Respiratory therapies for amyotrophic lateral sclerosis: A primer

Muscle and Nerve

Volumn 46, Issue 3, 2012, Pages 313-331

  Gruis, Kirsten L ^a   Lechtzin, Noah ^b  

^a UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

^b JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Amyotrophic lateral sclerosis; Mechanical insufflation exsufflation; Noninvasive ventilation; Practical guide; Respiratory therapy

Indexed keywords

AMITRIPTYLINE; TEMAZEPAM; TRAZODONE;

AERATION; AMYOTROPHIC LATERAL SCLEROSIS; ARTERIAL GAS; ARTIFICIAL VENTILATION; BREATHING MECHANICS; BREATHING MUSCLE; CHEST WALL OSCILLATION; COUGHING; DENERVATION; ELECTRODIAGNOSIS; FACE MASK; FUNCTIONAL RESIDUAL CAPACITY; GAS EXCHANGE; HEADGEAR; HUMAN; HUMIDIFIER; HYPERCAPNIA; HYPERSALIVATION; MASK; MEDICAL DEVICE; MUSCLE STRENGTH; MUSCLE WEAKNESS; NASAL PILLOW SYSTEM; NONINVASIVE VENTILATION; NOSE CONGESTION; OXYGEN THERAPY; POSITIVE END EXPIRATORY PRESSURE; POST TREATMENT SURVIVAL; PRACTICE GUIDELINE; PREDICTIVE VALUE; PRIORITY JOURNAL; QUALITY OF LIFE; RESPIRATORY FAILURE; RESPIRATORY FUNCTION; REVIEW; RHINITIS; SKIN IRRITATION; SLEEP DISORDER; SLEEP DISORDERED BREATHING; SLOW VITAL CAPACITY; TRACHEOSTOMY; VENTILATOR; VITAL CAPACITY;

AMYOTROPHIC LATERAL SCLEROSIS; EVIDENCE-BASED MEDICINE; HUMANS; RESPIRATORY INSUFFICIENCY; RESPIRATORY THERAPY;

EID: 84865342824     PISSN: 0148639X     EISSN: 10974598     Source Type: Journal    
DOI: 10.1002/mus.23282     Document Type: Review

References (86)

1. Kurian KM, Forbes RB, Colville S, Swingler RJ. Cause of death and clinical grading criteria in a cohort of amyotrophic lateral sclerosis cases undergoing autopsy from the Scottish Motor Neurone Disease Register. J Neurol Neurosurg Psychiatry 2009; 80: 84-87.
2. Corcia P, Pradat PF, Salachas F, Bruneteau G, Forestier N, Seilhean D, et al. Causes of death in a post-mortem series of ALS patients. Amyotroph Lateral Scler 2008; 9: 59-62.
3. Bergofsky EH. Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis 1979; 119: 643-669.
4. De Troyer A, Borenstein S, Cordier R. Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax 1980; 35: 603-610.
5. Pinto AC, Evangelista T, de Carvalho M, Alves MA, Sales Luis ML. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci 1995; 129(Suppl): 19-26.
6. Aboussouan LS, Khan SU, Meeker DP, Stelmach K, Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med 1997; 127: 450-453.
7. Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci 1999; 164: 82-88.
8. Gruis KL, Brown DL, Lisabeth LD, Zebarah VA, Chervin RD, Feldman EL. Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients. J Neurol Sci 2006; 247: 59-63.
9. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 2006; 5: 140-147.
10. Bourke SC, Bullock RE, Williams TL, Shaw PJ, Gibson GJ. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology 2003; 61: 171-177.
11. Lyall RA, Donaldson N, Fleming T, Wood C, Newsom-Davis I, Polkey MI, et al. A prospective study of quality of life in ALS patients treated with noninvasive ventilation. Neurology 2001; 57: 153-156.
12. Carratu P, Spicuzza L, Cassano A, Maniscalco M, Gadaleta F, Lacedonia D, et al. Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency. Orphanet J Rare Dis 2009; 4: 10.
13. Miller RG, Rosenberg JA, Gelinas D, Mitsumoto H, Newman D, Sufit R, et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology 1999; 52: 1311-1323.
14. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009; 73: 1218-1226.
15. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation-a consensus conference report. Chest 1999; 116: 521-534.
16. Bradley WG, Anderson F, Gowda N, Miller RG. Changes in the management of ALS since the publication of the AAN ALS practice parameter 1999. Amyotroph Lateral Scler Other Motor Neuron Disord 2004; 5: 240-244.
17. De Troyer A, Kirkwood PA, Wildon TA. Respiratory action of the intercostal muscles. Physiol Rev 2005; 85: 717-756.
18. Goldman MD, Loh L, Sears TA. The respiratory activity of human levator costae muscles and its modification by posture. J Physiol 1985; 362: 189-204.
19. De Troyer A, Sampson MG. Activation of the parasternal intercostals during breathing efforts in human subjects. J Appl Physiol 1982; 52: 524-529.
20. Loring SH. Action of human respiratory muscles inferred from finite element analysis of rib cage. J Appl Physiol 1992; 72: 1461-1465.
21. de Carvalho M, Pinto S, Swash M. Association of paraspinal and diaphragm denervation in ALS. Amyotroph Lateral Sclerosis 2010; 11: 63-66.
22. Uldry C, Fitting JW. Maximal values of sniff nasal inspiratory pressure in healthy subjects. Thorax 1995; 50: 371-375.
23. ATS/ERS Statement on respiratory muscle testing. Am J Respir Crit Care Med 2002; 166: 518-624.
24. Fallat RJ, Jewitt B, Bass M, Kamm B, Norris FH Jr. Spirometry in amyotrophic lateral sclerosis. Arch Neurol 1979; 36: 74-80.
25. Schiffman PL, Belsh JM. Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration. Chest 1993; 103: 508-513.
26. Gruis KL, Brown DL, Schoennemann A, Zebarah VA, Feldman EL. Predictors of noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis. Muscle Nerve 2005; 32: 808-811.
27. Lechtzin N, Wiener CM, Shade DM, Clawson L, Diette GB. Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest 2002; 121: 436-442.
28. Mendoza M, Gelinas DF, Moore DH, Miller RG. A comparison of maximal inspiratory pressure and forced vital capacity as potential criteria for initiating non-invasive ventilation in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2007; 8: 106-111.
29. Lyall RA, Donaldson N, Polkey MI, Leigh PN, Moxham J. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain 2001; 124: 2000-2013.
30. Hart N, Polkey MI, Sharshar T, Falaize L, Fauroux B, Raphael JC, et al. Limitations of sniff nasal pressure in patients with severe neuromuscular weakness. J Neurol Neurosurg Psychiatry 2003; 74: 1685-1687.
31. Beck M, Giess R, Wurffel W, Magnus T, Ochs G, Toyka KV. Comparison of maximal voluntary isometric contraction and Drachman's hand-held dynamometry in evaluating patients with amyotrophic lateral sclerosis. Muscle Nerve 1999; 22: 1265-1270.
32. Pinto A, De CM, Evangelista T, Lopes A, Sales-Luis L. Nocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients. Amyotroph Lateral Scler Other Motor Neuron Disord 2003; 4: 31-35.
33. Lechtzin N, Scott Y, Busse AM, Clawson LL, Kimball R, Wiener CM. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler 2007; 8: 185-188.
34. Jaye J, Chatwin M, Dayer M, Morrell MJ, Simonds AK. Autotitrating versus standard noninvasive ventilation: a randomised crossover trial. Eur Respir J 2009; 33: 566-571.
35. Navalesi P, Fanfulla F, Frigerio P, Gregoretti C, Nava S. Physiologic evaluation of noninvasive mechanical ventilation delivered with three types of masks in patients with chronic hypercapnic respiratory failure. Crit Care Med 2000; 28: 1785-1790.
36. Lechtzin N, Weiner CM, Clawson L. A fatal complication of noninvasive ventilation. N Engl J Med 2001; 344: 533.
37. Bach JR, Alba AS. Management of chronic alveolar hypoventilation by nasal ventilation. Chest 1990; 97: 52-57.
38. Gay PC, Edmonds LC. Severe hypercapnia after low-flow oxygen therapy in patients with neuromuscular disease and diaphragmatic dysfunction. Mayo Clin Proc 1995; 70: 327-330.
39. Melo J, Homma A, Iturriaga E, Frierson L, Amato A, Anzueto A, et al. Pulmonary evaluation and prevalence of non-invasive ventilation in patients with amyotrophic lateral sclerosis: a multicenter survey and proposal of a pulmonary protocol. J Neurol Sci 1999; 169: 114-117.
40. Roberts F. Respiratory physiology. Update in Anaesthesia 2000; 1-3.
41. Hillberg RE, Johnson DC. Noninvasive ventilation. N Engl J Med 1997; 337: 1746-1752.
42. Berry RB, Chediak A, Brown LK, Finder J, Gozal D, Iber C, et al. Best clinical practices for the sleep center adjustment of noninvasive positive pressure ventilation (NPPV) in stable chronic alveolar hypoventilation syndromes. J Clin Sleep Med 2010; 6: 491-509.
43. Peysson S, Vandenberghe N, Philit F, Vial C, Petitjean T, Bouhour F, et al. Factors predicting survival following noninvasive ventilation in amyotrophic lateral sclerosis. Eur Neurol 2008; 59: 164-171.
44. Butz M, Wollinsky KH, Wiedemuth-Catrinescu U, Sperfeld A, Winter S, Mehrkens HH, et al. Longitudinal effects of noninvasive positive-pressure ventilation in patients with amyotrophic lateral sclerosis. Am J Phys Med Rehabil 2003; 82: 597-604.
45. Janssens JP, Howarth-Frey C, Chevrolet JC, Abajo B, Rochat T. Transcutaneous PC02 to monitor noninvasive mechanical ventilation in adults: assessment of a new transcutaneous PC02 device. Chest 1998; 113: 768-773.
46. Chai CL, Pathinathan A, Smith B. Continuous positive airway pressure delivery interfaces for obstructive sleep apnoea. Cochrane Database of Systematic Reviews 2006(4)CD005308. DOI: 10.1002/14651858.CD005308.pub2.
47. Brown DL, Concannon M, Kaye AB, Zupancic M, Lisabeth LD. Comparison of two headgear systems for sleep apnea treatment of stroke patients. Cerebrovasc Dis 2009; 27: 183-186.
48. Criner GJ, Brennan K, Travaline JM, Kreimer D. Efficacy and compliance with noninvasive positive pressure ventilation in patients with chronic respiratory failure. Chest 1999; 116: 667-675.
49. Aboussouan LS, Khan SU, Banerjee M, Arroliga AC, Mitsumoto H. Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis. Muscle Nerve 2001; 24: 403-409.
50. Lo Coco D, Marchese S, Pesco MC, La Bella V, Piccoli F, Lo Coco A. Noninvasive positive-pressure ventilation in ALS: predictors of tolerance and survival. Neurology 2006; 67: 761-765.
51. Meyer TJ, Pressman MR, Benditt J, McCool FD, Millman RP, Natarajan R, et al. Air leaking through the mouth during nocturnal nasal ventilation: effect on sleep quality. Sleep 1997; 20: 561-569.
52. Tuggey JM, Delmastro M, Elliott MW. The effect of mouth leak and humidification during nasal non-invasive ventilation. Respir Med 2007; 101: 1874-1879.
53. Bachour A, Hurmerinta K, Maasilta P. Mouth closing device (chinstrap) reduces mouth leak during nasal CPAP. Sleep Med 2004; 5: 261-267.
54. Cooper-Knock J, Ahmedzai SH, Shaw P. The use of subcutaneous glycopyrrolate in the management of sialorrhoea and facilitating the use of non-invasive ventilation in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2011; 12: 464-465.
55. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009; 73: 1227-1233.
56. Pinto A, Almeida JP, Pinto S, Pereira J, Oliveira AG, de Carvalho M. Home telemonitoring of non-invasive ventilation decreases healthcare utilisation in a prospective controlled trial of patients with amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2010; 81: 1238-1242.
57. Bach JR. Update and perspective on noninvasive respiratory muscle aids. Part 2: the expiratory aids. Chest 1994; 105: 1538-1544.
58. Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne Muscular Dystrophy. Chest 1997; 112: 1024-1028.
59. Bach JR. Amyotrophic lateral sclerosis: predictors for prolongation of life by noninvasive respiratory aids. Arch Phys Med Rehabil 1995; 76: 828-832.
60. Sancho J, Servera E, Diaz J, Marin J. Predictors of ineffective cough during a chest infection in patients with stable amyotrophic lateral sclerosis. Am J Respir Crit Care Med 2007; 175: 1266-1271.
61. Mustfa N, Aiello M, Lyall RA, Nikoletou D, Olivieri D, Leigh PN, et al. Cough augmentation in amyotrophic lateral sclerosis. Neurology 2003; 61: 1285-1287.
62. Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest 2000; 118: 1390-1396.
63. Winck JC, Goncalves MR, Lourenco C, Viana P, Almeida J, Bach JR. Effects of mechanical insufflation-exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance. Chest 2004; 126: 774-780.
64. Sancho J, Servera E, Diaz J, Marin J. Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest 2004; 125: 1400-1405.
65. Baker WL, Lamb VJ, Marini JJ. Breath-stacking Increases the depth and duration of chest expansion by incentive spirometry. Am Rev Respir Dis 1990; 141: 343-346.
66. Robinson R. 'Breath stacking' reported to make swallowing safer in ALS. Neurol Today 2011; 11: 12.
67. Cleary S, Misiaszek J, Wheeler S, Kalra S, Johnston W. Using lung volume recruitment therapy to improve swallowing and airway pretection for individuals with ALS. Amyotroph Lateral Scler 2011; 11(Suppl 1): 58.
68. Bach JR. Mechanical Insufflation Exsufllation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest 1993; 104: 1553-1562.
69. Toussaint M, Boitano LJ, Gathot V, Steens M, Soudon P. Limits of effective cough-augmentation techniquest in patients with neuromuscular disease. Respir Care 2009; 54: 359-366.
70. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004; 125: 1S-39S.
71. Chaisson KM, Walsh S, Simmons Z, Vender RL. A clinical pilot study: high frequency chest wall oscillation airway clearance in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2006; 7: 107-111.
72. Lange DJ, Lechtzin N, Davey C. High-frequency chest wall oscillation in ALS: an exploratory randomized, controlled trial. Neurology 2006; 67: 991-997.
73. Onders RP, Elmo M, Khansarinia S, Bowman B, Yee J, Road J, et al. Complete worldwide operative experience in laparoscopic diaphragm pacing: results and differences in spinal cord injured patients and amyotrophic lateral sclerosis patients. Surg Endosc 2009; 23: 1433-1440.
74. Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest 2004; 126: 1502-1507.
75. Bradley MD, Orrell RW, Clarke J, Davidson AC, Williams J, Kullmann DM, et al. Outcome of ventilatory support for acute respiratory failure in motor neuron disease. J Neurol Neurosurg Psychiatry 2002; 72: 752-756.
76. Kuhnlein P, Kubler A, Raubold S, Worrell M, Kurt A, Gdynia H-J, et al. Palliative care and circumstances of dying in German ALS patients using non-invasive ventilation. Amyotroph Lateral Scler 2008; 9: 91-98.
77. Neudert C, Oliver D, Wasner M, Borasio GD. The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol 2001; 248: 612-616.
78. Moss AH, Oppenheimer EA, Casey P, Cazzolli PA, Roos RP, Stocking CB, et al. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Chest 1996; 110: 249-255.
79. Kaub-Wittemer D, von Steinbuchel N, Wasner M, Laier-Groeneveld G, Borasio GD. Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. J Pain Symptom Manage 2003; 26: 890-896.
80. Rabkin JG, Albert SM, Tider T, Del Bene ML, O'Sullivan I, Rowland LP, et al. Predictors and course of elective long-term mechanical ventilation: a prospective study of ALS patients. Amyotroph Lateral Scler 2006; 7: 86-95.
81. Chio A, Calvo A, Ghiglione P, Mazzini L, Mutani R, More G. Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry 2010; 81: 1141-1143.
82. Vianello A, Arcaro G, Palmieri A, Ermani M, Braccioni F, Gallan F, et al. Survival and quality of life after tracheostomy for acute respiratory failure in patients with amyotrophic lateral sclerosis. J Crit Care 2011; 26: 329.e7-329.e14.
83. Lo Coco D, Marchese S, La Bella V, Piccoli T, Lo Coco A. The amyotrophic lateral sclerosis functional rating scale predicts survival time in amyotrophic lateral sclerosis patients on invasive mechanical ventilation. Chest 2007; 132: 64-69.
84. Gelinas DF, O'Connor P, Miller RG. Quality of life for ventilator-dependent ALS patients and their caregivers. J Neurol Sci 2008; 160(Suppl 1): S134-S136.
85. Hayashi H, Oppenheimer EA. ALS patients on TPPV. Totally locked-in state, neurologic findings and ethical implications. Neurology 2003; 61: 135-137.
86. Borasio GD, Voltz R. Discontinuation of mechanical ventilation in patients with amyotrophic lateral sclerosis. J Neurol 1998; 245: 717-722.