Hemophilia A and B are rare bleeding disorders due to coagulation factor VIII (FVIII) and FIX deficiency, respectively. Repeated joint bleeding (hemarthrosis) leads to chronic arthropathy, disability, and early prosthetic surgery. Indeed, bleeding can be prevented by regular prophylactic treatment, although adherence is often suboptimal, in particular in young adults. Annual bleeding rate (ABR) has been traditionally used as an outcome measure of treatment efficacy, although it is a patient-reported outcome and there is evidence that patients often report as bleeding events also joint pain episodes due to chronic arthropathy. In addition, bleeding may even occur in asymptomatic joints. Therefore, a definite diagnosis of hemarthrosis is pivotal and a personalized management is warranted to avoid overtreatment or undertreatment. To date, a definite diagnosis of hemarthrosis can be confirmed by ultrasound or arthrocentesis at the Hemophilia Treatment Center (HTC).

The World Federation of Hemophilia acknowledges the importance of software-based diaries for treatment adherence and communication with the HTC. Electronic diaries may increase the quantity of information provided by the patients, while improving the data quality. The huge amount of data collected, properly integrated and made available through analytical and visual dashboards, may support clinicians in personalizing treatment.

On this background, we will develop a home-based integrated system for patient adherence to treatment by active assistance with reminders and collection of data on spontaneous and post-traumatic bleeding events, quality of life, productivity and pain through the use of a mobile application and wearable sensors to monitor physical activity. In a subgroup of patients, an exploratory study will be conducted with a portable wireless ultrasound probe compatible with smartphones and tablets. A user-friendly mobile app will guide the patient in the collection of images that will be transferred to the HTC. A computer-assisted diagnosis (CAD) tool prototyped by our research team will support physicians in providing the patient a quick response. Aim of the TEMPO study is to investigate the feasibility, acceptability and sustainability of such telemedicine system and to evaluate its efficacy in improving adherence to treatment; to explore the feasibility of integrating patient-based joint ultrasound assessment; and to pave the way for a personalized management in contrast with a “one-size-fits all” model. The ultimate goal is to improve patients’ quality of life, while also reducing disease burden. Moreover, we will explore beyond state of the art approaches to further improve the efficacy of at home sensors and devices in tackling spontaneous or traumatic bleeding.