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The Paediatric Haemophilia Activities List (pedHAL) in routine assessment: changes over time, child-parent agreement
Isolde A R Kuijlaars1, Janjaap van der Net2, Roger E G Schutgens1
1Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Insights
Children with haemophilia on prophylaxis show high and stable activity scores using the Paediatric Haemophilia Activities List (pedHAL). Optimal child-parent agreement is not consistently achieved, highlighting the need for both perspectives in assessing limitations.
Area of Science:
- Paediatric Haematology
- Quality of Life Assessment
- Clinical Outcome Measures
Background:
- The Paediatric Haemophilia Activities List (pedHAL) is a tool designed to evaluate self-reported activity and participation limitations in children diagnosed with haemophilia.
- Understanding the impact of haemophilia on a child's daily life is crucial for effective management and treatment.
Purpose of the Study:
- To longitudinally assess changes in activity and participation using the pedHAL in boys with haemophilia receiving early prophylaxis.
- To evaluate the agreement between child and parent reports on the pedHAL.
- To identify which pedHAL domains provide the most informative data for this patient group.
Main Methods:
- The pedHAL, comprising 53 items across 7 domains, was administered annually to boys aged 4-18 years with moderate to severe haemophilia and their parents.
- Data analysis focused on the development of pedHAL scores in relation to bleeding events, changes over 3-5 years, child-parent agreement (defined as ≤5% difference), and the identification of limitations (scores ≤95).
Main Results:
- A total of 73 patients and their parents completed at least one pedHAL assessment, with 92% having severe haemophilia and a median age of 13.1 years.
- Median pedHAL sum scores were high (99.5 for children, 99.6 for parents), indicating minimal limitations at a group level.
- Child-parent agreement varied significantly across domains, ranging from 92% for 'self-care' to 71% for 'sitting/kneeling/standing', with the latter domains and 'leisure activities and sports' reporting the most limitations.
Conclusions:
- In Dutch children with haemophilia on prophylaxis, pedHAL scores are generally high and stable over 3-5 years at the group level.
- For individual patients without joint or muscle bleeds, pedHAL scores remained high after one year.
- The observed suboptimal child-parent agreement underscores the importance of collecting and reporting both child self-reports and parent proxy reports for a comprehensive understanding of limitations.
Introduction:
The Paediatric Haemophilia Activities List (pedHAL) assesses self-reported limitations in activities and participation in children with haemophilia.
Aim:
To assess longitudinal changes, child-parent agreement and to identify which pedHAL domains yielded most information in boys with access to early prophylaxis.
Methods:
The pedHAL (53 items, 7 domains, optimum 100) was completed annually at the Van Creveldkliniek by boys aged 4-18 years with moderate/severe haemophilia and their parents. Development of the pedHAL in relation to bleeds, changes per domain over 3-5 years, child-parent agreement (% difference child-parent≤|5|) per domain and domain scores (limitations defined as ≤ 95) were determined.
Results:
Seventy-three patients and their parents (92% severe haemophilia, median age 13.1 years [range 5.4;18.0]) completed ≥1 pedHAL. Median (IQR) pedHAL sum score was 99.5 (95.2;100.0) for children and 99.6 (95.8;100.0) for parents. If patients scored >95 and had no joint and/or muscle bleed, 90.9% of the patients scored >95 at the next assessment. The median change in sum score was 0.0 for both the 3- and 5-year interval. Child-parent agreement varied between domains from 92% ('self-care') to 71% ('sitting/kneeling/standing'). Most limitations were reported in the domains 'sitting/kneeling/standing', 'functions of the legs' and 'leisure activities and sports.'
Conclusion:
In routine clinical practice in Dutch children on prophylaxis, pedHAL scores were high and remained stable in 3-5 years at group level. In individual patients without joint and/or muscle bleeds, pedHAL scores remained high after 1 year. Child-parent agreement was not optimal which indicated that both child report and parent proxy should be reported.
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