For a study, it was determined to see if long-term noninvasive ventilation (NIV), continuous or bilevel positive airway pressure, improves health outcomes in children with neuromuscular diseases compared to other treatment options. This systematic review was a follow-up to a scoping study. Medical Subject Headings and free-text terms for “kid” and “noninvasive ventilation” were employed in the search approach. Human studies were searched by Researchers in MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed from 1990 onwards. Articles on neuromuscular disorders and health outcomes, such as mortality, hospitalization, quality of life, lung function, sleep study parameters, and healthcare expenses, were screened. Study design, duration, sample size, age, type of NIV, follow-up period, underlying disease, and primary and secondary outcome measures were among the data extracted. The research was divided into three categories based on the primary disease: spinal muscular atrophy, Duchenne muscular dystrophy, and other/multiple neuromuscular illnesses. The review comprised 50 publications that covered 1,412 children with 36 different neuromuscular diseases. Across all neuromuscular disease categories, children who get long-term NIV had a lower mortality rate than those who received supportive treatment. When comparing NIV to invasive mechanical ventilation, mortality does not differ significantly. At the same time, heterogeneity implies that mortality with NIV was higher for spinal muscular atrophy type 1 and lower for other/multiple neuromuscular illnesses. Long-term NIV had a different effect on hospitalization rates depending on the type of neuromuscular disease, with lower rates compared to supportive care but higher rates compared to supportive care use for spinal muscular atrophy type 1 and lower rates compared to before NIV for other/multiple neuromuscular diseases. Long-term NIV usage improved sleep study measures from baseline and did not affect lung function. There was scant data on how long-term NIV usage affects the quality of life and healthcare costs.
For some subgroups of children with neuromuscular diseases, long-term NIV was beneficial in mortality, hospitalizations, and sleep study characteristics. Strong conclusions were impossible to reach due to the high risk of bias and inadequate study quality.
Reference:www.atsjournals.org/doi/full/10.1513/AnnalsATS.202009-1089OC
