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CFTR Modulator

Lung Perfusion Mapping for Cystic Fibrosis

Phase 4
Waitlist Available
Led By Mark DiFrancesco, PhD
Research Sponsored by Children's Hospital Medical Center, Cincinnati
Eligibility Criteria Checklist
Specific guidelines that determine who can or cannot participate in a clinical trial
Must have
Inclusion CF Cohort: diagnosis of CF by positive sweat test and genetic test
Inclusion CF Cohort: Absence of exacerbation defined as O2 saturation level at 90% or greater when laying flat
Timeline
Screening 3 weeks
Treatment Varies
Follow Up baseline and 6 months post trikafta initiation for cf group. baseline for cf and control groups.
Awards & highlights
Drug Has Already Been Approved
No Placebo-Only Group
Pivotal Trial

Summary

This trial will use a new MRI method to measure regional lung perfusion in children with CF and relate it to regional assessments of ventilation and to serum cytokines or proteomic markers of angiogenesis and inflammatory processes.

Who is the study for?
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.
What is being tested?
The study tests a new MRI technique that maps blood flow in the lungs without contrast agents, alongside hyperpolarized Xenon to map ventilation. It aims to understand how CF affects lung perfusion and its relationship with ventilation defects over time.
What are the potential side effects?
Since this trial involves non-invasive imaging techniques rather than drugs, side effects may be minimal but could include discomfort from lying still during MRI scans or mild anxiety due to claustrophobia.

Eligibility Criteria

Inclusion Criteria

You may be eligible if you check “Yes” for the criteria below
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I have been diagnosed with cystic fibrosis through a sweat and genetic test.
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My oxygen levels stay above 90% when I'm lying down.
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I am between 6 and 21 years old and healthy.
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My lung function is within 5% of my best result in the past 6 months.

Timeline

Screening ~ 3 weeks
Treatment ~ Varies
Follow Up ~baseline and 6 months post trikafta initiation for cf group. baseline for cf and control groups.
This trial's timeline: 3 weeks for screening, Varies for treatment, and baseline and 6 months post trikafta initiation for cf group. baseline for cf and control groups. for reporting.

Treatment Details

Study Objectives

Study objectives can provide a clearer picture of what you can expect from a treatment.
Primary study objectives
Degree of concordance between ventilation and perfusion defects
Perfusion Defect Percentage (PDP)
Proteome assays as global indicators of inflammatory/angiogenic processes
+1 more

Awards & Highlights

Drug Has Already Been Approved
The FDA has already approved this drug, and is just seeking more data.
No Placebo-Only Group
All patients enrolled in this study will receive some form of active treatment.
Pivotal Trial
The final step before approval, pivotal trials feature drugs that have already shown basic safety & efficacy.

Trial Design

2Treatment groups
Experimental Treatment
Group I: Control CohortExperimental Treatment1 Intervention
10 Healthy control study participants matched for age and gender will undergo one MRI imaging study visit. Hyperpolarized Xenon 129 will be administered through inhalation at one MRI imaging study visit.
Group II: CF CohortExperimental Treatment2 Interventions
16 Cystic Fibrosis Patients will undergo MRI imaging before and 6 months after initiation of triple-combination modulator therapy. Initiation of triple -combination modulator therapy will be determined by clinician and family prior to study enrollment. Hyperpolarized Xenon 129 will be administered through inhalation at two MRI imaging study visits.

Find a Location

Who is running the clinical trial?

Children's Hospital Medical Center, CincinnatiLead Sponsor
839 Previous Clinical Trials
6,565,577 Total Patients Enrolled
22 Trials studying Cystic Fibrosis
1,159 Patients Enrolled for Cystic Fibrosis
Mark DiFrancesco, PhDPrincipal InvestigatorCCHMC
Jason Woods, PhDStudy ChairCCHMC
8 Previous Clinical Trials
483 Total Patients Enrolled
4 Trials studying Cystic Fibrosis
166 Patients Enrolled for Cystic Fibrosis

Media Library

Initiation of CFTR Modulator (CFTR Modulator) Clinical Trial Eligibility Overview. Trial Name: NCT04467957 — Phase 4
Cystic Fibrosis Research Study Groups: CF Cohort, Control Cohort
Cystic Fibrosis Clinical Trial 2023: Initiation of CFTR Modulator Highlights & Side Effects. Trial Name: NCT04467957 — Phase 4
Initiation of CFTR Modulator (CFTR Modulator) 2023 Treatment Timeline for Medical Study. Trial Name: NCT04467957 — Phase 4
~0 spots leftby Dec 2024