History

Initial development of the Hemophilia Joint Health Score (HJHS) began in 2002), by experienced physiotherapists and physicians from Denver, Montreal, Stockholm, Toronto, and Utrecht through the International Prophylaxis Study Group (IPSG).  The goal of this collaborative initiative was to develop a single unified scale to standardize the methods and tools used for joint evaluation in persons with hemophilia.  Three scoring systems served as the basis for this evaluation tool.

The earliest scoring system, known as the Gilbert Scale or the World Federation of Hemophilia (WFH) Orthopaedic Joint Score or the Gilbert Scale, dated back to the early 1980s and was developed by a group of orthopedists from Germany, Israel, Venezuela, England and the USA (Gilbert M et al, Seminars in Hematology, 1993).    It proved insensitive to the earliest signs of joint disease in young boys with hemophilia, and psychometric properties of the scale were never established.

In 1994, in an effort to address identification of joint disease in young children, the hemophilia center at the University of Colorado, modified the WFH scale to make it more sensitive to identifying early joint disease in persons with hemophilia.  This modified scale, known as the Colorado Young Child scale, was used in the Joint Outcome Study, a multi-center, prospective, randomized clinical trial which compared joint outcome in children on prophylactic factor infusion with those who received enhanced episodic therapy.  (Manco-Johnson, NEJM, 2007). This tool was also used in the Canadian Hemophilia Escalating-Dose Prophylaxis Study (CHPS).  (Feldman, Lancet Haematol, 2018).

In the late 1990s, further modifications of this scale were made by the hemophilia center at Karolinska University Hospital in Stockholm, Sweden. (Hill, Haemophilia, 2003). This modification was used by the European Paediatric Network (PedNet) for Haemophilia Management in a study which assessed joint outcome in 116 children, comparing the scores to the WFH scale.  .

Strengths and weaknesses of both the Denver, Stockholm, and WFH Orthopaedic scales were identified and a tentative merged system was developed.  This scale became known as the Hemophilia Joint Health Score.  Studies were initiated to establish reliability (2003) and validity (2006-2007).  Reliability of the HJHS was excellent with an interrater coefficient of 0.83 and test-retest reliability of 0.89.  (Hilliard, Haemophilia 2006)  The HJHS was shown to be 97% more efficient than the WFH scale at differentiating severe from mild and moderate hemophilia, and 74% more efficient at differentiating subjects treated with prophylaxis from those treated on demand. It correlated moderately with a physician global joint score (rs = 0.42, P < 0.0001) and with overall arthropathy impact (rs  = 0.42, P < 0.0001). The HJHS and WFH scales both show evidence of strong construct validity, based on their moderate correlation with the physician arthropathy impact score, the physician’s individual joint score and the physician total score. However, the HJHS has been shown to be more sensitive for evaluating mild arthropathy.  (Feldman, Arthritis Care & Research, 2011).

A validation study of the use of the HJHS in adults was performed on the HJHS version 2.1 in 2017.  The HJHS was found to be a valid joint assessment tool with high internal reliability in the adult hemophilia population. It significantly discriminated between age groups and disease severity confirming its discriminant construct validity. The HJHS total score was also significantly greater in adults with hemophilia compared to healthy men without hemophilia. The HJHS showed excellent correlation with the WFH scale and it had moderate correlation with the SF-MPQ Pain Scale and the Functional Independence Score in Hemophilia (FISH), establishing convergent construct validity. (St. Louis, Research & Practice in Thrombosis & Haemostasis 2022)

The HJHS 2.1 is available in 10 languages, and  has now been studied in multiple language versions in both academic and commercial clinical trials. Work to improve and refine the HJHS remains active.

Important Statements

The HJHS has been tested in its integral, complete version. The developers strongly recommend that the tool be used in its entirety as outlined in the current instruction manual. The HJHS is a copyrighted and licensed tool and any alterations or modifications are prohibited.

Inclusion of the HJHS summary score sheet in an electronic database is acceptable. However, we do not recommend the use of an automated scoring system due to the complexity of specific HJHS items such as Range of motion and Non-evaluable. Inaccurate item scores can alter both individual joint and overall scores.

References

  1. Gilbert MS. Prophylaxis: musculoskeletal evaluation. Semin Hematol. 1993 Jul;30(3 Suppl 2):3-6.
  2. Manco-Johnson MJ, Nuss R, Funk S, Murphy J. Joint evaluation instruments for children and adults with haemophilia. Haemophilia. 2000 Nov;6(6):649-57.
  3. Hill F, Ljung R. Meeting Report – third & fourth workshops of the European Paediatric Network for Haemophilia Management. Haemophilia. 2003; 9:223-8.
  4. Feldman BM, Pai M, Rivard GE, Israels S, Poon MC, Demers C, et al. Tailored prophylaxis in severe hemophilia A: interim results from the first 5 years of the Canadian Hemophilia Primary Prophylaxis Study. Journal of Thrombosis & Haemostasis. 2006 Jun; 4(6):1228-36.
  5. Hilliard P, Funk S, Zourikian N, Bergstrom BM, Bradley CS, McLimont M, et al. Hemophilia joint health score reliability study. Haemophilia. 2006 Sep; 12(5):518-25.
  6. Feldman BM, Funk S, Bergstrom B-M, Zourikian N, Hilliard P, van der Net J, Engelbert RHH, Petrini P, van den Berg M , Manco-Johnson M, Rivard GE, Abad A, and Blanchette VS. Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the Hemophilia Joint Health Score (HJHS).  Arthritis Care & Research 2011 Feb; 63 (2):223-30.
  7. St-Louis J, Abad A, Funk S, Tilak M, Classey S, Zourikian N, McLaughlin P, Lobet S, Hernandez G, Akins S, Wells AJ, Manco-Johnson M, John J, Austin S, Chowdary P, Hermans C, Nugent D, Bakeer N, Mangles S, Hilliard P, Blanchette VS, Feldman BM. The Hemophilia Joint Health Score version 2.1 Validation in Adult Patients Study: A multicenter international study. Res Pract Thromb Haemost. 2022 Mar 25;6(2):e12690. doi: 10.1002/rth2.12690. PMID: 35356667; PMCID: PMC8956786.