Use of the Mechanical In-Exsufflator in Pediatric Patients With Neuromuscular Disease and Impaired Cough

Miske, Laura J.; Hickey, Eileen M.; Kolb, S; Weiner, Daniel J.; Panitch, Howard B.

doi:10.1378/chest.125.4.1406

Cited by 174 publications

(122 citation statements)

References 20 publications

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“…85 The care team must include a physician and therapist with skill in the initiation and management of non-invasive ventilation and associated interfaces, 36,[86][87][88][89][90][91] lung-volume recruitment techniques, [92][93][94] and manual and mechanically assisted cough. [95][96][97][98][99][100][101][102] Assessments and interventions will need to be re-evaluated as the condition changes ( figures 2 and 3, panel 1). In the ambulatory stage, minimum assessment of pulmonary function (such as measurement of forced vital capacity at least annually) allows familiarity with the equipment and Appropriateness Method data and was produced solely using expert discussion.…”

Section: Respiratory Managementmentioning

confidence: 99%

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

Bushby

Finkel

Birnkrant

et al. 2010

The Lancet Neurology

1,719

1,596

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Section: Respiratory Managementmentioning

confidence: 99%

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

Bushby

Finkel

Birnkrant

et al. 2010

The Lancet Neurology

1,719

1,596

View full text Add to dashboard Cite

“…Finally, scoliosis (frequently seen in DMD) can also diminish the effectiveness of the cough. 6,12 Inspiratory muscle weakness prevents the patient from taking a deep breath, thus limiting the volume of air available to be expired during the exhalation phase of the cough. 1,8,13 The diaphragm is the major muscle of inspiration, and its weakness greatly affects the patient's ability to achieve the adequate lung volume needed for an www.pediatrics.org/cgi…”

Section: Altered Cough In Dmdmentioning

confidence: 99%

“…8,10 During the inspiratory phase, the patient inhales, usually to 60% to 90% of total lung capacity. 2,3,10,12,13 In the contraction phase, the patient's glottis closes and the expiratory muscles begin to contract. This phase lasts only 0.2 seconds; however, during this time, elevated intrathoracic pressures are reached.…”

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confidence: 99%

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Airway Clearance in Duchenne Muscular Dystrophy

Kravitz

2009

Pediatrics

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This is a summary of the presentation on airway clearance in neuromuscular disorders presented as part of the program on airway clearance in Duchenne muscular dystrophy at the 30th annual Carrell-Krusen Neuromuscular Symposium on February 20, 2008. Pediatrics 2009123:S231-S235 D UCHENE MUSCULAR DYSTROPHY (DMD) is an X-linked recessive hereditary disease that affects both skeletal and cardiac muscle. [1][2][3][4][5] The protein dystrophin is made in inadequate amounts, which leads to the break down of muscle cell walls and the cell's ultimate death. This loss of muscle leads to numerous problems including axial skeletal muscle weakness and the loss of ambulation, diaphragmatic weakness with recurrent pneumonias and progressive respiratory failure, and cardiomyopathy with arrhythmias. [2][3][4][5] The combination of cardiopulmonary failure leads to early death, with the mean life span of affected males being ϳ25 years. 1,2,[4][5][6][7] Although there is currently no cure for DMD, supportive therapies can markedly decrease the morbidity of this condition, thus prolonging the life span of these young men and improving their quality of life. This review will focus on airway clearance and cough augmentation as ways of decreasing the incidence of atelectasis, pneumonia, and respiratory failure associated with this muscular dystrophy. NORMAL COUGHCoughing works by helping to clear airway secretions from the lungs. [8][9][10][11] These secretions act as a protective layer covering the bronchi and bronchioles. They trap environmental particulate matter as well as bacteria and viruses that are inhaled during respiration. 8,11 Cilia protruding from bronchial epithelial cells move this protective layer in a proximal direction. 11 Coughing augments the ciliary clearance of these secretions, with the ultimate expectoration or swallowing of this material. 9 Coughing consists of 3 components: an inspiratory phase; a contraction phase; and an expiratory phase. 8,10 During the inspiratory phase, the patient inhales, usually to 60% to 90% of total lung capacity. 2,3,10,12,13 In the contraction phase, the patient's glottis closes and the expiratory muscles begin to contract. This phase lasts only 0.2 seconds; however, during this time, elevated intrathoracic pressures are reached. 8 During the final (expiratory) phase of a cough, the glottis opens, and there is a sudden expiratory rush of air. It is during this phase of the cough that large shearing forces are generated, cleaving off parts of the secretary lining and carrying with them the trapped foreign material. 8,10 During this expiratory phase, velocities as high as 12 m/second can be reached, with flow rates of 360 to 1000 L/minute being obtained for a normal cough. 2,3,8,10,12 Many conditions can lead to an increase in airway secretions. In pediatrics, 2 of the more common etiologies of increased airway secretions include pneumonia and asthma. These illnesses are associated with an increase in airway inflammation and a resultant increase in secretion volume. One of...

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“…There have been case reports 4 and retrospective reviews 7,30 of the clinical use of mechanical insufflationexsufflation but no further prospective comparisons with other methods of assisting airway clearance. The mechanical insufflator-exsufflator is generally well tolerated and considered safe.…”

Section: Augmenting Inspiration and Expirationmentioning

confidence: 99%

Airway clearance in neuromuscular weakness

Gauld

2009

Developmental Medicine &Amp; Child Neurology

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FVC Forced vital capacity IVC Inspiratory vital capacity PCF Peak cough flowImpaired airway clearance leads to recurrent chest infections and respiratory deterioration in neuromuscular weakness. It is frequently the cause of death. Cough is the major mechanism of airway clearance. Cough has several components, and assessment tools are available to measure the different components of cough. These include measuring peak cough flow, respiratory muscle strength, and inspiratory capacity. Each is useful in assessing the ability to generate an effective cough, and can be used to guide when techniques of assisting airway clearance may be effective for the individual and which are most effective. Techniques to assist airway clearance include augmenting inspiration by air stacking, augmenting expiration by assisting the cough, and augmenting both inspiration and expiration with the mechanical insufflator-exsufflator or by direct suctioning via a tracheostomy. Physiotherapists are invaluable in assisting airway clearance, and in teaching patients and their families how to use these techniques. Use of the mechanical insufflator-exsufflator has gained popularity in recent times, but several simpler, more economical methods are available to assist airway clearance that can be used effectively alone or in combination. This review examines the literature available on the assessment and management of impaired airway clearance in neuromuscular weakness.

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Use of the Mechanical In-Exsufflator in Pediatric Patients With Neuromuscular Disease and Impaired Cough

Cited by 174 publications

References 20 publications

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

Airway Clearance in Duchenne Muscular Dystrophy

Airway clearance in neuromuscular weakness

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