Semin Respir Crit Care Med 2002; 23(3): 293-306
DOI: 10.1055/s-2002-33038
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Ventilator Management for Neuromuscular Disease

Nicholas S. Hill
  • Division of Pulmonary, Critical Care and Sleep Medicine, Tufts-New England Medical Center, Tufts University School of Medicine, Boston, Massachusetts
Further Information

Publication History

Publication Date:
02 August 2002 (online)

ABSTRACT

Many progressive neuromuscular disorders involve the muscles of the respiratory system, and respiratory insufficiency is a major cause of morbidity and mortality. Thus ventilatory assistance becomes an important part of disease management for patients with advanced neuromuscular disease. The following discusses the evaluation of patients with neuromuscular disease to characterize the nature and severity of respiratory muscle involvement, indications and contraindications for the use of noninvasive ventilation, and techniques for initiation and monitoring. Indications for invasive ventilation are also discussed, and major principles of management and monitoring of invasive mechanical ventilation are outlined. For optimal ventilator management of patients with neuromuscular disease, clinicians must be familiar with the principles discussed in this article so that treatment can be tailored to the individual's goals and desires. With proper implementation, ventilatory support of patients with neuromuscular disease can bring about improvements in quality of life and, for those who desire, potential prolongation of survival.

REFERENCES

  • 1 Wilson J L. Acute anterior poliomyelitis.  N Engl J Med . 1932;  206 887-893
  • 2 Alba A, Khan A, Lee M. Mouth IPPV for sleep.  Rehab Gazette . 1981;  24 47-49
  • 3 Kerby G R, Mayer L S, Pingleton S K. Nocturnal positive pressure ventilation vianasal mask.  Am Rev Respir Dis . 1987;  135 738-740
  • 4 Leger P, Hill N. Noninvasive ventilation for restrictive thoracic disease. In: Hill NS, ed. Long-Term Mechanical Ventilation New York: Marcel Dekker 2001
  • 5 Sanders M H, Kern N B, Costantino J P. Accuracy of end-tidal andtranscutaneous pCO2 monitoring during sleep.  Chest . 1994;  106 472-483
  • 6 Strumpf D A, Millman R P, Hill N S. The management of chronic hypo-ventilation.  Chest . 1990;  98 474-480
  • 7 Leger P, Bedicam J M, Cornette A. Nasal intermittent positive pressure: long-term follow-up in patients with severe chronic respiratory insufficiency.  Chest . 1994;  105 100-105
  • 8 Bach J R, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.  Chest . 1997;  112 1024-1028
  • 9 Raphael J C, Chevret S, Chastang C. French multicenter trial of prophylactic nasal ventilation in Duchenne muscular dystrophy.  Lancet . 1994;  343 1600-1604
  • 10 Bach J R, Brougher P, Hess D R. Consensus statement: noninvasive positive pressure ventilation.  Respir Care . 1997;  42 365-369
  • 11 Masa J F, Celli B R, Riesco J A, de Cos S J, Disdier C, Sojo A. Noninvasive positive pressure ventilation and not oxygen may prevent overt ventilatory failure in patients with chest wall diseases.  Chest . 1997;  112 207-213
  • 12 Bach J R, Alba A S, Saporito L R. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users.  Chest . 1993;  103 174-182
  • 13 Hill N S. Complications of noninvasive positive pressure ventilation.  Respir Care . 1997;  42 432-442
  • 14 Hill N S, Eveloff S E, Carlisle C C, Goff S G. Efficacy of nocturnal nasal ventilation in patients with restrictive thoracic disease.  Am Rev Respir Dis . 1992;  145 365-371
  • 15 Strumpf D A, Carlisle C C, Millman R P. An evaluation of the Respironics BiPAP bi-level CPAP device for delivery of assisted ventilation.  Respiratory Care . 1990;  35 415-422
  • 16 Bach J R. Mechanical insufflation-exsufflation: comparison of peak expiratory flows with manually assisted and unassisted coughing techniques.  Chest . 1993;  104 1553-1564
  • 17 Hill N S, Kacmarek R. Ventilators for noninvasive ventilation. In: Muir JT, Simonds A, Roussi A, eds. Noninvasive Mechanical Ventilation 2001 European Respiratory Society Monograph
  • 18 Hill N S. Clinical applications of body ventilators.  Chest . 1986;  90 897-905
  • 19 Mehta S, Hill N S. Noninvasive ventilation: state of the art.  Am J Resp Crit Care Med . 2001;  163 540-577
  • 20 Glenn W WL, Holcomb W G, Hogan J. Diaphragm pacing by radiofrequency transmission in the treatment of chronic ventilatory insufficiency.  J ThoracCardiovas Surg . 1973;  66 505-520
  • 21 Krieger A, Gropperm M R, Adler R J. Electrophrenic respiration after intercostal to Phrenic Nerve anastomosis in a patient with anterior spinal artery syndrome: technical case report.  Neurosurg . 1994;  35 760-764
  • 22 Bach J R, O'Connor K. Electrophrenic ventilation: a different perspective.  J Am Paraplegia Soc . 1991;  14 9-17
  • 23 Dail C W, Affeldt J E, Collier C R. Clinical aspects of glossopharayngeal breathing.  JAMA . 1953;  158 445-449
  • 24 Bach J R, Alba A S, Bodofsky E, Curran F J, Schultheiss M. Glossophrayngeal breathing and noninvasive aids in the management of post-polio respiratory insufficieny.  Birth Defects . 1987;  23 99-113
  • 25 Navalesi P, Fanfulla F, Frigerio P, Gregoretti C, Nava S. Physiologic evaluation of noninvasive mechanical ventilation delivered by three types of masks in patients with chronic hypercapnic respiratory failure.  Crit Care Med . 2000;  28 1785-1790
  • 26 Vitacca M, Rubini F, Foglio K. Noninvasive modalities of positive pressure ventilation improved the outcome of acute exacerbations in COLD patients.  Intensive Care Med . 1993;  19 450-455
  • 27 Schoenhofer B, Sonneborn M, Haide P, Bohrer H, Kohler D. Comparison of two different modes for noninvasive mechanical ventilation in chronic respiratory failure: volume versus pressure controlled device.  Eur Respir J . 1997;  10 184-191
  • 28 Meecham Jones J D, Wedzicha J A. Comparison of pressure volume and volume preset nasal ventilator systems in stable chronic respiratory failure.  Eur Respir J . 1993;  6 1060-1064
  • 29 Bach J R. Update and perspectives on noninvasive respiratory muscle aids, II: The expiratory muscle aids.  Chest . 1994;  105 1538-1544
  • 30 Schonhofer B, Geibel M, Sonneborn M, Haidl P, Kohler D. Daytime mechanical ventilation in chronic respiratory insufficiency.  Eur Respir J . 1997;  10 2840-2846
  • 31 Chervin R D, Theut S, Basssetti C, Aldrich S. Compliance with nasal CPAP therapy can be improved with simple interventions.  Sleep . 1997;  20 284-289
  • 32 Likar L, Panciera T M, Erickson A D, Rounds S. Group education sessions and compliance with nasal CPAP therapy.  Chest . 1997;  111 1273-1277
  • 33 Gay P C, Edmonds L C. Severe hypercapnia after low-flow oxygen therapy in patients with neuromuscular disease and diaphragmatic dysfunction.  Mayo Clin Proc . 1995;  50 327-330
  • 34 Ferguson G T, Gilmartin M. CO2 rebreathing during BiPAP ventilatory assistance.  Am J Respir Crit Care Med . 1995;  151 1126-1135
  • 35 Teague W G. Long-term mechanical ventilation in infants and children. In: Hill NS, ed. Long-Term Mechanical Ventilation New York: Marcel Dekker 2001
  • 36 Stocks J, Godfrey S. Nasal resistance during infancy.  Respir Physiol . 1978;  34 233-246
  • 37 Aboussouan L S, Khan S U, Meeker D P, Stelmach K, Mitsumoto H. Effect of noninvasive positive pressure ventilation on survival in amyotrophic lateral sclerosis.  Ann Intern Med . 1997;  127(6) 450-453
  • 38 Bach J R. A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver.  Chest . 1993;  104 1702-1706
  • 39 Branthwaite M A. Noninvasive and domiciliary ventilation: positive pressure techniques.  Thorax . 1991;  46 208-212
  • 40 Travaline J M, Criner G J. Management and monitoring of long-term invasive mechanical ventilation. In: Hill NS, ed. Long-Term Mechanical Ventilation New York: Marcel Dekker 2001
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