken to indicate increased/decreased bone mass accrual by microCT. |
Countries
United States
Contacts
CONTACTAshley Keller
Outcome results
None listed