Continuous noninvasive ventilatory support outcomes for patients with neuromuscular disease: a multicenter data collaboration

Autores da FMUP

• Miguel Ramalho Souto Goncalves

  Autor

• João Carlos Winck Fernandes Cruz

  Autor

Participantes de fora da FMUP

• Bach, JR
• Ishikawa, Y
• Saporito, L
• Int Study Grp Continuous Noninvasi

Unidades de investigação

• Cirurgia e Fisiologia

• Laboratório Associado RISE- Rede de Investigação em Saúde

  

  Investigador

  Fernando Carlos De Landér Schmitt

Abstract

Background: Typically, patients with progressive neuromuscular disorders (NMDs) develop acute respiratory failure (ARF), are intubated, and when failing spontaneous breathing trials (SBTs) undergo a tracheotomy and receive tracheostomy mechanical ventilation (TMV). However, increasing numbers of patients use nasal noninvasive ventilation (NIV), initially for sleep and this is extended to continuous dependence (CNVS). This can be used as a strategy to assist in success-ful extubation . We retrospectively reviewed 19 centers offering CNVS and mechanical insuffla-tion-exsufflation (MI-E) as an alternative to TMV. Methods: Centers with publications or presentations concerning CNVS outcomes data were pooled for amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy type 1 (SMA1). Progression to CNVS dependence without hospitalization, dura-tion of dependence, and extubations and decannulations to CNVS were recorded. Prolongation of life was defined by duration of CNVS dependence without ventilator free breathing ability (VFBA). Results: There were 1623 part time (<23 h/day) NVS users with ALS, DMD, and SMA1 from 19 centers in 16 countries of whom 761 (47%) were CNVS dependent for 2218 patient-years. This included: 335 ALS patients for a mean 1.2 +/- 1.0 (range to 8) years each; 385 DMD patients for 5.4 +/- 1.6 (range to 29) years; and 41 SMA1 patients for 5.9 +/- 1.8 (range to 20) years. Thirty-five DMD and ALS TMV users were decannulated to CNVS and MI-E. At data collection 494 (65%) patients were CNVS dependent but 110 (74 of whom with bulbar ALS), had undergone tracheotomies. Conclusions: ALS, DMD, and SMA1 patients can become CNVS dependent without requiring hospitalization but CNVS cannot be used indefinitely for many patients with advanced upper motor neuron diseases. (c) 2021 Sociedade Portuguesa de Pneumologia. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/bync-nd/4.0/).

Keywords

• Noninvasive ventilation; Neuromuscular disease; Long term survival