Options for Modifying and Improving the Efficiency of the HJHS Scoring System

Accurate but Time-Consuming

Assessing musculoskeletal health is a very important part of comprehensive care for patients with hemophilia. A well-established and frequently used tool for assessing joint health in practice is the Hemophilia Joint Health Score (HJHS).

Assessing the HJHS takes about 45–60 minutes, which some argue is not practical or achievable in practice. Recently, one of the programs for clinical practice has been the effort to develop a shortened version of the HJHS. However, this process must reflect a broad discussion involving not only doctors and nurses but particularly physiotherapists.

Discussion Basis

In the first step, a panel of authors evaluated the possibilities of developing a shortened version of the HJHS that would be easier to use. The work, whose results were published in the journal Haemophilia in July 2020, was based on a multicenter secondary analysis of pooled data from published studies using the HJHS scoring system in individuals with hemophilia A or B aged 4–30 years. The aim was to identify items that are least informative or to reconsider scoring options. An international panel of 19 experts from different specializations evaluated and voted on proposals to modify this scoring system.

Findings

Data from 499 patients from a total of 7 studies were assessed. The median age was 15 years (range 4.0–29.9), 83.2% of patients had severe hemophilia, and 61.5% were receiving regular prophylactic treatment. The median overall HJHS score was 6.0 (interquartile range [IQR] 1.0–17.0), but there was a wide variation in total scores: 0–63. Only 21% of evaluated patients had a total HJHS score of 0. The analysis showed different patterns according to the HJHS score in children versus young adults and in hemophilia patients treated with more or less intensive regimens.

For evaluated items, the authors agreed that “duration of swelling” and “crepitus” were the least informative parameters regarding joint involvement and could therefore be omitted as part of reducing the scope of the scoring system. The so-called HJHS_short scoring system was still able to reflect the diversity in age and treatment regimens. Another possible way to shorten the HJHS assessment, according to the authors, could be selective screening examinations of joints where the full HJHS scoring panel could be applied.

Conclusion

Reducing the HJHS is theoretically possible such that the score retains its informative value. However, several important steps, including subsequent validation, are still necessary to obtain a tool that is truly effective in clinical practice, i.e., as accurate as possible.

(eza)

Source: Kuijlaars I. A. R., van der Net J., Feldman B. M. et al. Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: options for a shorter HJHS. Haemophilia 2020 Oct 15, doi: 10.1111/hae.14180 [Epub ahead of print].