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The Hemophilia Joint Health Score version 2.1 Validation in Adult Patients Study: A multicenter international study
Jean St-Louis1,2, Audrey Abad3, Sharon Funk4
1Department of Hematology CHU Sainte-Justine and Hôpital Maisonneuve-Rosemont Montréal Québec Canada.
Research and Practice in Thrombosis and Haemostasis
|March 31, 2022
Summary
The Hemophilia Joint Health Score (HJHS) effectively detects joint disease in adults with hemophilia, showing strong validity and reliability for this population. This score is suitable for clinical use in adult hemophilia patients.
Area of Science:
- Rheumatology
- Hematology
- Orthopedics
Background:
- The Hemophilia Joint Health Score (HJHS) was validated for pediatric use.
- Evidence for its validity in adult populations with hemophilia is limited.
- Adult hemophilia patients require reliable tools to assess joint health and arthropathy.
Purpose of the Study:
- To evaluate the convergent and discriminant construct validity of the Hemophilia Joint Health Score version 2.1 (HJHSv2.1) in adults with hemophilia.
- To establish age-related normative reference values for the adult HJHSv2.1.
- To confirm the HJHS's utility in assessing arthropathy in adult hemophilia patients.
Main Methods:
- A cross-sectional study involving 192 adults with hemophilia and 120 healthy controls across four age groups.
- Data collection included the HJHS, World Federation of Hemophilia (WFH) joint score, Functional Independence Scale of Hemophilia (FISH), Hemophilia Activities List (HAL), and Short-Form McGill Pain Questionnaire (SF-MPQ).
- Trained physiotherapists administered the HJHS and WFH scores, with statistical analyses to assess construct validity and reliability.
Main Results:
- The HJHS demonstrated strong correlation with the WFH score (r=0.95, P<.001).
- Moderate correlations were observed with FISH (r=0.50, P<.001) and SF-MPQ Present Pain Intensity (r=0.50, P<.001), and a modest correlation with HAL (r=-0.37, P<.001).
- The HJHS reliably differentiated between age groups and disease severity, with high internal reliability (Cronbach's α=0.88). Duration of swelling was identified as a redundant item.
Conclusions:
- The HJHSv2.1 exhibits strong convergent and discriminant construct validity for detecting arthropathy in adults with hemophilia.
- The HJHS is well-suited for use in adult hemophilia populations for assessing joint health.
- The findings support the HJHS as a valuable tool in the management of adult hemophilia patients.
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