Abstract

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.

Highlights

  • Duchenne Muscular Dystrophy (DMD) is the most common and progressive muscular dystrophy in childhood

  • Upper limb functional status was assessed with the 6-point Brooke scale (BS) (1—the subject can abduct their arms in a full circle until they touch above their head, 6—the subject has no useful function of the hands) [36]

  • We found that all participants reported the benefits of home telemonitoring, and most of them pointed to an improvement in breathing as the most important benefit achieved

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Summary

Introduction

Duchenne Muscular Dystrophy (DMD) is the most common and progressive muscular dystrophy in childhood. The DELOS study, (Duchenne muscular dystrophy long-term idebenone study) showed that idebenone, a short-chain benzoquinone, significantly reduced the loss of pulmonary function over the 52-week study period in a DMD patient cohort not using a concomitant corticosteroid therapy. This treatment is only available in clinical trials [22,23,24]. The aim of the study was to evaluate the influence of the three-month home espirometry monitoring on the pulmonary function and strength of respiratory muscles as well as the patients’ benefits from this telemetric program

Study Design
Participants
Respiratory Muscle Assessment
Functional Status and Hand Grip Strength
Telemonitoring Benefits Survey
Statistical Analysis
Participants ID
Study Group Spirometry
Individual Spirometry
Relationship between FVC and Respiratory Muscles
Regression Analysis
Limitations
Summary
Conclusions
Full Text
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