Changing trends in the management of end-stage neuromuscular respiratory muscle failure: Recommendations of an international consensus

American Journal of Physical Medicine and Rehabilitation

Volumn 92, Issue 3, 2013, Pages 267-277

  Bach, John R ^a   Gonçalves, Miguel R ^b   Hon, Alice ^a   Ishikawa, Yuka ^c   De Vito, Eduardo Luis ^d   Prado, Francisco ^e   Dominguez, Marie Eugenia ^f  

^a RUTGERS NEW JERSEY MEDICAL SCHOOL   (United States)

^b HOSPITAL DE SÃO JOÃO   (Portugal)

^c National Organization Yakumo Hospital   (Japan)

^d INSTITUTO DE INVESTIGACIONES MÉDICAS ALFREDO LANARI   (Argentina)

^e UNIVERSITY OF CHILE   (Chile)

^f UNIVERSIDAD NACIONAL AUTÓNOMA DE MÉXICO   (Mexico)

Author keywords

Neuromuscular Disease; Pulmonary; Respiratory Muscle Failure; Ventilation

Indexed keywords

ARTIFICIAL VENTILATION; CONSENSUS DEVELOPMENT; COUGHING; DRUG CONTRAINDICATION; EXTUBATION; HUMAN; METHODOLOGY; NEUROMUSCULAR DISEASE; NONINVASIVE VENTILATION; OXIMETRY; OXYGEN THERAPY; POSITIVE END EXPIRATORY PRESSURE; RESPIRATORY FAILURE; REVIEW;

AIRWAY EXTUBATION; CONSENSUS DEVELOPMENT CONFERENCES AS TOPIC; COUGH; HUMANS; NEUROMUSCULAR DISEASES; NONINVASIVE VENTILATION; OXIMETRY; OXYGEN INHALATION THERAPY; POSITIVE-PRESSURE RESPIRATION; RESPIRATORY INSUFFICIENCY; RESPIRATORY THERAPY; VENTILATOR WEANING;

MLCS; MLOWN;

EID: 84874291816     PISSN: 08949115     EISSN: None     Source Type: Journal    
DOI: 10.1097/PHM.0b013e31826edcf1     Document Type: Article

References (124)

1. Bach J: The history of mechanical ventilation and respiratory muscle aids, in Bach JR (ed): Noninvasive Mechanical Ventilation. Philadephia, PA, Hanley & Belfus, 2002, pp. 45-72
2. Bach J: A historical perspective on the use of noninvasive ventilatory support alternatives. Respir Care Clin N Am 1996;2:161-81
3. Round Table Conference on Poliomyelitis Equipment. National Foundation for Infantile ParalysisYMarch of Dimes, Inc. Roosevelt Hotel, White Plaines, New York City, May 1953
4. Bach JR, Alba AS: Sleep and nocturnal mouthpiece IPPV efficiency in postpoliomyelitis ventilator users. Chest 1994;106:1705-10
5. Hodes H: Treatment of respiratory difficulty in poliomyelitis, in: Poliomyelitis: Papers and Discussion: Presented at the Third International Poliomyelitis Conference. Philadelphia, PA, Lippincott, 1955, pp 91-113
6. Bach JR: Update and perspectives on noninvasive respiratory muscle aids. Part 1: The inspiratory aids. Chest 1994;105:1230-40
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;103:174-82
8. Bach JR, Alba A, Mosher R, et al: Intermittent positive pressure ventilation via nasal access in the management of respiratory insufficiency. Chest 1987;92: 168-70
9. Bach JR, Alba AS: Management of chronic alveolar hypoventilation by nasal ventilation. Chest 1990; 97:52-7
10. Servera E, Sancho J, Zafra MJ, et al: Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil 2005;84:851-7
11. Toussaint M, Steens M, Wasteels G, et al: Diurnal ventilation via mouthpiece: Survival in end-stage Duchenne patients. Eur Respir J 2006;28:549-55
12. Brochard L, Isabey D, Piquet J, et al: Reversal of acute exacerbations of chronic obstructive lung disease by inspiratory assistance with a face mask. N Engl J Med 1990;323:1523-30
13. Carrey Z, Gottfried SB, Levy RD: Ventilatory muscle support in respiratory failure with nasal positive pressure ventilation. Chest 1990;97:150-8
14. Ellis ER, Bye PT, Bruderer JW, et al: Treatment of respiratory failure during sleep in patients with neuromuscular disease, positive pressure ventilation through a nose mask. Am Rev Respir Dis 1987; 135:148-52
15. Kerby G, Mayer L, Pingleton SK: Nocturnal positive pressure ventilation via nasal mask. Am Rev Respir Dis 1987;135:738-40
16. Bach JR, O'Brien J, Krotenberg R, et al: Management of end stage respiratory failure in Duchenne muscular dystrophy. Muscle Nerve 1987;10:177-82
17. Hotes LS, Johnson JA, Sicilian L: Long-term care, rehabilitation, and legal and ethical considerations in the management of neuromuscular disease with respiratory dysfunction. Clin Chest Med 1994;15: 783-95
18. Wallgren-Pettersson C, Laing NG: 109th ENMC International Workshop: 5th workshop on nemaline myopathy, 11thY13th October 2002, Naarden, The Netherlands. Neuromuscul Disord 2003;13:501-7
19. Tzeng AC, Bach JR: Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest 2000;118:1390-6
20. Make BJ, Hill NS, Goldberg AI, et al: Mechanical ventilation beyond the intensive care unit. Report of a consensus conference of the American College of Chest Physicians. Chest 1998;113:289S-344S
21. Gomez-Merino E, Bach JR: Duchenne muscular dystrophy: Prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil 2002;81:411-5
22. Vitacca M, Paneroni M, Trainini D, et al: At home and on demand mechanical cough assistance program for patients with amyotrophic lateral sclerosis. Am J Phys Med Rehabil 2010;89:401-6
23. Bach JR, Goncalves MR, Hamdani I, et al: Extubation of patients with neuromuscular weakness: A new management paradigm. Chest 2010;137:1033-9
24. Bach JR, Saporito LR: Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning. Chest 1996;110:1566-71
25. Guyatt G, Gutterman D, Baumann M, et al: Grading strength of recommendations and quality of evidence in clinical guidelines: Report from an American College of Chest Physicians Task Force. Chest 2006;129:174-81
26. Benditt JO: Full-time noninvasive ventilation: Possible and desirable. Respir Care 2006;51:1005-12. discussion 1012-5
27. Finder JD, Birnkrant D, Carl J, et al: Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004;170:456-65
28. Birnkrant DJ, Bushby KM, Amin RS, et al: The respiratory management of patients with Duchenne muscular dystrophy: A DMD care considerations working group specialty article. Pediatr Pulmonol 2010;45:739-48
29. Robert D, Willig TN, Leger P, et al: Long-term nasal ventilation in neuromuscular disorders: Report of a consensus conference. Eur Respir J 1993;6:599-606
30. Boitano LJ: Equipment options for cough augmentation, ventilation, and noninvasive interfaces in neuromuscular respiratory management. Pediatrics 2009;123(suppl 4):S226-30
31. Dean S, Bach JR: The use of noninvasive respiratory muscle aids in the management of patients with progressive neuromuscular diseases. Respir Care Clin N Am 1996;2:223-40
32. Shneerson JM, Simonds AK: Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J 2002;20:480-7
33. Mehta S: Neuromuscular disease causing acute respiratory failure. Respir Care 2006;51:1016-21. discussion 1021-3
34. Roper H, Quinlivan R: Implementation of Bthe consensus statement for the standard of care in spinal muscular atrophy[ when applied to infants with severe type 1 SMA in the UK. Arch Dis Child 2010;95:845-9
35. Wang CH, Finkel RS, Bertini ES, et al: Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol 2007;22:1027-49
36. Hughes RA, Bihari D: Acute neuromuscular respiratory paralysis. J Neurol Neurosurg Psychiatry 1993;56:334-43
37. Corrado A, Gorini M, De Paola E: Alternative techniques for managing acute neuromuscular respiratory failure. Semin Neurol 1995;15:84-9
38. Statements AC: Noninvasive positive pressure ventilation. Respir Care 1997;42:365-9
39. Teener JW, Raps EC: Evaluation and treatment of respiratory failure in neuromuscular disease. Rheum Dis Clin North Am 1997;23:277-92
40. Barthlen GM: Nocturnal respiratory failure as an indication of noninvasive ventilation in the patient with neuromuscular disease. Respiration 1997;64 (suppl 1):35-8
41. Bella I, Chad DA: Neuromuscular disorders and acute respiratory failure. Neurol Clin 1998;16:391-417
42. Gozal D: Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy. Pediatr Pulmonol 2000;29:141-50
43. Birnkrant DJ: The assessment and management of the respiratory complications of pediatric neuromuscular diseases. Clin Pediatr (Phila) 2002;41:301-8
44. Howard RS, Davidson C: Long term ventilation in neurogenic respiratory failure. J Neurol Neurosurg Psychiatry 2003;74(suppl 3):iii24-30
45. Farrero E, Prats E, Escarrabill J: [Series 4: Respiratory muscles in neuromuscular diseases and the chest cavity. Decision making in the clinical management of patients with lateral amyotrophic sclerosis]. Arch Bronconeumol 2003;39:226-32
46. Perrin C, Unterborn JN, Ambrosio CD, et al: Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve 2004;29:5-27
47. Wallgren-Pettersson C, Bushby K, Mellies U, et al: 117th ENMC workshop: Ventilatory support in congenital neuromuscular disordersVCongenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II) 4-6 April 2003, Naarden, The Netherlands. Neuromuscul Disord 2004;14:56-69
48. Simmons Z: Management strategies for patients with amyotrophic lateral sclerosis from diagnosis through death. Neurologist 2005;11:257-70
49. Mellies U, Dohna-Schwake C, Voit T: Respiratory function assessment and intervention in neuromuscular disorders. Curr Opin Neurol 2005;18:543-7
50. Simonds AK: Ethical aspects of home long term ventilation in children with neuromuscular disease. Paediatr Respir Rev 2005;6:209-14
51. Kang SW: Pulmonary rehabilitation in patients with neuromuscular disease. Yonsei Med J 2006;47: 307-14
52. Hess DR: Noninvasive ventilation in neuromuscular disease: Equipment and application. Respir Care 2006;51:896-911. discussion 911-2
53. Simonds AK: Recent advances in respiratory care for neuromuscular disease. Chest 2006;130:1879-86
54. Panitch HB: Respiratory issues in the management of children with neuromuscular disease. Respir Care 2006;51:885-93. discussion 894-5
55. Giannini A, Pinto AM, Rossetti G, et al: Respiratory failure in infants due to spinal muscular atrophy with respiratory distress type 1. Intensive Care Med 2006;32:1851-5
56. Kirk VG: Pulmonary complications of neuromuscular disease. Paediatr Respir Rev 2006;7(suppl 1): S232-4
57. Gregory SA: Evaluation and management of respiratory muscle dysfunction in ALS. NeuroRehabilitation 2007;22:435-43
58. Simonds AK: Respiratory support for the severely handicapped child with neuromuscular disease: Ethics and practicality. Semin Respir Crit Care Med 2007;28:342-54
59. Birnkrant DJ, Panitch HB, Benditt JO, et al: American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest 2007;132:1977-86
60. Annane D, Orlikowski D, Chevret S, et al: Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev 2007;CD001941
61. Manzur AY, Kinali M, Muntoni F: Update on the management of Duchenne muscular dystrophy. Arch Dis Child 2008;93:986-90
62. Hutchinson D, Whyte K: Neuromuscular disease and respiratory failure. Pract Neurol 2008;8:229-37
63. Weber M: Meeting report of European ALS Consortium (EALSC): 6th European ALS congress research workshop and young investigators' meeting. Amyotroph Lateral Scler 2008;9:318-20
64. Ozsancak A, D'Ambrosio C, Hill NS: Nocturnal noninvasive ventilation. Chest 2008;133:1275-86
65. Ambrosino N, Carpene N, Gherardi M: Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J 2009;34:444-51
66. Benditt JO: Initiating noninvasive management of respiratory insufficiency in neuromuscular disease. Pediatrics 2009;123(suppl 4):S236-8
67. Radunovic A, Annane D, Jewitt K, et al: Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2009; 4:CD004427
68. Schroth MK: Special considerations in the respiratory management of spinal muscular atrophy. Pediatrics 2009;123 (suppl 4):S245-9
69. Racca F, Del Sorbo L, Mongini T, et al: Respiratory management of acute respiratory failure in neuromuscular diseases. Minerva Anestesiol 2010; 76:51-62
70. Miller RG, Jackson CE, Kasarskis EJ, et al: Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptommanagement, and cognitive/behavioral impairment (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009;73:1227-33
71. Panitch HB: The pathophysiology of respiratory impairment in pediatric neuromuscular diseases. Pediatrics 2009;123(suppl 4):S215-8
72. Vazquez-Sandoval A, Huang EJ, Jones SF: Hypoventilation in neuromuscular disease. Semin Respir Crit Care Med 2009;30:348-58
73. Kennedy JD, Martin AJ: Chronic respiratory failure and neuromuscular disease. Pediatr Clin North Am 2009;56:261-73. xii
74. Bach JR, Bianchi C, Aufiero E: Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest 2004;126:1502-7
75. Farrero E, Prats E, Povedano M, et al: Survival in amyotrophic lateral sclerosis with home mechanical ventilation: The impact of systematic respiratory assessment and bulbar involvement. Chest 2005;127: 2132-8
76. Sancho J, Servera E, Diaz J, et al: Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest 2004;125:1400-5
77. Bach JR, Rajaraman R, Ballanger F, et al: Neuromuscular ventilatory insufficiency: Effect of home mechanical ventilator use vs. oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil 1998;77:8-19
78. Bach J, Robert D, Leger P, et al: Sleep fragmentation in Kyphoscoliotic individuals with alveolar hypoventilation treated by NIPPV. Chest 1995;107:1552-8
79. Bach JR: Update and perspective on noninvasive respiratory muscle aids. Part 2: The expiratory aids. Chest 1994;105:1538-44
80. MaessenM,Veldink JH, van denBergLH, et al:Requests for euthanasia: Origin of suffering in ALS, heart failure, and cancer patients. J Neurol 2010;257:1192-8
81. Kang SW, Bach JR: Maximum insufflation capacity. Chest 2000;118:61-5
82. Bach JR, Mahajan K, Lipa B, et al: Lung insufflation capacity in neuromuscular disease. Am J Phys Med Rehabil 2008;87:720-5
83. Kang SW, Bach JR: Maximum insufflation capacity: Vital capacity and cough flows in neuromuscular disease. Am J Phys Med Rehabil 2000;79:222-7
84. Bach JR, Bianchi C: Prevention of pectus excavatum for children with spinal muscular atrophy type 1. Am J Phys Med Rehabil 2003;82:815-9
85. Chatwin M, Bush A, Simonds AK: Outcome of goaldirected non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I. Arch Dis Child 2011;96:426-32
86. Bach JR: Amyotrophic lateral sclerosis: Predictors for prolongation of life by noninvasive respiratory aids. Arch Phys Med Rehabil 1995;76:828-32
87. Bach JR, Goncalves MR, Paez S: Expiratory flow maneuvers in patients with neuromuscular diseases. Am J Phys Med Rehabil 2006;85:105-11
88. Bach JR: Mechanical insufflation-exsufflation. Comparison of peak expiratory flows withmanually assisted and unassisted coughing techniques. Chest 1993; 104:1553-62
89. Bach JR: Continuous noninvasive ventilation for patients with neuromuscular disease and spinal cord injury. Semin Respir Crit Care Med 2002;23: 283-92
90. Schonhofer B, Sortor-Leger S: Equipment needs for noninvasivemechanical ventilation.Eur Respir J 2002; 20:1029-36
91. Fauroux B, LerouxK,Desmarais G, et al: Performance of ventilators for noninvasive positive-pressure ventilation in children. Eur Respir J 2008;31:1300-7
92. Bach JR, Martinez D: Duchenne muscular dystrophy: Continuous noninvasive ventilatory support prolongs survival. Respir Care 2011;56:744-50
93. Goncalves MR: Noninvasive ventilation and mechanical assisted cough: Efficacy from acute to chronic care [PhD thesis in BioMedicine]. Porto, Portugal, Faculty of Medicine, University of Porto, 2010
94. Boitano LJ, Benditt JO: An evaluation of home volume ventilators that support open-circuit mouthpiece ventilation. Respir Care 2005;50:1457-61
95. Bach JR: Bilevel pressure vs volume ventilators for amyotrophic lateral sclerosis patients. Chest 2006;130:1949. author reply 1949-50
96. Chatwin M: How to use a mechanical insufflatorexsufflator Bcough assist machine[. Breathe 2008; 4:321-5
97. Fishburn MJ, Marino RJ, Ditunno JF Jr: Atelectasis and pneumonia in acute spinal cord injury. Arch Phys Med Rehabil 1990;71:197-200
98. Goncalves MR, Honrado T, Winck JC, et al: Effects of mechanical insufflation-exsufflation in preventing respiratory failure after extubation: A randomized controlled trial. Crit Care 2012;16:R48
99. Bach JR, Intintola P, Alba AS, Holland I: The ventilator-assisted individual. Cost analysis of institutionalization vs rehabilitation and in-home management. Chest 1992;101:26-30
100. Bach J, Gupta K, Reyna M, Hon A: Spinal muscular atrophy type 1: Prolongation of survival by noninvasive respiratory aids. Pediatric Asthma Allergy Immunol 2009;22:151-62
101. Winck JC, Goncalves MR, Lourenco C, et al: Effects of mechanical insufflation-exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance. Chest 2004;126:774-80
102. Fauroux B, Guillemot N, Aubertin G, et al: Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. Chest 2008;133:161-8
103. Sancho J, Servera E, Marin J, et al: Effect of lung mechanics on mechanically assisted flows and volumes. Am J Phys Med Rehabil 2004;83:698-703
104. Soudon P, Steens M, Toussaint M: A comparison of invasive versus noninvasive full-time mechanical ventilation in Duchenne muscular dystrophy. Chron Respir Dis 2008;5:87-93
105. Vianello A, Bevilacqua M, Arcaro G, et al: Prevention of pulmonary morbidity in patients with neuromuscular disorders: A possible role for permanent cricothyroid minitracheostomy. Chest 1998;114:346-7
106. Bach JR: Tracheostomy for advanced neuromuscular disease. Con. Chron Respir Dis 2007;4:239-41
107. Bach JR, Goncalves M: Ventilator weaning by lung expansion and decannulation. Am J Phys Med Rehabil 2004;83:560-8
108. Bach JR, Alba AS: Noninvasive options for ventilatory support of the traumatic high level quadriplegic patient. Chest 1990;98:613-9
109. Bach JR: Amyotrophic lateral sclerosis: Prolongation of life by noninvasive respiratory AIDS. Chest 2002;122:92-8
110. Birnkrant DJ, Pope JF, Eiben RM: Management of the respiratory complications of neuromuscular diseases in the pediatric intensive care unit. J Child Neurol 1999;14:139-43
111. Baydur A, Kanel G: Tracheobronchomalacia and tracheal hemorrhage in patients with Duchenne muscular dystrophy receiving long-term ventilation with uncuffed tracheostomies. Chest 2003;123:1307-11
112. Biering-Sorensen M, Biering-Sorensen F: Tracheostomy in spinal cord injured: Frequency and follow up. Paraplegia 1992;30:656-60
113. Ishikawa Y, Miura T, Aoyagi T, et al: Duchenne muscular dystrophy: Survival by cardio-respiratory interventions. Neuromuscul Disord 2011;21:47-51
114. Bach JR: A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. Chest 1993;104:1702-6
115. Epstein SK: Late complications of tracheostomy. Respir Care 2005;50:542-9
116. Sancho J, Servera E, Vergara P, et al: Mechanical insufflation- exsufflation vs. tracheal suctioning via tracheostomy tubes for patients with amyotrophic lateral sclerosis: A pilot study. AmJ Phys Med Rehabil 2003;82:750-3
117. Bach JR, Niranjan V, Weaver B: Spinal muscular atrophy type 1: A noninvasive respiratory management approach. Chest 2000;117:1100-5
118. Bach JR, Saltstein K, Sinquée D, et al: Long-term survival in Werdnig-Hoffmann disease. Am J Phys Med Rehabil 2007;86:339-45. quiz 346-8, 379
119. Albert SM, Murphy PL, Del Bene ML, et al: Prospective study of palliative care in ALS: Choice, timing, outcomes. J Neurol Sci 1999;169:108-13
120. Kuhnlein P, Kubler A, Raubold S, et al: Palliative care and circumstances of dying in German ALS patients using non-invasive ventilation. Amyotroph Lateral Scler 2008;9:91-8
121. Kwiecinski H: [Symptomatic treatment and palliative care of ALS]. Neurol Neurochir Pol 2001;35:51-9
122. Laaksovirta H: [Palliative care tailored according to the symptoms of the ALS patient is an art form]. Duodecim 2005;121:220-4
123. Zimmerman EK, Eslinger PJ, Simmons Z, et al: Emotional perception deficits in amyotrophic lateral sclerosis. Cogn Behav Neurol 2007;20:79-82
124. Blackhall LJ: Amyotrophic lateral sclerosis and palliative care: Where we are, and the road ahead. Muscle Nerve 2012;45:311-8