Thromb Haemost 2017; 117(08): 1465-1470
DOI: 10.1160/TH16-12-0967
Coagulation and Fibrinolysis
Schattauer GmbH

Joint assessment in von Willebrand disease

Validation of the Haemophilia Joint Health score and Haemophilia Activities List
Karin P. M. van Galen
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands
,
Merel A. Timmer
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands
,
Piet de Kleijn
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands
,
Kathelijn Fischer
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands
,
Wouter Foppen
2   Department of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands
,
Roger E. G. Schutgens
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands
,
Jeroen Eikenboom
3   Department of Thrombosis and Hemostasis and Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, Leiden, The Netherlands
,
Karina Meijer
4   Department of Hematology, University of Groningen, University Medical Center Groningen, The Netherlands
,
Marjon H. Cnossen
5   Department of Pediatric Hematology, Erasmus University Medical Center-Sophia Children’s Hospital, Rotterdam, The Netherlands
,
Karin Fijnvandraat
6   Department of Pediatric Hematology, Academisch Medisch Centrum, Emma children’s hospital, Amsterdam, The Netherlands
,
Johanna G. van der Bom
7   Jon J van Rood Center for Clinical Transfusion Medicine, Sanquin Research, Leiden, The Netherlands
,
Britta A. P. Larosvan Gorkom
8   Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands
9   Department of Hematology, Radboud university medical center, Nijmegen, The Netherlands
,
Frank W. G. Leebeek
10   Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands
,
Eveline P. Mauser-Bunschoten
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands
,
on behalf of the Win study group › Author Affiliations
Financial support: This research was supported by an unrestricted grant from CLS Behring and the Dutch Hemophilia Foundation.
Further Information

Publication History

Received: 28 December 2016

Accepted after major revision: 10 April 2017

Publication Date:
22 November 2017 (online)

Summary

Assessment of clinical outcome after joint bleeding is essential to identify joint damage and optimise treatment, to prevent disability. However, disease-specific tools to assess the musculoskeletal status in patients with von Willebrand disease (VWD) are lacking. We aimed to determine validity and reliability of the Haemophilia Joint Health Score (HJHS) and Haemophilia Activities List (HAL) in patients with Von Willebrand disease (VWD). Ninety-six patients with VWD were included (mean age 46 years) of whom 27 had more than five documented joint bleeds. The HJHS was performed in all patients and all patients completed the HAL and Impact on Participation and Autonomy (IPA) questionnaires. Health-related quality of life (SF36) results were obtained from the prior ‘Willebrand in the Netherlands’ study. Joint X-rays of knees, elbows and ankles were scored according to Pettersson (PS). Internal consistency of the HJHS (Cronbach’s α (α)=0.75) and HAL (α=0.89) were good. Inter-observer agreement of the HJHS was good (ICC 0.84; Limits of Agreement ± 10.3). The HJHS showed acceptable correlation with the X-ray PS (Spearman’s r (rs)>0.60 all joints) and HAL (rs=0.71). The HAL also showed acceptable correlation with the SF36 physical functioning (rs=0.65) and IPA (rs=0.69). Hypothesis testing showed adequate discriminative power of both instruments: in patients with a history of >5 versus ≤ 5 joint bleeds (median HJHS 10 vs 2 (p<0.01); median HAL 77 vs 98 (p<0.01)), independent from age. In conclusion, both the HJHS and HAL are feasible to assess clinical outcome after joint bleeds in VWD.

 
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