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Measuring Physical Function Capacity in Persons With Haemophilia: A Systematic Review of Performance-Based Methods.

Catherine Holdsworth1, Melanie Bladen2, Hannah Harbidge3

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Summary

Physical function tests for hemophilia lack robust validation. The six-minute walk test (6MWT) shows moderate responsiveness, making it suitable for monitoring adults with hemophilia and joint issues.

Keywords:
core outcome sethaemophiliajoint healthmusculoskeletalphysical capacityphysical functionsystematic review

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Area of Science:

  • Orthopedics
  • Rehabilitation Medicine
  • Hematology

Background:

  • Current physical health assessments for individuals with hemophilia primarily focus on bleeding events and their consequences.
  • There is a need to evaluate the measurement properties of performance-based instruments for assessing physical capability in this population.

Purpose of the Study:

  • To systematically review and apply standardized criteria to assess the reliability, responsiveness, and construct validity of performance-based instruments for evaluating physical capability in individuals with hemophilia.

Main Methods:

  • A comprehensive literature search was conducted across multiple databases (Medline, CINAHL, Embase, EMCARE, Cochrane) using COSMIN filters and manual searches.
  • Seven performance-based tests were evaluated: six-minute walk test (6MWT), timed up and go test (TUG), timed up and down stairs (TUDS), 30-second sit-to-stand (30-STS), single leg stance (SLS), tandem stance (TS), and single hop for distance (SH).

Main Results:

  • Out of 88 abstracts, 25 studies were included, covering 5 of the 7 instruments (6MWT, TUG, TUDS, SLS, 30-STS).
  • No single performance-based test was evaluated for all measurement properties across all age groups.
  • The six-minute walk test (6MWT) was the only instrument graded as moderate, specifically for responsiveness in all age groups.

Conclusions:

  • Evaluating the measurement properties of performance-based instruments for physical function capacity is crucial.
  • The six-minute walk test (6MWT) is currently the most recommended instrument for monitoring responsiveness in adult individuals with hemophilia experiencing significant arthropathy, pending further evidence generation.