Understanding the use of NIV in ALS: results of an international ALS specialist survey

Terry D. Heiman-Patterson*, Merit E. Cudkowicz, Mamede De Carvalho, Angela Genge, Orla Hardiman, Carlayne E. Jackson, Noah Lechtzin, Hiroshi Mitsumoto, Vincenzo Silani, Jinsy A. Andrews, Dafeng Chen, Sarah Kulke, Stacy A. Rudnicki, Leonard H. van den Berg

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Objective: To identify common practices of noninvasive ventilation (NIV) use among ALS specialists and how they follow respiratory status in their patients. Methods: A 25-item questionnaire on NIV indications/initiation was sent via SurveyMonkey® to ALS specialists identified through membership in NEALS (114 sites in the US) and ENCALS (39 sites in Europe). Descriptive statistics and Cochran–Mantel–Haenszel test for general association were performed. Results: In their initial evaluation, US and European specialists (n = 186) use upright forced vital capacity (FVC) most (92.8% vs 91.1%; p = 0.752). Upright FVC results are most important for US respondents when deciding to prescribe NIV; European respondents consider symptoms of orthopnea and/or dyspnea as most important. European respondents use overnight pulse oximetry (69.8% vs 7.9%; p < 0.001) and arterial blood gas analyses (62.8% vs 3.2%; p < 0.001) more than US respondents. Insurance regulations/national health care coverage impact NIV initiation more in the US than in Europe (70.0% vs 47.5%; p = 0.025). When asked if insurance/other financial constraints affects when they prescribe NIV, more US respondents answered positively (77.2% vs 15.4%; p < 0.001). In patients with no respiratory symptoms, most US specialists (68.3%) initiated NIV at VC <50% predicted; European responses showed greater variability. Conclusions: Given the impact of NIV on respiratory function and the importance of respiratory function to quality of life and survival, understanding differences that influence NIV prescribing is critical. This information may inform future study design and identify areas warranting additional research to develop best practices for NIV implementation.

Original languageEnglish
Pages (from-to)331-341
Number of pages11
JournalAmyotrophic Lateral Sclerosis & Frontotemporal Degeneration
Issue number5-6
Publication statusPublished - 3 Jul 2018


  • Amyotrophic lateral sclerosis
  • noninvasive ventilation
  • respiratory function
  • vital capacity


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