Physical Activity, Bleedings and Quality of Life in Subjects with Haemophilia A without Inhibitors-A Multicenter, Observational Italian Study with a Wearable Device

Maria Elisa Mancuso; Chiara Biasoli; Renato Marino; Andrea Buzzi; Daniele Preti; Luigi Sannino; Rosaria Tempre; Sara Bendinelli; Elena Pompeo; Giacomo Siri; Giancarlo Castaman; POWER Study Sites Group

doi:10.3390/jcm13113036

Physical Activity, Bleedings and Quality of Life in Subjects with Haemophilia A without Inhibitors-A Multicenter, Observational Italian Study with a Wearable Device

J Clin Med. 2024 May 22;13(11):3036. doi: 10.3390/jcm13113036.

Authors

Affiliations

¹ Center for Thrombosis and Hemorrhagic Diseases, IRCCS Humanitas Research Hospital and Humanitas University, 20089 Milan, Italy.
² Centro Emofilia, Unità Opertaiva Complessa Medicina Trasfusionale, Dipartimento Patologia, Clinica Ospedale M. Bufalini, CESENA, 47521 Cesena, Italy.
³ Ospedale Policlinico, S.S.D. Centro Emofilia e Trombosi, Piazza Giulio Cesare, 70124 Bari, Italy.
⁴ Fondazione Paracelso, 20155 Milan, Italy.
⁵ Federazione Associazioni Emofilici, 20155 Milan, Italy.
⁶ Roche Italia, Viale G. B. Stucchi, 110, 20900 Monza, Italy.
⁷ Alira Health S.r.l., Via Dante 14, 20121 Milan, Italy.
⁸ Center for Bleeding Disorders and Coagulation, Department of Oncology, Careggi University Hospital, 50134 Florence, Italy.

Abstract

Background: This study aimed to gather data on physical activity (PA), bleeding, health-related quality of life, and health status, using a wearable device and an electronic patient-reported outcome (ePRO) app, in individuals with moderate or severe hemophilia A (HA) without inhibitors receiving treatment according to the clinical practice. Methods: This is a 12-month multicenter cohort study conducted in Italy. The primary outcomes included the description of PA by type and intensity, adherence to World Health Organization guidelines, bleeding, and health-related quality of life by EQ-5D questionnaire. PA data were collected continuously through a fitness tracker worn by the patient; all the other variables were collected through ePRO questionnaires. Results: Only 54 of the 103 enrolled subjects (52.4%) used their fitness tracker for the defined valid period; adolescents were the least compliant age group. PA was performed at low rates and intensity. Approximately 52% of the subjects had sedentary behavior. The mean EQ-5D values did not change over time. At least one bleeding was reported in 43.7% of the subjects, mostly with sedentary behavior. The PA in the 2 days preceding the bleeding was comparable to the one observed in the overall observational period. Conclusions: The systematic recording of data through a fitness tracker and ePRO app shows that subjects with HA without inhibitors have lower-than-expected PA and that they still experience issues related to bleeding.

Keywords: activity; bleeding; cohort studies; congenital; factor VIII deficiency; hemophilia A; pain; patient outcome; physical; quality of life.

Grants and funding

The study was fully sponsored by Roche Italia S. p. A. Alira Health was responsible for data collection and performed statistical analyses. The authors thank Andrea Rossi, who provided medical writing support for the preparation of the article. All the activities were funded by Roche, whose employees were fully involved in the study design, in the collection, analysis, and interpretation of data, in the writing of the report, and in the decision to submit the article for publication. All authors had full access to aggregated data (including statistical reports and tables) in the study and can take responsibility for the integrity of the data and the accuracy of the data analysis.