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New Possibilities for Home E-monitoring of Pulmonary Function in Patients with Duchenne Muscular Dystrophy - ePULMoDMD Project
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927779
ABSTRACT
RATIONALE. Duchenne muscular dystrophy (DMD) is the most common and severe muscular dystrophy recognized in childhood. Genetically determined progressive dystrophin dysfunction in skeletal and multiple organ muscles is fatal before the age of 20 years without multi-specialized care. The most common cause of death is respiratory muscle failure. The primary task in caring for patients with DMD is the early monitoring of pulmonary functions. This has become difficult during the pandemic SARS-CoV-2 as the procedures generate aerosols, i.e. high risk of SARS-CoV-2 virus transmission. The aim of the study was to evaluate the capacity of the patients with DMD to perform longitudinal daily spirometry examinations at home. METHODS. The patients with DMD were recruited from the Rare Disease Centre, Medical University of Gdask, Poland (a member of the TREAT NMD Alliance Neuromuscular Network). Physical examination with anthropometry measurement, Vignos scale (VS), Brooke scale (BS), and Pulmonary Function Tests were measured during 3 months of monitoring. Participants used home spirometry (AioCare System, Healthup), twice every day. The adherence and correctness (separately for FEV1 and FVC) of spirometry examinations were evaluated. RESULTS. We recruited 20 boys with mean age 11.7(3.0) years (range 7-16 years) and BMI 22.3(5.5) kg/m∧2 with a median VS of 2.5 (IQR 6.5) and BS of 2 (IQR 4.0). During 3 months of monitoring 1301 spirometry examinations were performed;32% of them meet ATS/ERS criteria for FEV1 and FVC, 35% for FEV1 and 33% for FVC, however 50% of patients performed examinations with at least one maneuver with correct FVC. The mean adherence considering performance spirometry examinations twice a day was 39(26)% (range 6%-98%). We observed a significant increase of FVC values during the monitoring period in 4/20 of patients based on linear regression model estimation. The study is still ongoing. CONCLUSIONS. The systematic pulmonary function monitoring at home in patients with restrictive diseases is an innovative project. The results showed that patients with DMD are able to monitor the lung function using a mobile spirometry system. We observed that in some cases, based on patient feedback, systematic spirometry tests were a form of pulmonary exercises and could be one of the factors that increase FVC after 3 months of monitoring. Personal spirometers could be used as a part of the therapy of patients with muscular dystrophy.
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Full text: Available Collection: Databases of international organizations Database: EMBASE Language: English Journal: American Journal of Respiratory and Critical Care Medicine Year: 2022 Document Type: Article

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Full text: Available Collection: Databases of international organizations Database: EMBASE Language: English Journal: American Journal of Respiratory and Critical Care Medicine Year: 2022 Document Type: Article