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Keywords:

  • cough;
  • muscular atrophy;
  • spinal;
  • muscular dystrophy;
  • Duchenne;
  • neuromuscular diseases;
  • quality of life

Aim

Mechanical in-exsufflation (MI-E) augments the weakened cough of patients with neuromuscular disease (NMD), clearing secretions and overcoming atelectasis. Little has been published on the impact of MI-E alone on rates of hospitalisation and quality of life (QOL). The aim of this study was to assess the impact of home MI-E on hospital admissions and life-style in children with NMD.

Methods

A retrospective chart review was performed on children using MI-E, including data on the number of admissions to hospital, length of stay and hours of ventilation. A parental survey was used to gather information on the impact of MI-E on life-style for the child and family.

Results

Ten children with NMD (seven spinal muscular atrophy, two Duchenne muscular dystrophy and one centronuclear myopathy) using MI-E at home were identified. MI-E use commenced at mean age of 8.5 years (range 1.1–16.9) with 1.4 years of use (range 0.3–3.8). MI-E pressures ranged from +/−30 to 40 cmH2O with no complications reported. There was a significant reduction in hospital days at 6 (P = 0.036) and 12 (P = 0.028) months following commencement of home MI-E compared with the same period preceding MI-E use. The survey highlighted positive benefits of MI-E use, in particular the ability to treat many pulmonary exacerbations at home.

Conclusions

Home MI-E use by children with NMD can reduce hospitalisation and benefit families by maintaining their child at home.