Active lung volume recruitment to preserve vital capacity in Duchenne muscular dystrophy

Chiou, Michael; Bach, John R.; Jethani, Lavina; Gallagher, Michael

doi:10.2340/16501977-2144

Cited by 39 publications

(32 citation statements)

References 27 publications

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“…To preserve lung compliance, lung volume recruitment is indicated when FVC is 60% predicted or less, achieved with a self-inflating manual ventilation bag or mechanical insufflation– exsufflation device to provide deep lung inflation once or twice daily. 25–27 …”

Section: Respiratory Managementmentioning

confidence: 99%

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management

Birnkrant

Bushby

Bann

et al. 2018

The Lancet Neurology

789

886

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A coordinated, multidisciplinary approach to care is essential for optimum management of the primary manifestations and secondary complications of Duchenne muscular dystrophy (DMD). Contemporary care has been shaped by the availability of more sensitive diagnostic techniques and the earlier use of therapeutic interventions, which have the potential to improve patients’ duration and quality of life. In part 2 of this update of the DMD care considerations, we present the latest recommendations for respiratory, cardiac, bone health and osteoporosis, and orthopaedic and surgical management for boys and men with DMD. Additionally, we provide guidance on cardiac management for female carriers of a disease-causing mutation. The new care considerations acknowledge the effects of long-term glucocorticoid use on the natural history of DMD, and the need for care guidance across the lifespan as patients live longer. The management of DMD looks set to change substantially as new genetic and molecular therapies become available.

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Section: Respiratory Managementmentioning

confidence: 99%

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management

Birnkrant

Bushby

Bann

et al. 2018

The Lancet Neurology

789

886

View full text Add to dashboard Cite

show abstract

“…In cohort of 282 patients with various NMDs, lung volume recruitment applied over several years resulted in increased MIC and LIC, despite a decrease in VC, indicating a higher number of recruitable lung units [76]. In a study including 151 patients with DMD, lung volume recruitment delayed the maximal VC decline by at least 5 years compared to previously reported series [129]. In addition, in a retrospective study of 16 individuals with DMD followed over a median of 6 years, the prescription of lung volume recruitment decreased the rate of VC decline from 4.5% predicted/year to 0.5% predicted/year [128].…”

Section: Respiratory Management Of Patients With Neuromuscular Diseasesmentioning

confidence: 76%

Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review

et al. 2019

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Respiratory muscle weakness is a major cause of morbidity and mortality in patients with neuromuscular diseases (NMDs). Respiratory involvement in NMDs can manifest broadly, ranging from milder insufficiency that may affect only sleep initially to severe insufficiency that can be life threatening. Patients with neuromuscular diseases exhibit very often sleep-disordered breathing, which is frequently overlooked until symptoms become more severe leading to irreversible respiratory failure necessitating noninvasive ventilation (NIV) or even tracheostomy. Close monitoring of respiratory function and sleep evaluation is currently the standard of care. Early recognition of sleep disturbances and initiation of NIV can improve the quality of life and prolong survival. This review discusses the respiratory impairment during sleep in patients with NMDs, the diagnostic tools available for early recognition of sleep-disordered breathing and the therapeutic options available for overall respiratory management of patients with NMDs.

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“…8 Lung volume recruitment techniques providing deep lung inflation have been suggested to recruit volume by preserving lung and chest wall compliance when used once or twice daily. 8 Studies have investigated inexpensive techniques, such as breath-stacking using volume-preset ventilation or a manual resuscitator bag, [9][10][11] as well as expensive techniques, such as MI-E, 12,13 which has a cost nearly 100 times greater than that of breathstacking via a resuscitator bag. It is not clear why Cesareo et al 1 decided to investigate the more expensive option of MI-E when less expensive techniques are available and effective.…”

Section: To the Editormentioning

confidence: 99%

Effects of Mechanical Insufflation-Exsufflation on the Breathing Pattern in Stable Subjects With Duchenne Muscular Dystrophy: A Step in a Wrong Direction

Toussaint¹,

Gonçalves

Chatwin

2019

Respir Care

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Active lung volume recruitment to preserve vital capacity in Duchenne muscular dystrophy

Abstract: For patients with Duchenne muscular dystrophy, active lung volume recruitment may help to preserve vital capacity. Effects on post-plateau vital capacity may be a useful outcome measure for therapeutic trials.

Cited by 39 publications

References 27 publications

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management

Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review

Effects of Mechanical Insufflation-Exsufflation on the Breathing Pattern in Stable Subjects With Duchenne Muscular Dystrophy: A Step in a Wrong Direction

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