Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.

STUDY OBJECTIVE To evaluate the effects of a new respiratory management protocol on respiratory morbidity and hospitalization rates for patients with Duchenne muscular dystrophy (DMD). DESIGN A retrospective cohort study. METHODS Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive pressure ventilation (IPPV) and assisted coughing as needed, the hospitalization rates and days for 24 protocol DMD ventilator users were compared with those of 22 nonprotocol DMD tracheostomy IPPV users. RESULTS The 22 conventionally managed patients were hospitalized a mean of 72.2+/-112 days when undergoing tracheostomy. This included a 16.1+/-5.4-day period of translaryngeal intubation. The 24 protocol patients were hospitalized a mean of 6.0+/-2.4 days (p<0.005) when beginning ventilator use. Over their next 126.2 patient-years of ventilator use, the 24 protocol patients had significantly lower rates of hospitalization (p<0.008) and hospitalization days (p<0.005) than had the tracheostomy IPPV users over a 167.2 patient-year period. This is true although 14 of the 24 protocol patients went on to require 24-h noninvasive IPPV for 4.5+/-3.6 years. Five of the 14 have yet to be hospitalized. CONCLUSION The use of inspiratory and expiratory aids can prolong survival while significantly decreasing the pulmonary morbidity and hospitalization rates associated with conventional resort to tracheostomy IPPV.

[1]  B. Katirji.  Diagnostic Criteria for Neuromuscular Disorders , 1994, Neurology.

[2]  J. Mendell,et al.  Recent advances in Duchenne and Becker muscular dystrophy. , 1988, Neurologic clinics.

[3]  J. Bach,et al.  Management of chronic alveolar hypoventilation by nasal ventilation. , 1990, Chest.

[4]  P. Vignos.  Respiratory function and pulmonary infection in Duchenne muscular dystrophy. , 1977, Israel journal of medical sciences.

[5]  M. Sanders,et al.  Obstructive sleep apnea treated by independently adjusted inspiratory and expiratory positive airway pressures via nasal mask. Physiologic and clinical implications. , 1990, Chest.

[6]  J. Bach.  Pulmonary rehabilitation : the obstructive and paralytic conditions , 1996 .

[7]  J. Bach.  A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. , 1993, Chest.

[8]  J. Miller,et al.  Duchenne muscular dystrophy , 1989, Neurology.

[9]  J. Bach,et al.  Prolongation of life in Duchenne's muscular dystrophy. , 1983, Acta neurologica.

[10]  J. Bach,et al.  Intermittent Positive Pressure Ventilation via the Mouth as an Alternative to Tracheostomy for 257 Ventilator Users , 2006 .

[11]  J. Bach.  Ventilator use by muscular dystrophy association patients. , 1992, Archives of physical medicine and rehabilitation.

[12]  N. Hill,et al.  Efficacy of nocturnal nasal ventilation in patients with restrictive thoracic disease. , 1992, The American review of respiratory disease.

[13]  J. Bach,et al.  Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning. , 1996, Chest.

[14]  K. Hizawa,et al.  Life spans of Duchenne muscular dystrophy patients in the hospital care program in Japan , 1987, Journal of the Neurological Sciences.

[15]  J. M. Masa Jiménez,et al.  Nasal intermittent positive pressure ventilation. Analysis of its withdrawal. , 1995, Chest.

[16]  P. Calverley,et al.  Protriptyline treatment of sleep hypoxaemia in Duchenne muscular dystrophy. , 1989, Thorax.

[17]  M. Green,et al.  Respiratory muscle weakness during upper respiratory tract infections. , 1988, The American review of respiratory disease.

[18]  A. Emery.  Duchenne muscular dystrophy. Genetic aspects, carrier detection and antenatal diagnosis. , 1980, British medical bulletin.

[19]  J. S. D. C. Escuín,et al.  Nasal intermittent positive pressure ventilation. Analysis of its withdrawal. , 1995, Chest.

[20]  J. Bach,et al.  Amyotrophic lateral sclerosis: predictors for prolongation of life by noninvasive respiratory aids. , 1995, Archives of physical medicine and rehabilitation.

[21]  J. Bach,et al.  The ventilator-assisted individual. Cost analysis of institutionalization vs rehabilitation and in-home management. , 1992, Chest.

[22]  Emery.  Diagnostic Criteria for Neuromuscular Disorders , 1997 .

[23]  S. Inkley,et al.  Pulmonary function in Duchenne muscular dystrophy related to stage of disease. , 1974, The American journal of medicine.

[24]  J. Bach.  Mechanical Insufflation-Exsufflation: Comparison of Peak Expiratory Flows With Manually Assisted and Unassisted Coughing Techniques , 1993 .

[25]  P. Wollan,et al.  Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy. , 1996, Pediatric neurology.