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Continuous noninvasive ventilatory support for patients with neuromuscular or chest wall disease

Author
John R Bach, MD
Section Editors
Talmadge E King, Jr, MD
R Sean Morrison, MD
Deputy Editor
Geraldine Finlay, MD

INTRODUCTION

Patients with neuromuscular or chest wall disease develop respiratory muscle dysfunction that can result in difficulty clearing airway secretions with ventilation-perfusion mismatching, and alveolar hypoventilation. Noninvasive ventilatory assistance is usually first needed at night, but eventually, daytime assistance may also be needed until many patients become dependent on continuous noninvasive ventilatory support (CNVS). Indeed, virtually all patients with neuromuscular disease except those with bulbar amyotrophic lateral sclerosis (ALS) that causes stridor and upper airway collapse can avoid tracheostomy indefinitely by using noninvasive inspiratory and expiratory aids [1].

The use of CNVS will be reviewed here. Nocturnal ventilatory assistance/support, the types of ventilators, and the role of tracheostomy are discussed separately (see "Practical aspects of nocturnal noninvasive ventilation in neuromuscular and chest wall disease"). In addition, intubated patients and those dependent on up to continuous tracheostomy mechanical ventilation (CTMV) can be extubated or decannulated to CNVS.

INDICATIONS

Symptomatic alveolar hypoventilation is the primary factor determining the need for ongoing nocturnal ventilatory assistance. Typical symptoms include fatigue, exertional dyspnea, reduced appetite, inattention, and impaired concentration and memory. Initially, hypoventilation occurs during rapid eye movement (REM) sleep and is manifest by oxyhemoglobin desaturation and hypercapnia. Hypoventilation subsequently extends throughout sleep and eventually into daytime hours [2,3]. (See "The effect of sleep in patients with neuromuscular and chest wall disorders".)

Symptoms (eg, dyspnea, somnolence, fatigue) and blood gas derangements related to chronic hypoventilation are typically relieved by nocturnal noninvasive positive pressure ventilatory assistance/support (NVS; noninvasive ventilatory support). While the effect of limiting the application of NVS to nocturnal-only does not result in markedly prolonged survival [4], clinicians who understand and use up to continuous NVS (CNVS) report decades of prolonged survival for patients with neuromuscular (NMD) and chest wall diseases. When the need for ventilatory support extends into daytime hours and is ultimately needed up to continuously, the properly informed patient can use it indefinitely as an alternative to tracheostomy ventilation.

TYPES

The respiratory muscles can be aided by manually or mechanically applying forces to the body or delivering intermittent pressure to the airway. Some devices assist inspiratory muscles, whereas others facilitate coughing, predominantly by assisting expiratory muscles. Specific types of useful devices include the following [5,6]:

                    

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Literature review current through: Nov 2016. | This topic last updated: Thu Aug 06 00:00:00 GMT+00:00 2015.
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References
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  1. Bach JR. Tracheostomy for advanced neuromuscular disease. Con. Chron Respir Dis 2007; 4:239.
  2. Redding GJ, Okamoto GA, Guthrie RD, et al. Sleep patterns in nonambulatory boys with Duchenne muscular dystrophy. Arch Phys Med Rehabil 1985; 66:818.
  3. Soudon, P. Ventilation assistee au long cours dans les maladies neuro-musculaire: Experience actuelle. Readaptation Revalidatie 1987; 3:45.
  4. Hannan LM, Dominelli GS, Chen YW, et al. Systematic review of non-invasive positive pressure ventilation for chronic respiratory failure. Respir Med 2014; 108:229.
  5. Bach JR. Update and perspectives on noninvasive respiratory muscle aids. Part 1: The inspiratory aids. Chest 1994; 105:1230.
  6. Bach JR. Update and perspective on noninvasive respiratory muscle aids. Part 2: The expiratory aids. Chest 1994; 105:1538.
  7. Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest 1993; 103:174.
  8. Bach JR, Alba AS. Intermittent abdominal pressure ventilator in a regimen of noninvasive ventilatory support. Chest 1991; 99:630.
  9. Bach JR. The Management of Patients with Neuromuscular Disease, Hanley & Belfus, Philadelphia 2004. p.331.
  10. Bach JR, Gupta K, Reyna M, Hon A. Spinal muscular atrophy type 1: prolongation of survival by noninvasive respiratory aids. Pediatric Asthma, Allergy & Immunology 2009; 22:151.
  11. Bach JR, Alba AS. Noninvasive options for ventilatory support of the traumatic high level quadriplegic patient. Chest 1990; 98:613.
  12. Bach JR, Martinez D. Duchenne muscular dystrophy: continuous noninvasive ventilatory support prolongs survival. Respir Care 2011; 56:744.
  13. Kang SW, Bach JR. Maximum insufflation capacity: vital capacity and cough flows in neuromuscular disease. Am J Phys Med Rehabil 2000; 79:222.
  14. Morrow B, Zampoli M, van Aswegen H, Argent A. Mechanical insufflation-exsufflation for people with neuromuscular disorders. Cochrane Database Syst Rev 2013; :CD010044.
  15. BARACH AL, BECK GJ. Exsufflation with negative pressure; physiologic and clinical studies in poliomyelitis, bronchial asthma, pulmonary emphysema, and bronchiectasis. AMA Arch Intern Med 1954; 93:825.
  16. Bach JR. Mechanical insufflation-exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest 1993; 104:1553.
  17. Bach JR, Saporito LR, Shah HR, Sinquee D. Decanulation of patients with severe respiratory muscle insufficiency: efficacy of mechanical insufflation-exsufflation. J Rehabil Med 2014; 46:1037.
  18. Bach JR, Sinquee DM, Saporito LR, Botticello AL. Efficacy of mechanical insufflation-exsufflation in extubating unweanable subjects with restrictive pulmonary disorders. Respir Care 2015; 60:477.
  19. Bach JR, Alba AS, Bodofsky E, et al. Glossopharyngeal breathing and noninvasive aids in the management of post-polio respiratory insufficiency. Birth Defects Orig Artic Ser 1987; 23:99.
  20. Bach JR, Rajaraman R, Ballanger F, et al. Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil 1998; 77:8.
  21. LEINER GC, ABRAMOWITZ S, SMALL MJ, et al. EXPIRATORY PEAK FLOW RATE. STANDARD VALUES FOR NORMAL SUBJECTS. USE AS A CLINICAL TEST OF VENTILATORY FUNCTION. Am Rev Respir Dis 1963; 88:644.
  22. Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest 2004; 126:1502.
  23. Bach, JR, Sortor, S, Saporito, LR. Interfaces for non-invasive intermittent positive pressure ventilatory support in North America. Eur Respir Rev 1993; 3:254.
  24. Bach JR, Gonçalves MR, Hamdani I, Winck JC. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest 2010; 137:1033.
  25. Bach JR. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory AIDS. Chest 2002; 122:92.
  26. Richard I, Giraud M, Perrouin-Verbe B, et al. Laryngotracheal stenosis after intubation or tracheostomy in patients with neurological disease. Arch Phys Med Rehabil 1996; 77:493.
  27. Bach JR, Tilton MC. Life satisfaction and well-being measures in ventilator assisted individuals with traumatic tetraplegia. Arch Phys Med Rehabil 1994; 75:626.
  28. Bach JR. A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. Chest 1993; 104:1702.
  29. Mahajan KR, Bach JR, Saporito L, Perez N. Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. Muscle Nerve 2012; 46:851.
  30. DiPALS Writing Committee, DiPALS Study Group Collaborators, McDermott CJ, et al. Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial. Lancet Neurol 2015; 14:883.
  31. Griffiths RD, Edwards RH. A new chart for weight control in Duchenne muscular dystrophy. Arch Dis Child 1988; 63:1256.
  32. Griffiths, RD. Controlling weight in muscle disease to reduce the burden. Physiotherapy 1989; 75:190.
  33. Bach JR, Gonzalez M, Sharma A, et al. Open gastrostomy for noninvasive ventilation users with neuromuscular disease. Am J Phys Med Rehabil 2010; 89:1.
  34. Duport G, Gayet E, Pries P, et al. Spinal deformities and wheelchair seating in Duchenne muscular dystrophy: twenty years of research and clinical experience. Semin Neurol 1995; 15:29.
  35. Fowler WM, Goodgold J. Rehabilitation management of neuromuscular diseases. In: Rehabilitation Medicine, Goodgold J (Ed), CV Mosby Co, Washington 1988. p.298.
  36. Bach JR, Valenza J, Ogin S. Stage 2: Stage of loss of ambulation. In: The Management of Patients with Neuromuscular Disease, Bach JR (Ed), Hanley & Belfus, Philadelphia 2004. p.125.