Abstract

To date, none of the available assessment instruments to evaluate functional abilities for individuals with haemophilia has been translated and validated in Brazil. To translate, and test the construct validity, internal consistency and the reliability of the Haemophilia Activities List (HAL) for the Brazilian population with severe and moderate haemophilia (type A or B) and to investigate differences in the self-perception of functional abilities in patients adults with haemophilia classified according to the presence of joint bleeding and the performance of orthopaedic surgeries. The translation and transcultural adaptation following the steps: (a) translation; (b) synthesis (consensual version); (c) back-translation; (d) review by the committee of experts; (e) pretest in the target population; and (f) final version of the instrument. Correlations between HAL scores and the scores of the Health Assessment Questionnaire (HAQ) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) were determined for construct validation. Reliability was tested using the test and retest method, and internal consistency was calculated by Cronbach's α. The HAL-BR was applied to 52 individuals with haemophilia (34.51±12.87years). The process of translation and cross-cultural adaptation produced similar versions between the translations A moderate correlation was observed between HAL and the HAQ (r=-0.55) and WOMAC scores (r=-0.58). The reliability was ICC=0.972, CI (0.917-0.997) ICC=0.876, CI (0.631-0.978) for inter- and intra-examiners. No difference was found in the total HAL score between the groups with and without bleeding. The group subjected to surgery compared to the group of not subjected to surgery presented in the HAL domains worse function in 'lying down/sitting/kneeling/standing', 'self-care' and 'complex lower extremities activities' as well as in total HAL score. The Brazilian version of the HAL was proven to be a valid and reliable evaluation tool for adults with haemophilia in Brazil.

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