Noninvasive ventilation in patients with amyotrophic lateral sclerosis in palliative care

Authors

Keywords:

Palliative care, motor neuron disease, amyotrophic lateral sclerosis, noninvasive ventilation, quality of life, survival

Abstract

The study highlights that respiratory failure is one of the main causes of mortality in patients with advanced-stage amyotrophic lateral sclerosis (ALS), with non-invasive ventilation (NIV) being the recommended standard treatment. The objective was to analyze the use of NIV in palliative care for ALS patients through a systematic review of the scientific literature. Searches were conducted in databases such as Cochrane Library, Medline, Scielo, and TripDatabase, applying the JBI critical appraisal tool and synthesizing the findings narratively. Out of 951 articles identified, 15 were selected, including 3,001 adult patients with a mean age ranging from 58.7 to 69.1 years, with a predominance of males. The reviewed studies reported significant benefits of NIV, particularly in improving respiratory function, correcting nocturnal hypoxemia, and slowing the decline of respiratory muscle strength, all of which contribute to better quality of life. In conclusion, early initiation of NIV in advanced ALS patients was shown to comprehensively improve their well-being and overall quality of life.

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References

Ackrivo, J., Hsu, J. Y., Hansen-Flaschen, J., Elman, L., & Kawut, S. M. (2021). Noninvasive ventilation use is associated with better survival in amyotrophic lateral sclerosis. Annals of the American Thoracic Society, 18(3), 486–494. https://doi.org/10.1513/ANNALSATS.202002-169OC

Barry, C., Larner, E., Copsey, H., Smith, M., & Peryer, G. (2021). Non-invasive ventilation support for people with amyotrophic lateral sclerosis: Multidisciplinary team management. Current Opinion in Supportive and Palliative Care, 15(4), 214–218. https://doi.org/10.1097/SPC.0000000000000570

Berlowitz, D. J., & Sheers, N. (2021). Not Only about the Drugs: Improved Survival with Noninvasive Ventilation in Amyotrophic Lateral Sclerosis. Annals of the American Thoracic Society, 18(3), 419–420. https://doi.org/10.1513/AnnalsATS.202011-1404ED

Bertran-Recasens, B., Vidal-Notari, S., Hernández Guillamet, G., López Seguí, F., Vidal-Alaball, J., Jiménez-Balado, J., & Rubio, M. A. (2025). Epidemiology of amyotrophic lateral sclerosis: A population-based analysis, 2015–2020. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. https://doi.org/10.1080/21678421.2025.2527887

Engel, M., Glatz, C., Helmle, C., Young, P., Dräger, B., & Boentert, M. (2021). Respiratory parameters on diagnostic sleep studies predict survival in patients with amyotrophic lateral sclerosis. Journal of Neurology, 268(11), 4321–4331. https://doi.org/10.1007/s00415-021-10563-0

Filipe, C. B., Carreira, N. R., & Reis-Pina, P. (2024). Optimizing breathlessness management in amyotrophic lateral sclerosis: insights from a comprehensive systematic review. BMC palliative care, 23(1), 100. https://doi.org/10.1186/s12904-024-01429-z

Grassano, M., Koumantakis, E., Manera, U., Canosa, A., Vasta, R., Francesca, P., Fuda, G., Salamone, P., Marchese, G., Casale, F., Charrier, L., Mora, G., Moglia, C., Calvo, A., & Chiò, A. (2023). Non-invasive mechanical ventilation reduces the motor decline in amyotrophic lateral sclerosis. medRxiv. https://doi.org/10.1101/2023.05.26.23290514

Grassano, M., Koumantakis, E., Manera, U., Canosa, A., Vasta, R., Palumbo, F., Fuda, G., Salamone, P., Marchese, G., Casale, F., Charrier, L., Mora, G., Moglia, C., Calvo, A., & Chiò, A. (2024). Giving breath to motor neurons: Noninvasive mechanical ventilation slows disease progression in amyotrophic lateral sclerosis. Annals of Neurology, 95(4), 817–822. https://doi.org/10.1002/ana.26875

Jackson, C. E., Heiman-Patterson, T. D., Sherman, M., Yu, D., Kasarskis, E. J., Mitchell, M., Sattazahn, R., Scelsa, S. N., Imperato, T., Shefner, J. M., Watson, M. L., Rollins, Y., Cumming, J., Newman, D., Foley, H., Verma, A., Koggan, D., … Lu, X. (2021). Factors associated with noninvasive ventilation compliance in patients with ALS/MND. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22(S1), 40–47. https://doi.org/10.1080/21678421.2021.1917617

Jiménez, J., Tang, M., Wilson, M., Morrison, A., Ackrivo, J., & Choi, P. (2024). Initiation of noninvasive ventilation in patients with amyotrophic lateral sclerosis. Muscle & Nerve, 70(5), 1099–1103. https://doi.org/10.1002/mus.28250

Mercadante, S., Petronaci, A., Terranova, A., & Casuccio, A. (2025). Characteristics of patients with amyotrophic lateral sclerosis followed by a home palliative care team. American Journal of Hospice and Palliative Medicine, 42(5). https://doi.org/10.1177/10499091241266985

Orlova, A., Malygin, Y., Gofman, A., Sotulenko, S., Gandalian, V., Kartashov, I., Brylev, L., Bolevich, S., Nikolic Turnic, T., & Jakovljevic, V. (2024). Survival prognostic factors of non-invasive ventilation in amyotrophic lateral sclerosis: A systematic review. Life, 14(12), 1664. https://doi.org/10.3390/life14121664

Orr, J., Chen, K., Vaida, F., Schmickl, C., Laverty, C., Ravits, J., Lesser, D., Bhattacharjee, R., Malhotra, A., & Owens, R. L. (2023). Effectiveness of long-term noninvasive ventilation measured by remote monitoring in neuromuscular disease. ERJ Open Research, 9(5), 163–174. https://doi.org/10.1183/23120541.00163-2023

Plowman, E. K., Gray, L. T., Chapin, J., Anderson, A., Vasilopoulos, T., Gooch, C., Vu, T., & Wymer, J. P. (2023). Respiratory strength training in amyotrophic lateral sclerosis. Neurology, 100(15), e1634–e1642. https://doi.org/10.1212/wnl.0000000000206830

Réginault, T., Bouteleux, B., Pillet, O., Grassion, L., Wibart, P., Mathis, S., & Masson, G. L. (2023). At-home noninvasive ventilation initiation with telemonitoring in amyotrophic lateral sclerosis patients: A retrospective study. ERJ Open Research, 9(1). https://doi.org/10.1183/23120541.00438-2022

Rudnicki, S. A., Andrews, J. A., Bian, A., Cockroft, B. M., Cudkowicz, M. E., Hardiman, O., Malik, F. I., Meng, L., Wolff, A. A., & Shefner, J. M. (2021). Noninvasive ventilation use by patients enrolled in VITALITY-ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22(7–8), 486–494. https://doi.org/10.1080/21678421.2021.1904993

Sales de Campos, P., Olsen, W. L., Wymer, J. P., & Smith, B. K. (2023). Respiratory therapies for amyotrophic lateral sclerosis: A state of the art review. Chronic Respiratory Disease, 20, 1–12. https://doi.org/10.1177/14799731231175915

Sarasate, M., González, N., Córdoba-Izquierdo, A., Prats, E., Gonzalez-Moro, J. M. R., Martí, S., Lujan, M., Calle, M., Antón, A., Povedano, M., & Farrero, E. (2023). Impact of early non-invasive ventilation in amyotrophic lateral sclerosis: A multicenter randomized controlled trial. Journal of Neuromuscular Diseases, 10(4), 627–637. https://doi.org/10.3233/jnd-221658

Schmidt, R., Slotina, E., Meissner, F., Metelmann, M., Ilse, B., Vogt, V., & Freytag, A. (2025). Palliative care pathways in amyotrophic lateral sclerosis (ALS): A sequence analysis of health claims data. BMC Palliative Care, 24(1). https://doi.org/10.1186/s12904-025-01843-x

Sheers, N., Hannan, L., Rautela, L., Graco, M., Jones, J., Retica, S., Saravanan, K., Burgess, N., McGaw, R., Donovan, A., Clohessy, T., Chao, C., Charles, C., Howard, M., & Berlowitz, D. (2025). NIV@Home: A pilot randomized controlled trial of in-home noninvasive ventilation initiation compared to a single-day admission model. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 26(3–4), 239–248. https://doi.org/10.1080/21678421.2024.2416668

Spittel, S., Maier, A., Kettemann, D., Walter, B., Koch, B., Krause, K., Norden, J., Münch, C., & Meyer, T. (2021). Non-invasive and tracheostomy invasive ventilation in amyotrophic lateral sclerosis: Utilization and survival rates in a cohort study over 12 years in Germany. European Journal of Neurology, 28(4), 1160–1171. https://doi.org/10.1111/ene.14647

Volpato, E., Vitacca, M., Ptacinsky, L., Lax, A., D'Ascenzo, S., Bertella, E., Paneroni, M., Grilli, S., & Banfi, P. (2022). Home-Based Adaptation to Night-Time Non-Invasive Ventilation in Patients with Amyotrophic Lateral Sclerosis: A Randomized Controlled Trial. Journal of clinical medicine, 11(11), 3178. https://doi.org/10.3390/jcm11113178

Walsh, L., Deasy, K., Gomez, F., O’Sullivan, E., Eustace, J., Ryan, A., & Murphy, D. (2021). Use of non-invasive ventilation in motor neuron disease: A retrospective cohort analysis. Chronic Respiratory Disease, 18, 1–9. https://doi.org/10.1177/14799731211063886

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Published

2025-10-01

How to Cite

Molina-Onofa, L. C., Pujos-Espín, T. P., & Bonifaz-Díaz, D. R. (2025). Noninvasive ventilation in patients with amyotrophic lateral sclerosis in palliative care. Revista UGC, 3(S3), 330–341. Retrieved from https://universidadugc.edu.mx/ojs/index.php/rugc/article/view/256

Issue

Vol. 3 No. S3 (2025): Retos, innovaciones y perspectivas multidisciplinarias en investigación y práctica universitaria (Octubre)

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Copyright (c) 2025 Lucía Cristina Molina-Onofa, Tania Paola Pujos-Espín, Diego Raúl Bonifaz-Díaz

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