The pediatric Hemophilia Activities List: towards a next generation of activities and participation assessment

Introduction and Objective: The pediatric Hemophilia Activities List (pedHAL) assesses self-reported limitations in activities in children with hemophilia (child and parent forms). The pedHAL contains 53 items, divided in seven domains. The present study aimed to explore patient-parent agreement, assessment of social participation and identify non-informative items. Materials and Methods: We analyzed cross-sectional pedHAL data collected at the annual routine assessment at the Van Creveldkliniek (University Medical Center Utrecht, The Netherlands) between October 2010 - June 2017. Patients (4-18 years) with moderate/severe hemophilia A/B and their parents completing ≥1 pedHAL were included. We determined the distribution of scoring per item with descriptive analyses and patient-parent agreement per domain with limits of agreement and Wilcoxon signed-rank tests. In addition, 7 children, 4 parents and 11 health care professionals rated the relevance of all 15 participation items of the domains ‘use of transport’, ‘household tasks’ and ‘leisure activities and sports’. Results: Eighty-two patients with hemophilia A/B (severe 94%, median 13.1 years [range 5.4-18.0]) were included. Mean domain scores ranged between 98 and 92. Limits of agreement of domain scores of patients and parents (n=69 pairs) varied from 8 (‘selfcare’, mean score 98) to 19 (‘functions of the legs’, mean score 93). In parents 15 items were scored as ‘never problems’ in >90% and 28 items were scored as ‘never problems’ in 80-90%. The 13 most informative items (<80% ‘never problems’) belonged to the domains ‘sitting/kneeling/standing’, ‘functions of the legs’ and ‘leisure activities and sports’. The evaluation of participation items showed that sports items were considered most relevant; sports items were scored as relevant by >64% of the participants. Conclusions: Patient-parent agreement for the pedHAL was acceptable. This explorative analysis of single-center data identified several non-informative items. In addition, participation assessment will be further evaluated in focus groups. Item reduction is expected enhance feasibility of the pedHAL for research and clinical proposes, but can only be performed after testing in other populations.