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

Authors:

Holdsworth, C., Bladen, M., Harbidge, H., Drechsler, W., Mahaffey, R., Perez-Alenda, S., Sayers, F., Strike, K., Timmer, M., and Stephensen, D.

Abstract:

INTRODUCTION: Currently, physical health assessments in persons with haemophilia focus on bleed-related events and after-effects. The aim of the systematic review was to review and apply standardised criteria to evaluate reliability, responsiveness and construct validity of performance-based instruments evaluating physical capability in persons with haemophilia.

METHODS: Medline, CINAHL, Embase, EMCARE, and Cochrane (inception-March 2024) were searched using COSMIN filters for 7 performance-based tests in haemophilia, supplemented by manual searches. Reliability, responsiveness and construct validity of the 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) were evaluated.

RESULTS: The search yielded 88 abstracts; 25 studies remained after full-text screening, covering 5 of 7 performance-based instruments: 6MWT, TUG, TUDS, SLS, and 30-STS. No performance-based test was evaluated for all properties across all ages. Only TUG in adults and older adults and 6MWT in children and adolescents has been tested for all properties. No test received a high grading. Low and very low grades were given mostly for indeterminate results, small or single studies and lack of a similar construct of comparator. The 6MWT in all age groups was the only performance-based test graded moderate, and this was for responsiveness.

CONCLUSION: With increasing use of performance-based methods of physical function capacity, evaluating measurement properties is a priority. Until evidence is generated, we can only advocate the 6MWT to monitor responsiveness in adult persons with haemophilia affected with marked arthropathy.

DOI:

10.1111/hae.70081

URL:

https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/hae.70081?download=true

Journal:

Haemophilia

issn:

1351-8216

Publication year:

2025

pages:

Functional status:

Physical functioning

Age:

Adults (18-65)
Children (0-18)
Seniors (65+)

Disease:

Diseases of and symptoms related to the blood and blood-forming organs and certain disorders involving the immune mechanism

PRO / non-PRO:

Non-patient Reported Outcome

Type of measurement instrument:

4 - Performance-based tests
6 - Clinical rating scales

Instrument:

6MWT - 6 Minute Walk Test (versions: 6MWT app; 6MWT Lite app; M6MWT - Modified 6MWT; 6mWTPC - 6MWT on parquet and carpet)    |  | also see: database.cosmin.nl
SLST - Single Leg Stance Test [ZIE: One-leg standing balance]
STS - Sit-to-stand test (versions: 5STS or STS-5; 5times STS/FTSTS; 20sSTS; 30sSTS; 60sSTS; STST1 - 1 min STS; 2minSTS; 3minSTS; 10times STS or STS-10; STS-60; STS-30)
TUDS - Timed Up-and-Down Stairs   
TUG - Timed Up and Go test (versions: TUG 3m)    |