RECRUITINGEarly Phase 1INTERVENTIONAL

Regional Monitoring of CF Lung Disease

Regional Monitoring of CF Lung Disease After Changes in Mechanical Airway-clearance Treatment

About This Trial

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 U.S. 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 Children's 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.

Who May Be Eligible (Plain English)

Who May Qualify: - 1 Written willing to sign a consent form (and assent when applicable) obtained from subject or subject's legal representative. 2 Use of highly effective modulators for more than 30 days (ie. Trikafta) 3 Willingness and ability to adhere to the study visit schedule and other protocol requirements. 4 Documentation of a CF diagnosis with prescription of Mechanical ACT 5 Ages 12-21 inclusive, at the time of consent. 6 Clinically stable with no respiratory tract infection or recent exacerbations. 7 Treating CF physician agreeable to study procedures. Only applicable to Aim 3. 8 No change in chronic maintenance therapies in the 28 days prior to enrollment. 9 Ability to cooperate with MRI procedures. Who Should NOT Join This Trial: 1. Standard MRI exclusions (metal implants, claustrophobia). 2. For females of childbearing potential: Positive urine pregnancy test or Lactating. 3. Acute respiratory symptoms (e.g., wheezing) at the time of the MRI 4. Chronic lung or liver or pancreatic disease not related to CF. 5. Any other condition that, in the opinion of the Investigator, would preclude willing to sign a consent form or assent, make study participation unsafe, complicate interpretation of study outcome data, or otherwise interfere with achieving the study objectives. Always talk to your doctor about whether this trial is right for you.

Original Eligibility Criteria

View original clinical language

Inclusion Criteria: * 1 Written informed consent (and assent when applicable) obtained from subject or subject's legal representative. 2 Use of highly effective modulators for more than 30 days (ie. Trikafta) 3 Willingness and ability to adhere to the study visit schedule and other protocol requirements. 4 Documentation of a CF diagnosis with prescription of Mechanical ACT 5 Ages 12-21 inclusive, at the time of consent. 6 Clinically stable with no respiratory tract infection or recent exacerbations. 7 Treating CF physician agreeable to study procedures. Only applicable to Aim 3. 8 No change in chronic maintenance therapies in the 28 days prior to enrollment. 9 Ability to cooperate with MRI procedures. Exclusion Criteria: 1. Standard MRI exclusions (metal implants, claustrophobia). 2. For females of childbearing potential: Positive urine pregnancy test or Lactating. 3. Acute respiratory symptoms (e.g., wheezing) at the time of the MRI 4. Chronic lung or liver or pancreatic disease not related to CF. 5. Any other condition that, in the opinion of the Investigator, would preclude informed consent or assent, make study participation unsafe, complicate interpretation of study outcome data, or otherwise interfere with achieving the study objectives.

Treatments Being Tested

PROCEDURE

Airway-clearance vest

Airway clearance devices will be re-initiated for those who have self withdrawn and will be withdrawn temporarily for those who are using them regularly and have high FEV1 and low Xe MRI ventilation defects.

DRUG

Hyperpolarized Xe129

Hyperpolarized Xe MRI is FDA Approved for evaluation of ventilation in adults and children 12 and above and will be used as a diagnostic test here. Earlier studies using Xe MRI have shown its efficacy in exploring CF and other lung diseases in pediatric and adult populations, and it has been found to be much more sensitive to early lung disease than traditional metrics like spirometry and MBW.

Locations (1)

Cincinnati Children's Hospital

Cincinnati, Ohio, United States