Ventilatory support and respiratory infection in patients with Amyotrophic Lateral Sclerosis
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Amyotrophic Lateral Sclerosis is a progressive neurodegenerative disease that causes significant and progressive functional loss, leading to a high degree of dependence. The disease’s consequences include hypoventilation, inefficient cough, dysphagia, and malnutrition, which predispose patients to recurrent respiratory infections and reduced life expectancy. A growing number of Amyotrophic Lateral Sclerosis patients are being treated with mechanical ventilation at home. Ventilatory support can be invasive or non-invasive. Since little has been reported on the association between ventilatory support type and the incidence of pneumonia in patients with Amyotrophic Lateral Sclerosis, this study aims to evaluate the incidence density of pneumonia in Brazilian patients with Amyotrophic Lateral Sclerosis who received private home care, correlating it with ventilatory support type and comparing it with the global incidence among home care patients.
This observational retrospective cohort study analyzed the electronic medical records of patients treated between January and December 2022.
A total of 91 patients with Amyotrophic Lateral Sclerosis were treated between January and December 2022 with mean age of 63.3 years. Of these 91 patients, 56 (62%) were tracheostomized. 33 (36%) developed pneumonia during the study period, of whom 20 had more than one infectious episode, totaling 69 pneumonia events. The incidence density of pneumonia among Amyotrophic Lateral Sclerosis patients was 3.1 cases/1000 patient days. A total of 56 (62%) Amyotrophic Lateral Sclerosis patients received invasive mechanical ventilation, and the incidence density of pneumonia was 4.1 cases/1000 ventilation days. The incidence density of pneumonia among Amyotrophic Lateral Sclerosis patients who received non-invasive ventilatory support was 1.6 cases/1000 non-invasive ventilation days and Amyotrophic Lateral Sclerosis patients who did not receive ventilatory support was 0.3 cases/1000 patient days.
Amyotrophic Lateral Sclerosis patients who receive specialized home care in Brazil often receive invasive mechanical ventilation. The modality of ventilatory support is correlated with the incidence of pneumonia, and those who receive invasive ventilatory support are more affected.
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2. Logroscino G;Traynor BJ;Hardiman O;Chiò A;Mitchell D;Swingler RJ;Millul A;Benn E;Beghi E; ; Incidence of amyotrophic lateral sclerosis in Europe. Journal of neurology, neurosurgery, and psychiatry. https://pubmed.ncbi.nlm.nih.gov/19710046/. Accessed January 10, 2023
3. Jordan H, Rechtman L, Wagner L, Kaye WE. Amyotrophic lateral sclerosis surveillance in Baltimore and Philadelphia. Muscle & Nerve. 2015;51(6):815-821. doi:10.1002/mus.24488
4. Mehta P, Kaye W, Raymond J, et al. Prevalence of Amyotrophic Lateral Sclerosis - United States, 2014. MMWR Morb Mortal Wkly Rep 2018; 67(7):216-218. doi: 10.15585/mmwr.mm6707a3
5. Camacho‐Soto A, Searles Nielsen S, Faust IM, Bucelli RC, Miller TM, Racette BA. Incidence of amyotrophic lateral sclerosis in older adults. Muscle & Nerve. 2022;66(3):289-296. doi:10.1002/mus.27652
6. Calvo AC, Manzano R, Mendonça DM, Muñoz MJ, Zaragoza P, Osta R. Amyotrophic lateral sclerosis: A focus on disease progression. BioMed Research International. 2014;2014:1-12. doi:10.1155/2014/925101
7. Calvo A, Vasta R, Moglia C, et al. Prognostic role of slow vital capacity in amyotrophic lateral sclerosis. Journal of Neurology. 2020;267(6):1615-1621. doi:10.1007/s00415-020-09751-1
8. Pisa FE, Logroscino G, Giacomelli Battiston P, Barbone F. Hospitalizations due to respiratory failure in patients with amyotrophic lateral sclerosis and their impact on survival: A population-based Cohort Study. BMC Pulmonary Medicine. 2016;16(1). doi:10.1186/s12890-016-0297-y
9. Zakharova MN, Abramova AA. Lower and upper motor neuron involvement and their impact on disease prognosis in amyotrophic lateral sclerosis. Neural Regeneration Research. 2022;17(1):65. doi:10.4103/1673-5374.314289
10. Chiò A, Calvo A, Moglia C, et al. Non-invasive ventilation in amyotrophic lateral sclerosis: A 10 year population based study. Journal of Neurology, Neurosurgery & Psychiatry. 2011;83(4):377-381. doi:10.1136/jnnp-2011-300472
11. Simonds AK. Home mechanical ventilation: An overview. Annals of the American Thoracic Society. 2016;13(11):2035-2044. doi:10.1513/annalsats.201606-454fr
12. Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73(15):1218-1226. doi:10.1212/wnl.0b013e3181bc0141
13. Bae JS, Hong Y-H, Baek WK, et al. Current status of the diagnosis and management of amyotrophic lateral sclerosis in Korea: A multi-center cross-sectional study. Journal of Clinical Neurology. 2012;8(4):293. doi:10.3988/jcn.2012.8.4.293
14. Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M. Home ventilation for amyotrophic lateral sclerosis patients: Outcomes, costs, and patient, family, and physician attitudes. Neurology. 1993;43(2):438-438. doi:10.1212/wnl.43.2.438
15. Gordon PH, Corcia P, Lacomblez L, et al. Defining survival as an outcome measure in amyotrophic lateral sclerosis. Archives of Neurology. 2009;66(6). doi:10.1001/archneurol.2009.1
16. Ceriana P, Surbone S, Segagni D, Schreiber A, Carlucci A. Decision-making for tracheostomy in amyotrophic lateral sclerosis (ALS): A retrospective study. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2017;18(7-8):492-497. doi:10.1080/21678421.2017.1317812
17. Vitacca M, Montini A, Lunetta C, et al. Impact of an early respiratory care programme with non‐invasive ventilation adaptation in patients with amyotrophic lateral sclerosis. European Journal of Neurology. 2018;25(3):556. doi:10.1111/ene.13547
18. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: A randomised controlled trial. The Lancet Neurology. 2006;5(2):140-147. doi:10.1016/s1474-4422(05)70326-4
19. Sancho J, Martínez D, Bures E, Díaz JL, Ponz A, Servera E. Bulbar impairment score and survival of stable amyotrophic lateral sclerosis patients after noninvasive ventilation initiation. ERJ Open Research. 2018;4(2):00159-02017. doi:10.1183/23120541.00159-2017
20. Vandenberghe N, Vallet A-E, Petitjean T, et al. Absence of airway secretion accumulation predicts tolerance of noninvasive ventilation in subjects with amyotrophic lateral sclerosis. Respiratory Care. 2013;58(9):1424-1432. doi:10.4187/respcare.02103
21. Baxter SK, Baird WO, Thompson S, et al. The initiation of non-invasive ventilation for patients with motor neuron disease: Patient and carer perceptions of obstacles and outcomes. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2012;14(2):105-110. doi:10.3109/17482968.2012.719238
22. Pinto AC, Evangelista T, Carvalho M, Alves MA, Sales Luís ML. Respiratory assistance with a non-invasive ventilator (bipap) in MND/ALS patients: Survival rates in a controlled trial. Journal of the Neurological Sciences. 1995;129:19-26. doi:10.1016/0022-510x(95)00052-4
23. Aboussouan LS;Khan SU;Meeker DP;Stelmach K;Mitsumoto H; Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Annals of internal medicine. https://pubmed.ncbi.nlm.nih.gov/9313002/. Accessed February 22, 2023
24. Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson T. BIPAP improves survival and rate of pulmonary function decline in patients with ALS. Journal of the Neurological Sciences. 1999;164(1):82-88. doi:10.1016/s0022-510x(99)00045-3
25. Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for amyotrophic lateral sclerosis: Nasal compared to tracheostomy-intermittent positive pressure ventilation. Journal of the Neurological Sciences. 1996;139:123-128. doi:10.1016/0022-510x(96)00099-8
26. JR; B. Amyotrophic lateral sclerosis. communication status and survival with ventilatory support. American journal of physical medicine & rehabilitation. https://pubmed.ncbi.nlm.nih.gov/8260126/. Accessed February 22, 2023
27. Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M. Home ventilation for amyotrophic lateral sclerosis patients: Outcomes, costs, and patient, family, and physician attitudes. Neurology. 1993;43(2):438-438. doi:10.1212/wnl.43.2.438
28. Moss AH, Oppenheimer EA, Casey P, et al. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Chest. 1996;110(1):249-255. doi:10.1378/chest.110.1.249
29. Hayashi H. Ventilatory support: Japanese experience. Journal of the Neurological Sciences. 1997;152. doi:10.1016/s0022-510x(97)00254-2
30. Borasio GD, Gelinas DF, Yanagisawa N. Mechanical ventilation in amyotrophic lateral sclerosis: A cross-cultural perspective. Journal of Neurology. 1998;245(14). doi:10.1007/s004150050641
31. Yamaguchi M, Hideaki H, Kuniko H. Ventilatory support in Japan: A new life with ALS and a positive approach to living with the disease. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders. 2001;2(4):209-211. doi:10.1080/14660820152882223
32. Caderno 2 - Critérios diagnósticos de infecção relacionada à assistência à saúde.pdf. Caderno 2 - Critérios Diagnósticos de Infecção Relacionada à Assistência à Saúde.pdf - Agência Nacional de Vigilância Sanitária - Anvisa. https://www.gov.br/anvisa/pt-br/centraisdeconteudo/publicacoes/servicosdesaude/publicacoes/publicacoes/caderno-2-criterios-diagnosticos-de-infeccao-relacionada-a-assistencia-a-saude.pdf/view. Accessed January 10, 2023
33. CDC/NHSN surveillance definitions for specific types of infections. https://www.cdc.gov/nhsn/pdfs/pscmanual/17pscnosinfdef_current.pdf. Accessed January 10, 2023
34. Pugliese R, Sala R, Regondi S, Beltrami B, Lunetta C. Emerging technologies for management of patients with amyotrophic lateral sclerosis: From telehealth to Assistive Robotics and neural interfaces. Journal of Neurology. 2022;269(6):2910-2921. doi:10.1007/s00415-022-10971-w
35. Sorenson EJ, Crum B, Clarke Stevens J. Incidence of aspiration pneumonia in ALS in Olmsted County, MN. Amyotrophic Lateral Sclerosis. 2007;8(2):87-89. doi:10.1080/17482960601147461
36. Sorenson EJ, Stalker AP, Kurland LT, Windebank AJ. Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology. 2002;59(2):280-282.