Generation and optimization of the self‐administered bleeding assessment tool and its validation as a screening test for von Willebrand disease

Abstract

Summary Introduction/Aim Our aim was to generate, optimize and validate a self-administered bleeding assessment tool (BAT) for von Willebrand disease (VWD). Methods In Phase 1, medical terminology in the expert-administered ISTH-BAT was converted to 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 was determined and test-retest reliability analyzed. In Phase 3, the optimized Self-BAT was tested as a screening tool for first time referrals to the Hematology clinic. Results BS 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 bleeding scores 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 analyzed; sensitivity of 100%, specificity of 21%, PPV=0.17 and NPV=1.0. Conclusion 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.

The diagnostic accuracy of bleeding assessment tools for the identification of patients with mild bleeding disorders: A systematic review

“…Many studies investigating the BAT claim that a MBD can be excluded with a negative BAT and that the greatest clinical utility lies in the high negative predictive value of the BAT and its ability to exclude subjects in which further testing may be unnecessary . Our findings do not support this claim.…”

Fifth Åland Island conference on von Willebrand disease

“…In 2010, the International Society on Thrombosis and Haemostasis (ISTH) proposed a single BAT to standardize reporting of bleeding symptoms for use in adult and paediatric populations . In addition, a self‐administered BAT based on the ISTH‐BAT has been developed …”

A diagnostic approach to mild bleeding disorders

“…We have previously used a self‐administered MCMDM‐1 VWD BS in a research setting, which gave similar results to the expert‐administered version . A self‐administered version of the ISTH‐BAT called the self‐BAT seems to be comparable to the expert‐administered ISTH‐BAT, particularly in women being assessed for von Willebrand disease, although relatively few patients were included in this study .…”