0866 A SYSTEMATIC REVIEW OF HEALTH OUTCOMES FOR CHILDREN WITH NEUROMUSCULAR DISORDERS USING LONG TERM NON-INVASIVE VENTILATION.

Abstract

Introduction: With increasing survival, recognition of the benefits of earlier intervention for respiratory insufficiency, and improvement in the technology used for non-invasive ventilation, the use of long-term non-invasive ventilation in children with neuromuscular disease has increased dramatically over the last 10 years. To date, while individual studies have looked at the impact of NIV on important outcomes, there has been no summary of these results. The aim of this systematic review is to summarize the published data relevant to health outcomes in children with neuromuscular disease using non-invasive ventilation. Methods: A scoping review was performed as the first step for identifying all published literature on children using long-term non-invasive ventilation and identified 289 publications. These articles were then screened for inclusion of discrete data on children with neuromuscular disease. Outcomes of interest included survival, healthcare usage, quality of life, respiratory function, sleep quality, neurocognition, or care giver burden. Grey literature sources were excluded. Results: A total of 35 publications were identified for inclusion in this review. A total 823 children were across these studies. While the majority of children using non-invasive ventilation had spinal muscular atrophy (59%) or Duchenne muscular dystrophy (19%), 20 additional neuromuscular diseases were present in the remaining children. An outcome of interest was studied in 71% of studies with the remaining studies only describing subject characteristics. The most common outcomes studied were change in polysomnography results (34%), healthcare usage (20%), and survival (20%); the remaining studies addressed respiratory function, neurocognition, sleep and quality of life. Conclusion: The majority of information on non-invasive ventilation use in children with neuromuscular diseases comes from two predominant conditions with use in a large number of additional unique diseases. Outcomes focus on polysomnography results and healthcare usage with less emphasis on outcomes of potential greater relevance to children and their families.