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Joint Scoring using the Haemophilia Joint Health Score System


Table 1: Studies that Have Used the Haemophilia Joint Health Score for Assessing Musculoskeletal Status in Patients with Haemophilia


Authors, Year Hilliard et al., 20068 Chen et al., 200852


Engelbert et al., 200850


Trakymiene et al., 201053


Christoforidis at al., 201151


Bladen et al., 201054


Feldman et al., 201049


Number of Patients


8 20 47 20 26 39 226 Type of Haemophilia Severe


Severe/moderate/ unknown: 5/13/2


Severe/moderate/mild: 21/7/19 Severe


Severe/moderate Severe Severe/moderate/mild Treatment Regimen Prophylaxis


On-demand/sporadic/ none


Prophylaxis/on-demand: 21/26


On-demand Prophylaxis Primary prophylaxis Prophylaxis/on-demand Mean Age (Range) Median/Mean HJHS 8.7 (4–12) NS (5–17) NS 11.5 (4–17.2) 12.08 (NS) 10 (4–18) 10.8


15 (First day) 14 (Second day) 12


NS (0–6) 24.5


11.6 (<10 years of age) 31.5 ( ≥10 years of age) 8.27 (severe)


4.83 (moderate)


1 (4–6 years of age) 4 (14–18 years of age) 5 (in severe with primary


prophylaxis) 9 (in severe with secondary prophylaxis) 11.5 (in severe with on-demand therapy)


HJHS = Haemophilia Joint Health Score; NS = not specified.


Until recently, most long-term musculoskeletal outcome studies in haemophilia have used clinical and radiological scores to determine the efficacy of different treatment regimens. Physical joint assessment, being readily available and inexpensive, is commonly used to measure structural and functional joint damage. The physical status of the musculoskeletal system can be judged by quantifying the extent of musculoskeletal damage.


Several systems have been adopted in the past as instruments for musculoskeletal assessment in children and adults. The earliest reported and most widely used instrument for the assessment of haemophilic arthropathy is the World Federation of Hemophilia (WFH) Physical Examination (PE) scale, which was developed in the early 1980s for use in persons with haemophilia worldwide, primarily in the evaluation of adults.31


This instrument was developed in the


However, with the introduction of prophylaxis and heightened interest in preserving joints and preventing complications related to recurrent haemarthroses, the WFH PE scale was found to lack sensitivity in detecting the earliest signs of joint disease and to be inadequate for the evaluation of joints in children.17,35,36


has allowed the natural history of haemophilia to be documented and has determined the progressive improvement in outcomes that has been achieved with comprehensive haemophilia care.17,32


The WFH


instrument contains many tasks that cannot be performed by young children owing to their developmental immaturity, and was not designed to detect changes that are part of normal physiological musculoskeletal development in healthy young children.37–39 Furthermore, the score does not assess muscle strength – an important function that affects physical activity.


To address these inadequacies, the system was repeatedly modified from 1993 onwards by adding analysis of gait and strength, among


EUROPEAN ONCOLOGY & HAEMATOLOGY


pre-prophylaxis era, at a time when much lower quantities of replacement therapy were available for the management of haemophilia patients. Severe joint disease was to be expected in haemophilia patients at that time. The global application of this tool29,32–34


other factors, in US and Canadian haemophilia centres.35,40,41 Additionally, in the late 1990s Swedish researchers implemented similar revisions to increase the sensitivity of the WFH PE tool.42


Eventually, the


WFH scale with its various modifications was judged inadequate for evaluating the results of prophylaxis. In addition, the WFH PE tool lacks documented reliability and validity.43


Subsequently, progress has been


accomplished through work delivered by multinational expert groups reviewing the existing joint health scoring systems to produce consensus on the most relevant items to be adopted internationally.8,40 The Physiotherapy Expert Working Group of the International Prophylaxis Study Group (IPSG) was formed by a group of interested expert clinicians and scientists,44


who combined and harmonised


existing joint health scores (WFH, Colorado and Stockholm) to develop a more sensitive tool. In 2003, a new international consensus tool, the Haemophilia Joint Health Score (HJHS) version 1.0, was announced. The aim was to produce a score that would be sensitive to early change, accounting for normal development in children and reliable, valid and practical to administer. It aimed to provide an international scoring instrument for children with haemophilia as a measure of joint health in order to evaluate and monitor the effectiveness of haemophilia treatment.8,35,36,44–46


Reliability and Validity of the Haemophilia Joint Health Score


The HJHS is an 11-item scoring tool for assessing joint impairment in children four to 18 years of age.8


The tool focuses on the six joints most


affected by haemophilia: ankles, knees and elbows. Each of the six index joints is assessed individually on different items and numerically scored in categories of severity. A global gait score (walking, stairs, running and hopping on one leg) is assessed separately. The total joint score (the sum of the six joint scores) and the global gait score, when combined, provide an overall total score ranging from zero to 124 in version 2.0 (148 in version 1.0), where a score of zero corresponds to no identifiable joint impairment. The new scale was piloted during a meeting in September 2003. Four physiotherapists from Canada, the US and Sweden conducted an initial reliability study of the HJHS


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