The Hemophilia Joint Health Score version 2.1 Validation in Adult Patients Study: A multicenter international study

Abstract

Background The Hemophilia Joint Health Score (HJHS) was developed and validated to detect arthropathy in children. Additional evidence is required to show validity in adults. We studied the convergent and discriminant construct validity of the HJHS version 2.1(HJHSv2.1) in adults with hemophilia. A secondary aim was to define age‐related normative adult HJHSv2.1 reference values. Methods We studied 192 adults with hemophilia, and 120 healthy adults in four age‐matched groups—18 to 29, 30 to 40, 41 to 50, and >50 years—at nine centers. Trained physiotherapists scored the HJHS and World Federation of Hemophilia (WFH) joint score. Health history, the Functional Independence Scale of Hemophilia (FISH), Hemophilia Activities List (HAL), and Short‐Form McGill Pain Questionnaire (SF‐MPQ) were also collected. Results The median age was 35.0 years. Of participants with hemophilia, 68% had severe, 14% moderate, and 18% mild disease. The HJHS correlated strongly with WFH score (Spearman’s rho [rs] = .95, P < .001). Moderate correlations were seen between the FISH (rs = .50, P < .001) and SF‐MPQ Present Pain Intensity (rs = .50, P < .001), while a modest correlation was found with the HAL (rs = −.37, P < .001). The HJHS significantly differentiated between age groups (Kruskal‐Wallis T = 35.02, P < .001) and disease severity in participants with hemophilia. The HJHS had high internal reliability (Cronbach’s α = .88). We identified duration of swelling as a redundant item in the HJHS. Conclusions The HJHS shows evidence of strong convergent and discriminant construct validity to detect arthropathy in adults with hemophilia and is well suited for use in this population.

Hemophilic Arthropathy: Barriers to Early Diagnosis and Management

“…This scale was designed to assess joint deterioration in pediatric patients with hemophilia, 40 although a study has recently been published that shows its validity in the adult population. 41 This measuring instrument measures 8 items: Swelling, Duration of swelling, Muscle Atrophy and Strength, Crepitus, Mobility and Joint Pain. The scoring range of this additive scale is 0-20 points in each joint.…”

LongHest project: A prospective, observational study of extended half‐life treatment in the musculoskeletal health of patients with severe haemophilia A

“….88). 17 The scale measures eight items: swelling, duration of swelling, atrophy and muscle strength, crepitus, mobility and joint pain. The results of this scale express the degree of joint deterioration in patients with haemophilia ranging from 0 (joint without degenerative injury) to 20 (maximum joint damage) points.…”

Adaptación cultural del cuestionario PedHAL (pediatric haemophilia activities list), Cali, Colombia (Cultural adaptation of the PedHAL (pediatric hemophilia activity list), Cali, Colombia)

“…Estas escalas están en su mayoría diseñadas bajo el modelo de la Clasificación Internacional del Funcionamiento, la Discapacidad y la Salud, de la Organización Mundial de la Salud (CIF) (Echeverría et al, 2021). Dentro de estas escalas se encuentran: Hemophilia Joint Health Score (HJHS) (Hilliard et al, 2006;St-Louis et al, 2022), Gilbert Score (Fischer & de Kleijn, 2013), Haemophilia Activities List (HAL) (van Genderen et al, 2004;Van Genderen et al, 2006), Pediatric Haemophilia Activities List (PedHAL) (Groen et al, 2010), Functional Independence Score in Hemophilia (FISH) (Poonnoose et al, 2005;Timmer et al, 2018). Estas herramientas presentan datos de confiabilidad y validez para población de países en los cuales la oferta de tratamiento y las condiciones económicas para la adquisición de tratamiento de última generación es distinta, no se conocen datos de validez para países latinoamericanos (Gouw et al, 2019).…”