VPPI does indeed appear to have a positive effect on chest growth in children with type I SMA

A French retrospective study assessed the effect of intermittent positive pressure ventilation (IPPV) using the Alpha 300® in seven children with type I spinal muscular atrophy, with a median age of 4.8 years, who were being treated with Zolgensma® (four of them) or Nusinersen®.

  • After four months of daily use for around 20 minutes, the ratio of chest circumference to head circumference increased significantly.
  • Only one child did not respond to IPPV.
  • The duration of the study was too short to measure a significant effect on the number of hospitalisations due to exacerbations.

Randomised controlled trials involving larger cohorts will be required to confirm the results of this pilot study.