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Eur Respir J 2007; 30:179-180
Copyright ©ERS Journals Ltd 2007

Tracheostomy tubes are not needed for Duchenne muscular dystrophy

J. R. Bach1, C. Bianchi2, J. Finder3, T. Fragasso4, M. R. Goncalves5, Y. Ishikawa6, A. K. Ramlall7, D. McKim8, E. Servera9, A. Vianello10, M. Villanova11 and J. C. Winck5

1 Dept of Physical Medicine and Rehabilitation, University Hospital, Newark, NJ, and, 3 Dept of Paediatrics, University of Pittsburgh, Pittsburgh, PA, USA. 2 Rehabilitation Dept, Sacra Famiglia Institute Foundation, Cesano Boscone, Milan, 4 General Intensive Care Unit, Policlinico A. Gemelli, Catholic University, Rome, 10 Respiratory Pathophysiology Unit, University Hospital of Padova, Padova, and, 11 Neuromuscular Unit, Nigrisoli Hospital, Bologna, Italy. 5 Lung Function and Rehabilitation Unit, Pulmonology Dept, University Hospital of S. João, Porto, Portugal. 6 Dept of Paediatrics, National Hospital Organization Yakumo Hospital, Hokkaido, Japan. 7 Division of Paediatric Respirology, Alberta Children's Hospital, Calgary, Alberta, and, 8 Division of Respiratory Medicine, The Rehabilitation Centre, Ottawa Hospital, Ottawa, Canada. 9 Rehabilitation and Ventilation Unit, Dept of Respiratory Medicine, University Hospital, Valencia, Spain.

To the Editors:

Respiratory failure is the main cause of death in patients with Duchenne muscular dystrophy (DMD). Some respiratory management paradigms recommend tracheostomy for ventilator-dependent DMD patients. Prolongation of survival by years and, in many cases, decades using continuous mechanical noninvasive ventilation (NIV) without tracheostomy has been reported 1.

Toussaint et al. 2 reported their experience on the use of NIV along with assisted coughing to prolong life and avoid tracheostomy for patients with DMD. However, the commentary by Lofaso et al. 3 on this outstanding work was uninformed. The commentators failed to present the outcomes of continuous NIV from other centres 4–6 and they have, apparently, no experience in continuous long-term NIV. These authors seem to be unaware that more than 250 long-term (mostly 24-h dependent) NIV users whose main interface was a simple mouthpiece during the day and a mouthpiece with a plastic phalange during sleep have been described 7. Together, we currently have more than 1,000 continuous NIV users in our centres, some aged >50 yrs and most using a simple mouthpiece during daytime hours. Lofaso et al. 3 are correct to state that there are "no controlled studies that demonstrate that NIV prolongs life." The evidence to date suggests that there is no clinical equipoise regarding the use of NIV. As such, withholding such support would be unethical. Lofaso et al. 3 quote a conservative consensus statement which suggested that when NIV is inadequate "tracheostomy may be considered", but they have never reported using continuous NIV for their own patients. The recent American Thoracic Society consensus panel endorsed NIV as the therapy of choice in supporting breathing in DMD, while allowing for the possibility of tracheostomy in cases in which bulbar weakness precluded its use or expert support for NIV was unavailable locally 8. Lofaso et al. 3 also seem to be unaware that there were signicantly fewer hospitalisations and pulmonary complications in the NIV group as compared with the tracheostomy group 9.

In reality, NIV has never been ineffective for continuous ventilatory support in competent hands unless the ventilator settings or interfaces are inadequate, patients are too mentally impaired to cooperate with it, or bulbar-innervated musculature is too impaired to protect the airway from continuous saliva aspiration that results in oxyhaemoglobin desaturation 10. This has not occurred in the 200 or more DMD patients managed by continuous NIV in our centres. Lofaso et al. 3 note that tracheostomy ventilation is "more effective" than NIV without ever having permitted their own patients to use long-term continuous NIV or equipped and trained them in how to avoid serious chest infections by using manually and mechanically assisted coughing when needed 5. Lofaso et al. 3 confirmed that respiratory failure is not completely eliminated by 24-h NIV without ever discussing the cough aids that prevent it 11.

Lofaso et al. 3 state that "tracheostomy may also reduce the number of hours of ventilatory support needed per day", without understanding that DMD patients who were not using ventilators at all but are tracheotomised for acute respiratory failure often become and remain continuously ventilator dependent, whereas continuously ventilator-dependent DMD patients who are decannulated can be weaned to nocturnal-only NIV 1. Lofaso et al. 3 would be well advised to learn why patients with tubes require more ventilatory support than NIV-managed patients 12. They should know that patients who have used both tracheostomy and NIV ventilatory support for ≥1 month and who are decannulated to continuous NIV unanimously prefer NIV for safety, comfort, speech, swallowing, appearance and in general 13. Such patients often refuse to undergo tracheostomy a second time unless bulbar-innervated musculature deteriorates to the point that this becomes necessary for survival (motor neuron disease patients only) 10.

Our centres consider tracheostomy as a last resort and have been able to successfully avoid this procedure in all but a few patients, in some centres virtually never resorting to it. We would recommend that Lofaso et al. 3 try the approach for themselves before they conclude that this is neither possible nor desirable.

We disagree with the contentions that "daytime noninvasive ventilation via a mouthpiece should not be viewed as an alternative to tracheostomy" and "determining the best date for tracheostomy in patients with Duchenne muscular dystrophy remains a challenge." Indeed, instead of the latter, the challenge should be to remove tracheostomy tubes for continuous Duchenne muscular dystrophy ventilator users if the patient so desires, if there have been complications of tracheostomy, or if tube removal can facilitate social functioning like deinstitutionalisation 14.

REFERENCES

  1. 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.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]
  2. Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J 2006;28:549–555.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]
  3. Lofaso F, Orlikowski D, Raphael JC. Ventilatory assistance in patients with Duchenne muscular dystrophy. Eur Respir J 2006;28:468–469.[Free Full Text]
  4. Ishikawa Y, Bach JR, Minami R. Cardioprotection for Duchenne's muscular dystrophy. Am Heart J 1999;137:895–902.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]
  5. Bach JR, Ishikawa Y, Kim H. Prevention of the pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997;112:1024–1028.[Web of Science][Medline] [Order article via Infotrieve]
  6. Servera E, Sancho J, Zafra MJ, Catala A, Vergara P, Marin J. Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil 2005;84:851–857.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]
  7. Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheotomy for 257 ventilator users. Chest 1993;103:174–182.[Web of Science][Medline] [Order article via Infotrieve]
  8. Finder J, 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;170:456–465.[Free Full Text]
  9. Respiratory muscle aids: Patient evaluation, respiratory aid protocol, and outcomes. In: Bach JR, ed. Management of Patients with Neuromuscular Disease. Philadelphia, Hanley & Belfus, 2004; pp. 271–308
  10. Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest 2004;126:1502–1507.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]
  11. Bach JR. Mechanical insufflation–exsufflation: comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest 1993;104:1553–1562.[Web of Science][Medline] [Order article via Infotrieve]
  12. Home mechanical ventilation for neuromuscular ventilatory failure: conventional approaches and their outcomes. In: Bach JR, ed. Noninvasive Mechanical Ventilation. Philadelphia, Hanley & Belfus, 2002; pp. 103–128
  13. Bach JR. A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. Chest 1993;104:1702–1706.[Web of Science][Medline] [Order article via Infotrieve]
  14. Bach J, Goncalves M. Ventilator weaning by lung expansion and decannulation. Am J Phys Med Rehabil 2004;83:560–568.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]




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