Validation of the pedHALshort and HALshort in Dutch children and adults with haemophilia

Isolde A R Kuijlaars; Janjaap van der Net; Lize F D van Vulpen; Mariette H E Driessens; Saskia E M Schols; Melanie Tan; Samantha C Gouw; Kathelijn Fischer

doi:10.1111/hae.14628

Validation of the pedHAL_short and HAL_short in Dutch children and adults with haemophilia

Haemophilia. 2022 Nov;28(6):1007-1015. doi: 10.1111/hae.14628. Epub 2022 Jul 29.

Authors

Isolde A R Kuijlaars¹, Janjaap van der Net², Lize F D van Vulpen¹, Mariette H E Driessens³, Saskia E M Schols^{4

5}, Melanie Tan⁶, Samantha C Gouw^{7

8}, Kathelijn Fischer¹

Affiliations

¹ Center for Benign Haematology, Thrombosis and Haemostasis, Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
² Center for Child Development, Exercise and Physical Literacy, University Medical Center Utrecht, Utrecht University, University Children's Hospital, Utrecht, the Netherlands.
³ Netherlands Haemophilia Society (NVHP), Nijkerk, the Netherlands.
⁴ Department of Haematology, Radboud University Medical Center, Nijmegen, the Netherlands.
⁵ Haemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Radboud University Medical Center, Nijmegen, the Netherlands.
⁶ Department of Vascular Medicine, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands.
⁷ Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Pediatric Hematology, Amsterdam, the Netherlands.
⁸ Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands.

Abstract

Introduction: The Haemophilia Activities List (HAL) and paediatric HAL assess self-reported limitations in various daily activities. To reduce patient burden, shorter versions of the pedHAL (22 items) and HAL (18 items) have been developed.

Aim: This study aimed to determine the agreement between the pedHAL/HAL_full and pedHAL/HAL_short and construct validity and internal consistency of the pedHAL_/ HAL_short in persons with haemophilia (PWH).

Methods: A cross-sectional secondary analysis of the Hemophilia in the Netherlands-6 national survey was performed. Adult and paediatric PWH completed the original pedHAL/HAL_full , from which pedHAL_/ HAL_short were derived. Score differences between the original and short versions were calculated. Construct validity was studied by testing hypotheses regarding the relationship of the pedHAL/HAL_short with the pedHAL/HAL_full , Haemophilia & Exercise Project Test-Questionnaire (HEP-Test-Q), Canadian Haemophilia Outcomes-Kids' Life Assessment Tool (CHO-KLAT) and RAND 36-item Health Survey (RAND-36) (convergent/discriminant validity) as well as its ability to discriminate between subgroups (known-group validity). Internal consistency was assessed with Cronbach's α.

Results: We included 113 children (median 10y [range 4-17], 53% severe haemophilia) and 691 adults (median 51y [range 18-88], 35% severe). Scores of the pedHAL/HAL_full and pedHAL/HAL_short were similar with high correlations (>0.9). Construct validity was confirmed for the pedHAL/HAL_short . The HAL_short was able to discriminate between different disease severities and ages. Cronbach's α of the pedHAL/HAL_short was 0.95-0.97.

Conclusion: This study confirmed the agreement between the pedHAL/HAL_full and the pedHAL/HAL_short and the construct validity of the pedHAL/HAL_short . The next step is to study construct validity of the pedHAL/HAL_short when administered as short forms.

Keywords: activities; haemophilia; participation; patient-reported outcome; validity.

MeSH terms

Adult
Canada
Child
Cross-Sectional Studies
Hemophilia A*
Humans
Quality of Life
Reproducibility of Results
Self Report
Surveys and Questionnaires