Résumé :
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Introduction : Patients with neuromuscular disease (NMD) frequently have impaired cough. Inefficient bronchial secretion clearance may cause episodes of severe acute ventilatory failure (ARF). During hospital stay, mechanical assisted cough with mechanical insufflation-exsufflation (MI-E) has been successfully applied as first-line intervention in patients with neuromuscular diseases with acute chest infections and at risk of ARF, however limited data exist on its home long-term use. Objective : To determine the physiologic effects, compliance, feasibility and safety of home MI-E treatment in patients with NMD. Material and methods : Retrospective analysis performed in two centers with a neuromuscular clinic organization. Thirty two patients (9 females) with NMD (24 with amyotrophic lateral sclerosis and 8 with other neuromuscular disorders) with mean age of 57.8±14,8 years. Inclusion criteria were confirmed NMD diagnose with impaired cough, inadequate Vital Capacity (< 30%), reduced respiratory muscle strength (PImax and PEmax < 30%) and the need of home mechanical ventilation. Nineteen patients were on noninvasive mechanical ventilation (NIV) and had a mean peak cough flow (PCF) of 122±89 L/min. Thirteen patients were on tracheostomy ventilation and had a mean peak expiratory flow (PEF) of 78,8±39,1L/min. MI-E was applied at home in all patients following a pulse oximetry feedback protocol and performed either by caregivers previously trained by respiratory physiotherapists (RT) or by our department and home care staff (RT and nurses) with an on-call regime supported by a tele-assisted integrated care programme. Patients were previously taught to detect early symptoms of secretion encumbrance. Data were recorded and analysed as function of, spirometry, respiratory muscle strength, cough efficacy and oxygen saturation (SpO2) during routine clinical evaluations and during episodes of secretion encumbrance and ARF. Safety, tolerance, compliance and efficacy were assessed based the number of MI-E sessions at home and the number of complications and hospitalizations related to secretion encumbrance and respiratory tract infections. Intolerance and side effects of the technique were also assessed. Results : Pulmonary function previous to initiation of MI-E was : FVC=0.99±0,49L (29%pred), FEV1=0.88±0,45L(28% pred),MIP=29±24,7cmH2O, median MEP=27,5± 17,5cmH2O. Mean follow-up under home MI-E was 25 months. Eight patients (7 with ALS, 1 with DMD) needed daily MI-E sessions (at least once a day). Twenty five patients used it intermittently, during episodes of severe secretion encumbrance. In 17 patients (5 with tracheostomy), timely initiation of MI-E (guided by oximetry feed-back) totally avoided hospitalization and admissions to emergency department for ARF. Fifteen patients (8 with tracheostomy) were hospitalized at least one time (maximum 4 times) for respiratory problems that could not be solved only by MI-E at home. During ARF episodes we could monitor some physiologic effects ofMI-E : SpO2 improved (92±1 vs 95±1, p<0,05) , number of deep airway suctions per day decreased (8±1 vs 3±1, p<0,05 ) and dyspnoea borg scale improved (7,3±0,9 vs 2±1, p<0,05). MI-E was well-tolerated and there were no complications related to the treatment. In general, MI-E was considered effective by caregivers and by all the home care team. Conclusion : Home MI-E is safe if used by well prepared caregivers. An on-call regime supported by a tele-assisted integrated care programme is efficient in providing secretion clearance in time to prevent ARF.MI-E use during respiratory infections avoided hospitalization in non-tracheostomized patients as well as decreased frequency of tracheobronchial infections in tracheostomized patients.
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