Description

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

It is primarily designed for adults and children with established arthropathy. It may be used when there is a need for orthopedic intervention, or as an outcome measure of physiotherapy interventions.

Date of this review:
March 7, 2011

Utility

Overall utility:
Until recently, the WFH Physical Examination Score was the main tool used to assess joint health. Although somewhat less sensitive than the Hemophilia Joint Health Score, it distinguishes between different groups of patients (e.g. mild, moderate, severe disease; prophylaxis vs. on-demand). It is probably best used for older children and adults, and in severely affected populations such as patients with uncontrolled inhibitors, or little access to factor replacement.

Limitations:
The WFH Physical Examination Score takes a long time to administer.

It may not be appropriate for younger children as normal development axial alignment changes may be scored as abnormal. It may not be appropriate for persons with mild arthropathy, such as those treated with lifelong prophylaxis, as it is insensitive to mild joint changes.

Administration

1. Time to complete: 30-45 minutes, plus computing and recording score.
2. Equipment/space required:
   • plinth/bed
   • goniometer
   • tape measure
3. Training required:
   • use of goniometer to measure joint range of motion and axial deformity
   • familiarity with ligament (instability) testing and other examination techniques to assess joint crepitus and swelling.
4. Cost: None
5. Scoring/scaling/interpretation of results: The WFH Physical Examination Score provides a total score (higher score is worse) and joint specific scores.

Psychometrics

Psychometric properties:

1. Construct validity: The WFH Physical Examination Score shows adequate 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=0.43) and physician global assessment of joint health (r_s=0.47).

2. Criterion validity: The WFH Physical Examination Score compares less favourably with the Hemophilia Joint Health Score 1.0 when discriminating known groups (63% to 97% less efficient).
3. Reliability has not been tested.
4. Responsiveness/sensitivity has not been tested longitudinally.

Languages studied:
Swedish, English, Dutch

Groups tested with this measure:
North American and European children with well-treated hemophilia A and B (mild, moderate and severe factor deficiency).

Glossary of Psychometric Properties

References

1. Gilbert MS. Prophylaxis: musculoskeletal evaluation. Semin Hematol 1993 Jul; 30(3 Suppl 2):3-6.
2. Gilbert MS, Weidel JD. The Treatment of Hemophilia: Current Orthopedic Management; National Hemophilia Foundation 1995.