Monitoring lung changes in cystic fibrosis patients

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

This study is testing new ways to see how stopping and starting airway clearance therapy affects lung health in people with cystic fibrosis.

Quick facts

• Phase: Early Phase 1
• Study type: Interventional
• Enrollment: 60 (estimated)
• Ages: 12 Years to 21 Years
• Sex: All
• Sponsor: Children's Hospital Medical Center, Cincinnati (Academic / other)
• Drugs / interventions: radiation
• Locations: 1 site (Cincinnati, Ohio)
• Trial ID: NCT06339593 on ClinicalTrials.gov

What this trial studies

This observational study aims to evaluate new tests for measuring lung changes in patients with cystic fibrosis (CF), specifically focusing on the effects of stopping and restarting airway clearance therapy (ACT). The study utilizes hyperpolarized xenon gas with MRI to enhance imaging of lung function and employs the Lung Clearance Index (LCI) to assess lung performance. Participants will be monitored to understand how these new imaging techniques can provide insights into lung health and the impact of ACT on their condition. The MRI technology used is FDA-approved, ensuring safety and reliability in the assessment process.

Who should consider this trial

Good fit: Ideal candidates for this study are cystic fibrosis patients aged 12-21 who have been using effective CFTR modulators and are stable without recent respiratory infections.

Not a fit: Patients with acute respiratory symptoms, those who cannot undergo MRI procedures, or those with certain medical exclusions may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to improved monitoring and management strategies for cystic fibrosis patients, potentially reducing the burden of daily therapies.

How similar studies have performed: While the use of hyperpolarized xenon MRI is investigational, similar imaging techniques have shown promise in other studies, indicating potential for success in this approach.

Eligibility criteria

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.

Where this trial is running

Cincinnati, Ohio

• Cincinnati Children's Hospital — Cincinnati, Ohio, United States (Recruiting)

Study contacts

• Principal investigator: Jason C Woods, PhD — Cincinnati Children's Hospital Medica Center
• Study coordinator: Carrie Stevens, BS

Conditions Cystic Fibrosis