176
Participants
Start Date
October 1, 2018
Primary Completion Date
May 1, 2026
Study Completion Date
May 1, 2026
Ventilation Inhomogeneity
Ventilation inhomogeneity will be measured using the Lung Clearance Index (LCI), derived from multiple breath Nitrogen washout during tidal breathing, measured by a mouthpiece or mask covering nose and mouth.
Diaphragm Ultrasound
Diaphragm thickness and contractile activity measured during tidal breathing.
Respiratory Inductance Plethysmography
Measure of thoraco-abdominal asynchrony during tidal breathing
Spirometry
Measurement of Forced Expiratory Volume (FEV1), Forced Vital Capacity and other lung volumes using standard pulmonary function techniques
Functional Residual Capacity
Measurement using body box plethysmography of functional residual capacity and other lung volumes using standard pulmonary function techniques
MIP/MEP
Measurement of maximal inspiratory and expiratory pressures during airway occlusion
6 minute walk test
Measurement of cardio-respiratory function and capabilities during treadmill walking for 6 minutes.
Neurocognitive Testing
Detailed in person neuro-cognitive testing using standardized inventories using either the Battelle Developmental Inventory, second edition (Battelle-2) (age birth to 5 years 11 months) and the Wechsler Intelligence Scale for Children, fifth edition (WISC-5, for age 6 years to 19 years).
Emotional Health Assessment
In person and written assessments of children and parents using the Behavioral Assessment System for Children, third edition (BASC-3) for children ≥ 2 years and the UCLA PTSD Reaction Index (UCLA RI) for children ≥ 8 years.
Health Related Quality of Life
Standardized instruments to assess (in person, over the phone, or via mail) health related quality of life in children. Parent and child questionnaires.
Functional Status
Survey of overall functional status, administrated by asking a series of questions to patient and families.
Respiratory Status Questionnaire
Survey of respiratory health, administrated by asking a series of questions to patient and families.
Children's Hospital Los Angeles, Los Angeles
Children's Hospital Los Angeles
OTHER