Abstract

Patients at risk of developing respiratory dysfunctions, such as patients with severe forms of muscular dystrophy, need a careful respiratory assessment, and periodic follow-up visits to monitor the progression of the disease. In these patients, at-home continuous monitoring of respiratory activity patterns could provide additional understanding about disease progression, allowing prompt clinical intervention. The core aim of the present study is thus to investigate the feasibility of using an innovative wearable device for respiratory monitoring, particularly breathing frequency variation assessment, in patients with muscular dystrophy. A comparison of measurements of breathing frequency with gold standard methods showed that the device based on the inertial measurement units (IMU-based device) provided optimal results in terms of accuracy errors, correlation, and agreement. Participants positively evaluated the device for ease of use, comfort, usability, and wearability. Moreover, preliminary results confirmed that breathing frequency is a valuable breathing parameter to monitor, at the clinic and at home, because it strongly correlates with the main indexes of respiratory function.

Highlights

  • In the severe forms of muscular dystrophy, such as Duchenne Muscular Dystrophy (DMD), respiratory failure is still the principal cause of death, followed by cardiomyopathy

  • The dataset was divided into two groups: patients with DMD and patients with limb girdle muscular dystrophy (LGMD)

  • Given the ad-hoc nature of this questionnaire, there is no available literature to evaluate the mean score; we considered the scores item-by-item

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Summary

Introduction

In the severe forms of muscular dystrophy, such as Duchenne Muscular Dystrophy (DMD), respiratory failure is still the principal cause of death, followed by cardiomyopathy. Ineffective coughing, and reduced ventilation often leads to pneumonia, atelectasis, and respiratory insufficiency during sleep and while awake [1]. Periodic measurement of respiratory function and respiratory muscle strength allow the clinician to predict when to introduce assisted coughing and ventilation. Recommended respiratory evaluation includes measurement of oxyhemoglobin saturation, spirometric parameters, maximum inspiratory and expiratory pressures, and peak cough flow once or twice per year. These patients need a careful respiratory assessment, and periodic follow-up visits to monitor the progression of the disease

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