Long-term Nasal Intermittent Positive Pressure Ventilation in Advanced Duchenne's Muscular Dystrophy

doi:10.1378/chest.105.2.445

Chest

Volume 105, Issue 2, February 1994, Pages 445-448

https://doi.org/10.1378/chest.105.2.445 Get rights and content

The aim of our study was to evaluate the long-term effect of nasal ventilation in patients with advanced Duchenne's muscular dystrophy (DMD). To this end, we compared the clinical and pulmonary function course of five subjects affected with chronic ventilatory failure due to DMD and treated with nasal intermittent positive pressure ventilation (NIPPV) with that of an unventilated comparison group; the latter consisted of another five patients with DMD, with a similar degree of clinical and respiratory functional impairment, who refused long-term mechanical ventilation. The duration of the follow-up was 24 months. At the conclusion of the trial, all patients treated with NIPPV were still alive; in contrast, four of five patients who underwent simple conservative treatment had already died (mean survival, 9.7 ± 5.8 months). After 6 months of follow-up, mean loss of FVC and maximal voluntary ventilation was considerably higher in nonventilated subjects (respectively: –0.23 L vs + 0.03 L and –5 L/min vs –1.5 L/min). These are the first comparative results confirming that long-term NIPPV helps to stabilize pulmonary function and to prolong the expectancy of life of patients with DMD.

Section snippets

Material and Methods

Between January 1987 and June 1990, ten unselected young male patients affected with advanced DMD were referred to the Respiratory Pathophysiology Department of the City Hospital of Padua, because of symptoms consistent with chronic hypercapnia (ie, hypersomnolence, insomnia, morning headaches, fatigue, enuresis).

The diagnosis of DMD was based on standard clinical, enzymatic, electromyographic, and biopsy criteria.

At the beginning of the study, the mean age was 20.1 ± 5.2 years (range, 12 to 27

Results

Anthropometric, radiographic, pulmonary function, and blood gas data at detection of stable hypercapnia are reported in Table 1.

With regard to these parameters, there are no significant differences between the two groups.

As in other studies,¹⁰ in our group of patients, FVC and MW values showed a great variation at the onset of daytime hypercapnia.

Clinical, pulmonary function, and blood gas data collected at the end of the study are summarized in Table 2.

At the end of the trial, all the patients

Discussion

The success of NIPPV in the long-term treatment of chronic ventilatory failure due to neuromuscular effectiveness of this technique in prolonging survival of the patients and slowing the loss of FVC could not be demonstrated definitively.

In our experience, five subjects with DMD refused to undergo long-term mechanical ventilation, in spite of a severe respiratory impairment. Since the severity of their clinical status and the degree of their respiratory function disability were in the mean the

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This investigation was supported by a grant from TELETHON 1991.

revision accepted June 11.

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Chest

Clinical InvestigationsSleep, Breathing and Nasal VentilationLong-term Nasal Intermittent Positive Pressure Ventilation in Advanced Duchenne's Muscular Dystrophy

Section snippets

Material and Methods

Results

Discussion

J Med

Chest

Chest

Chest

Chest

Chest

J Thorac Cardiovasc Surg

Cardiac and pulmonary complications in Duchenne's progressive muscular dystrophy

Circulation

Prolongation of life in Duchenne's muscular dystrophy

Acta Neurol

Clinical Investigations
Sleep, Breathing and Nasal Ventilation
Long-term Nasal Intermittent Positive Pressure Ventilation in Advanced Duchenne's Muscular Dystrophy