title: Web-enabled registry of inherited bleeding disorders in Portugal: conditions and perception of the patients.
abstract: Local and national haemophilia registries are powerful instruments to support the healthcare and researchers and improve the communication between Comprehensive Haemophilia Diagnostic and Treatment Centres (HTCs) and patients. Hemo@care is an example of a Local Haemophilia Registry Systems (LHR_Sys) based on the Web, developed in collaboration with a HTC located in Portugal, to support the haemophilia treatments registry, collect and manage the clinical information and provide mechanisms to control the clotting factor concentrates (CFC) stock. To extend this solution (the hemo@care) to other Portuguese HTCs and consequently to meet the preconditions to create a National Haemophilia Registry Systems (NHR_Sys), a study based on a questionnaire was carried out at nationwide. This study aims to assess the conditions and motivations of people with haemophilia (PWH) geographically scattered throughout the country, to use a potential Web-enabled registry with the purpose of replacing the traditional paper-diaries, to understand their judgment about a potential NHR_Sys currently non-existent in Portugal, and at the same time, to characterize demographically and pathologically those people at the nationwide. The results based on the analysis of 168 responses (response rate of 31%) confirmed the high prevalence of the disease in haemophilia A (75%) compared with haemophilia B (11.3%) and a large incidence in the severe levels, or the existence of people with mild severity without diagnosis and treatment. Furthermore, the results also revealed the need, conditions and motivation for using a registry system by PWH; thus it is deemed to justify the extension of the hemo@care to other HTCs in Portugal and consequently to create the NHR_Sys.
authors.affiliation: Department of Economics, Management and Industrial Engineering, University of Aveiro, Aveiro, Portugal. lteixeira@ua.pt
authors.forename: L
authors.initials: L
authors.lastname: Teixeira
authors.forename: C
authors.initials: C
authors.lastname: Ferreira
authors.forename: B S
authors.initials: BS
authors.lastname: Santos
authors.forename: V
authors.initials: V
authors.lastname: Saavedra
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D025861:Blood Coagulation Disorders, Inherited / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D003258:Consumer Behavior; D005260:Female; D006801:Humans; D007223:Infant; D020407:Internet; D008297:Male; D008875:Middle Aged; D009042:Motivation; D011174:Portugal / Q000453:epidemiology; D012042:Registries; D011795:Surveys and Questionnaires; D055815:Young Adult