Hostname: page-component-586b7cd67f-r5fsc Total loading time: 0 Render date: 2024-11-25T21:02:36.615Z Has data issue: false hasContentIssue false
  • English
  • Français

Effects of prolonged interruption of rehabilitation routines in amyotrophic lateral sclerosis patients

Published online by Cambridge University Press:  04 May 2021

Filipe Gonçalves Open the ORCID record for Filipe Gonçalves [Opens in a new window]  and
Bruno Magalhães Open the ORCID record for Bruno Magalhães [Opens in a new window]
Filipe Gonçalves*
Affiliation:
APELA-Portuguese Amyotrophic Lateral Sclerosis Association, Porto, Portugal
Bruno Magalhães
Affiliation:
Santa Maria Health School, Porto, Portugal CINTESIS – Center for Health Technology and Services Research (NursID: Innovation and Development in Nursing), Porto, Portugal
*
Author for correspondence: Filipe Gonçalves, APELA, Centro Hospitalar Conde de Ferreira, Rua de Costa Cabral 1659, 4200-227 Porto, Portugal. E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective

Patients with amyotrophic lateral sclerosis (ALS) experienced prolonged interruption of their rehabilitation palliative care routines due to restrictive COVID-19 pandemic public health measures. This study assesses the effects of before and after the lockdown on functionality rates and quality of life (QoL) in patients with ALS.

Methods

A longitudinal observational study was conducted. Participants were assessed three times — early January (T0), before mandatory lockdown (T1), and during lockdown (T2) — using the ALS Functional Rating Scale-revised (ALSFRS-R), Fatigue Severity Scale (FSS), and the ALS-Specific Quality of Life-Short Form (ALSSQOL-SF). The paired-sample t-test and Wilcoxon signed-rank test were used.

Results

Thirty-two patients were included with a mean age of 56.9 (SD 14.2) years and mean symptoms onset of 27.1 (SD 14.3) months. ALSFRS-R mean scores decayed significantly over time when comparing T0–T1 (0.26 ± 0.38) and T1–T2 (1.36 ± 1.43) slopes (p < 0.001). Significant differences were observed between T1 and T2 for ALSSQOL-SF scores (115.31 ± 17.06 vs. 104.31 ± 20.65), especially in four specific domains, and FSS scores (34.06 ± 16.84 vs. 40.09 ± 17.63). Negative correlations between negative emotions and physical symptoms assessed by ALSSQOL-SF and FSS were found.

Significance of the results

Rehabilitation treatment routines in palliative care, such as physiotherapy and speech therapy, appear to mitigate the ALSFRS-R slope. Prolonged interruption of rehabilitation during the lockdown may have accelerated the functional decline in ALS patients’ motor skills with as measured after 2 months by the ALSFRS-R in the limb and bulbar subscores, but not respiratory subscore. Other short-term effects, increased fatigue and negative impact on QoL, were also verified.

Keywords

ALS Functional Rating ScaleAmyotrophic lateral sclerosisPhysiotherapyQuality of lifeRehabilitation in palliative careSpeech therapy
Type
Original Article
Information
Palliative & Supportive Care , Volume 20 , Issue 3 , June 2022 , pp. 369 - 374
Check for updates
Copyright
Copyright © The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

REFERENCES

Ackrivo, J, Hansen-Flaschen, J, Jones, BL, et al. (2019) Classifying patients with amyotrophic lateral sclerosis by changes in FVC. A group-based trajectory analysis. American Journal of Respiratory and Critical Care Medicine 200(12), 1513–1521.CrossRefGoogle ScholarPubMed
Andersen, PM, Abrahams, S, Borasio, GD, et al. (2012) EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)–revised report of an EFNS task force. European Journal of Neurology 19(3), 360–375.Google ScholarPubMed
Andrews, JA, Berry, JD, Baloh, RH, et al. (2020) Amyotrophic lateral sclerosis care and research in the United States during the COVID-19 pandemic: Challenges and opportunities. Muscle & Nerve 62(2), 182–186.CrossRefGoogle ScholarPubMed
Bakker, LA, Schröder, CD, van Es, MA, et al. (2017) Assessment of the factorial validity and reliability of the ALSFRS-R: A revision of its measurement model. Journal of Neurology 264(7), 1413–1420.CrossRefGoogle ScholarPubMed
Bello-Haas, VD (2018) Physical therapy for individuals with amyotrophic lateral sclerosis: Current insights. Degenerative Neurological and Neuromuscular Disease 8, 45–54.CrossRefGoogle ScholarPubMed
Braga, ACM, Pinto, A, Pinto, S, et al. (2018) The role of moderate aerobic exercise as determined by cardiopulmonary exercise testing in ALS. Neurology Research International 2018, 10.CrossRefGoogle ScholarPubMed
Chiò, A, Gauthier, A, Montuschi, A, et al. (2004) A cross sectional study on determinants of quality of life in ALS. Journal of Neurology, Neurosurgery, and Psychiatry 75(11), 1597–1601.CrossRefGoogle ScholarPubMed
Chio, A, Logroscino, G, Traynor, BJ, et al. (2013) Global epidemiology of amyotrophic lateral sclerosis: A systematic review of the published literature. Neuroepidemiology 41(2), 118–130.CrossRefGoogle ScholarPubMed
Connors, KA, Mahony, LM and Morgan, P (2019) Adaptive equipment use by people with motor neuron disease in Australia: A prospective, observational consecutive cohort study. Disability and Rehabilitation Assistive Technology 14(1), 62–67.CrossRefGoogle ScholarPubMed
de Almeida, JP, Silvestre, R, Pinto, AC, et al. (2012) Exercise and amyotrophic lateral sclerosis. Neurological Sciences 33(1), 9–15.CrossRefGoogle ScholarPubMed
Felgoise, SH, Feinberg, R, Stephens, HE, et al. (2018) Amyotrophic lateral sclerosis-specific quality of life-short form (ALSSQOL-SF): A brief, reliable, and valid version of the ALSSQOL-R. Muscle Nerve 58(5), 646–654.CrossRefGoogle ScholarPubMed
Gayoso, MV, Domingues, FS, Franca Junior, MC, et al. (2020) Cross-cultural adaptation and validation for the Brazilian population of the instrument amyotrophic lateral sclerosis-specific quality of life-short form (ALSSQOL-SF). Quality of Life Research 29(3), 805–813.CrossRefGoogle Scholar
Gibbons, CJ, Thornton, EW and Young, CA (2013) The patient experience of fatigue in motor neurone disease. Frontiers in Psychology 4, 788.CrossRefGoogle ScholarPubMed
Gordon, PH (2013) Amyotrophic lateral sclerosis: An update for 2013 clinical features, pathophysiology, management and therapeutic trials. Aging and Disease 4(5), 295–310.CrossRefGoogle ScholarPubMed
Gordon, PH, Miller, RG and Moore, DH (2004) ALSFRS-R. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders 5(Suppl 1), 90–93.CrossRefGoogle ScholarPubMed
Gordon, PH, Cheng, B, Salachas, F, et al. (2010) Progression in ALS is not linear but is curvilinear. Journal of Neurology 257(10), 1713–1717.CrossRefGoogle Scholar
Hardiman, O, Al-Chalabi, A, Chio, A, et al. (2017) Amyotrophic lateral sclerosis.Nature Reviews Disease Primers 3, 17071.CrossRefGoogle ScholarPubMed
Helleman, J, Kruitwagen, ET, van den Berg, LH, et al. (2020) The current use of telehealth in ALS care and the barriers to and facilitators of implementation: A systematic review. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration 21(3–4), 167–182.CrossRefGoogle ScholarPubMed
Hogden, A, Foley, G, Henderson, RD, et al. (2017) Amyotrophic lateral sclerosis: Improving care with a multidisciplinary approach. Journal of Multidisciplinary Healthcare 10, 205–215.CrossRefGoogle ScholarPubMed
Kasarskis, EJ, Dempsey-Hall, L, Thompson, MM, et al. (2005) Rating the severity of ALS by caregivers over the telephone using the ALSFRS-R. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders 6(1), 50–54.CrossRefGoogle ScholarPubMed
Leveque, N (2006) [Speech therapy guidelines in patients with amyotrophic lateral sclerosis]. Revue Neurologique (Paris) 162(Spec No 2), 4s269–4s272.Google Scholar
Lisle, S and Tennison, M (2015) Amyotrophic lateral sclerosis: The role of exercise. Current Sports Medicine Reports 14(1), 45–46.CrossRefGoogle ScholarPubMed
Londral, A, Pinto, A, Pinto, S, et al. (2015) Quality of life in amyotrophic lateral sclerosis patients and caregivers: Impact of assistive communication from early stages. Muscle Nerve 52(6), 933–941.CrossRefGoogle ScholarPubMed
Lou, JS (2012) Techniques in assessing fatigue in neuromuscular diseases. Physical Medicine and Rehabilitation Clinics of North America 23(1), 11–22, ix.CrossRefGoogle ScholarPubMed
Lou, JS, Weiss, MD and Carter, GT (2010) Assessment and management of fatigue in neuromuscular disease. American Journal of Hospice & Palliative Care 27(2), 145–157.CrossRefGoogle ScholarPubMed
Lunetta, C, Lizio, A, Sansone, VA, et al. (2016) Strictly monitored exercise programs reduce motor deterioration in ALS: Preliminary results of a randomized controlled trial. Journal of Neurology 263(1), 52–60.CrossRefGoogle ScholarPubMed
Maier, A, Holm, T, Wicks, P, et al. (2012) Online assessment of ALS Functional Rating Scale compares well to in-clinic evaluation: A prospective trial. Amyotrophic Lateral Sclerosis 13(2), 210–216.CrossRefGoogle ScholarPubMed
Makkonen, T, Ruottinen, H, Puhto, R, et al. (2018) Speech deterioration in amyotrophic lateral sclerosis (ALS) after manifestation of bulbar symptoms. International Journal of Language & Communication Disorders 53(2), 385–392.CrossRefGoogle ScholarPubMed
Merico, A, Cavinato, M, Gregorio, C, et al. (2018) Effects of combined endurance and resistance training in amyotrophic lateral sclerosis: A pilot, randomized, controlled study. European Journal of Translational Myology 28(1), 132–140.Google ScholarPubMed
Ng, L, Khan, F, Young, C, et al. (2017) Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 1, CD011776.Google ScholarPubMed
O'Brien, D, Stavroulakis, T, Baxter, S, et al. (2019) The optimisation of noninvasive ventilation in amyotrophic lateral sclerosis: A systematic review. European Respiratory Journal 54, 3.CrossRefGoogle ScholarPubMed
Paganoni, S, Karam, C, Joyce, N, et al. (2015) Comprehensive rehabilitative care across the spectrum of amyotrophic lateral sclerosis. NeuroRehabilitation 37(1), 53–68.CrossRefGoogle ScholarPubMed
Paipa, AJ, Povedano, M, Barcelo, A, et al. (2019) Survival benefit of multidisciplinary care in amyotrophic lateral sclerosis in Spain: Association with noninvasive mechanical ventilation. Journal of Multidisciplinary Healthcare 12, 465–470.CrossRefGoogle ScholarPubMed
Pfohl, SR, Kim, RB, Coan, GS, et al. (2018) Unraveling the complexity of amyotrophic lateral sclerosis survival prediction. Frontiers in Neuroinformatics 12, 36.CrossRefGoogle ScholarPubMed
Pinto, S and de Carvalho, M (2015) The R of ALSFRS-R: Does it really mirror functional respiratory involvement in amyotrophic lateral sclerosis? Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration 16(1–2), 120–123.CrossRefGoogle Scholar
Pinto, A, Almeida, JP, Pinto, S, et al. (2010) Home telemonitoring of non-invasive ventilation decreases healthcare utilisation in a prospective controlled trial of patients with amyotrophic lateral sclerosis. Journal of Neurology, Neurosurgery, and Psychiatry 81(11), 1238–1242.CrossRefGoogle Scholar
Proudfoot, M, Jones, A, Talbot, K, et al. (2016) The ALSFRS as an outcome measure in therapeutic trials and its relationship to symptom onset. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration 17(5–6), 414–425.CrossRefGoogle ScholarPubMed
Robbins, RA, Simmons, Z, Bremer, BA, et al. (2001) Quality of life in ALS is maintained as physical function declines. Neurology 56(4), 442–444.CrossRefGoogle ScholarPubMed
Rooney, J, Byrne, S, Heverin, M, et al. (2015) A multidisciplinary clinic approach improves survival in ALS: A comparative study of ALS in Ireland and northern Ireland. Journal of Neurology, Neurosurgery, and Psychiatry 86(5), 496–501.CrossRefGoogle ScholarPubMed
Rooney, J, Burke, T, Vajda, A, et al. (2017) What does the ALSFRS-R really measure? A longitudinal and survival analysis of functional dimension subscores in amyotrophic lateral sclerosis. Journal of Neurology, Neurosurgery, and Psychiatry 88(5), 381–385.CrossRefGoogle Scholar
Simmons, Z (2015) Patient-perceived outcomes and quality of life in ALS. Neurotherapeutics 12(2), 394–402.CrossRefGoogle ScholarPubMed
Stegmann, GM, Hahn, S, Liss, J, et al. (2020) Early detection and tracking of bulbar changes in ALS via frequent and remote speech analysis. npj Digital Medicine 3(1), 132.CrossRefGoogle ScholarPubMed
Van den Berg, JP, Kalmijn, S, Lindeman, E, et al. (2005) Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology 65(8), 1264–1267.CrossRefGoogle ScholarPubMed
Vucic, S, Krishnan, AV and Kiernan, MC (2007) Fatigue and activity dependent changes in axonal excitability in amyotrophic lateral sclerosis. Journal of Neurology, Neurosurgery, and Psychiatry 78(11), 1202–1208.CrossRefGoogle ScholarPubMed