23
Participants
Start Date
April 30, 2015
Primary Completion Date
September 1, 2020
Study Completion Date
September 1, 2020
Pulmonary function testing
Each enrolled patient will receive pulmonary function testing every three months. Pulmonary function testing includes spirometry, plethysmography, and diffusion capacity measurements.
FAM Therapy
All patients who have evidence of early airflow obstruction on pulmonary function testing will be started on FAM therapy. Early airflow obstruction is defined by a FEV1 decline of ≥10% predicted in addition to a FVC \<0.8.
Ann & Robert H Lurie Children's Hospital of Chicago, Chicago
Ann & Robert H Lurie Children's Hospital of Chicago
OTHER