Protocol - Hemophilia Joint Health Score
- Counts of Acute Bleeds in Hemophilia
- Hemophilia-specific Functional Ability - Adult
- Hemophilia-specific Functional Ability - Child
- Quality of Life - Adult
- Quality of Life - Pediatric
- Total Physical Activity - Comprehensive - Adolescent
- Total Physical Activity - Comprehensive - Adult
- Total Physical Activity - Comprehensive - Older Adult
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.
Availability
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.
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 Category | Required |
|---|---|
| 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
Lifestage
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, Other languages available at source
Standards
| Standard | Name | ID | Source |
|---|
Derived Variables
None
Process and Review
Not applicable.
Protocol Name from Source
Hemophilia Joint Health Score (HJHS), 2.1
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 Name | Variable ID | Variable Description | dbGaP Mapping | |
|---|---|---|---|---|
| PX911701000000 | Protocol 911701 - proprietary. Check DCW for more | N/A | ||
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
Measure Protocols
| Protocol ID | Protocol Name |
|---|---|
| 911701 | Hemophilia Joint Health Score |
Publications
There are no publications listed for this protocol.