Survival and multidisciplinary amyotrophic lateral sclerosis clinic care at a United States Veterans Affairs medical center
DOI:
https://doi.org/10.17161/rrnmf.v5i1.20037Keywords:
ALS, multidisciplinary, PEG, NIV, edaravone, survivalAbstract
Introduction/Aims: The purpose of this work was to investigate survival outcomes in patients with amyotrophic lateral sclerosis (ALS) at our Veteran’s Affairs Medical Center multidisciplinary ALS clinic and compare this to relevant data from several European studies.
Methods: Our sample consisted of 56 total Veterans (n=56; 54 males, 2 females) that had been seen between June 24, 2013 and February 1, 2021 at our multidisciplinary ALS clinic.
Results: The median survival time of our Veterans from symptom onset was 40.96 months (95% CI of 32.17, 76.07), and the median survival time from diagnosis was 23.77 months (95% CI of 18.64, 38.58). This was consistent with the literature. Further consistent with the literature is that multidisciplinary clinics, including ours, have survival advantage over general neurology clinics. Analyzing factors that contributed to this survival, we found significant protective effect on survival from Edaravone use (HR = 0.32, p = 0.036). Otherwise, there was no significant effect on survival noted from use of percutaneous endoscopic gastrostomy (PEG), non-invasive ventilation (NIV), or Riluzole.
Conclusion: We found no significant difference in survival rates between our U.S. Veterans in our multidisciplinary ALS clinic and European multidisciplinary ALS clinics, and both are better than general neurology clinics. We also found that Edaravone use may provide some benefit to survival in this patient population.
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Bensimon G, Lacomblez L, Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med. 1994;330(9):585-91. doi: 10.1056/NEJM199403033300901. PMID: 8302340.
Writing Group; Edaravone (MCI-186) ALS 19 Study Group. Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2017;16(7):505-512. doi: 10.1016/S1474-4422(17)30115-1. Epub 2017 May 15. PMID: 28522181.
Khairoalsindi OA, Abuzinadah AR. Maximizing the Survival of Amyotrophic Lateral Sclerosis Patients: Current Perspectives. Neurol Res Int. 2018; 12; 2018:6534150. doi: 10.1155/2018/6534150. PMID: 30159171; PMCID: PMC6109498.
Oliver DJ. Palliative care in motor neurone disease: where are we now? Palliat Care. 2019; Jan 21;12:1178224218813914. doi: 10.1177/1178224218813914. PMID: 30718958; PMCID: PMC6348498.
Miller RG, Jackson CE, Kasarskis EJ, et al. Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73(15):1227-33. doi: 10.1212/WNL.0b013e3181bc01a4. PMID: 19822873; PMCID: PMC2764728.
EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis:, Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force. Eur J Neurol. 2012;19(3):360-75. doi: 10.1111/j.1468-1331.2011.03501.x. Epub 2011 Sep 14. PMID: 21914052.
Traynor BJ, Alexander M, Corr B, et al. Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996-2000. J Neurol Neurosurg Psychiatry. 2003; 74(9):1258-61. doi: 10.1136/jnnp.74.9.1258. PMID: 12933930; PMCID: PMC1738639.
Chiò A, Bottacchi E, Buffa C, et al. PARALS. Positive effects of tertiary centres for amyotrophic lateral sclerosis on outcome and use of hospital facilities. J Neurol Neurosurg Psychiatry. 2006; 77(8):948-50. doi: 10.1136/jnnp.2005.083402. Epub 2006 Apr 13. PMID: 16614011; PMCID: PMC2077622.
Zoccolella S, Beghi E, Palagano G, et al. ALS multidisciplinary clinic and survival. Results from a population-based study in Southern Italy. J Neurol. 2007; 254(8):1107-12. doi: 10.1007/s00415-006-0401-y. Epub 2007 Apr 13. PMID: 17431705.
Van den Berg JP, Kalmijn S, Lindeman E, et al. Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology. 2005;65(8):1264-7. doi: 10.1212/01.wnl.0000180717.29273.12. PMID: 16247055.
Institute of Medicine. Amyotrophic Lateral Sclerosis in Veterans: Review of the Scientific Literature. Washington, DC: The National Academies Press.2006; https://doi.org/10.17226/11757.
Beard JD, Engel LS, Richardson DB, et al. Military service, deployments, and exposures in relation to amyotrophic lateral sclerosis survival. PLoS One. 2017;12(10):e0185751. doi: 10.1371/journal.pone.0185751. PMID: 29016608; PMCID: PMC5634564.
Re DB, Yan B, Calderón-Garcidueñas L, et al. A perspective on persistent toxicants in veterans and amyotrophic lateral sclerosis: identifying exposures determining higher ALS risk. J Neurol. 2022. doi: 10.1007/s00415-021-10928-5. Epub ahead of print. PMID: 34973105.
Office of the Federal Register, National Archives and Records Administration. (2009, November 3). 74 FR 57072 - Presumption of Service Connection for Amyotrophic Lateral Sclerosis. [Government]. Office of the Federal Register, National Archives and Records Administration. https://www.govinfo.gov/app/details/FR-2009-11-04/E9-26580
Office of the Federal Register, National Archives and Records Administration. (2011, December 19). 76 FR 78823 - Schedule for Rating Disabilities; Evaluation of Amyotrophic Lateral Sclerosis. [Government]. Office of the Federal Register, National Archives and Records Administration. https://www.govinfo.gov/app/details/FR-2011-12-20/2011-32531
Paipa AJ, Povedano M, Barcelo A, et al. Survival benefit of multidisciplinary care in amyotrophic lateral sclerosis in Spain: association with noninvasive mechanical ventilation. J Multidiscip Healthc. 2019;12:465-470. doi: 10.2147/JMDH.S205313. PMID: 31354285; PMCID: PMC6590410.
Aridegbe T, Kandler R, Walters SJ, et al. The natural history of motor neuron disease: assessing the impact of specialist care. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14(1):13-9. doi: 10.3109/17482968.2012.690419. Epub 2012 May 29. PMID: 22642305.
Rooney J, Byrne S, Heverin M, Tobin K, Dick A, Donaghy C, Hardiman O. A multidisciplinary clinic approach improves survival in ALS: a comparative study of ALS in Ireland and Northern Ireland. J Neurol Neurosurg Psychiatry. 2015 May;86(5):496-501. doi: 10.1136/jnnp-2014-309601. Epub 2014 Dec 30. PMID: 25550416.
Martin S, Trevor-Jones E, Khan S, Shaw K, Marchment D, Kulka A, Ellis CE, Burman R, Turner MR, Carroll L, Mursaleen L, Leigh PN, Shaw CE, Pearce N, Stahl D, Al-Chalabi A. The benefit of evolving multidisciplinary care in ALS: a diagnostic cohort survival comparison. Amyotroph Lateral Scler Frontotemporal Degener. 2017 Nov;18(7-8):569-575. doi: 10.1080/21678421.2017.1349151. Epub 2017 Jul 18. PMID: 28719997.
Fattal D, Hester S, Wendt L. Body Weight and Obstructive Sleep Apnea: a Mathematical Relationship between Body Mass Index and Apnea Hypopnea Index in Veterans: For every 7-lb. drop in weight, expect a 7% drop in AHI. Submitted, J Clin Sleep Med. 2022.
United States Census Bureau. (2020, June 2). Release Number CB20-TPS.30 - Census Bureau Releases New Report on Veterans. https://www.census.gov/newsroom/press-releases/2020/veterans-report.html
Lacomblez L, Bensimon G, Leigh PN, et al. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet. 1996;347(9013):1425-31. doi: 10.1016/s0140-6736(96)91680-3. PMID: 8676624.
Aboussouan LS, Khan SU, Meeker DP, et al. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med. 1997; 127(6):450-3. doi: 10.7326/0003-4819-127-6-199709150-00006. PMID: 9313002.
Chiò A, Finocchiaro E, Meineri P, et al. Safety and factors related to survival after percutaneous endoscopic gastrostomy in ALS. ALS Percutaneous Endoscopic Gastrostomy Study Group. Neurology. 1999;53(5):1123-5. doi: 10.1212/wnl.53.5.1123. PMID: 10496278.
Turner MR, Scaber J, Goodfellow JA, et al (2010). The diagnostic pathway and prognosis in bulbar-onset amyotrophic lateral sclerosis. J Neurol Sci. 2010;294(1-2):81-5. doi: 10.1016/j.jns.2010.03.028. Epub 2010 May 10. PMID: 20452624.
Chiò A, Logroscino G, Hardiman O, et al (2009). Eurals Consortium. Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler. 2009;10(5-6):310-23. doi: 10.3109/17482960802566824. PMID: 19922118; PMCID: PMC3515205.
Burkhardt C , Neuwirth C , Sommacal A, et al. Is survival improved by the use of NIV and PEG in amyotrophic lateral sclerosis (ALS)? A post-mortem study of 80 ALS patients. PLoS One. 2017;12(5):e0177555. doi: 10.1371/journal.pone.0177555. eCollection.
Shoesmith CL, Findlater K, Rowe A, et al. Prognosis of amyotrophic lateral sclerosis with
respiratory onset. J Neurol Neurosurg Psychiatr. 2007;78:629–31. [PubMed: 17088331]
Tysnes O-B, Vollset SE, Aarli JA. Epidemiology of amyotrophic lateral sclerosis in Hordaland
county, western Norway. Acta Neurol Scand. 1991;83:280–5. [PubMed: 2063649]
Pinto AC, Evangelista T, Carvalho M, et al. Respiratory assistance with a
non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol
Sci. 1995;129(Suppl):19–26. [PubMed: 7595610]
Pinto A, deCarvalho M, Evangelista T, et al. Nocturnal pulse oximetry: a new
approach to establish the appropriate time for non-invasive ventilation in ALS patients.
Amyotroph Lateral Scler Other Motor Neuron Disord. 2003;4:31–5. [PubMed: 12745616]
Chio A, Mora G, Leone M, et al. for the Piemonte and Valle
d’Aosta Register for ALS. Early symptom progression rate is related to ALS outcome: a
prospective population-based study. Neurology. 2002;59:99–103. [PubMed: 12105314]
Houzen H, Kano T, Horiuchi K, Wakita M, Nagai A, Yabe I. Improved Long-Term Survival with Edaravone Therapy in Patients with Amyotrophic Lateral Sclerosis: A Retrospective Single-Center Study in Japan. Pharmaceuticals (Basel). 2021 Jul 21;14(8):705. doi: 10.3390/ph14080705. PMID: 34451802; PMCID: PMC8398196.
Okada M, Yamashita S, Ueyama H, Ishizaki M, Maeda Y, Ando Y. Long-term effects of edaravone on survival of patients with amyotrophic lateral sclerosis. eNeurologicalSci. 2018 May 17;11:11-14. doi: 10.1016/j.ensci.2018.05.001. PMID: 29928711; PMCID: PMC6006910.
Brooks BR, Berry JD, Ciepielewska M, Liu Y, Zambrano GS, Zhang J, Hagan M. Intravenous edaravone treatment in ALS and survival: An exploratory, retrospective, administrative claims analysis. EClinicalMedicine. 2022 Aug 4;52:101590. doi: 10.1016/j.eclinm.2022.101590. PMID: 35958519; PMCID: PMC9358426.
Witzel S, Maier A, Steinbach R, Grosskreutz J, Koch JC, Sarikidi A, Petri S, Günther R, Wolf J, Hermann A, Prudlo J, Cordts I, Lingor P, Löscher WN, Kohl Z, Hagenacker T, Ruckes C, Koch B, Spittel S, Günther K, Michels S, Dorst J, Meyer T, Ludolph AC; German Motor Neuron Disease Network (MND-NET). Safety and Effectiveness of Long-term Intravenous Administration of Edaravone for Treatment of Patients With Amyotrophic Lateral Sclerosis. JAMA Neurol. 2022 Feb 1;79(2):121-130. doi: 10.1001/jamaneurol.2021.4893. Erratum in: JAMA Neurol. 2022 Jun 13;:null. PMID: 35006266; PMCID: PMC8749709.
Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1(5):293-9. doi: 10.1080/146608200300079536.
Department of Veterans Affairs. Veterans Health Administration. (2021, August 30). VHA Directive 1101.07. Amyotrophic Lateral Sclerosis System of Care. https://www.va.gov/vhapublications/ViewPublication.asp?pub_ID=9423
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Copyright (c) 2024 Stephen Rostad MD, Linder Wendt MS, Mia Poleksic BS, Bryan Hutchinson-Reuss LISW, Heather Bingham MD, Deema Fattal MD
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