Abstract

AimTo determine the prevalence of long‐term mechanical insufflation‐exsufflation (MI‐E) and concomitant mechanical ventilation in children with neurological conditions, with reported reasons behind the initiation of treatment.MethodThis was a population‐based, cross‐sectional study using Norwegian national registries and a questionnaire.ResultsIn total, 114 of 19 264 children with a neurological condition had an MI‐E device. Seventy‐three of 103 eligible children (31 females, 42 males), median (min–max) age of 10 years 1 month (1y 5mo–17y 10mo), reported their MI‐E treatment initiation. Overall, 76% reported airway clearance as the main reason to start long‐term MI‐E. A prophylactic use was mainly reported by children with neuromuscular disorders (NMDs). Prevalence and age at initiation differed by diagnosis. In spinal muscular atrophy and muscular dystrophies, MI‐E use was reported in 34% and 7% of children, of whom 83% and 57% respectively received ventilator support. One‐third of the MI‐E users were children with central nervous system (CNS) conditions, such as cerebral palsy and degenerative disorders, and ventilator support was provided in 31%. The overall use of concomitant ventilatory support among the long‐term MI‐E users was 56%.InterpretationThe prevalence of MI‐E in a neuropaediatric population was 6 per 1000, with two‐thirds having NMDs and one‐third having conditions of the CNS. The decision to initiate MI‐E in children with neurological conditions relies on clinical judgment.What this paper adds The prevalence and age at initiation of mechanical insufflation/exsufflation (MI‐E) differed between diagnoses.MI‐E was most commonly used in spinal muscular atrophy, where it generally coincided with ventilatory support.One‐third of MI‐E devices were given to children with central nervous system conditions, and one‐third also received ventilatory support.

Highlights

  • The children were divided into two groups based on their main diagnosis: (1) neuromuscular disorders (NMDs) including (ICD-10 code) spinal muscular atrophy (SMA) (G12) and muscular dystrophies/myotonies (G70–72); (2) conditions originating from the central nervous system (CNS) including encephalitis (G00–09), degenerative disorders in the CNS (G30–32), cerebral palsy (CP) (G80–83), and other CNS disorders (G90–99)

  • In total, 114 of the 19 264 children (45 females, 69 males; median [min–max] age 10y 8mo [1y 5mo–18y]) in the neuropaediatric population were equipped with an mechanical insufflation-exsufflation (MI-E) device for long-term use, giving an overall point prevalence of 6 per 1000

  • Prevention of airway infections, without present signs of mucus retention, was reported in 17 (24%) children. Such prophylactic MI-E use was more commonly reported among children with NMDs (n=15) compared to those with CNS conditions (n=2, p=0.011) (Table S2)

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Summary

Objectives

Our aim was to determine the overall prevalence of long-term MI-E in the Norwegian paediatric population with a neurological diagnosis and to identify the clinical assessments and reported reasons behind initiation of the MI-E treatment. We aimed to describe rather than test associations, the requirements for internal validity are less rigid

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