“Pulmonary dysfunction in children with Duchenne muscular dystrophy may appear earlier than we thought – analysis using novel methodology based on z-scores."

Mański

Jabłońska-Brudło

et al. 2021

IJERPH

Self Cite

Background: The COVID-19 pandemic forced reorganization of the multidisciplinary healthcare system for Duchenne muscular dystrophy. Digital solutions seem to be optimal for providing rehabilitation at this time. The aim of this study was to investigate whether it is possible to conduct respiratory physical therapy with the use of telerehabilitation in Duchenne muscular dystrophy. Methods: The study was conducted during an online conference for families with DMD. During the physical therapy panel we showed the video with the instructions of respiratory exercises. All participants (n = 152) were asked to fill in the online survey evaluating the quality, acceptance, and understanding of the instructions. Results: The survey was filled in by 45 (29.6%) participants. The mean rating of satisfaction was 4.70/5, and for intelligibility was 4.78/5. Thirty-seven (82.2%) patients declared that they had performed the exercises, all caregivers declared that it was possible to perform the proposed exercises a few times a week or daily, and only two respondents replied to invitations to individual online sessions. Conclusions: Findings from the study show that respiratory telerehabilitation may be implemented for DMD patients; however, the interest in digital rehabilitation among caregivers of DMD boys in Poland is low. The reasons for this situation require further research.

Section: Introductionmentioning

confidence: 99%

Respiratory Telerehabilitation of Boys and Young Men with Duchenne Muscular Dystrophy in the COVID-19 Pandemic

Mański

Jabłońska-Brudło

et al. 2021

IJERPH

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“…Innovative forms of lung function monitoring at home are extremely important during the COVID-19 pandemic, especially for patients with chronic diseases and dysfunction of the respiratory system. Many studies showed that respiratory function declines at a rate of 6-11% annually in patients with DMD [3][4][5]. Moreover, respiratory muscle weakness leads to secondary changes, such as decreased lung compliance, ineffective cough with deterioration of airway clearance, and repeated infections [3,4], which causes DMD patients to have a higher risk of a severe course of COVID-19 [7].…”

Section: Discussionmentioning

confidence: 99%

“…Many studies showed that respiratory function declines at a rate of 6-11% annually in patients with DMD [3][4][5]. Moreover, respiratory muscle weakness leads to secondary changes, such as decreased lung compliance, ineffective cough with deterioration of airway clearance, and repeated infections [3,4], which causes DMD patients to have a higher risk of a severe course of COVID-19 [7].…”

Section: Discussionmentioning

confidence: 99%

“…Many studies confirm that from the age of 7 years, lung function in children with DMD does not increase as in healthy peers. Moreover, when the child loses the ability to walk independently (usually between 10 and 13 years of age), values of pulmonary function tests rapidly deteriorate [3,4]. Basic care at this stage includes the initiation of early monitoring of respiratory system functions.…”

Section: Introductionmentioning

confidence: 99%

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Is It Possible to Have Home E-Monitoring of Pulmonary Function in Our Patients with Duchenne Muscular Dystrophy in the COVID-19 Pandemic?—A One Center Pilot Study

Wasilewska

Małgorzewicz

et al. 2021

IJERPH

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Background: Duchenne muscular dystrophy (DMD) is the most common, progressive, irreversible muscular dystrophy. Pulmonary function is crucial for duration of life in this disease. Currently, the European Respiratory Society is focused on digital health, seeking innovations that will be realistic for digital respiratory medicine to support professionals and patients during the COVID-19 pandemic. Aims: The aim of this study was to investigate whether it is possible to monitor pulmonary function at home using an individual electronic spirometry system in boys with Duchenne muscular dystrophy. Materials and methods: In this observational, prospective study, conducted from March 2021 to June 2021, twenty boys with DMD (aged 8–16) were enrolled. The patients were recruited from the Rare Disease Centre, University Clinical Centre, of Gdańsk, Poland. Medical history and anthropometric data were collected, and spirometry (Jaeger, Germany) was performed in all patients at the start of the study. Each patient received an electronic individual spirometer (AioCare) and was asked to perform spirometry on their own every day, morning and evening, at home for a period of 4 weeks. The number of measurements, correctness of performing measurements, forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and peak expiratory flow (PEF) were evaluated. Results: Finally, 14 out of 20 boys enrolled in the study with a mean age of 12.5 years (7 non-ambulatory) applied and received a home spirometer (AioCare). A total of 283 measurements were performed by all patients at home for 4 weeks. Half of the patients were able to perform measurements correctly. There were no significant differences between mean values of FVC, FE1, PEF between home and hospital spirometry (p > 0.05) expect PEF pv% (p < 0.00046). Patients with higher FEV1 (p = 0.0387) and lower BMI (p = 0.0494) were more likely to take home spirometer measurements. The mean general satisfaction rating of home-spirometry was 4.33/5 (SD 0.78), the mean intelligibility rating was 4.83/5 (SD 0.58). Reasons for irregular measurements were: forgetting (43%), lack of motivation (29%), difficulty (14%), lack of time (14%). Conclusion: Home electronic monitoring of pulmonary function in patients with DMD is possible to implement in daily routines at home. This protocol should be introduced as early as possible in patients 7–8 years old with good, preserved lung function. Patients accept this form of medical care but require more education about the benefits of e-monitoring. There is a need to implement a system to remind patients of the use of electronic medical devices at home, e.g., via SMS (short message service).

“…It is known that pulmonary function declines at a rate of 6-11% annually in patients with DMD [3][4][5]. Moreover, respiratory muscle weakness leads to secondary changes such as decreased lung compliance, ineffective cough with deterioration of airway clearance, and repeated infections [6,7].…”

Section: Introductionmentioning

confidence: 99%

Benefits of Telemonitoring of Pulmonary Function—3-Month Follow-Up of Home Electronic Spirometry in Patients with Duchenne Muscular Dystrophy

Wasilewska

Małgorzewicz

et al. 2022

JCM

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Background: In patients with Duchenne Muscular Dystrophy (DMD), the respiratory system determines the quality and length of life; therefore, the search for easy and safe everyday monitoring of the pulmonary function is currently extremely important, particularly in the COVID-19 pandemic. The aim of the study was to evaluate the influence of a three-month home electronic spirometry (e-spirometry) monitoring of the pulmonary function and strength of respiratory muscles as well as the patients’ benefits from this telemetric program. Methods: Twenty-one boys with DMD (aged 7–22; non-ambulatory-11) received a remote electronic spirometer for home use with a special application dedicated for patients and connected with a doctor platform. Control of the hospital spirometry (forced vital capacity-FVC, forced expiratory volume in 1 second-FEV1, peak expiratory flow-PEF) and respiratory muscle strength (maximal inspiratory-MIP and expiratory pressures-MEP) before and after the three-month monitoring were performed as well telemonitoring benefit survey. Results: A total of 1403 measurements were performed; 15 of the participants were able to achieve correct attempts. There were no differences between the hospital and the home spirometry results as well as between respiratory muscle strength during v1 vs. v2 visits for the whole study group (all parameters p > 0.05); the six participants achieved increased value of FVC during the study period. There was a positive correlation between ΔFVC and the number of assessments during the home spirometry (r = 0.7, p < 0.001). Differences between FVC and MIPcmH2O (r = 0.58; p = 0.01), MEPcmH2O (r = 0.75; p < 0.001) was revealed. The mean general satisfaction rating of the telemonitoring was 4.46/5 (SD 0.66) after one month and 4.91/5 (SD 0.28) after three months. The most reported benefit of the home monitoring was the improvement in breathing (38% of participants after one month, 52% after three months of telemonitoring). Forgetting about the procedures was the most common reason for irregular measurements; the participants reported also increased motivation but less time to perform tests. Conclusions: The study indicates high compliance of the home telemonitoring results with the examination in the hospital. Benefits from home spirometry were visible for all participants; the most important benefit was breathing improvement. The remote home spirometry is usable for everyday monitoring of the pulmonary function in DMD patients as well can be also treated as respiratory muscle training.