Respiratory Care in Amyotrophic Lateral Sclerosis

2005 ◽  
pp. 769-792
2021 ◽  
Author(s):  
Letícia Aquino ◽  
Juliana Victor dos Santos ◽  
Jaqueline Donola Scandoleira ◽  
Jéssica Elen Gonçalves Nascimento ◽  
Letícia Moraes de Aquino

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a progressive and degenerative motor disease of the nervous system. Symptoms are variable, the main one being muscle weakness. Treatment is based on medication and monitoring by a multidisciplinary team to maintain quality of life (QoL) and autonomy. There are barriers, like mobility, and telehealth (TH) can be a possibility of care. Objectives: To identify evidence of the use of TH in patients with ALS to improve symptoms and QoL. Design and settings: Study carried out at Centro Universitario São Camilo. Methodology: Literature review in the PubMed, Lilacs and PEDro, between 2011 and 2021, in Portuguese, English or Spanish, with “ALS”, “telemedicine”, “TH”. Results: Of the 14 studies found, 13 were selected after review. The majority (93%) made use of video and telephone calls for monitoring and new orientations, after face-to-face evaluation; but all showed the possibility of remote assessment, associated or no with technological resources (such apps, accelerometers, smartwatches). 31% of the studies reported indication of TH for respiratory care in critically ill patients. In general, 93% of the papers demonstrated that TH brought benefits in maintaining QoL and improving respiratory parameters. Conclusion: Use of TH in patients with ALS seems to be to viable, safe and beneficial for assessment and monitoring, especially in advanced stages and for respiratory symptoms.


BMJ Open ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. e042780
Author(s):  
Karen Pondofe ◽  
Guilherme A F Fregonezi ◽  
Ozana Brito ◽  
Mario Emilio Dourado Júnior ◽  
Rodrigo Torres-Castro ◽  
...  

IntroductionThis study aims to investigate the effects of an optimal home-based respiratory care protocol in individuals with amyotrophic lateral sclerosis (ALS).Methods and analysisThis is a randomised, blinded controlled trial involving patients diagnosed with ALS, both sexes, age between 18 and 80 years. Patients will be randomly allocated into the conventional respiratory care (CRC) group and the optimised respiratory care home-based (ORC) group. Primary outcomes will be peak cough flow, the number of exacerbations and ALS Functional Rating Scale Revised. Secondary outcomes will include chest wall volumes, maximal respiratory pressures, sniff nasal inspiratory pressure, nasal expiratory pressure and forced vital capacity (FVC), forced expiratory volume in the 1st second (FEV1) and FEV1/FVC. The CRC group will receive educational information about respiratory care at the clinic. The ORC group will receive conventional care and home-based care. The clinical status of all individuals will be monitored weekly through telephone calls. A 6-month intervention is planned, the outcomes will be assessed every 3 months and 3 and 6 months follow-up after final evaluation. The primary and secondary results will be described as average or median for continuous variables and absolute and relative frequencies for qualitative variables. Treatment effects or differences between the outcomes (baseline, 3 months and 6 months) of the study groups will be analysed using an analysis of variance. The level of significance will be set as p≤0.05.Ethics and disseminationThe research ethics committee approved the study. It is expected to evaluate respiratory function in patients with ALS in the short, medium and long terms with home-based care protocol applied. The disease’s rapid progression is a limitation for performing a long-term clinical study.Trial registration numberRBR-3z23ts; Pre-results.


2015 ◽  
Vol 73 (10) ◽  
pp. 873-876 ◽  
Author(s):  
Marco Orsini ◽  
Agnaldo José Lopes ◽  
Sara Lucia Silveira de Menezes ◽  
Acary Bulle Oliveira ◽  
Marcos Raimundo Gomes de Freitas ◽  
...  

Amyotrophic lateral sclerosis is a progressive neuromuscular disease, resulting in respiratory muscle weakness, reduced pulmonary volumes, ineffective cough, secretion retention, and respiratory failure. Measures as vital capacity, maximal inspiratory and expiratory pressures, sniff nasal inspiratory pressure, cough peak flow and pulse oximetry are recommended to monitor the respiratory function. The patients should be followed up by a multidisciplinary team, focused in improving the quality of life and deal with the respiratory symptoms. The respiratory care approach includes airway clearance techniques, mechanically assisted cough and noninvasive mechanical ventilation. Vaccination and respiratory pharmacological support are also recommended. To date, there is no enough evidence supporting the inspiratory muscle training and diaphragmatic pacing.


1993 ◽  
Vol 7 (4_suppl) ◽  
pp. 49-64 ◽  
Author(s):  
Edward Anthony Oppenheimer

As respiratory function starts to deteriorate in those with amyotrophic lateral sclerosis, one of the principal questions that has to be answered is whether it is appropriate to provide ventilatory support. Although expensive, it is perfectly feasible to provide this at home, and this article examines many of the issues surrounding home mechanical ventilation.


Respiration ◽  
1994 ◽  
Vol 61 (2) ◽  
pp. 61-67 ◽  
Author(s):  
Michael S. Sherman ◽  
Harold L. Paz

2020 ◽  
Vol 4 (1) ◽  
pp. 2514183X2091418
Author(s):  
Junghyun Kim ◽  
John Daniel Bireley ◽  
Ghazala Hayat ◽  
Jafar Kafaie

Amyotrophic lateral sclerosis is a neurodegenerative illness that causes gradual loss of muscle function. Patients eventually develop bulbar impairment, requiring extensive respiratory support. Noninvasive ventilation (NIV) has gained attention as an easily accessible method with promising benefits. We conducted this systematic review to outline the therapeutic effects of NIV, add to previous publications discussing this topic by providing updates on newly completed and ongoing studies, and identify limitations that must be addressed in future trials. A search of PubMed and Cochran for relevant primary studies yielded 26 publications. Studies indicate NIV use is associated with improvements in quality of life, regardless of the severity of bulbar impairment. However, NIV’s benefits on survival were limited to patients with less bulbar impairment. In addition, our review found several limitations that undermine the efforts to establish a definitive treatment regimen. Future studies will need to address these problems in order to provide patients with better respiratory care.


2020 ◽  
Vol 63 (1) ◽  
pp. 59-73 ◽  
Author(s):  
Panying Rong

Purpose The purpose of this article was to validate a novel acoustic analysis of oral diadochokinesis (DDK) in assessing bulbar motor involvement in amyotrophic lateral sclerosis (ALS). Method An automated acoustic DDK analysis was developed, which filtered out the voice features and extracted the envelope of the acoustic waveform reflecting the temporal pattern of syllable repetitions during an oral DDK task (i.e., repetitions of /tɑ/ at the maximum rate on 1 breath). Cycle-to-cycle temporal variability (cTV) of envelope fluctuations and syllable repetition rate (sylRate) were derived from the envelope and validated against 2 kinematic measures, which are tongue movement jitter (movJitter) and alternating tongue movement rate (AMR) during the DDK task, in 16 individuals with bulbar ALS and 18 healthy controls. After the validation, cTV, sylRate, movJitter, and AMR, along with an established clinical speech measure, that is, speaking rate (SR), were compared in their ability to (a) differentiate individuals with ALS from healthy controls and (b) detect early-stage bulbar declines in ALS. Results cTV and sylRate were significantly correlated with movJitter and AMR, respectively, across individuals with ALS and healthy controls, confirming the validity of the acoustic DDK analysis in extracting the temporal DDK pattern. Among all the acoustic and kinematic DDK measures, cTV showed the highest diagnostic accuracy (i.e., 0.87) with 80% sensitivity and 94% specificity in differentiating individuals with ALS from healthy controls, which outperformed the SR measure. Moreover, cTV showed a large increase during the early disease stage, which preceded the decline of SR. Conclusions This study provided preliminary validation of a novel automated acoustic DDK analysis in extracting a useful measure, namely, cTV, for early detection of bulbar ALS. This analysis overcame a major barrier in the existing acoustic DDK analysis, which is continuous voicing between syllables that interferes with syllable structures. This approach has potential clinical applications as a novel bulbar assessment.


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