Treatment of Duchenne Muscular Dystrophy with Respiratory Muscle Weakness and Hypoventilation

In Duchenne muscular dystrophy, progressive loss of muscle function eventually affects the muscles responsible for breathing. When the respiratory muscles weaken sufficiently, hypoventilation follows — a situation that requires structured, staged respiratory support.

Clinical Scenario

Weakness of the breathing (respiratory) muscles with hypoventilation. As the muscles that assist in breathing get weaker, the respiratory system must be adequately supported.

Approach Overview

Management addresses two complementary goals: keeping the airways clear of secretions through assisted coughing techniques, and supporting ventilation with noninvasive respiratory assistance used on a part-time basis.

The complete protocol — including specific devices, sequencing, escalation criteria, and monitoring — is available via the structured regimen below.

Instant Access to Structured Evidence-Based Regimens

References

As the muscles that assist in breathing get weaker, the respiratory system must be adequately supported.

Support of the respiratory system can be thought of in two ways: (1) keeping the bronchial system free of secretions, either by using a cough assist device or by manual assisted coughing and (2) addressing hypoventilation with ventilator supported breathing, such as with a BiPAP device.

Typically, noninvasive ventilation is initially required only part time, often only during sleep.

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