Duchenne muscular dystrophy: Continuous noninvasive ventilatory support prolongs survival

Respiratory Care

Volumn 56, Issue 6, 2011, Pages 744-750

  Bach, John R ^a   Martinez, Daniel ^b  

^a University of Medicine and Dentistry of New Jersey   (United States)

^b HOSPITAL CLÍNICO UNIVERSITARIO   (Spain)

Author keywords

Cough; Cough flows; Duchenne muscular dystrophy; Mechanical insufflation exsufflation; Mechanical ventilation; Noninvasive ventilation; Respiratory paralysis; Respiratory therapy; Vital capacity

Indexed keywords

ADOLESCENT; ADULT; ARTICLE; CHILD; CONTROLLED STUDY; COUGHING; DEATH; DUCHENNE MUSCULAR DYSTROPHY; END TIDAL CARBON DIOXIDE TENSION; EXTUBATION; HEART FAILURE; HUMAN; HYPOVENTILATION; MAJOR CLINICAL STUDY; MORTALITY; NONINVASIVE VENTILATION; OXIMETRY; RESPIRATORY TRACT INFECTION; SCHOOL CHILD; SURVIVAL RATE; TRACHEOSTOMY; TREATMENT DURATION; TREATMENT OUTCOME; VITAL CAPACITY;

ADULT; CAUSE OF DEATH; COUGH; FEMALE; HUMANS; MALE; MUSCULAR DYSTROPHY, DUCHENNE; OXIMETRY; RESPIRATION, ARTIFICIAL; STATISTICS, NONPARAMETRIC; SURVIVAL ANALYSIS; TRACHEOTOMY; TREATMENT OUTCOME; VITAL CAPACITY;

EID: 79960665553     PISSN: 00201324     EISSN: 19433654     Source Type: Journal    
DOI: 10.4187/respcare.00831     Document Type: Article

References (46)

1. Bach J, Alba A, Pilkington LA, Lee M. Long-term rehabilitation in advanced stage of childhood onset, rapidly progressive muscular dystrophy. Arch Phys Med Rehabil 1981;62(7):328-331.
2. Inkley SR, Oldenburg FC, Vignos PJ Jr. Pulmonary function in Duchenne muscular dystrophy related to stage of disease. Am J Med 1974;56(3):297-306.
3. Rideau Y, Gatin G, Bach J, Gines G. Prolongation of life in Duchenne muscular dystrophy. Acta Neurol 1983;5(2):118-124.
4. Vignos PJ Jr. Respiratory function and pulmonary infection in Duchenne muscular dystrophy. Isr J Med Sci 1977;13(2):207-214.
5. Emery AE. Duchenne muscular dystrophy: genetic aspects, carrier detection and antenatal diagnosis. Br Med Bull 1980;36(2):117-122.
6. Eagle M, Baudouin SV, Chandler C, Giddings DR, Bullock R, Bushby K. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 2002;12(10):926-929.
7. Bach JR, Rajaraman R, Ballanger F, Tzeng AC, Ishikawa Y, Kulessa R, Bansal T. Neuromuscular ventilatory insufficiency: the effect of home mechanical ventilator use vs. oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil 1998;77(1):8-19.
8. Gomez-Merino E, Bach JR. Duchenne muscular dystrophy: prolongation of life by noninvasive respiratory muscle aids. Am J Phys Med Rehabil 2002;81(6):411-415.
9. Bach JR, Holland I. Conventional "end-stage" management. In: Bach JR, editor. The management of patients with neuromuscular disease. Philadelphia: Hanley & Belfus; 2004:155-184.
10. Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J 2006;28(3):549-555.
11. Kohler M, Clarenbach CF, Böni L, Brack T, Russi EW, Bloch KE. Quality of life, physical disability, and respiratory impairment in Duchenne muscular dystrophy. Am J Respir Crit Care Med 2005; 172(8):1032-1036.
12. Bach JR. Risk factors for extubation success in patients following an unsuccessful spontaneous breathing trial. Chest 2007;131(5):1615.
13. Kang SW, Bach JR. Maximum insufflation capacity: the relationships with vital capacity and cough flows for patients with neuromuscular disease. Am J Phys Med Rehabil 2000;79(3):222-227.
14. Bach JR, Kang SW. Disorders of ventilation: weakness, stiffness, and mobilization. Chest 2000;117(2):301-303.
15. Birnkrant DJ, Bushby K, Amin RS, Bach JR, Benditt JO, Eagle M, et al. The respiratory management of patients with Duchenne muscular dystrophy: a DMD care considerations working group specialty article. Pediatr Pulmonol 2010;45(8):739-748.
16. Bach JR, Baird JS, Plosky D, Nevado J, Weaver B. Spinal muscular atrophy type 1: management and outcomes. Pediatr Pulmonol 2002; 34(1):16-22.
17. Bach JR, Niranjan V, Weaver B. Spinal muscular atrophy type 1: a noninvasive respiratory management approach. Chest 2000;117(4): 1100-1105.
18. Bach JR, Gonçalves MR, Hamdani I, Winck JC. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest 2010;137(5):1033-1039.
19. Bach JR, Bianchi C, Vidigal-Lopes M, Turi S, Felisari G. Lung inflation by glossopharyngeal breathing and "air stacking" in Duchenne muscular dystrophy. Am J Phys Med Rehabil 2007;86(4):295-300.
20. Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997;112(4): 1024-1028.
21. Magdalena Ferrán Aranaz M, editor. SPSS para Windows. Programación y análisis estadístico. Madrid: McGraw-Hill; 1996. Book in Spanish.
22. Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy in amyotrophic lateral sclerosis. Chest 2004;126(5):1502-1507.
23. Eagle M, Bourke J, Bullock R, Gibson M, Mehta J, Giddings D, et al. Managing Duchenne muscular dystrophy-the additive effect of spinal surgery and home nocturnal ventilation in improving survival. Neuromuscul Disord 2007;17(6):470-475.
24. Yasuma F, Sakai M, Matsuoka Y. Effects of noninvasive ventilation on survival in patients with Duchenne's muscular dystrophy. Chest. 1996;109(2):590.
25. Simonds AK, Muntoni F, Heather S, Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax 1998;(11):949-952.
26. Raphael JC, Chevret S, Chastang C, Bouvet F. Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. Lancet 1994;343(8913):1600-1604.
27. Hukins CA, Hillman DR. Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med 2000;161(1):166-170.
28. Kohler M, Clarenbach CF, Bahler C, Brack T, Russi EW, Bloch KE. Disability and survival in Duchenne muscular dystrophy. J Neurol Neurosurg Psychiatry 2009;80(3):320-325.
29. Ishikawa Y, Miura T, Ishikawa Y, Aoyagi T, Ogata H, Hamada S, Minami R. Duchenne muscular dystrophy: Survival by cardio-respiratory interventions. Neuromuscular Disorders 2011;21(1):47-51.
30. Bach JR, Bianchi C, Finder JD, Gonçalves M, Ishikawa Y, Kumar R, et al. Tracheostomy tubes are not needed for Duchenne muscular dystrophy. Eur Respir J 2007;30(1):179-180.
31. Bach JR, Gonçalves M. Ventilator weaning by lung expansion and decannulation. Am J Phys Med Rehabil 2004;83(7):560-568.
32. Ishikawa Y, Bach JR, Minami R. Cardioprotection for Duchenne's muscular dystrophy. Am Heart J 1999;137(5):895-902.
33. Baydur A, Layne E, Aral H, Krishnareddy N, Topacio R, Frederick G, Bodden W. Long term non-invasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review. Thorax 2000;55(1):4-11.
34. Hodes HL. Treatment of respiratory difficulty in poliomyelitis. In: Poliomyelitis: papers and discussions presented at the Third International Poliomyelitis Conference. Philadelphia: Lippincott; 1955:91-113.
35. Bach JR, Rogers B, King A. Noninvasive respiratory muscle aids: Intervention goals, and mechanisms of action. In: Bach JR. The management of patients with neuromuscular disease. Philadelphia: Hanley & Belfus; 2004:211-270.
36. Bach JR, O'Brien J, Krotenberg R, Alba A. Management of end stage respiratory failure in Duchenne muscular dystrophy. Muscle Nerve 1987;10(2):177-182.
37. Gatin G. Intêret de la ventilation assisée dans les dystrophies musculaires. Ann Readapt Med Phys 1983;26:111-128. Article in French
38. Splaingard ML, Frates RC, Harrison GM, Carter RE, Jefferson LS. Home positive-pressure ventilation: twenty years' experience. Chest 1983;84(4):376-382.
39. Fukunaga H, Okubo R, Moritoyo T, Kawashima N, Osame M. Longterm follow-up of patients with Duchenne muscular dystrophy receiving ventilatory support. Muscle Nerve 1993;16(5):554-558.
40. Racca F, Appendini L, Berta G, Barberis L, Vittone F, Gregoretti C, et al. Helmet ventilation for acute respiratory failure and nasal skin breakdown in neuromuscular disorders. Anesth Analg 2009;109(1):164-167.
41. Bach JR, Intintola P, Alba AS, Holland I. The ventilator-assisted individual: cost analysis of institutionalization versus rehabilitation and in-home management. Chest 1992;101(1):26-30.
42. Kollef MH. Avoidance of tracheal intubation as a strategy to prevent ventilator associated pneumonia. Intensive Care Med 1999;25(6): 553-555.
43. Bach JR. A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. Chest 1993;104(6): 1702-1706.
44. 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(2):87-93.
45. Birnkrant DJ. The American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Pediatrics 2009;123(Suppl 4):S242-S244.
46. Annane D, Orlikowski D, Chevret S, Chevrolet JC, Raphaël JC. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Hoboken, NJ: Wiley; 2009.