Lung Perfusion Mapping for Cystic Fibrosis
What You Need to Know Before You Apply
What is the purpose of this trial?
Cystic fibrosis (CF) results in the thickening of mucus in the lungs and other organs due to dysfunction of a transmembrane conductance protein. This allows buildup of bacteria that results in inflammation, leading to tissue breakdown and loss of function. In the lungs, this process causes loss of air exchange structures progressing to diminished lung function. The exchange of oxygen in the lungs depends on both the integrity of air conduits and vasculature. Most clinical assessments, however, focus on ventilatory function, with the assumption that any vascular compromise is secondary. Nevertheless, there is evidence, some from the investigator's lab, to suggest that perfusion anomalies in the lung occur before signs of ventilatory dysfunction. Thus, the inflammatory processes of CF may impact pulmonary microvasculature specifically and concurrently or prior to damage to ventilatory structures. This study aims to apply a new MRI method to serially measure regional lung perfusion, without the use of contrast agent, in children with CF and to associate it with regional assessments of ventilation and to serum cytokines or proteomic markers of angiogenesis and inflammatory processes.The investigator's lab has recently developed a noninvasive, non-contrast, method of labeling blood flowing into the lungs and generating a map of perfusion. The investigator aims to couple this technique to existing methods using hyperpolarized Xenon to map ventilation. The investigator will apply these methods over time in CF patients, monitoring the relationship between regional perfusion and ventilation defects.This pilot work will provide the foundation for larger studies to establish the essential etiological role of perfusion deficits in CF.
Who Is on the Research Team?
Mark DiFrancesco, PhD
Principal Investigator
CCHMC
Jason Woods, PhD
Principal Investigator
CCHMC
Are You a Good Fit for This Trial?
This trial is for children and young adults aged 6-21 with cystic fibrosis (CF) confirmed by tests, who are about to start Trikafta treatment. Healthy individuals of the same age without lung issues can also join as controls. Participants need stable lung function and no recent exacerbations or antibiotic treatments.Inclusion Criteria
Exclusion Criteria
Timeline for a Trial Participant
Screening
Participants are screened for eligibility to participate in the trial
Baseline Assessment
Initial MRI imaging and baseline measurements for CF and control groups
Treatment Monitoring
CF patients undergo MRI imaging and blood sample analysis before and 6 months after initiation of triple-combination modulator therapy
Follow-up
Participants are monitored for safety and effectiveness after treatment
What Are the Treatments Tested in This Trial?
Interventions
- Hyperpolarized Xenon 129
- Initiation of CFTR Modulator
Find a Clinic Near You
Who Is Running the Clinical Trial?
Children's Hospital Medical Center, Cincinnati
Lead Sponsor