Clinical-demographic characteristics and ventilatory requirements of subjects with amyotrophic lateral sclerosis in a weaning and rehabilitation center
A descriptive study
Abstract
Objective: To describe the clinical-demographic characteristics and ventilatory requirements in subjects with amyotrophic lateral sclerosis (ALS) admitted to a weaning and rehabilitation center (WRC). The secondary objectives were to analyze survival time within the WRC and describe the condition at discharge.
Materials and methods: An observational, cross-sectional, and retrospective study was conducted. Subjects over 18 years of age admitted between January 1, 2016, and March 30, 2023, were included. Clinical-demographic characteristics were described, and a time-based survival analysis was performed.
Results: Thirty-three subjects were included. Of these, 84.8 % had a tracheostomy and invasive mechanical ventilation (IMV), while 5 had non-invasive mechanical ventilation (NIMV). Four subjects achieved partial weaning, and no subject with NIMV required an artificial airway. Twelve subjects (41.4 %) achieved leak speech, and 9 (27.3 %) received therapeutic feeding. The length of stay was 174 days (57-319), and the median survival time was 254 days (95 % CI 89-344).
Conclusion: Subjects with ALS admitted to the CDVMR were mostly tracheostomized, required IMV, and were bedridden upon admission. Additionally, they had prolonged stays, with goals that generally did not focus on weaning from mechanical ventilation. However, it was possible to work on other aspects that improve their daily life, such as phonation with leak ventilation and recreational oral feeding.
References
Aktein M, Uysal H. Epidemiology of amyotrophic lateral sclerosis. Turk J Neurol 2020;26:187-196. doi: 10.4274/tnd.2020.45549
Crossref
| Google Scholar
Pérez Akly M, Schiavab M, Melcomc M, Rodríguez G, Gargiulo G, Mariela Bettini, et al. Estudio epidemiológico multicéntrico sobre esclerosis lateral amiotrófica en la Ciudad de Buenos Aires. Neurología Argentina. 2017;9(4):225–230. doi: 10.1016/j.neuarg.2017.07.004
Crossref
| Google Scholar
Schoser B, Fong E, Geberhiwot T, Hughes D, Kissel JT, Madathil SC, et al. Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature. Orphanet J Rare Dis [Internet]. 2017;12(1). Disponible en: http://dx.doi.org/10.1186/s13023-017-0598-0 doi: 10.1186/s13023-017-0598-0
Crossref
| Google Scholar
Aguilera N, Carlos B, Bertoti A, de Vito E, Dubrovsky AL, Fiszman ML, et al. Consenso argentino para el diagnóstico y tratamiento de la esclerosis lateral amiotrofica (caditela) [Internet]. Com.mx. [citado el 31 de julio de 2024]. Disponible en: https://www.imbiomed.com.mx/articulo.php?id=15964
Borasio GD, Gelinas DF, Yanagisawa N. Mechanical ventilation in amyotrophic lateral sclerosis: a cross-cultural perspective. J Neurol. 1998 Aug;245 Suppl 2:S7-12; discussion S29. doi: 10.1007/s004150050641
Crossref
| Google Scholar
Zaga CJ, Chao C, Cameron T, Ross J, Rautela L, Rollinson TC, et al. A Multidisciplinary Approach to Verbal Communication Interventions for Mechanically Ventilated Adults With a Tracheostomy. Respir Care. 2023 May;68(5):680-691. doi: 10.4187/respcare.10511
Crossref
| Google Scholar
Tabor L, Gaziano J, Watts S, Robison R, Plowman EK. Defining Swallowing-Related Quality of Life Profiles in Individuals with Amyotrophic Lateral Sclerosis. Dysphagia. 2016 Jun;31(3):376-82. doi: 10.1007/s00455-015-9686-2
Crossref
| Google Scholar
De Souza LC, da Silva CT Jr, Lugon JR. Evaluation of the inspiratory pressure using a digital vacuometer in mechanically ventilated patients: Analysis of the time to achieve the inspiratory peak. Respir Care. 2012;57(2):257–62. doi: 10.4187/respcare.01228
Crossref
| Google Scholar
Jubran A, Grant BJB, Duffner LA, Collins EG, Lanuza DM, Hoffman LA, et al. Effect of pressure support vs unassisted breathing through a tracheostomy collar on weaning duration in patients requiring prolonged mechanical ventilation: A randomized trial. JAMA. 2013;309(7):671. doi: 10.1001/jama.2013.159
Crossref
| Google Scholar
Bach JR, Hon A. Amyotrophic lateral sclerosis: noninvasive ventilation, uncuffed tracheostomy tubes, and mechanically assisted coughing. Am J Phys Med Rehabil. 2010 May;89(5):412-4. doi: 10.1097/PHM.0b013e3181d8a0c7
Crossref
| Google Scholar
Garguilo M, Leroux K, Lejaille M, Pascal S, Orlikowski D, Lofaso F, Prigent H. Patient-controlled positive end-expiratory pressure with neuromuscular disease: effect on speech in patients with tracheostomy and mechanical ventilation support. Chest. 2013 May;143(5):1243-1251. doi: 10.1378/chest.12-0574
Crossref
| Google Scholar
Skoretz SA, Anger N, Wellman L, Takai O, Empey A. A Systematic Review of Tracheostomy Modifications and Swallowing in Adults. Dysphagia. 2020 Dec;35(6):935-947. doi: 10.1007/s00455-020-10115-0
Crossref
| Google Scholar
Béchet S, Hill F, Gilheaney Ó, Walshe M. Diagnostic Accuracy of the Modified Evan's Blue Dye Test in Detecting Aspiration in Patients with Tracheostomy: A Systematic Review of the Evidence. Dysphagia. 2016 Dec;31(6):721-729. doi: 10.1007/s00455-016-9737-3
Crossref
| Google Scholar
Hayashi N, Atsuta N, Yokoi D, Nakamura R, Nakatochi M, Katsuno M, et al. Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan. J Neurol Neurosurg Psychiatry. 2020 Mar;91(3):285-290. doi: 10.1136/jnnp-2019-322213
Crossref
| Google Scholar










