Abstract
The primary causes of death in Duchenne muscular dystrophy (DMD) patients are both respiratory and cardiac, with the former being the main cause between the 1980s and 1990s. More recently, a more structured approach to respiratory problems and, in particular, the use of nocturnal NIV, have had a major impact on increasing life expectancy rates for DMD patients. Because respiratory failure is still among the primary causes of death in those affected by DMD, it is critical to measure lung and respiratory muscle function regularly to identify early indicators of respiratory failure, monitor the progression of the disease, and plan the most ideal interventions to improve longevity and quality of life in these patients. A combination of home NIV, cough assistance, and gastrostomy feeding to manage respiratory symptoms and to use intensively during chest infections may decrease hospital admissions, facilitate care at home, and increase life expectancy for individuals with DMD.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Abbreviations
- BIPAP:
-
Bilevel positive airway pressure
- CPAP:
-
Continuous positive airway pressure
- CSA:
-
Central sleep apnea
- DMD:
-
Duchenne muscular dystrophy
- FEV1:
-
Forced expiratory volume in 1 s
- FVC:
-
Forced vital capacity
- IPAP:
-
Inspiratory positive airway pressures
- MEP:
-
Maximal expiratory mouth pressures
- MIP:
-
Maximal inspiratory mouth pressures
- NIV:
-
Noninvasive ventilation
- NIV/MPV:
-
Mouthpiece intermittent positive pressure ventilation
- OSA:
-
Obstructive sleep apnea
- PCV:
-
Pressure controlled ventilation
- PEEP:
-
Positive end-expiratory pressure
- PFT:
-
Pulmonary function tests
- PSG:
-
Polysomnography
- REM:
-
Rapid eye movement
- RR:
-
Respiratory rate
- SDB:
-
Sleep disordered breathing
- VC:
-
Vital capacity
- VCV:
-
Volume controlled ventilation
- VTPCV:
-
Volume-targeted pressure-controlled ventilation
References
MacKintosh EW, Chen ML, Benditt JO. Lifetime care of duchenne muscular dystrophy. Sleep Med Clin. 2020;15(4):485–95.
LoMauro A, D’Angelo MG, Aliverti A. Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Ther Clin Risk Manag. 2015;11:1475.
Bach JR, Martinez D. Duchenne muscular dystrophy: continuous noninvasive ventilatory support prolongs survival. Respir Care. 2011;56(6):744–50.
McKim DA, Griller N, LeBlanc C, Woolnough A, King J. Twenty-four hour noninvasive ventilation in Duchenne muscular dystrophy: a safe alternative to tracheostomy. Can Respir J. 2013;20(1):e5–9.
Sheehan DW, Birnkrant DJ, Benditt JO, Eagle M, Finder JD, Kissel J, et al. Respiratory management of the patient with Duchenne muscular dystrophy. Pediatrics. 2018;142(Supplement 2):S62–71.
Manzur AY, Kinali M, Muntoni F. Update on the management of Duchenne muscular dystrophy. Arch Dis Child. 2008;93(11):986–90.
Wagner KR, Lechtzin N, Judge DP. Current treatment of adult Duchenne muscular dystrophy. Biochim Biophys Acta. 2007;1772(2):229–37.
Deo P, Bach J. Noninvasive ventilatory support to reverse weight loss in Duchenne muscular dystrophy: a case series. Pulmonology. 2019;25(2):79–82.
Crimi C, Pierucci P, Carlucci A, Cortegiani A, Gregoretti C. Long-term ventilation in neuromuscular patients: review of concerns, beliefs, and ethical dilemmas. Respiration. 2019;97(3):185–96.
Toussaint M, Chatwin M, Soudon P. Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience. Chron Respir Dis. 2007;4(3):167–77.
Finder JD, Birnkrant D, Carl J, Farber HJ, Gozal D, Iannaccone ST, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004;170(4):456–65.
Finder JD. A 2009 perspective on the 2004 American Thoracic Society statement,“respiratory care of the patient with Duchenne muscular dystrophy”. Pediatrics. 2009;123(Supplement 4):S239–S41.
Pascoe JE, Sawnani H, Hater B, Sketch M, Modi AC. Understanding adherence to noninvasive ventilation in youth with Duchenne muscular dystrophy. Pediatr Pulmonol. 2019;54(12):2035–43.
Lee S, Lee H, Eun LY, Gang SW. Cardiac function associated with home ventilator care in Duchenne muscular dystrophy. Korean J Pediatr. 2018;61(2):59.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2023 The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Ozercan, S. (2023). Cardiopulmonary Interventions to Prolong Survival in Patients with Duchenne Muscular Dystrophy. In: Esquinas, A.M. (eds) Noninvasive Mechanical Ventilation. Springer, Cham. https://doi.org/10.1007/978-3-031-28963-7_29
Download citation
DOI: https://doi.org/10.1007/978-3-031-28963-7_29
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-031-28962-0
Online ISBN: 978-3-031-28963-7
eBook Packages: MedicineMedicine (R0)