Initiation of home mechanical ventilation in children with neuromuscular diseases

Objective: To determine how often home mechanical ventilation (HMV) is instituted electively in children with respiratory failure from neuromuscular diseases and whether there were opportunities to discuss therapeutic options with patients/families before respiratory failure. Methods: Patients with neuromuscular disease (n = 73) requiring HMV (age, 2 months to 24 years) were studied. Whether HMV was initiated nonelectively because of acute respiratory failure or electively before acute respiratory failure, and opportunities for health care providers to discuss therapeutic options with patients/families before acute respiratory failure (hospitalization with pneumonia, clinic visits for preoperative evaluation, pulmonary function testing [PFT] and/or polysomnography [PSG]) were recorded. Results: HMV was initiated electively in 21% of patients with neuromuscular disease; 69% of the nonelective HMV group had HMV initiated after respiratory failure caused by pneumonia. In the nonelective group, opportunities for discussion of therapeutic options with the patients and families could have occurred before respiratory failure during 111 hospitalizations for pneumonia, 13 preoperative evaluations, 43 abnormal PETs, and 24 abnormal PSGs. Conclusions: Most patients with neuromuscular disease had HMV initiated nonelectively after acute respiratory failure caused by pneumonia. Opportunities for discussing the therapeutic options with patients and families before respiratory failure were missed or ineffective.