Children With SMA Type 1 May Benefit More From Invasive Ventilation

Contrary to children with other neuromuscular diseases, children with type 1 spinal muscular atrophy have higher mortality with long-term noninvasive ventilation compared with invasive mechanical ventilation, suggest results from a systematic review and meta-analysis published online in the Annals of the American Thoracic Society. 

Children with other neuromuscular diseases have lower mortality with long-term noninvasive ventilation, researchers reported. 

The systematic review and meta-analysis spanned 50 articles and 1412 children with 36 different neuromuscular disorders. 

Across all neuromuscular disorder types, mortality was lower for children using long-term non-invasive ventilation compared with supportive care, according to the study. When researchers compared noninvasive ventilation to invasive mechanical ventilation, they found that, overall, mortality did not differ between the two—although “heterogeneity suggests that mortality with noninvasive ventilation is higher for spinal muscular atrophy type 1 and lower for other/multiple neuromuscular diseases,” they wrote. 

Hospitalization rates with long-term noninvasive ventilation were generally lower compared with invasive mechanical ventilation and varied with neuromuscular disease type, the study found. Again, however, children with type 1 spinal muscular atrophy had higher hospitalization rates with noninvasive mechanical ventilation. 

“Long-term noninvasive ventilation for children provides benefit for mortality, hospitalizations, and sleep study parameters for some subgroups of children with neuromuscular disorders,” researchers concluded. “High risk of bias and low study quality preclude strong conclusions.”

Reference: 

AlBalawi MM, Castro-Codesal M, Featherstone R, et al. Outcomes of Long-term Non-invasive Ventilation Use in Children with Neuromuscular Disease: Systematic Review and Meta-analysis [published online ahead of print, 2021 Jun 28]. Ann Am Thorac Soc. 2021;10.1513/AnnalsATS.202009-1089OC. doi:10.1513/AnnalsATS.202009-1089OC