Use of air stacking to improve pulmonary function in Indonesian Duchenne muscular dystrophy patients: bridging the standard of care gap in low middle income country setting

Kristy Iskandar, Sunartini, Andika Priamas Nugrahanto, Nissya Ilma, Alvin Santoso Kalim, Guritno Adistyawan, Siswanto, Roni Naning, Kristy Iskandar, Sunartini, Andika Priamas Nugrahanto, Nissya Ilma, Alvin Santoso Kalim, Guritno Adistyawan, Siswanto, Roni Naning

Abstract

Background: Duchenne Muscular Dystrophy (DMD) is a fatal X-linked recessive neuromuscular disease, characterized by progressive loss of muscle strength. Respiratory failure is the main cause of morbidity and mortality in DMD patients. Respiratory devices have been reported to increase the effectiveness of cough and pulmonary function, thus prolong the survival rate. However, there is scarcity of studies about DMD patients' respiratory profiles and usage of respiratory devices in Indonesia.

Methods: We recruited 8 Indonesian DMD patients in Dr. Sardjito Hospital and UGM Academic Hospital, Yogyakarta. Baseline pulmonary function was measured using spirometry. Peak Cough Flow was measured at baseline, with chest compression, after air stacking with manual ventilation bag, and with the combined techniques. Data recorded was presented as mean ± SD and analysed using ANOVA.

Results: Here we show the respiratory profiles from 8 non-ambulatory DMD patients (mean age: 13.25 ± 3.96 years old) confirmed by genetic testing. None of them had access to respiratory devices. Spirometry measurements showed 7 of 8 patients had severe restrictive pulmonary function with mean FEV1/FVC 22.40 ± 10.30% of predictive values (normal ratio > 70%). In addition, all patients showed poor cough performances measured by peak cough flowmeter (160 ± 44.58 L/min (normal value > 270 L/min)) that were improved by air stacking using a manual ventilation bag (167.4 ± 46.72 L/min). Three patients who had nocturnal hypoventilation did not have daytime hypercapnia. Manual ventilation bag or mechanical in-/ex-sufflation was indicated in 75% of patients while nocturnal assisted ventilation was indicated in 50% of patients. Neither daytime assisted ventilation nor tracheostomy was indicated in these patients.

Conclusion: Use of manual exsufflation in combination with the manual ventilation bag for air stacking to improve cough performance is recommended as the first step of respiratory management in DMD patients. Provision of manual ventilation bag serve as an affordable and effective device for respiratory support in the early stage of respiratory involvement in those non-ambulatory patients with DMD.

Keywords: Duchenne muscular dystrophy; Respiratory devices; Respiratory function.

Conflict of interest statement

Competing interestsThe authors declare that they have no competing interests.

© The Author(s). 2019.

Figures

Fig. 1
Fig. 1
Peak Cough Flow (PCF) value at baseline, after chest compression, after air stacking using ambu bag and after combined technique (chest compression and ambu bag air stacking), PCF  160 L/min: 3 patients. *p-value< 0.05.
Fig. 2
Fig. 2
Correlation between lung function (FVC and FEV1) and clinical characteristic (age and degree of cobbs angle)

References

    1. Buu MC. Respiratory complications, management and treatments for neuromuscular disease in children. Curr Opin Pediatr. 2017;29:326–333. doi: 10.1097/MOP.0000000000000498.
    1. Kravits RM. Airway clearance in Duchenne muscular dystrophy. Pediatrics. 2009;123:S231–S235. doi: 10.1542/peds.2008-2952G.
    1. LoMauro Antonella, Romei Marianna, D'Angelo Maria Grazia, Aliverti Andrea. Determinants of cough efficiency in Duchenne muscular dystrophy. Pediatric Pulmonology. 2013;49(4):357–365. doi: 10.1002/ppul.22836.
    1. Sheehan Daniel W., Birnkrant David J., Benditt Joshua O., Eagle Michelle, Finder Jonathan D., Kissel John, Kravitz Richard M., Sawnani Hemant, Shell Richard, Sussman Michael D., Wolfe Lisa F. Respiratory Management of the Patient With Duchenne Muscular Dystrophy. Pediatrics. 2018;142(Supplement 2):S62–S71. doi: 10.1542/peds.2018-0333H.
    1. Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol. 2018;17(4):347–361. doi: 10.1016/S1474-4422(18)30025-5.
    1. Gauld LM, Boynton A. Relationship between peak cough flow and spirometry in Duchenne muscular dystrophy. Pediatr Pulmonol. 2005;39:457–460. doi: 10.1002/ppul.20151.
    1. Toussaint M, Pernet K, Steen M, Haan J, Sheers N. Cough augmentation in subjects with Duchenne muscular dystrophy: comparison of air stacking via a resuscitator bag versus mechanical ventilation. Respir Care. 2016;61(1):61–67. doi: 10.4187/respcare.04033.
    1. Bach John R., Ishikawa Yuka, Kim Heakyung. Prevention of Pulmonary Morbidity for Patients With Duchenne Muscular Dystrophy. Chest. 1997;112(4):1024–1028. doi: 10.1378/chest.112.4.1024.
    1. Berry RB, Budhiraja R, Gottlieb DJ, et al. Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberation of the Sleep Apnea Definitions Task Force of the American Academy of Sleep Medicine. J Clin Sleep Med. 2012;8:597–619.
    1. Nardi J, Pringent H, Adala A, Bohic M, Lebargy F, Quera-Salva MA, Orlikowski D, Lofaso F. Nocturnal oximetry and transcutaneus carbon dioxide in home ventilated neuromuscular patients. Respir Care. 2012;57(9):1425–1430. doi: 10.4187/respcare.01658.
    1. Kang SW, Kang YS, Moon JH, Yoo TW. Assisted cough and pulmonary compliance in patients with Duchenne muscular dystrophy. Yonsei Med J. 2005;46(2):233–238. doi: 10.3349/ymj.2005.46.2.233.
    1. Brito MF, Moreira GA, Pradella-Halinnan M, Tufik S. Air stacking and chest compression increase peak cough flow in patients with Duchenne muscular dystrophy. J Bras Pneumol. 2009;35(10):973–979. doi: 10.1590/S1806-37132009001000005.
    1. American Thoracic Society Consensus Statement Respiratory care of the patient with Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2004;170:456–465. doi: 10.1164/rccm.200307-885ST.

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