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 PFTs, 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.
Sritippayawan
Suchada
S
Division of Pediatric Pulmonology, Childrens Hospital Los Angeles, California 90027, USA.
Kun
Sheila S
SS
Keens
Thomas G
TG
Davidson Ward
Sally L
SL
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006699:Home Care Services; D006760:Hospitalization; D006801:Humans; D008297:Male; D009468:Neuromuscular Diseases / Q000150:complications* / Q000534:rehabilitation*; D011014:Pneumonia / Q000150:complications / Q000534:rehabilitation; D017286:Polysomnography / Q000295:instrumentation / Q000706:statistics & numerical data; D012121:Respiration, Artificial / Q000295:instrumentation* / Q000706:statistics & numerical data; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000534:rehabilitation*; D012189:Retrospective Studies