Current practice and evolution of pediatric respiratory management of Duchenne muscular dystrophy in Canada

Abstract

RATIONALE: Respiratory management practices for Duchenne muscular dystrophy (DMD) vary across Canada, according to a web-based survey conducted in 2010. Since then, new guidelines for respiratory management of DMD have been published. OBJECTIVES: This study aimed to describe current respiratory management practices of Canadian Pediatric Respirologists for children with DMD across the country. METHODS: A web-based survey on timing and indications for respiratory diagnostic tests and interventions was sent to all practicing Pediatric Respirologists at tertiary care centers in Canada who are involved in the longitudinal management of boys with DMD (N = 20). MEASUREMENTS AND MAIN RESULTS: Responses were received from 17 (85%) Pediatric Respirologists. Pulmonary function testing is regularly performed at least twice yearly after 5-6 years of age or after a patient becomes nonambulatory (13/17, 76%). Maximal expiratory pressures (15/17, 88%) and peak cough flow (12/17, 71%) are used to assess respiratory muscle strength. Sleep evaluation is most commonly performed with polysomnography (14/17, 82%), and is requested with the onset of symptoms (14/17, 82%) or with a forced vital capacity (FVC) < 60% predicted (6/9, 67%). At least once-daily lung volume recruitment exercises are prescribed by 94% (16/17) of respondents during periods of clinical stability, with the most common indication being an FVC < 80% predicted (9/17, 53%). CONCLUSIONS: The majority of Canadian Pediatric Respirologists are providing respiratory care to boys with DMD according to the current guidelines. The greatest change over time is the increased usage of regular LVR/airway clearance during periods of clinical stability.