Hemophilia Joint Health Score (HJHS)

Description

The HJHS measures joint health, in the domain of body structure and function (i.e. impairment), of the joints most commonly affected by bleeding in hemophilia: the knees, ankles, and elbows.

It is primarily designed for children with hemophilia aged 4-18 years with mild joint impairment (e.g. treated with prophylaxis). It can be used when there is a need for orthopedic intervention, or as an outcome measure of physiotherapy interventions.

Contact person(s):
International Prophylaxis Study Group, [email protected]

Date of this review:
February 07, 2011

Utility

Overall utility:
The HJHS is a physical examination assessment tool sensitive enough to pick up the subtle early signs of joint damage. It is appropriate for monitoring joint change over time or assessing efficacy of treatment regimens in children receiving both prophylactic and on-demand therapy.

Limitations:
The HJHS takes a long time to administer. It has not yet been adequately studied for use in adults, more severe joint disease, or in very young children.

Administration

1. Time to complete: Approximately 90 minutes, including computing and recording onto score sheet. Less time may be required with increased experience using the tool.
2. Equipment/space required:
   • plinth/bed
   • room with a sufficient source of lighting
   • goniometer (1⁰ gradations are recommended)
   • floor with a smooth firm surface
   • space to assess gait (walking, running)
   • set of stairs (nearby)

3. Training required: Instruction manual, worksheets, score sheet, addendums and video are available on www.ipsg.ca. The instruction manual is available in English, French (France), Spanish (Spain), and German (Germany).

   Please contact [email protected] if you do not receive an approval email within 48 hours of submitting your registration.

   NOTE: It is recommended that you add [email protected] to your safe list.

4. Cost: None
5. Scoring/scaling/interpretation of results: The HJHS 2.1 provides a total score (higher score is worse; max=124), joint specific scores, and a global gait score.

Psychometrics

Psychometric properties:

1. Construct validity: The HJHS 1.0 shows good ability to discriminate between known groups of patients, for example:
   • severe vs. moderate vs. mild hemophilia
   • treated with prophylaxis vs. not treated with prophylaxis

   It correlates appropriately with bleeding rates (r_s=0.50) and physician global assessment of joint health (r_s=0.42). It correlates well with radiographic changes.

2. Criterion validity: The HJHS 1.0 compares favorably with the World Federation of Hemophilia Physical Examination Score when discriminating known groups (63% to 97% more efficient).
3. Reliability: Test-retest (ICC=0.89) and inter-observer (ICC=0.83) reliability are excellent, as is internal reliability (Cronbach’s alpha=0.86).
4. Responsiveness/sensitivity has not been tested longitudinally.

The validation of the HJHS version 1.0 led to the development of the HJHS 2.0 and 2.1. The HJHS 2.1 instruction manual includes a complete description of the differences between the versions of the tool.

Groups tested with this measure:

• North American and European children with well-treated hemophilia A and B (mild, moderate, and severe factor deficiency)
• Chinese boys with moderate to severe arthropathy

Glossary of Psychometric Properties

References

1. 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.
2. Hilliard P, Funk S, Zourikian N. Bergstrom BM, Bradley CS, McLimont M, Manco-Johnson M, Petrini P, Van Den Berg M, Feldman BM. Hemophilia joint health score reliability study. Haemophilia 2006; 12(5):pp 518-525.
3. Feldman BM. Funk S, Hilliard P, Van Der Net J, Zourikian N, Berstrom B-M, Engelbert RHH, Abad A, Petrini P, Manco-Johnson M, and On behalf of the International Prophylaxis Study Group. The Haemophilia Joint Health Score (HJHS) International Validation Study. XXVIIIth International Congress of the World Federation of Hemophilia, Istanbul, Turkey. Haemophilia 2008; 14 (Suppl. 2):pp 83.
4. Hilliard P, Blanchette VS, Doria A, Blanchette C, Hang M, Feldman BM. The Hemophilia Joint Health Score (HJHS) correlates highly with radiographic damage. XXVIIIth International Congress of the World Federation of Hemophilia, Istanbul, Turkey. Haemophilia 2008; 14 (Suppl. 2):pp 80.