Mekanik İnsuflasyon-Eksuflasyon Cihazının Nöromusküler Hastalıkları Olan Çocuklarda Yaşam Kalitesine ve Hastane Yatışına Etkisi

Year 2023, Volume: 9 Issue: 1, 90 - 97, 01.01.2023

Abdurrahman Erdem Basaran , Ayşen Başaran , Tuğba Kazlı , Suzan Yılmaz Durmuş , Özgür Duman , Şenay Haspolat , Ayşen Bingöl

https://doi.org/10.53394/akd.1164632

Abstract

Giriş: Nöromusküler hastalığı (NMH) olan olgularda en önemli morbidite ve mortalite nedeni solunumsal komplikasyonlardır. Çalışmanın amacı Mekanik İnsuflasyon-Eksuflasyon (Mİ-E) cihazı kullanan NMH’li olguların demografik verilerini ve Mİ-E cihazının yaşam kalitesine ve hastane yatışına olan etkisini incelemekti.
Gereç ve Yöntemler: Aralık 2019 ve Aralık 2020 tarihleri arasında Çocuk Göğüs Hastalıkları Polikliniği’nde NMH tanısıyla takip edilen 18 yaşından küçük hastalar çalışmaya dahil edildi. En az 3 aydır Mİ-E cihazı kullanan 27 NMH ‘li hasta çalışma grubuna ve sadece manuel solunum yolu temizleme teknikleri kullanan 30 hasta kontrol grubuna alındı. Klinik ve demografik özellikleri, Mİ-E cihazı ayarları, balgam çıkarmadaki kolaylık vizüel analog skorları (BÇK) ve yaşam kalitesi skorlarıyla ilgili veriler toplandı. Mİ-E cihazını 12 aydan uzun süre kullanan 13 olgunun Mİ-E cihazı öncesi ve sonrası solunumsal nedenlerle hastaneye yatış verileri değerlendirildi.
Bulgular: Mİ-E cihazı kullanımı sonrası BÇK skorları cihaz kullanımı öncesine göre istatiksel anlamlı yüksekti. Mİ-E cihazı kullanımı öncesi ve sonrası değerlendirilen olgularda hastaneye yatış sayısında ve süresinde istatistiksel anlamlı azalma vardı. Çalışma grubu içerisindeki trakeostomili spinal musküler atrofi (SMA) tanılı olguların yaşam kalitesi skorları ortalaması kontrol grubundaki trakeostomili SMA’lı olgulara göre istatistiksel anlamlı yüksek saptandı.
Sonuç: Mİ-E cihazı kullanımı NMH'li hastalarda BÇK’ya, hastaneye yatış sayı ve süresine olumlu katkı sağladı ve SMA'lı hastalarda yaşam kalitesine olumlu katkıda bulundu.

Keywords

Mekanik İnsuflasyon-Eksuflasyon, nöromusküler hastalık, Mİ-E cihazı, yaşam kalitesi

The Effect of a Mechanical Insufflation-Exsufflation Device on Quality of Life and Hospitalization of Children with Neuromuscular Disorders

Abstract

Background: Respiratory complications are a significant cause of morbidity and mortality in patients with neuromuscular diseases (NMD). The aim of this study was to examine the demographic data of subjects with NMD using a mechanical insufflation-exsufflation (MI-E) device and the effect of an MI-E device on quality of life (QoL) and hospitalization.
Methods: The study included patients under 18 years with NMD followed up in the Pediatric Pulmonology Polyclinic between December 2019 and December 2020. Twenty-seven patients with NMD using an MI-E for at least 3 months were enrolled in the study group and 30 patients with NMD using only manuel airway clearance techniques were enrolled in the control group. Data were collected with respect to clinical and demographic characteristics, the MI-E device settings, ease of expectoration visual analogue scores (EE), and the QoL scores. Thirteen subjects, who used an MI-E device for more than 12 months, were assessed pre-and post-MI-E use hospitalization data for respiratory reasons.
Results: The mean EE scores were significantly higher after MI-E device use compared to before. Among participants who were evaluated pre- and post-MI-E use there was a significant decrease in the number of hospital admissions and length of stay. The mean QoL scores of subjects diagnosed with spinal muscular atrophy (SMA) with tracheostomy in study group were determined to be significantly higher than SMA with tracheostomy in control group.
Conclusions: Use of an MI-E device made a positive contribution to EE, number of hospital admissions and length of stay in patients with NMD, and it contributed positively to QoL in patients with SMA.

Keywords

mechanical insufflation-exsufflation, neuromuscular disorder, MI-E device, quality of life

References

• 1- Mellies U, Dohna-Schwake C, Voit T. Respiratory function assessment and intervention in neuromuscular disorders. Curr Opin Neurol 2005; 18: 543–7.
• 2- Gauld LM. Airway clearance in neuromuscular weakness. Developmental Medicine & Child Neurology 2009; 51:350–355.
• 3- Benditt JO. The neuromuscular respiratory system: physiology, pathophysiology, and a respiratory care approach to patients. Respir Care 2006; 51(8):829-837.
• 4- Deem S. Intensive-care-unit-acquired muscle weakness. Respir Care 2006; 51(9):1042-1052.
• 5- Bott J, Blumenthal S, Buxton M, Ellum S, Falconer C, Garrod R, Harvey A, Hughes T, Lincoln M, Mikelsons C, Potter C, Pryor J, Rimington L, Sinfield F, Thompson C, Vaughn P, White J; British Thoracic Society Physiotherapy Guideline Development Group. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax. 2009 May;64 Suppl 1:i1-51.
• 6- Finder JD. Airway clearance modalities in neuromuscular disease. Paediatr Respir Rev 2010;11:31-34.
• 7- Toussaint M, Chatwin M, Gonzales J, Berlowitz DJ; ENMC Respiratory Therapy Consortium. 228th ENMC International Workshop:: Airway clearance techniques in neuromuscular disorders Naarden, The Netherlands, 3-5 March, 2017. Neuromuscul Disord. 2018 Mar;28(3):289-298.
• 8- Chatwin M, Toussaint M, Gonçalves MR, Sheers N, Mellies U, Gonzales-Bermejo J, Sancho J, Fauroux B, Andersen T, Hov B, Nygren-Bonnier M, Lacombe M, Pernet K, Kampelmacher M, Devaux C, Kinnett K, Sheehan D, Rao F, Villanova M, Berlowitz D, Morrow BM. Airway clearance techniques in neuromuscular disorders: A state of the art review. Respir Med. 2018 Mar;136:98-110.
• 9- Morrow B, Argent A, Zampoli M, Human A, Corten L, Toussaint M. Cough augmentation techniques for people with chronic neuromuscular disorders. Cochrane Database Syst Rev. 2021 Apr 22;4(4):CD013170.
• 10- Veldhoen ES, Verweij-van den Oudenrijn LP, Ros LA, Hulzebos EH, Papazova DA, van der Ent CK, van der Pol LW, Nijman J, Wösten-van Asperen RM. Effect of mechanical insufflation-exsufflation in children with neuromuscular weakness. Pediatr Pulmonol. 2020 Feb;55(2):510-513.
• 11- Moran FC, Spittle A, Delany C, Robertson CF, Massie J. Effect of home mechanical in-exsufflation on hospitalisation and life-style in neuromuscular disease: a pilot study. J Paediatr Child Health. 2013 Mar;49(3):233-7.
• 12- Osadnik C, Stuart-Andrews C, Ellis S, Thompson B, McDonald CF, Holland AE. Positive expiratory pressure via mask does not improve ventilation inhomogeneity more than huffing and coughing in individuals with stable chronic obstructive pulmonary disease and chronic sputum expectoration. Respiration. 2014;87(1):38-44.
• 13- Iannaccone ST, Hynan LS, Morton A, Buchanan R, Limbers CA, Varni JW; AmSMART Group. The PedsQL in pediatric patients with Spinal Muscular Atrophy: feasibility, reliability, and validity of the Pediatric Quality of Life Inventory Generic Core Scales and Neuromuscular Module. Neuromuscul Disord. 2009 Dec;19(12):805-12.
• 14- Davis SE, Hynan LS, Limbers CA, Andersen CM, Greene MC, Varni JW, Iannaccone ST. The PedsQL in pediatric patients with Duchenne muscular dystrophy: feasibility, reliability, and validity of the Pediatric Quality of Life Inventory Neuromuscular Module and Generic Core Scales. J Clin Neuromuscul Dis. 2010 Mar;11(3):97-109.
• 15- Chatwin M, Simonds AK. Long-term mechanical insufflation- exsufflation cough assistance in neuromuscular disease: patterns of use and lessons for application. Respir Care 2020; 65: 135–43.
• 16- Hull J, Aniapravan R, Chan E, Chatwin M, Forton J, Gallagher J, Gibson N, Gordon J, Hughes I, McCulloch R, Russell RR, Simonds A. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. 2012 Jul;67 Suppl 1:i1-40.
• 17- Chatwin M, Bush A, Simonds AK. Outcome of goal-directed non-invasive ventilation and mechanical insufflation/ exsufflation in spinal muscular atrophy type I. Arch Dis Child 2011; 96: 426–32.
• 18- Hov B, Andersen T, Toussaint M, Vollsaeter M, Mikalsen IB, Indrekvam S, Hovland V. Prevalence of long-term mechanical insufflation-exsufflation in children with neurological conditions: a population-based study. Dev Med Child Neurol. 2021 May;63(5):537-544.
• 19- Camela F, Gallucci M, Ricci G. Cough and airway clearance in Duchenne muscular dystrophy. Paediatr Respir Rev 2019; 31:35-39.
• 20- Schroth MK. Special Considerations in the Respiratory Management of Spinal Muscular Atrophy. Pediatrics 2009; S245-S249.
• 21- Boitano LJ. Management of airway clearance in neuromuscular disease. Respir Care. 2006; 51(8):913-22.
• 22- Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997; 112: 1024–1028.
• 23- Hov B, Andersen T, Hovland V, Toussaint M. The clinical use of mechanical insufflation-exsufflation in children with neuromuscular disorders in Europe. Paediatr Respir Rev 2018; 27: 69-73.
• 24- Fauroux B, Guillemot N, Aubertin G, Nathan N, Labit A, Clément A, Lofaso F. Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. Chest. 2008 Jan;133(1):161-8.
• 25- Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest 2000; 118(5): 1390–6.
• 26- Chatwin M, Ross E, Hart N, Nickol AH, Polkey MI, Simonds AK. Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J. 2003 Mar;21(3):502-8.
• 27- Miske LJ, Hickey EM, Kolb SM, Weiner DJ, Panitch HB. Use of the mechanical inexsufflator in pediatric patients with neuromuscular disease and inpaired cough. Chest 2004;125(4):1406–12.
• 28- Vianello A, Corrado A, Arcaro G, Gallan F, Ori C, Minuzzo M, Bevilacqua M. Mechanical insufflation-exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections. Am J Phys Med Rehabil. 2005 Feb;84(2):83-8; discussion 89-91.
• 29- McDonald LA, Berlowitz DJ, Howard ME, Rautela L, Chao C, Sheers N. Pneumothorax in neuromuscular disease associated with lung volume recruitment and mechanical insufflation-exsufflation. Respirol Case Rep. 2019 Jun 13;7(6):e00447.
• 30- Yasokawa N, Tanaka H, Kurose K, Abe M, Oga T. Mechanical insufflation-exsufflation-related bilateral pneumothorax. Respiratory Medicine Case Reports 2020; 29:101017.
• 31- Meric H, Falaize L, Pradon D, Lacombe M, Petitjean M, Orlikowski D, Prigent H, Lofaso F. Short-term effect of volume recruitment-derecruitment manoeuvre on chest-wall motion in Duchenne muscular dystrophy. Chron Respir Dis. 2017 May;14(2):110-116.
• 32- Mahede T, Davis G, Rutkay A, Baxendale S, Sun W, Dawkins HJ, Molster C, Graham CE. Use of mechanical airway clearance devices in the home by people with neuromuscular disorders: effects on health service use and lifestyle benefits. Orphanet J Rare Dis. 2015 May 6;10:54.