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. 2021 Jan;27(1):108-112.
doi: 10.1111/hae.14226. Epub 2020 Dec 13.

Assessing the test-retest reliability and smallest detectable change of the Haemophilia Activities List

Affiliations

Assessing the test-retest reliability and smallest detectable change of the Haemophilia Activities List

Isolde A R Kuijlaars et al. Haemophilia. 2021 Jan.

Abstract

Introduction: The Haemophilia Activities List (HAL) is a preferred instrument to measure self-reported limitations in activities in persons with haemophilia (PWH). Information on reliability and interpretability of HAL scores is lacking.

Aim: To examine the test-retest reliability and smallest detectable change (SDC) of the HAL in adult PWH.

Methods: Fifty adult (≥18 years) persons with mild to severe haemophilia completed the HAL (42 items, 7 domains, optimum 100) at baseline (T0) and 3-4 weeks later (T1). The intraclass correlation coefficient (ICC) and SDC were calculated for sum and component scores.

Results: Fifty persons with haemophilia were included (median age 49 years; 92% haemophilia A; 70% severe haemophilia). The median (interquartile ranges) HAL sum score was 77 (62 to 99) at T0 and 81 (64 to 98) at T1. Reliability was good with ICCs for sum and component scores >0.9. The SDC for the sum score was 10.2, for the upper extremity component score 9.2, for the basic lower extremity component score 16.7 and for the complex lower extremity component score 13.4.

Conclusion: The HAL has a good reliability for the sum and component scores. Score changes of the normalized sum HAL score greater than the SDC 10.2 indicate that the change was not a result of measurement error.

Keywords: activities; haemophilia; participation; patient-reported outcome; questionnaire; reliability.

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Conflict of interest statement

None of the authors reported any conflict of interest regarding this manuscript other than membership of the group that developed the HAL.

Figures

Figure 1
Figure 1
Bland and Altman plot of the HAL sum score, with limits of agreement of −0.92 ± 10.14.

References

    1. Srivastava A, Santagostino E, Dougall A, et al.WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia; 2020. - PubMed
    1. van Vulpen LFD, Holstein C, Martinoli K. Joint disease in haemophilia: Pathophysiology, pain and imaging. Haemophilia. 2018;24:44‐49. - PubMed
    1. Shapiro S, Makris M. Haemophilia and ageing. Br J Haematol. 2019;184:712‐720. - PubMed
    1. Miesbach W, O’Mahony B, Key NS, Makris M. How to discuss gene therapy for haemophilia? A patient and physician perspective. Haemophilia. 2019;25:545‐557. - PMC - PubMed
    1. Timmer MA, Gouw SC, Feldman BM, et al. Measuring activities and participation in persons with haemophilia: A systematic review of commonly used instruments. Haemophilia. 2017;24(2):e33‐e49. - PubMed