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Comparison of PCF values by diagnosis. Boxplot comparing PCF by type of NMD. The means of PCF of DMD and CMD were statistically significantly (*) lower than that of CMT (p = 0.007 and p = 0.02). Mean PCF of DMD and CMD were lower than BMD but difference was not statistically significant. PCF peak cough flow, DMD Duchenne muscular dystrophy, CMD congenital muscular dystrophy, CMT Charcot Marie Tooth, BMD Becker’s muscular dystrophy
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PurposePatients with neuromuscular disease (NMD) experience weakened cough due to progressive respiratory muscle weakness. Peak cough flow (PCF) measurements derived from adult populations are used to recommend initiation of assisted cough therapies. The objective of this study was to characterize PCF values among pediatric patients with NMD.Method...
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Objectives:
To evaluate the impact of the COVID-19 pandemic on outpatient care in Japanese patients with neuromuscular diseases (NMDs).
Design:
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Critical care management of acute respiratory failure in patients with neuromuscular disease (NMD) such as amyotrophic lateral sclerosis (ALS) is not standardized and is challenging for many critical care specialists. Progressive hypercapnic respiratory failure and ineffective airway clearance are key issues in this patient population. Often at the...
Citations
... 3,4 These mechanisms can be affected in many NMDs. [4][5][6] Cough strength can be assessed by measuring the airflow velocity generated during coughing. Adult subjects with a cough peak flow (CPF) of <270 L/min are at increased risk of complications from respiratory infections. ...
... 7 Therefore, the initiation of cough-assist devices is indicated in these patients. 4,5 Currently, to perform these tests, a complex device setup (facemasks and infection control filters) and trained staff are needed. In addition, these tests place a substantial burden on some patients with NMDs. ...
Introduction/Aims
Cough impairment is common in individuals with neuromuscular disorders and is associated with respiratory infections and shorter survival. Cough strength is assessed by measuring cough peak flow (CPF) using a flow meter, but this method requires a complex device setup and trained staff. The aim of the study is to evaluate the reliability of a smartphone app to estimate CPF based on cough sounds in a cohort of individuals with neuromuscular disorders.
Methods
Individuals with neuromuscular disorders underwent CPF measurement with a flow meter and a smartphone app. A CPF <270 L/min was considered abnormal.
Results
Of the 50 patients studied, 26 had amyotrophic lateral sclerosis (52%), 15 had hereditary myopathies (30%) and 9 had myasthenia gravis (18%). The Intraclass correlation coefficient (ICC) between the CPF measured with a flow meter and CPF estimated with cough sounds was 0.774 ( p < .001) even if the patients had orofacial weakness (ICC = 0.806, p < .001). The smartphone app had 94.4% sensitivity and 100% specificity to detect patients with CPF <270 L/min.
Discussion
Our findings suggest that sounds measured with a smartphone app provide a reliable estimate of CPF in patients with neuromuscular disorders, even in the presence of with orofacial weakness. This may be a convenient way to monitor respiratory involvement in patients with neuromuscular disorders, but larger studies of more diverse patient cohorts are needed.
... The studies have excluded the patients without RTI (149), which introduces significant selection and reporting bias, limiting generalisability. Furthermore, baseline PCF values in young children with NMDs are below these adult-specific values suggested for starting assisted cough techniques (152), and hence it is unclear if these thresholds are useful predictors in paediatric NMDs. ...
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.
... Chez l'adulte, un débit de pointe de 180 L/min (ou valeurs approchantes, en fonction des auteurs et des pays) est souvent proposé comme valeur seuil pour définir l'efficacité de la toux [30,31]. En pédiatrie, ces seuils sont inappropriés pour les enfants de moins de 12 ans et encore moins interprétables chez le nourrisson [32][33][34]. En effet, comme nous l'avons vu plus haut, les voies respiratoires des jeunes enfants sont plus souples que celles des enfants plus âgés et peuvent donc se rétrécir ou s'affaisser pour des pressions trans-pulmonaires (différence entre la pression alvéolaire et la pression pleurale) plus faibles. Ainsi, les nourrissons peuvent probablement générer des débits transitoires supra-maximaux en utilisant des pressions expiratoires plus faibles [33]. ...
Résumé
L’encombrement des voies aériennes supérieures (VAS) et inférieures (VAI) est un symptôme fréquent dans différentes pathologies respiratoires aiguës ou chroniques de l’enfance. Des techniques de kinésithérapie de drainage des VAS et des VAI sont régulièrement prescrites pour éliminer ces sécrétions. Le rationnel d’application de ces techniques est le même que chez l’adulte, mais elles devront être adaptées pour être transposées au contexte pédiatrique. Le kinésithérapeute (également dénommé masseur kinésithérapeute ou physiothérapeute en fonction des pays) dispose d’un large éventail de techniques, et la plus adéquate sera choisie en fonction de l’âge et des préférences de l’enfant et de l’indication. Le drainage des VAS, notamment par des techniques d’irrigation nasale, est aujourd’hui recommandé dans les rhino-sinusites aiguës et chroniques de l’enfant. Il est aussi l’un des traitements symptomatiques proposés aux nourrissons atteints de bronchiolite aiguë. Certaines indications de drainage des VAI, comme la mucoviscidose, la dyskinésie ciliaire ou les pathologies neuromusculaires sont aujourd’hui largement reconnues alors que leur place dans d’autres indications comme dans le traitement des nourrissons atteints de bronchiolite est très contestée. L’application raisonnée de ces techniques souffre encore aujourd’hui d’un manque d’outils robustes et précis pour évaluer objectivement la présence d’un encombrement bronchique et le traiter en conséquence. La même limite existe également pour évaluer de façon fiable l’efficacité propre de ces techniques. Cette revue propose de faire un état des lieux des techniques utilisées par le kinésithérapeute pour drainer les VAS et les VAI chez l’enfant et un état des connaissances actuelles sur les indications des techniques de drainage, ainsi que les adjuvants couramment utilisés en complément de celles-ci.
... 79 However, the baseline CPF values suggested for starting assisted cough techniques in young children may have to be lower than the adultspecific values. 80 ...
Background:
Cough characteristics vary between patients and this can impact clinical diagnosis and care. The purpose of Part 2 of this state-of-the-art review is to update the American College of Chest Physicians (CHEST) 2006 guideline on global physiology and pathophysiology of cough.
Methods:
A review of the literature was conducted using PubMed and Medline databases from 1951 to 2019 using pre-specified search terms.
Results:
We describe the demographics of typical cough patients in the clinical setting, including how cough characteristics changes across age. We summarize the effect of common clinical conditions impacting cough mechanics and the physical properties of mucus on airway clearance.
Conclusions:
This is the second of a two-part update to the 2006 CHEST Cough Guideline; it complements part one on basic phenomenology of cough by providing an extended clinical picture of cough along with the factors that alter cough mechanics and efficiency in patients. A greater understanding of the physiology and pathophysiology of cough will improve clinical management.
... Limited studies in children with muscular dystrophies have reported single cough assessment parameters. These values, however, were not compared to normative values from age-matched healthy individuals to indicate cough deficits (Kotwal et al., 2020). A typical cough is a three-phase expulsive motor task with an inspiratory phase, a compressive phase against a closed glottis, and an expiratory airflow phase (Fontana and Widdicombe, 2007). ...
We investigated the impact of spinal cord injury (SCI) on cough capacity in 10 children (Mean ± SD, age 8 ± 4 years) and compared it to 15 typically developing children (age 8 ± 3 years). Participants underwent spirometry, single and sequential cough assessment with surface-electromyography from respiratory muscles. Inspiratory phase duration, inspiratory phase peak flow, inspiratory phase rise time, compression phase duration, expiratory phase rise time, expiratory phase peak airflow (EPPF) and cough volume acceleration (CVA) parameters of single and sequential cough were measured. Root mean square (RMS) values of right pectoralis-major, intercostal, rectus-abdominus (RA), and oblique (OB) muscles were calculated and mean of three trials were compared. The significance criterion was set at P < 0.05. The SCI group produced significantly lower lung volumes, EPPF, CVA, and RMS values of RA and OB during expiratory phases of single and sequential coughs. The decrease in activation in expiratory muscles in the SCI group accounts for the impaired expiratory flow and may contribute to risk of respiratory complications.
Completely revised and updated, the second edition of this authoritative guide provides the latest information on the diagnosis, treatment, and ongoing management of pulmonary issues in children. Available for purchase at https://www.aap.org/Pediatric-Pulmonology-2nd-Edition-Paperback
Significant inconsistencies in respiratory care provision for Duchenne muscular dystrophy (DMD) are reported across different specialist neuromuscular centres in the UK. The absence of robust clinical evidence and expert consensus is a barrier to the implementation of care recommendations in public healthcare systems as is the need to increase awareness of key aspects of care for those living with DMD. Here, we provide evidenced-based and/or consensus-based best practice for the respiratory care of children and adults living with DMD in the UK, both as part of routine care and in an emergency.
Methodology
Initiated by an expert working group of UK-based respiratory physicians (including British Thoracic Society (BTS) representatives), neuromuscular clinicians, physiotherapist and patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK respiratory teams and neuromuscular services, consensus was achieved on these best practice recommendations for respiratory care in DMD.
Result
The resulting recommendations are presented in the form of a flow chart for assessment and monitoring, with additional guidance and a separate chart setting out key considerations for emergency management. The recommendations have been endorsed by the BTS.
Conclusions
These guidelines provide practical, reasoned recommendations for all those managing day-to-day and acute respiratory care in children and adults with DMD. The hope is that this will support patients and healthcare professionals in accessing high standards of care across the UK.
