title: [Indications and compliance of home mechanical insufflation-exsufflation in patients with neuromuscular diseases].
abstract: BACKGROUND
  Neuromuscular disease (NMD) patients frequently have impaired cough. Mechanical insufflation-exsufflation (MI-E) has proven efficacy in improving airway clearance, however data related to its long-term home use is lacking. The purpose of this study was to describe indications, safety and compliance of home MI-E in NMD patients.
  
  
  METHODS
  Four years observational analysis of 21 NMD patients on home MI-E. Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis (ALS) and other NMD. Median age was 58 years. Only cooperative patients with unassisted baseline Peak Cough Flow (PCF) <270 L/min were included. All patients were under continuous mechanical ventilation (6 by tracheostomy). Pulmonary function before initiation of MI-E (median): FVC=0.81 L, MIP=28cmH(2)O, MEP=22 cmH(2)O and PCF=60 L/min. MI-E was performed by previously trained non-professional caregivers, with an on-call support of a trained health care professional. Patients had pulse oximetry monitorization and applied MI-E whenever SpO(2)<95%. Median follow-up was 12 months (3-41 months).
  
  
  RESULTS
  Ten patients (9 ALS) used MI-E daily. Eleven patients used MI-E intermittently, during exacerbations, and in 8 patients early application of MI-E (guided by oximetry feed-back) avoided hospitalization. All tracheostomized patients used MI-E daily and more times a day than patients under NIV. Four patients (3 bulbar ALS), were hospitalized due to secretion encumbrance. MI-E was well-tolerated and there were no complications. In general, caregivers considered MI-E effective. During this period, 4 patients died, related to disease progression.
  
  
  CONCLUSIONS
  Home MI-E is well tolerated, effective and safe if used by well trained caregivers. MI-E should be considered as a complement to mechanical ventilation.
authors.affiliation: Pulmonology Department, HS João, Oporto, Portugal. joao.a.p.bento@gmail.com
authors.forename: João
authors.initials: J
authors.lastname: Bento
authors.forename: Miguel
authors.initials: M
authors.lastname: Gonçalves
authors.forename: Nuno
authors.initials: N
authors.lastname: Silva
authors.forename: Tiago
authors.initials: T
authors.lastname: Pinto
authors.forename: Anabela
authors.initials: A
authors.lastname: Marinho
authors.forename: João Carlos
authors.initials: JC
authors.lastname: Winck
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006699:Home Care Services; D006801:Humans; D008297:Male; D008875:Middle Aged; D009468:Neuromuscular Diseases / Q000628:therapy*; D010349:Patient Compliance; D012138:Respiratory Therapy