title: Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease.
abstract: OBJECTIVE
  Our aim was to generate, optimize and validate a self-administered bleeding assessment tool (self-BAT) for von Willebrand disease (VWD).
  
  
  METHODS
  In Phase 1, medical terminology in the expert-administered International Society on Thrombosis and Haemostasis (ISTH)-BAT was converted into a Grade 4 reading level to produce the first version of the Self-BAT which was then optimized to ensure agreement with the ISTH-BAT. In Phase 2, the normal range of bleeding scores (BSs) was determined and test-retest reliability analysed. In Phase 3, the optimized Self-BAT was tested as a screening tool for first time referrals to the Haematology clinic.
  
  
  RESULTS
  Bleeding score from the final optimized version of the Self-BAT showed an excellent intra-class correlation coefficient (ICC) of 0.87 with ISTH-BAT BS in Phase 1. In Phase 2, the normal range of BSs for the optimized Self-BAT was determined to be 0 to +5 for females and 0 to +3 for males and excellent test-retest reliability was shown (ICC = 0.95). In Phase 3, we showed that a positive Self-BAT BS (≥6 for females, ≥4 for males) has a sensitivity of 78%, specificity of 23%, positive predictive value (PPV) of 0.15 and negative predictive value (NPV) of 0.86 for VWD; these figures improved when just the females were analysed; sensitivity of 100%, specificity of 21%, PPV = 0.17 and NPV = 1.0.
  
  
  CONCLUSIONS
  We show an optimized Self-BAT can generate comparable BS to the expert-administered ISTH-BAT and is a reliable, effective screening tool to incorporate into the assessment of individuals, particularly women, referred for a possible bleeding disorder.
authors.affiliation: Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada.
authors.forename: M
authors.initials: M
authors.lastname: Deforest
authors.affiliation: Department of Medicine, Queen's University, Kingston, ON, Canada.
authors.forename: J
authors.initials: J
authors.lastname: Grabell
authors.affiliation: Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada.
authors.forename: S
authors.initials: S
authors.lastname: Albert
authors.affiliation: Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada.
authors.forename: J
authors.initials: J
authors.lastname: Young
authors.affiliation: Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada.
authors.forename: A
authors.initials: A
authors.lastname: Tuttle
authors.affiliation: Clinical Research Centre, Kingston General Hospital, Queen's University, Kingston, ON, Canada.|Department of Public Health Sciences, Queen's University, Kingston, ON, Canada.
authors.forename: W M
authors.initials: WM
authors.lastname: Hopman
authors.affiliation: Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada.|Department of Medicine, Queen's University, Kingston, ON, Canada.
authors.forename: P D
authors.initials: PD
authors.lastname: James
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D003710:Demography; D005260:Female; D006470:Hemorrhage / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D015203:Reproducibility of Results; D012646:Self Administration; D055815:Young Adult; D014842:von Willebrand Diseases / Q000175:diagnosis*
keywords: bleeding assessment tools; inherited bleeding disorders; symptom assessment; von Willebrand disease