Chest
Volume 97, Issue 1, January 1990, Pages 91-96
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Clinical Investigations
Long-Term Follow-up of Nocturnal Ventilatory Assistance in Patients with Respiratory Failure Due to Duchenne-Type Muscular Dystrophy

https://doi.org/10.1378/chest.97.1.91Get rights and content

We followed eight patients with Duchenne-type muscular dystrophy for an average of 39 months after initiation of noninvasive intermittent ventilatory assistance using body ventilators. After one to three months of nocturnal use averaging 8 h, mean daytime PaCOs fell from 63 ± 2 to 45 ± 3 mm Hg. At late follow-up, PaCO2 remained stable at 47 ± 4 mm Hg, but vital capacity fell 33 percent compared with the initial value and the average duration of ventilator use had increased to 18 ± 2 h daily. Three patients died and five survived; two continued using negative pressure ventilators and three had tracheostomies placed for administration of positive pressure ventilation. We conclude that noninvasive intermittent ventilatory assistance effectively reverses hypoventilation and symptoms in patients with late-stage Duchenne muscular dystrophy, but pulmonary function continues to deteriorate necessitating longer periods of ventilation, and often tracheostomy, within a few years. (Chest 1990; 97:91-96)

Section snippets

METHODS

Patients were referred for the management of hypercarbia from the Muscular Dystrophy Association Clinic at New England Medical Center. Patients had been diagnosed as having Duchenne or Duchenne-type muscular dystrophy using standard clinical criteria including male sex, elevation of creatine phosphokinase, and a compatible muscle biopsy and clinical picture. All patients were in functional class 93 and had severe quadraparesis.

All patients expressed a desire to try intermittent ventilatory

RESULTS

Table 1 shows age at initiation of assisted ventilation, respiratory parameters at initiation of assisted ventilation and at times of early and extended follow-up and clinical outcome in the eight patients comprising our series. Most patients developed respiratory failure at approximately 20 years of age, as is typical for Duchenne muscular dystrophy. Delayed onset in patients 1 and 6 suggests that they may have had a more slowly progressing variety. All patients had severe restriction of their

DISCUSSION

In our series of eight patients with Duchenne or Duchenne-type muscular dystrophy with respiratory failure, we found, as others have,6, 7, 8, 9, 10 that nocturnal ventilatory assistance using noninvasive ventilators brought about a sustained reversal of hypoventilation and improvement in symptoms of chronic hypercarbia. The clinical course was stabilized in most patients who required infrequent hospitalizations despite minimal pulmonary reserve. Although our patients tried a variety of negative

ADDENDUM

Since acceptance of this manuscript, patient 4 died suddenly 62 months after initiation of ventilation when his ventilator became accidentally detached from his tracheostomy tube. Autopsy revealed a well-healed tracheostomy without stricture, severe dystrophy of skeletal muscle, and moderate fatty infiltration of the myocardium without evidence of cardiac failure.

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  • Cited by (57)

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    Manuscript received March 7; revision accepted June 23.

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