title: Feasibility of Lung Volume Recruitment in Early Neuromuscular Weakness: A Comparison Between Amyotrophic Lateral Sclerosis, Myotonic Dystrophy, and Postpolio Syndrome.
abstract: BACKGROUND
  Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown.
  
  
  OBJECTIVE
  To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment.
  
  
  METHODS
  Feasibility study.
  
  
  METHODS
  Academic tertiary care center.
  
  
  METHODS
  Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV.
  
  
  METHODS
  Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months.
  
  
  METHODS
  Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference.
  
  
  RESULTS
  Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L (P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics.
  
  
  CONCLUSIONS
  In patients with NMDs who have early restrictive respiratory defects but do not yet require NIV, regular use of LVR is feasible with no negative impact on QOL over a 3-month period and may have physiological benefits. Further work is needed to determine whether early institution of LVR can improve respiratory system mechanics and help delay ventilatory failure in persons with NMDs.
authors.affiliation: Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2504 Montreal, Quebec, Canada H4A 3J1.|Respiratory Epidemiology and Clinical Research Unit, McGill University, Montreal, Quebec, Canada.
authors.forename: Marta
authors.initials: M
authors.lastname: Kaminska
authors.affiliation: Department of Respiratory Therapy, Montreal Chest Institute, McGill University Health Centre, Montreal, Quebec, Canada.
authors.forename: Franceen
authors.initials: F
authors.lastname: Browman
authors.affiliation: Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, Quebec, Canada.
authors.forename: Daria A
authors.initials: DA
authors.lastname: Trojan
authors.affiliation: Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, Quebec, Canada.
authors.forename: Angela
authors.initials: A
authors.lastname: Genge
authors.affiliation: Respiratory Epidemiology and Clinical Research Unit, McGill University, Montreal, Quebec, Canada.|Departments of Medicine and Epidemiology, Biostatistics & Occupational Health, McGill University Health Centre, Montreal, Quebec, Canada.
authors.forename: Andrea
authors.initials: A
authors.lastname: Benedetti
authors.affiliation: Respiratory Division, McGill University Health Centre, Montreal, Quebec, Canada.|Meakins-Christie Laboratories, McGill University and Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada.
authors.forename: Basil J
authors.initials: BJ
authors.lastname: Petrof
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000503:physiopathology / Q000534:rehabilitation*; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008168:Lung / Q000503:physiopathology; D008176:Lung Volume Measurements / Q000379:methods; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications / Q000503:physiopathology / Q000534:rehabilitation*; D009929:Organ Size; D016262:Postpoliomyelitis Syndrome / Q000150:complications / Q000503:physiopathology / Q000534:rehabilitation*; D011788:Quality of Life; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000534:rehabilitation*; D012138:Respiratory Therapy / Q000379:methods*; D012189:Retrospective Studies; D013990:Tidal Volume / Q000502:physiology*; D013997:Time Factors