Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS

• Authors: Isolde A.R. Kuijlaars, Utrecht University; Janjaap van der Net, Universitair Medisch Centrum Utrecht; Brian M. Feldman, Hospital for Sick Children; Magnus Aspdahl, Karolinska University Hospital; Melanie Bladen, Great Ormond Street Hospital for Children NHS Foundation Trust; Wypke de Boer, University of Amsterdam; Rubén Cuesta-Barriuso, European University of Madrid; Ruth E.D. Matlary, Oslo University Hospital; Sharon M. Funk, University of Colorado; Pamela Hilliard, Hospital for Sick Children; Judy A. John, Christian Medical College & Hospital; Christine Kempton, Emory University; Piet de Kleijn, Utrecht University; Marilyn Manco-Johnson, University of Colorado; Pia Petrini, Karolinska University Hospital; Pradeep Poonnoose, Christian Medical College & Hospital; Jean St-Louis, CHU St Justine; Sylvia Thomas, Federal University of Rio de Janeiro; Merel A. Timmer, Utrecht University; Sonata Saulyte Trakymiene, Vilnius University; Leo van Vlimmeren, Radboud University Nijmegen; Kathelijn Fischer, Utrecht University
• Journal Title: Haemophilia
• Volume: Volume 26, Number 6
• Publisher: Wiley | 2020-10-15, Pages 1072-1080
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.1111/hae.14180
• Subjects: Health Sciences, Human Development; Health Sciences, Health Care Management; Health Sciences, Rehabilitation and Therapy; Health Sciences, Radiology
• Keywords: Science & Technology; Life Sciences & Biomedicine; Hematology; expert opinion; haemophilia; joints; outcome measures; physical examination; Prophylaxis

Abstract

Introduction: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption. Aim: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion. Methods: A cross-sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0-124, optimum score 0) in persons with haemophilia A/B aged 4-30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%). Results: Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0-29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0-17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction. Conclusion: Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHSshort, which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.