Yonsei Med J.  2006 Jun;47(3):307-314. 10.3349/ymj.2006.47.3.307.

Pulmonary Rehabilitation in Patients with Neuromuscular Disease

• Kang SW

• KMID: 1715847
• DOI: http://doi.org/10.3349/ymj.2006.47.3.307

Abstract

In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.

Keyword

Pulmonary rehabilitation; neuromuscular disease; inspiratory muscle aid; expiratory muscle aid

MeSH Terms

Respiratory Insufficiency/etiology/*rehabilitation/*therapy
Respiration, Artificial
Neuromuscular Diseases/complications/*rehabilitation
Humans
Breathing Exercises

Figure

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  Fig. 1 Patient with congenital muscular dystrophy using nasal intermittent positive pressure ventilation.

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  Fig. 2 Cough Assist™ is a commercially available device which performs mechanical issufflation-exsufflation.

Reference

1. Gibson GJ, Pride NB, Davis JN, Loh LC. Pulmonary mechanics in patients with respiratory muscle weakness. Am Rev Respir Dis. 1977. 115:389–395.

2. Griggs RC, Donohoe KM, Utell MJ, Goldblatt D, Moxley RT 3rd. Evaluation of pulmonary function in neuromuscular disease. Arch Neurol. 1981. 38:9–12.

3. Smith PE, Calverley PM, Edwards RH, Evans GA, Cambell EJ. Practical problems in the respiratory care of patients with muscular dystrophy. N Engl J Med. 1987. 316:1197–1205.

4. Lynn DJ, Woda RP, Mendell JR. Respiratory dysfunction in muscular dystrophy and other myopathy. Clin Chest Med. 1994. 15:661–674.

5. Schramm CM. Current concepts of respiratory complications of neuromuscular disease in children. Curr Opin Pediatr. 2000. 12:203–207.

6. Braun NM, Arora NS, Rochester DF. Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax. 1983. 38:616–623.

7. Bach JR, Alba AS. Management of chronic alveolar hypoventilation by nasal ventilation. Chest. 1990. 97:52–57.

8. Leith DE. Brain JD, Proctor D, Reid L, editors. Cough. Lung biology in health and disease. 1977. New York: Marcel Dekker;545–592.

9. Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest. 1997. 112:1024–1028.

10. Bach JR, Kang SW. Disorders of ventilation weakness, stiffness, and mobilization. Chest. 2000. 117:301–303.

11. Kang SW, Kang YS, Moon JH, Yoo TW. Assisted Cough and Pulmonary Compliance in Patients with Duchenne Muscular Dystrophy. Yonsei Med J. 2005. 46:233–238.

12. Kang SW, Bach JR. Maximum insufflation capacity: vital capacity and cough flows in neuromuscular disease. Am J Phys Med Rehabil. 2000. 79:222–227.

13. Kreitzer SM, Saunders NA, Tyler HR, Ingram RH Jr. Respiratory muscle function in amyotrophic lateral sclerosis. Am Rev Respir Dis. 1978. 117:437–447.

14. McCool FD, Tzelepis GE. Inspiratory muscle training in the patient with neuromuscular disease. Phys Ther. 1995. 75:1006–1014.

15. McDonald CM, Abresch RT, Carter GT, Flwler WM, Johnson ER, Kilmer DD, Sigford BJ. Profiles of neuromuscular diseases: Duchenne muscular dystrophy. Am J Phys Med Rehabil. 1995. 74:S70–S92.

16. 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. 121:436–422.

17. Varrato J, Siderowf A, Damiano P, Gregory S, Feinberg D, McCluskey L. Postural change of forced vital capacity predicts some respiratory symptoms in ALS. Neurology. 2001. 57:357–359.

18. Baydur A, Adkins RH, Milic-Emili J. Lung mechanics in individuals with spinal cord injury: effects of injury level and posture. J Appl Physiol. 2001. 90:405–411.

19. Estenne M, De Troyer A. The effects of tetraplegia on chest wall statics. Am Rev Respir Dis. 1986. 134:121–124.

20. Winslow C, Rozovsky J. Effect of spinal cord injury on the respiratory system. Am J Phys Med Rehabil. 2003. 82:803–814.

21. Allen SM, Hunt B, Green M. Fall in vital capacity with posture. Br J Dis Chest. 1985. 79:267–271.

22. Bach JR. Bach JR, editor. Respiratory considerations. Guide to the evaluation and management of neuromuscular disease. 1999. Philadelphia: Hanley & Belfus;67–87.

23. Estenne M, Heilporn A, Delhez L, Yerault JC, De Troyer A. Chest wall stiffness in patients with chronic respiratory muscle weakness. Am Rev Respir Dis. 1983. 128:1002–1007.

24. Estenne M, Gevenois PA, Kinnear W, Soudon P, Heilporn A, De Troyer A. Lung volume restriction in patients with chronic respiratory muscle weakness: the role of microatelectasis. Thorax. 1993. 48:698–701.

25. Kang SW, Bach JR. Maximum insufflation capacity. Chest. 2000. 118:61–65.

26. Hanayama K, Ishikawa Y, Bach JR. Amyotrophic lateral sclerosis: successful treatment of mucus plugging by mechanical insufflation-exsufflation. Am J Phys Med Rehabil. 1997. 76:338–339.

27. Beachey W. Wilkins RL, Stroller JK, Scanlan CL, editors. Regulation of breathing. Egan's fundamentals of respiratory care. 2003. 8th ed. St. Louis: Mosby;297–305.

28. Bach JR. Bacg JR, editor. Physiology and pathophysiology of hypoventilation: ventilatory vs oxygenation impairment. Noninvasive mechanical ventilation. 2002. Philadelphia: Hanley & Belfus;25–43.

29. Bach JR. A comparison of long-term ventilatory support alternatives from the perspective of the patient and caregiver. Chest. 1993. 104:1702–1706.

30. Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation. J Neurol Sci. 1996. 139:123–128.

31. Gomez Merino E, Bach JR. Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil. 2002. 81:411–415.

32. Bach JR. Indications for tracheostomy and decannulation of tracheostomized ventilator users. Monaldi Arch Chest Dis. 1995. 50:223–227.

33. Bach JR. Bach JR, editor. Noninvasive ventilation. Guide to the evaluation and management of neuromuscular disease. 1999. Philadelphia: Hanley & Belfus;89–122.

34. Ellis ER, Bye PT, Bruderer JW, Sullivan CE. Treatment of respiratory failure during sleep in patients with neuromuscular disease, positive-pressure ventilation through a nose mask. Am Rev Respir Dis. 1987. 135:148–152.

35. Vianello A, Bevilacqua M, Salvador V, Cardaioli C, Vincenti E. Long-term nasal intermittent positive pressure ventilation in advanced Duchenne's muscular dystrophy. Chest. 1994. 105:445–448.

36. Bach JR. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory AIDS. Chest. 2002. 122:92–98.

37. Goldstein RS, DeRosie JA, Avendano MA, Dolmage TE. Influence of noninvasive positive pressure ventilation on inspiratory muscles. Chest. 1991. 99:408–415.

38. Bach JR, Saporito LR. Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure: a different approach to weaning. Chest. 1996. 110:1566–1571.

39. Bach JR. Bacg JR, editor. The history of mechanical ventilation and respiratory muscle aids. Noninvasive mechanical ventilation. 2002. Philadelphia: Hanley & Belfus;45–72.

40. Mislinski MJ, Scanlan CL. Wilkins RL, Stroller JK, Scanlan CL, editors. Bronchial hygiene therapy. Egan's fundamentals of respiratory care. 2003. 8th ed. St. Louis: Mosby;297–305.

41. Bach JR. Mechanical insufflation-exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest. 1993. 104:1553–1562.

42. Bach JR. Amyotrophic lateral sclerosis: predictors for prolongation of life by noninvasive respiratory aids. Arch Phys Med Rehabil. 1995. 76:828–832.

43. Bach JR, Saporito LR. Indications and criteria for decannulation and transition from invasive to non-invasive long-term ventilator support. Respir Care. 1994. 39:515–531.

44. Newth CJL, Asmler B, Anderson GP, Morley J. The effects of varying inflation and deflation pressures on the maximal expiratory deflation flow volume relationship in anesthetized Rhesus monkeys. Am Rev Respir Dis. 1991. 144:807–813.

45. Bach JR. Update and perspective on noninvasive respiratory muscle aids. Part 2: The expiratory aids. Chest. 1994. 105:1538–1544.

46. Bickerman HA. Exsufflation with negative pressure: elimination of radioopaque material and foreign bodies from bronchi of anesthetized dogs. AMA Arch Intern Med. 1954. 93:698–704.

47. 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. 93:825–841.