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Assessment of CFTR-Modulator Treatment in Cystic Fibrosis Lung Disease Using Novel Structural and Functional MRI

Group 4: 4-8 yo CF Participants Starting Triple Combination Modulator...

This is a prospective, longitudinal observational study conducted at The Hospital for Sick Children (SickKids). The purpose of this study is to evaluate the ability of MRI to detect changes in lung structure and function in CF patients receiving CFTR-modulator therapy. Participants who meet eligibility criteria and consent to the study will undergo study procedures which include spirometry and body plethysmography (optional), multiple breath washout testing, MRI and measurement of quality of life. The study will consist of three to six study visits for Group 1, 2 and 3: baseline, one month (±1 week) and 6 month (±2 week) and if they re-consent then 3 additional visits of 1 year (±6 months). (2 years (±6 months) and 3 years (±6 months) follow-up. Some participants in Group 2 may transition to Group 3.

This study includes three populations of interest: Group 1: 25 healthy volunteers, Group 2: 25 patients with stable CF lung disease , Group 3: 20 patients with CF lung disease anticipated to receive treatment with CFTR-modulator therapy and Group 4: 18 patients with CF who are between 4-8 years old and are starting triple combination modulator therapy using the rapid lung and abdominal imaging methods.

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Participation Requirements

  • Sex:

    ALL

  • Eligible Ages:

    4 to 18

Participation Criteria

Inclusion Criteria:

* Participants must be greater than or equal to 4 years of age
* Informed consent by patient or parent/guardian consent and participant assent when appropriate.
* Able to perform reproducible spirometry and achieve a breath hold duration sufficient for MRI acquisition

Exclusion Criteria:

* Medical instability that would preclude the ability to undergo the required investigations
* FEV1 % predicted \< 40%
* Severe claustrophobia
* Does not meet MRI screening criteria
* Cough within the past 3 days prior to study visit
* Usage of oral antibiotics within 3 weeks prior to study visit

Study Location

The Hospital for Sick Children
The Hospital for Sick Children
Toronto, Ontario
Canada

Contact Study Team

Backup Contact

Sharon Braganza, M.Sc

[email protected]
416 813 7654
Primary Contact

Giles Santyr, PhD FCCPM

[email protected]
Study Sponsored By
The Hospital for Sick Children
Participants Required
More Information
Study ID: NCT04391322