Management and Long-Term Outcome of Patients With Chronic Neuromuscular Disease Admitted to the Intensive Care Unit for Acute Respiratory Failure: A Single-Center Retrospective Study

Abstract

BACKGROUND:Patients with chronic neuromuscular disease represent less than 10% of those receiving mechanical ventilation in the intensive care unit (ICU). Little has been reported regarding either ICU management of acute respiratory failure (ARF) in the era of noninvasive mechanical ventilation (NIV) or long-term outcomes. OBJECTIVE: To describe the respiratory management of patients with chronic neuromuscular diseases admitted to our university hospital ICU for ARF, and the long-term outcomes. METHODS: We retrospectively analyzed patients with chronic neuromuscular diseases admitted to our ICU for a first episode of ARF between January 1, 1996, and February 27, 2007. We assessed severity of illness on ICU admission, respiratory management during ICU stay, and outcomes on June 15, 2008. RESULTS: During the study period, 87 patients (44 with hereditary and 43 with acquired neuromuscular diseases) had their first ARF episode that required ICU admission. In the hereditary-diseases group and the acquired-diseases group, respectively, the rates of NIV use during the ICU stay were 82% and 63% (P ‫؍‬ .040), the intubation rates were 30% and 56% (P ‫؍‬ .02), and the tracheotomy rates were 9% and 12% (difference not significant). At the final assessment (median 3 years) the mortality rate was 58%, and mortality was not significantly related to the type of neuromuscular disease. At final assessment, 46% of the patients were on NIV and 29% had tracheotomy. CONCLUSIONS: In our ICU, chronic neuromuscular disease is an uncommon cause of ARF, for which we often use NIV. These patients had a low probability of death in the ICU. Long-term outcome was independent of the type of neuromuscular disease.

Acute neuromuscular respiratory failure: a population-based study of aetiology and outcome in Northern Ireland

“…This may support the role for short-term escalation of respiratory support in degenerative conditions with chronic neuromuscular respiratory difficulties beyond the established management with supportive non-invasive ventilation on a mainly out-of-hospital basis 19 20. ICU admission in patients with chronic NMRF is rare (between 1% and 10%), and there are limited data on post-ICU outcome 21 22. We did not specifically examine the management of acute-on-chronic respiratory failure here, but it would be interesting to look at in more detail.…”

Emergency Neurological Life Support: Airway, Ventilation, and Sedation

“…Although some patients with neuromuscular disease require immediate intubation, those with preserved bulbar function and reasonable functional ventilatory reserves may undergo a trial of non-invasive ventilation combined with airway clearance by the frequent use of chest physiotherapy and a cough-assist device [28][29][30].…”

“…Basic principles of cervical spine stabilization have been developed and refined and can be reviewed in the American College of Surgeons' Advanced Trauma Life Support (ATLS) course [30]. The use of cricoid pressure is no longer recommended during intubation, it definitely should not be implemented in patients with cervical spine injury, as it may cause posterior displacement of the cervical spine [32,33].…”

Benefits of non‐invasive ventilation in acute hypercapnic respiratory failure

“…Flandreau et al . described the outcome of 87 NMD patients experiencing their first AHRF episode requiring ICU admission.…”