Enhanced Functional Independence Score for Haemophilia (E-FISH) for Early Detection of Musculoskeletal Changes.

Abstract

Background: The functional independence score in haemophilia (FISH) is a widely used performance-based assessment tool designed to evaluate musculoskeletal function in children with haemophilia (CWH). Nevertheless, FISH exhibits a ceiling effect in children who are undergoing early prophylaxis or those who present with minimal musculoskeletal impairment. This report describes the development of an enhanced FISH (e-FISH) with more challenging tasks to detect early physical function changes in CWH.

Methods: Two performance-based assessments, the basketball throw test (BBT) and the 10×5-meter sprint test (ST) were included as a subtest of FISH. Age-specific normative scores were established from healthy boys. A scoring system, which ranges from 1-4, was created and compared with CWH, who showed no joint deformities after obtaining the necessary consent and assent.

Results: Twenty CWH with FISH scores ranging from 31-32 and on prophylactic treatment with clotting factor concentrate were evaluated using these two tests. Only one (5%) CWH attained a higher score of 4 in both assessments. In the BBT, two (10%) achieved a score of 3, six (30%) received a score of 2, and 11 (55%) were assigned an e-FISH score of 1. In the 10×5 meter ST, eight (40%) scored 3, one (5%) scored 2, and 10 (50%) scored 1 in e-FISH. No bleeding was reported.

Conclusion: We have created e-FISH as a clinimetric instrument designed for the assessment of individuals with CWH who may exhibit minimal musculoskeletal alterations and demonstrate a ceiling effect when using FISH.