Long-term management of acute respiratory failure in metabolic myopathy.

OBJECTIVE

To describe how patients cope with the proposal of treatment with intermittent artificial ventilation after acute respiratory failure due to progressive respiratory muscle weakness.

DESIGN

Case series, follow-up study.

SETTING

Neurological intensive care unit (ICU).

PATIENTS

7 consecutive patients with metabolic myopathy treated for acute respiratory failure between 1983 and 1992.

INTERVENTIONS

Intermittent positive pressure ventilation (IPPV) via tracheostomy.

MEASUREMENTS AND RESULTS

Symptoms of chronic hypoventilation preceded acute respiratory failure for months. With one exception, patients were mainly disabled from respiratory muscle weakness and sleep-related breathing disorders. IPPV was recommended to prevent recurrent respiratory failure. Two of three patients who accepted home IPPV returned to full-time jobs. One patient, who decided against IPPV, died from CO2 narcosis several months after discharge. All patients adhered to the respiratory regimen once instituted.

CONCLUSIONS

Acute respiratory failure in chronic myopathy is heralded by daytime drowsiness. IPPV, or at least regular monitoring of waking and sleeping partial pressure of carbon dioxide, is highly recommended even if weaning is successful. IPPV improved quality of life. The treatment strategy at discharge from the ICU should be optimal, as patients are reluctant to modify regimens.