Abstract
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).
Highlights
Neuromuscular disorders include a heterogeneous group of diseases that impair the function of muscles, motor neurons, peripheral nerves, and neuromuscular junctions.Duchenne muscular dystrophy (DMD) is the most common, progressive, irreversible muscular dystrophy, caused by mutations in the X-linked dystrophin gene
14 out of 20 boys enrolled in the study applied and received a home spirometer during visit 1
We showed that almost a third of our patients with DMD performed daily measurements of spirometry at home, whereas in another study with asthmatics, only 13% of patients succeeded every single day over the three-week study period [22]
Summary
Duchenne muscular dystrophy (DMD) is the most common, progressive, irreversible muscular dystrophy, caused by mutations in the X-linked dystrophin gene. Determined and progressive with age, the atrophy of the dystrophin protein in the muscles includes respiratory muscles. 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. Pulmonary function is assessed by spirometry, which should be performed at least once a year, at least every 6 months after the loss of independent walking function, and every months after starting non-invasive ventilation (NIV) [2,5]
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