Chest
Volume 112, Issue 4, October 1997, Pages 1024-1028
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Clinical Investigations: Breathing
Prevention of Pulmonary Morbidity for Patients With Duchenne Muscular Dystrophy

https://doi.org/10.1378/chest.112.4.1024Get rights and content

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).

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.

Section snippets

Materials and Methods

Of all patients referred to a regional Jerry Lewis Muscular Dystrophy Association clinic since 1977, 92 were diagnosed as having DMD on the basis of onset and progression of weakness before age 5 years, calf muscle pseudohypertrophy, loss of unassisted ambulation before age 13 years,1 and characteristic elevations of serum creatinine kinase, muscle biopsy in the patient or male relative, and electrodiagnostic examination. More recently, documentation of a Duchenne-type mutation or identical

Results

Forty-eight patients required treatment for respiratory failure. Two patients became dependent on 24-h noninvasive IPPV without being equipped with oximeters or the expiratory aids of this protocol. Both died from pneumonia and respiratory failure complicating intercurrent URTIs. They are no longer considered. The remaining 46 patients included the following: (1) 22 nonprotocol tracheostomy IPPV users who were referred before 1983, referred already using tracheostomy IPPV and for whom the tube

Discussion

The initial objective, that of identifying DMD patients at risk of developing acute respiratory failure, appears to have been achieved because no patients with (assisted) PCF above 270 L/min developed acute respiratory distress. All but one patient for whom assisted PCF were below 270 L/min had VCs below 1,000 mL. Despite 14 patients eventually requiring full-time noninvasive IPPV, two with VCs under 100 mL, and three with severe dysphagia necessitating indwelling gastrostomy tubes, assisted

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