Dr. Bortcosh is an Assistant Professor in the Division of Pediatric Critical Care and a practicing pediatric intensivist at the University of Florida. 

Learning Objectives for this series:

• Discuss unique physiology of the neuromuscular patient and how it relates to work of breathing
• Define obstacles to effective ventilation in patients with neuromuscular disease
• Describe ventilation strategies to utilize in patients with neuromuscular disease

References

1.Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Cripe L, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9(2):177-89.

2.Mercuri E, Finkel RS, Muntoni F, Wirth B, Montes J, Main M, et al. Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103-15.

3.Finkel RS, Mercuri E, Meyer OH, Simonds AK, Schroth MK, Graham RJ, et al. Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscul Disord. 2018;28(3):197-207.

4.Marino PL SK. The ICU Book. Wilkins LW, editor. Philadelphia: Lippincott Williams & Wilkins; 2007.

5.Machado DL, Silva EC, Resende MB, Carvalho CR, Zanoteli E, Reed UC. Lung function monitoring in patients with duchenne muscular dystrophy on steroid therapy. BMC Res Notes. 2012;5:435.

6.Wheeler DS, Wong HR, Zingarelli B. Pediatric Sepsis - Part I: "Children are not small adults!". Open Inflamm J. 2011;4:4-15.

7.Lo Mauro A, Aliverti A. Physiology of respiratory disturbances in muscular dystrophies. Breathe (Sheff). 2016;12(4):318-27.

8.Diaz CE, Deoras KS, Allen JL. Chest wall motion before and during mechanical ventilation in children with neuromuscular disease. Pediatr Pulmonol. 1993.

9.Perez A, Mulot R, Vardon G, Barois A, Gallego J. Thoracoabdominal pattern of breathing in neuromuscular disorders. Chest. 1996;110(2):454-61.

10.Testa MB, Pavone M, Bertini E, Petrone A, Pagani M, Cutrera R. Sleep-disordered breathing in spinal muscular atrophy types 1 and 2. Am J Phys Med Rehabil. 2005;84(9):666-70.

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