Effectiveness of Telerehabilitation on Pulmonary Function in Children with Duchenne Muscular Dystrophy: A Pre-Post Intervention Study.
-
Priyanka Gupta
,
Seveka Bali
,
Amrit Pal Singh Sood
,
Amit Kumar Mital
,
Priyanka Choudhary
,
Sudhansu Sekhar Baral
,
Somya Saxena
,
Reeta Upadhyay
,
Renu Suthar
Abstract
Background - Duchenne Muscular Dystrophy (DMD) is an inherited neuromuscular disorder caused by mutations in the dystrophin gene, leading to progressive muscle weakness. This study aimed to evaluate the efficacy of telerehabilitation in the improvement of pulmonary function in DMD patients.
Materials and Methods - An interventional study was conducted among boys aged 5 to 12 years with DMD enrolled in the Pediatric Neurology clinic between September 2023 to February 2024. Telemedicine-based respiratory exercises were explained to the patients and their parents every 15 days for a 3 month-period, and patients were encouraged to do exercise for at least 5 days a week. Pulmonary function tests, including Forced Vital Capacity (FVC) and Forced Expiratory Volume in 1 second (FEV1), were recorded before and after intervention.
Results –67 children participated in this study. There was significant improvement in forced vital capacity (FVC) [p value <0.001, Cohen’s d value=1.93] and forced expiratory volume in one second (FEV1) [p value <0.001, Cohen’s d value=1.06]. The FEV1/FVC ratio, a parameter that can be elevated in restrictive diseases, was also significantly decreased [p value <0.001, Cohen’s d value = -0.63]. For a one-unit increase in age, the FVC change after intervention was predicted to decrease significantly by 0.24 units, holding other variables constant.
Conclusions: Telemedicine-based pulmonary rehabilitation for 3 months markedly improved the pulmonary function, thus concluding that this intervention is effective in improving the pulmonary function of DMD patients, and effectiveness is more if telerehabilitation is started at an early age.
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12. Finder J, Mayer OH, Sheehan D, et al. Pulmonary endpoints in Duchenne muscular dystrophy. A workshop summary. Am J Respir Crit Care Med. 2017;196(4):512-9.
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15. D'Souza AF, Rebello SR. Perceptions and Willingness of Physiotherapists in India to Use Telerehabilitation During the COVID-19 Pandemic. Int J Telerehabil. 2021 Dec 16;13(2):e6425. doi: 10.5195/ijt.2021.6425. PMID: 35646229; PMCID: PMC9098138.
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18. Seron P, Oliveros MJ, Gutierrez-Arias R, et al. Effectiveness of telerehabilitation in physical therapy: a rapid overview. Phys Ther. 2021 Jun 1;101(6):pzab053. doi: 10.1093/ptj/pzab053. PMID: 33561280; PMCID: PMC7928601.
19. Kenis-Coskun O, Imamoglu S, Karamancioglu B, Kurt K, Ozturk G, Karadag-Saygi E. Comparison of telerehabilitation versus home-based video exercise in patients with Duchenne muscular dystrophy: a single-blind randomized study. Acta Neurol. Belg. 2022;122(5):1269-80.
20. Khanna M, Gowda GS, Bagevadi VI, et al. Feasibility and utility of tele-neurorehabilitation service in India: Experience from a quaternary center. J. Neurosci. Rural Pract. 2018;9(4):541.
21. Shaw MT, Best P, Frontario A, Charvet LE. Telerehabilitation benefits patients with multiple sclerosis in an urban setting. J Telemed Telecare. 2021;27(1):39-45.
22. Akulwar-Tajane I, Bhatt GD. Telerehabilitation: an alternative service delivery model for pediatric neurorehabilitation services at a tertiary care center in India. Int J Phys Med Rehabil. 2021;9:S4.
23. Erden Güner A, Öztürk D, Sarı M, et al. Maintaining Physical Health in Individuals with Duchenne Muscular Dystrophy Through Telerehabilitation. Phys Occup Ther Pediatr. 2024;44(6):812-828. doi: 10.1080/01942638.2024.2376055. Epub 2024 Jul 16. PMID: 39014867.
24. LoMauro A, D’Angelo MG, Aliverti A. Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Ther Clin Risk Manag. 2015:1475-88.
25. Fromageot C, Lofaso F, Annane D, et al. Supine fall in lung volumes in the assessment of diaphragmatic weakness in neuromuscular disorders. Arch. Phys. Med. Rehabil. 2001;82(1):123-8.
26. Nicot F, Hart N, Forin V, et al. Respiratory muscle testing: a valuable tool for children with neuromuscular disorders. Am. J. Respir. Crit. Care Med. 2006;174(1):67-74.
27. Joo S, Shin D, Song C. The effects of game-based breathing exercise on pulmonary function in stroke patients: a preliminary study. Med Sci Monit. 2015;21:1806.
28. Kim K. The effect of chest expansion and pulmonary function of stroke patients after breathing exercise. J Kor Phys Ther. 2009;21(3):25-32.
29. Wasilewska E, Sobierajska-Rek A, Małgorzewicz S, Soliński M, Jassem E. Benefits of Telemonitoring of Pulmonary Function—3-Month Follow-Up of Home Electronic Spirometry in Patients with Duchenne Muscular Dystrophy. J. Clin. Med. 2022;11(3):856.
30. Rodrigues MR, Carvalho CR, Santaella DF, Lorenzi-Filho G, Marie SK. Effects of yoga breathing exercises on pulmonary function in patients with Duchenne muscular dystrophy: an exploratory analysis. J Bras Pneumol. 2014;40:128-33.
31. Dhargave P, Nalini A, Nagarathna R, et al. Effect of yoga and physiotherapy on pulmonary functions in children with Duchenne muscular dystrophy–a comparative study. Int J Yoga. 2021;14(2):133-40.
32. Wanke T, Toifl K, Merkle M, Formanek D, Lahrmann H, Zwick H. Inspiratory muscle training in patients with Duchenne muscular dystrophy. Chest. 1994;105(2):475-82.
33. Bach JR, Bianchi C, Vidigal-Lopes M, Turi S, Felisari G. Lung inflation by glossopharyngeal breathing and “air stacking” in Duchenne muscular dystrophy. Am. J. Phys. Med. 2007;86(4):295-300.
34. Yu MK, Chiu AY, Chau SK, Rosa Duque JS, Wong WH, Chan SH. A pilot study of an integrated, personalized, respiratory and motor telerehabilitation program for pediatric patients with hereditary neuromuscular disorders. Muscle Nerve. 2023;68(6):857-64.
2. 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):347-61.
3. Gomez-Merino E, Bach JR. Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil. 2002;81(6):411-5.
4. Yiu EM, Kornberg AJ. Duchenne muscular dystrophy. J Paediatr Child Health. 2015 Aug;51(8):759-64.
5. Wasilewska E, Małgorzewicz S, Sobierajska-Rek A, et al. Transition from childhood to adulthood in patients with Duchenne muscular dystrophy. Med. 2020;56(9):426.
6. Passamano L, Taglia A, Palladino A, et al. Improvement of survival in Duchenne Muscular Dystrophy: retrospective analysis of 835 patients. Acta Myol. 2012;31(2):121.
7. Matthews E, Brassington R, Kuntzer T, Jichi F, Manzur AY. Corticosteroids for the treatment of Duchenne muscular dystrophy. Cochrane Database Syst. Rev. 2016(5).
8. LoMauro A, Romei M, Gandossini S, et al. Evolution of respiratory function in Duchenne muscular dystrophy from childhood to adulthood. Eur Respir J. 2018;51(2).
9. Hoque R. Sleep-disordered breathing in Duchenne muscular dystrophy: an assessment of the literature. J. Clin. Sleep Med. 2016;12(6):905-11.
10. Phillips MF, Quinlivan RC, Edwards RH, Calverley PM. Changes in spirometry over time as a prognostic marker in patients with Duchenne muscular dystrophy. Am. J. Respir. Crit. Care Med. 2001;164(12):2191-4.
11. Balaban B, Matthews DJ, Clayton GH, Carry T. Corticosteroid treatment and functional improvement in Duchenne muscular dystrophy: long-term effect. Am J Phys Med Rehabil. 2005;84(11):843-50.
12. Finder J, Mayer OH, Sheehan D, et al. Pulmonary endpoints in Duchenne muscular dystrophy. A workshop summary. Am J Respir Crit Care Med. 2017;196(4):512-9.
13. McDonald CM, Henricson EK, Abresch RT, et al. The cooperative international neuromuscular research group Duchenne natural history study—a longitudinal investigation in the era of glucocorticoid therapy: design of protocol and the methods used. Muscle Nerve. 2013;48(1):32-54.
14. Pradnya D, Nalini A, Adoor M, et al. Respiratory dysfunctions in children with Duchenne muscular dystrophy. Int J Physiother Res. 2016;4(1):1365-69.
15. D'Souza AF, Rebello SR. Perceptions and Willingness of Physiotherapists in India to Use Telerehabilitation During the COVID-19 Pandemic. Int J Telerehabil. 2021 Dec 16;13(2):e6425. doi: 10.5195/ijt.2021.6425. PMID: 35646229; PMCID: PMC9098138.
16. Sobierajska-Rek A, Mański Ł, Jabłońska-Brudło J, Śledzińska K, Wasilewska E, Szalewska D. Respiratory Telerehabilitation of Boys and Young Men with Duchenne Muscular Dystrophy in the COVID-19 Pandemic. Int J Environ Res Public Health. 2021 Jun 8;18(12):6179. doi: 10.3390/ijerph18126179. PMID: 34200985; PMCID: PMC8229414.
17. Maltais F. Glossopharyngeal breathing. Am J Respir Crit Care Med 2011;184:381. doi: https://doi.org/10.1164/rccm.201012-2031IM
18. Seron P, Oliveros MJ, Gutierrez-Arias R, et al. Effectiveness of telerehabilitation in physical therapy: a rapid overview. Phys Ther. 2021 Jun 1;101(6):pzab053. doi: 10.1093/ptj/pzab053. PMID: 33561280; PMCID: PMC7928601.
19. Kenis-Coskun O, Imamoglu S, Karamancioglu B, Kurt K, Ozturk G, Karadag-Saygi E. Comparison of telerehabilitation versus home-based video exercise in patients with Duchenne muscular dystrophy: a single-blind randomized study. Acta Neurol. Belg. 2022;122(5):1269-80.
20. Khanna M, Gowda GS, Bagevadi VI, et al. Feasibility and utility of tele-neurorehabilitation service in India: Experience from a quaternary center. J. Neurosci. Rural Pract. 2018;9(4):541.
21. Shaw MT, Best P, Frontario A, Charvet LE. Telerehabilitation benefits patients with multiple sclerosis in an urban setting. J Telemed Telecare. 2021;27(1):39-45.
22. Akulwar-Tajane I, Bhatt GD. Telerehabilitation: an alternative service delivery model for pediatric neurorehabilitation services at a tertiary care center in India. Int J Phys Med Rehabil. 2021;9:S4.
23. Erden Güner A, Öztürk D, Sarı M, et al. Maintaining Physical Health in Individuals with Duchenne Muscular Dystrophy Through Telerehabilitation. Phys Occup Ther Pediatr. 2024;44(6):812-828. doi: 10.1080/01942638.2024.2376055. Epub 2024 Jul 16. PMID: 39014867.
24. LoMauro A, D’Angelo MG, Aliverti A. Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Ther Clin Risk Manag. 2015:1475-88.
25. Fromageot C, Lofaso F, Annane D, et al. Supine fall in lung volumes in the assessment of diaphragmatic weakness in neuromuscular disorders. Arch. Phys. Med. Rehabil. 2001;82(1):123-8.
26. Nicot F, Hart N, Forin V, et al. Respiratory muscle testing: a valuable tool for children with neuromuscular disorders. Am. J. Respir. Crit. Care Med. 2006;174(1):67-74.
27. Joo S, Shin D, Song C. The effects of game-based breathing exercise on pulmonary function in stroke patients: a preliminary study. Med Sci Monit. 2015;21:1806.
28. Kim K. The effect of chest expansion and pulmonary function of stroke patients after breathing exercise. J Kor Phys Ther. 2009;21(3):25-32.
29. Wasilewska E, Sobierajska-Rek A, Małgorzewicz S, Soliński M, Jassem E. Benefits of Telemonitoring of Pulmonary Function—3-Month Follow-Up of Home Electronic Spirometry in Patients with Duchenne Muscular Dystrophy. J. Clin. Med. 2022;11(3):856.
30. Rodrigues MR, Carvalho CR, Santaella DF, Lorenzi-Filho G, Marie SK. Effects of yoga breathing exercises on pulmonary function in patients with Duchenne muscular dystrophy: an exploratory analysis. J Bras Pneumol. 2014;40:128-33.
31. Dhargave P, Nalini A, Nagarathna R, et al. Effect of yoga and physiotherapy on pulmonary functions in children with Duchenne muscular dystrophy–a comparative study. Int J Yoga. 2021;14(2):133-40.
32. Wanke T, Toifl K, Merkle M, Formanek D, Lahrmann H, Zwick H. Inspiratory muscle training in patients with Duchenne muscular dystrophy. Chest. 1994;105(2):475-82.
33. Bach JR, Bianchi C, Vidigal-Lopes M, Turi S, Felisari G. Lung inflation by glossopharyngeal breathing and “air stacking” in Duchenne muscular dystrophy. Am. J. Phys. Med. 2007;86(4):295-300.
34. Yu MK, Chiu AY, Chau SK, Rosa Duque JS, Wong WH, Chan SH. A pilot study of an integrated, personalized, respiratory and motor telerehabilitation program for pediatric patients with hereditary neuromuscular disorders. Muscle Nerve. 2023;68(6):857-64.
| Issue | Articles in Press | |
| Section | Research Article(s) | |
| Keywords | ||
| Telerehabilitation; Duchenne muscular dystrophy; Spirometry; Breathing exercises; Pulmonary function tests | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Gupta P, Bali S, Sood AP, Mital A, Choudhary P, Baral S, Saxena S, Upadhyay R, Suthar R. Effectiveness of Telerehabilitation on Pulmonary Function in Children with Duchenne Muscular Dystrophy: A Pre-Post Intervention Study. jmr. 2025;(-).
