Reviewing HJHS as a tool to assess outcome measures in patients with haemophilia A in Australia

Abstract

Background : It is widely recommended that management of patients with haemophilia include the use of standardised joint assessment tools. The tool incorporated into the Australian Bleeding Disorders Registry (ABDR) is the UK MOD-HJHS, a validated but time-intensive tool of high sensitivity. Aims : To characterise clinician practices regarding the use of HJHS in routine assessment of patients with Haemophilia A (HA) in Australia; to identify potential barriers to HJHS tool usage via ABDR; and to examine relationships between HJHS score in individual patients and other treatment and outcome measures. Methods : Demographic data was derived from the ABDR for HA patients from whom consent had been obtained. Data was obtained on severity, treatment regimen, product usage, inhibitor status and age for patients with at least one HJHS recorded. Results : There were 547 HA patients (368 severe) with 1169 HJHS in the ABDR. 53.5% (368/687) of severe HA patients in Australia had at least one HJHS. 36.7% (429/1169) of HJHS assessments were performed in a paediatric setting, median baseline age 11years (8-14). Median baseline HJHS of severe HA patients on prophylaxis for at least 6months was 1 (0-3) for paediatric and 11 (3-28) for adult patients. 293 HA patients prescribed prophylaxis had more than one HJHS with median observation time of 14months (11-25) and median delta HJHS 0 (-2,2). Results demonstrated that high titre inhibitor, non-adherence and joint bleeds/surgery had significant impact on longitudinal HJHS. All HJHS were performed by physiotherapists (around 55% in clinics, 45% in outpatient/outreach settings). HJHS was usually recorded on paper at assessment and then later transcribed into ABDR. Conclusions : This study provided the first baseline assessment of joint health data for HA patients in Australia, and an initial understanding of how individual and treatment factors may influence long-term joint outcomes. It also provided insights into practical limitations of the HJHS tool.L6364069412021-11-12 <br />