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Protocol - Hemophilia Joint Health Score

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Description:

The Hemophilia Joint Health Score (HJHS) 2.1 is a physiotherapist-administered assessment that measures the impact of bleeding on the elbows, knees, and ankles. The HJHS requires approximately 45-60 minutes to complete.

Specific Instructions:

Although the Hemophilia Joint Health Score (HJHS) 2.1 has not yet been validated for adults, the guidance from the PhenX Hemophilia Inhibitor Research Working Group is that it is also appropriate for use in adult populations.

Protocol:

Summary of the Hemophilia Joint Health Score (HJHS) 2.1

The Hemophilia Joint Health Score (HJHS) 2.1 is a physiotherapist-administered assessment that takes approximately 45-60 minutes to complete. The HJHS measures the impact of recurrent bleeding on the elbows, knees, and ankles across 11 symptoms: swelling, duration of swelling, muscle atrophy, axial alignment, crepitus on motion, flexion loss, instability, joint pain, strength, and gait. Each of the 11 symptoms is rated on a Likert-style scale ranging from 0-1 to 0-4, with higher numbers indicating greater severity of symptoms. Scores from the individual items are added together to give a joint score, gait score, and a total score, with higher scores indicating greater joint damage.

Protocol Name from Source:

Hemophilia Joint Health Score (HJHS) 2.1

Availability:

Publicly available

Personnel and Training Required

The Hemophilia Joint Health Score (HJHS) 2.1 should be administered by experienced physiotherapists who have reviewed the training manual and instructional DVD. Training manual and DVD are available from the International Prophylaxis Study Group website (http://www.ipsg.ca/) by request.

Equipment Needs

Investigators will need the following:

  • 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), and
  • set of stairs (nearby).

Requirements
Requirement CategoryRequired
Major equipment No
Specialized training Yes
Specialized requirements for biospecimen collection No
Average time of greater than 15 minutes in an unaffected individual Yes
Mode of Administration

Clinical Examination

Life Stage:

Infant, Toddler, Child, Adolescent, Adult, Senior, Pregnancy

Participants:

Hemophilia patients on prophylaxis treatment ages 4 and older.

Selection Rationale

The Hemophilia Joint Health Score (HJHS) 2.1 is a valid, reliable, and widely used instrument that is sensitive to early joint changes in hemophilia patients on prophylaxis.

Language

English, Available in other languages

Standards
StandardNameIDSource
Common Data Elements (CDE) Hemophilia Emicizumab Therapy Coagulation Factor VIII Inhibitor Nijmegen-Bethesda Assay Bovine Reagent Chromogenic Substrate Clotting Factor Assay 6733007 CDE Browser
Derived Variables

None

Process and Review

Not applicable.

Source

The Hemophilia Joint Health Score (HJHS) 2.1 score sheet, manual, and training video can be obtained through the International Prophylaxis Study Group website (http://www.ipsg.ca/).

General References

Feldman, B. M., Funk, S., Bergstrom, B.-M., Zourikian, N., Hilliard, P., van der Net, J., Engelbert, R. H. H., Petrini, P., van den Berg, M., Manco-Johnson, M., Rivard, G. E., Abad, A., & Blanchette, V. S. (2011). 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, 63(2), 223-230.

Feldman, B. M., Funk, S., Hilliard, P., Van Der Net, J., Zourikian, N., Bergstrom, B.-M., Engelbert, R. H. H., Abad, A., Petrini, P., & Manco-Johnson, M.; International Prophylaxis Study Group. (2008). The Haemophilia Joint Health Score (HJHS) International Validation Study. XXVIIIth International Congress of the World Federation of Hemophilia, Istanbul, Turkey. Haemophilia, 14(Suppl. 2), 83.

Hilliard, P., Blanchette, V. S., Doria, A., Blanchette, C., Hang, M., & Feldman, B. M. (2008). The Hemophilia Joint Health Score (HJHS) correlates highly with radiographic damage. XXVIIIth International Congress of the World Federation of Hemophilia, Istanbul, Turkey. Haemophilia, 14 (Suppl. 2), 80.

Hilliard, P., Funk, S., Zourikian, N., Bergstrom, B. M., Bradley, C. S., McLimont, M., Manco-Johnson, M., Petrini, P., Van Den Berg, M., & Feldman, B. M. (2006). Hemophilia Joint Health Score reliability study. Haemophilia, 12(5), 518-525.

Protocol ID:

911701

Variables:
Export Variables
Variable NameVariable IDVariable DescriptionVersiondbGaP Mapping
PX911701000000 Protocol 911701 - proprietary. Check DCW for more
contact. show less
N/A
Hemophilia Inhibitor Research
Measure Name:

Joint Damage Assessment in Hemophilia Patients

Release Date:

May 7, 2019

Definition

A measure to assess joint damage resulting from bleeding in hemophilia patients.

Purpose

This measure can be used to detect the early signs of joint damage and monitor the results of orthopedic and physiotherapy interventions in hemophilia patients treated with prophylaxis. Additionally, it can be used to track joint change over time and assess the efficacy of treatment regimens.

Keywords

Bleeding, target joint, joint bleed, pain, joint