Ignite Creation Date:
2024-05-06 @ 8:18 PM
Last Modification Date:
2024-10-26 @ 3:25 PM
Study NCT ID:
NCT06339593
Status:
RECRUITING
Last Update Posted:
2024-04-01
First Post:
2024-02-06
Brief Title:
Regional Monitoring of CF Lung Disease
Sponsor:
Childrens Hospital Medical Center Cincinnati
Organization:
Childrens Hospital Medical Center Cincinnati
Study Overview
Official Title:
Regional Monitoring of CF Lung Disease After Changes in Mechanical Airway-clearance Treatment
Status:
RECRUITING
Status Verified Date:
2024-03
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
The main reason for this research study is to learn more about some new tests that are being developing for patients with Cystic Fibrosis CF to measure changes in the lungs In this study the focus will be to learn how stopping Airway Clearance ACT and re-starting ACT can affect these tests These new tests include using a breathable gas called Xenon Xe with MRI magnetic resonance imaging to improve the pictures of changes in the lungs The Xenon Xe gas that has been treated to have a larger MRI signal also called hyperpolarized The other new test is called LCI Lung Clearance Index that can measure how well the lungs are working The MRI machine used in this study has been approved by the US Food and Drug Administration FDA and is commercially available for sale in the USA Hyperpolarized Xe gas is an FDA-approved inhaled contrast agent for lung ventilation MRI The new Xe MRI techniques that are being developed and used for this research study are investigational meaning these new Xe MRI techniques are not FDA approved but they are similar to FDA-approved techniques that are used clinically at Cincinnati Childrens Hospital Medical Center CCHMC Xe gas and the new MRI techniques used in this research study have been used for many years in research including in many research studies conducted at CCHMC like this one
Detailed Description:
Cystic fibrosis CF is a progressive systemic disease affecting an estimated 30000 children and adults in the United States 70000 worldwide and is caused by mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator CFTR protein--a chloride and bicarbonate channel that regulates ion transport and mucus composition in CF-affected tissues such as the lung In airways this leads to mucus stasis infection inflammation and remodeling that result in mucus plugs regional lung obstruction and progressive airway destruction and bronchiectasis Highly-effective CFTR modulators which are recently available to 90 of patients have revolutionized CF clinical care with large increases in pulmonary function as a result of more effective mucociliary clearance As a result burdensome maintenance therapies like mechanical airway clearance treatment ACT requiring nearly 2 dedicated hours per day have been questioned by patients families and medical providers In a recent survey of CF community members ACT was ranked as the most burdensome chronic therapy yet is the least studied Prospective studies of maintenance-therapy withdrawal pose potential ethical risks since traditional testing via spirometry andor multiple-breath washout is relatively insensitive to small or regional changes and long-term lung-function reductions often have permanent consequences Nevertheless many patients have withdrawn these maintenance therapies against advice from their providers A major gap in CF management is our ability to monitor lung function sensitively and rapidly as a result of treatment changes such as partial withdrawal of ACT
Breakthroughs in structural and functional magnetic resonance imaging MRI have demonstrated exquisite sensitivity to regional CF lung disease and can monitor regional and subtle changes over time without ionizing radiation even in patients with normal spirometry As demonstrated in the previous R01 that ultrashort echo time UTE MRI provides structural images that rival computered tomography CT imaging with sensitivity to detect all of the structural hallmarks of treatable eg mucus plugs and permanent lung disease eg bronchiectasis It has been demonstrated that hyperpolarized 129Xe MRI is more sensitive than any other technique at detecting changes in regional pulmonary ventilation and gas exchange For the first time a single modality MRI is available to safely monitor regional lung disease and treatment changes before FEV1 declines become permanent This is a unique opportunity to safely evaluate ACT in CF populations that remain at risk of long-term lung function decline
Breakthroughs in structural and functional magnetic resonance imaging MRI have demonstrated exquisite sensitivity to regional CF lung disease and can monitor regional and subtle changes over time without ionizing radiation even in patients with normal spirometry As demonstrated in the previous R01 that ultrashort echo time UTE MRI provides structural images that rival computered tomography CT imaging with sensitivity to detect all of the structural hallmarks of treatable eg mucus plugs and permanent lung disease eg bronchiectasis It has been demonstrated that hyperpolarized 129Xe MRI is more sensitive than any other technique at detecting changes in regional pulmonary ventilation and gas exchange For the first time a single modality MRI is available to safely monitor regional lung disease and treatment changes before FEV1 declines become permanent This is a unique opportunity to safely evaluate ACT in CF populations that remain at risk of long-term lung function decline
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
True
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None