Respiratory measurements and airway clearance device prescription over one year in amyotrophic lateral sclerosis

Tattersall, Rachel; Murray, Deirdre; Heverin, Mark; Rooney, James; Tobin, Katy; Vance, Roisin; Hardiman, Orla; Meldrum, Dara

doi:10.1080/21678421.2019.1697887

Cited by 10 publications

(7 citation statements)

References 21 publications

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“…A recent study noted that in ALS, cough PEF values decline rapidly at a rate of 124.8 L/minute annually, further highlighting the potential clinical effect of these results and potential translation to improved secretion management and airway defense physiologic capacity. 31 The attenuated one-year ALSFRS-R bulbar subscale decline results may be related to the noted cough improvements, given that it includes a secretion management item. In addition, oral intake was preserved in 86% of active RST participants, compared with only 64% in the sham RST group.…”

Section: Discussionmentioning

confidence: 99%

Respiratory Strength Training in Amyotrophic Lateral Sclerosis

Plowman¹,

Gray

Chapin

et al. 2023

Neurology

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Objective:Evaluate the short-term physiologic and one-year functional impact of a 12-week inspiratory and expiratory respiratory strength training (RST) program in individuals with amyotrophic lateral sclerosis (ALS).Methods:A double-blind, randomized, sham-controlled trial was conducted in 45individuals with early-stage ALS. Participants were randomized into 12-weeks of active-RST (30% load, n=23) or sham-RST (0% load, n=22). An intent-to-treat analysis was conducted. Linear regression of pre-post change with group status and pre-test scores as predictors was conducted. Primary outcomes included maximum expiratory and inspiratory pressure (MEP, MIP) and secondary outcomes were cough spirometry and forced vital capacity. Exploratory follow-up outcomes included one-year global and bulbar decline (ALS Functional Rating Scale-Revised- ALSFRS-R total and Bulbar subscale slope), oral-intake status, and time to noninvasive ventilation (NIV).Results:RST completion rate was 91% with no RST-related adverse events. A 12-week RST program led to increases in MEP (p=0.004) but not MIP (p=0.33). On average, MEP increased by 20.8 cmH20 following active-RST (95% CI: +7.6, +33.9) and decreased by 1.0 cmH20 (95% CI: -9.1, +7.2) following sham-RST. Mean MIP increased by 8.9 cmH20 (95% CI: +1.5, +16.3) and 4.8 cmH20 (95% CI: -0.6, +10.2) for the active and sham groups respectively.Secondary Outcomes: RST led to significant increases in cough peak inspiratory flow (p=0.02), however, did not impact cough expiratory flow (p= 0.06) or FVC (p=0.60).Twelve-Month Outcomes: A significant difference in ALSFRS-R Bulbar subscale slope was observed across treatment groups, with a more than two-fold faster rate of bulbar decline in the sham versus active-RST groups observed (-0.29 vs. -0.12 points/month,p=0.02). Total ALSFRS-R slope, feeding status, and time to NIV did not differ across treatment groups (p>0.05).Conclusions:RST was well tolerated and led to improvements in some, but not all, short- and long-term outcomes. RST represents a proactive rehabilitative intervention that could increase physiologic capacity of specific breathing and airway clearance functions during the early stages of ALS. Further work is needed to determine optimal training intensity, resistance load specifications, and potential long-term functional outcomes.

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Section: Discussionmentioning

confidence: 99%

Respiratory Strength Training in Amyotrophic Lateral Sclerosis

Plowman¹,

Gray

Chapin

et al. 2023

Neurology

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“…Patients who begin using LVR when PCF is already below the critical threshold of 160 L/min may yield a limited clinical benefit on cough efficacy. 61…”

Section: Airway Clearancementioning

confidence: 99%

Respiratory therapies for Amyotrophic Lateral Sclerosis: A state of the art review

et al. 2023

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition noteworthy for upper and lower motor neuron death. Involvement of respiratory motor neuron pools leads to progressive pathology. These impairments include decreases in neural activation and muscle coordination, progressive airway obstruction, weakened airway defenses, restrictive lung disease, increased risk of pulmonary infections, and weakness and atrophy of respiratory muscles. These neural, airway, pulmonary, and neuromuscular changes deteriorate integrated respiratory-related functions including sleep, cough, swallowing, and breathing. Ultimately, respiratory complications account for a large portion of morbidity and mortality in ALS. This state-of-the-art review highlights applications of respiratory therapies for ALS, including lung volume recruitment, mechanical insufflation-exsufflation, non-invasive ventilation, and respiratory strength training. Therapeutic acute intermittent hypoxia, an emerging therapeutic tool for inducing respiratory plasticity will also be introduced. A focus on emerging evidence and future work underscores the common goal to continue to improve survival for patients living with ALS.

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“…Reported RTI rates vary widely, for example, from 9% to 75% in people with ALS/MND ( 23 , 88 , 94 , 144 – 146 ). The variance in rates may be explained by differences in study methodology (retrospective chart review vs. prospective data), RTI definition (viral, bacterial, community-acquired, etc.…”

Section: Respiratory Tract Infections In Neuromuscular Diseasementioning

confidence: 99%

The role of lung volume recruitment therapy in neuromuscular disease: a narrative review

Sheers

O’Sullivan

Howard

et al. 2023

Front. Rehabil. Sci.

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Respiratory muscle weakness results in substantial discomfort, disability, and ultimately death in many neuromuscular diseases. Respiratory system impairment manifests as shallow breathing, poor cough and associated difficulty clearing mucus, respiratory tract infections, hypoventilation, sleep-disordered breathing, and chronic ventilatory failure. Ventilatory support (i.e., non-invasive ventilation) is an established and key treatment for the latter. As survival outcomes improve for people living with many neuromuscular diseases, there is a shift towards more proactive and preventative chronic disease multidisciplinary care models that aim to manage symptoms, improve morbidity, and reduce mortality. Clinical care guidelines typically recommend therapies to improve cough effectiveness and mobilise mucus, with the aim of averting acute respiratory compromise or respiratory tract infections. Moreover, preventing recurrent infective episodes may prevent secondary parenchymal pathology and further lung function decline. Regular use of techniques that augment lung volume has similarly been recommended (volume recruitment). It has been speculated that enhancing lung inflation in people with respiratory muscle weakness when well may improve respiratory system “flexibility”, mitigate restrictive chest wall disease, and slow lung volume decline. Unfortunately, clinical care guidelines are based largely on clinical rationale and consensus opinion rather than level A evidence. This narrative review outlines the physiological changes that occur in people with neuromuscular disease and how these changes impact on breathing, cough, and respiratory tract infections. The biological rationale for lung volume recruitment is provided, and the clinical trials that examine the immediate, short-term, and longer-term outcomes of lung volume recruitment in paediatric and adult neuromuscular diseases are presented and the results synthesised.

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Respiratory measurements and airway clearance device prescription over one year in amyotrophic lateral sclerosis

Cited by 10 publications

References 21 publications

Respiratory Strength Training in Amyotrophic Lateral Sclerosis

Respiratory Strength Training in Amyotrophic Lateral Sclerosis

Respiratory therapies for Amyotrophic Lateral Sclerosis: A state of the art review

The role of lung volume recruitment therapy in neuromuscular disease: a narrative review

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