Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

Abstract

BackgroundThe Functional Independence Score in Hemophilia (FISH) is a performance‐based assessment tool used to measure the patients functional ability so far only used in patients with severe hemophilia. Its aim is to determine if FISH is useful in patients with mild and moderate disease.ProcedureIn a cross‐sectional study 90 children 60 hemophilic and 30 sex‐, race‐, and age‐matched healthy males were assessed. Patients between 5 and 16 years of age were selected each patient was evaluated in seven activities under three categories: self‐care (grooming and eating, bathing, and dressing), transfers (chair and squat), and locomotion (walking and step climbing). Each activity was graded from 1 to 4 according to the amount of assistance required to perform the activity with total scores ranging from 7 to 28.ResultsAs a whole, the mean age of the patients was 10.0 ± 3.4 years with a mean FISH of 25.8 ± 3.6 (range 15–28). There were no differences in the FISH between healthy males and patients with mild hemophilia; however, the score was significantly higher in patients with mild hemophilia (28 ± 0) than patients with moderate (26.2 ± 2.5; P = 0.004) or severe hemophilia (24.0 ± 4.7; P = 0.0006). The most affected activities were squatting, walking, and step climbing.ConclusionsA significant decrease in functional ability was demonstrated according to the severity of hemophilia, especially for those activities involving weight‐bearing demands like locomotion and step climbing. Of seven activities evaluated, changes were observed in the group with moderate and severe hemophilia, but no changes detected in patients with mild disease. Pediatr Blood Cancer 2010;54:394–397. © 2009 Wiley‐Liss, Inc.

Assessment of musculoskeletal function and mood in haemophilia A adolescents: a cross-sectional study

“…In our study, the mean FISH score was 23.32 ± 4.69 (range 13–28).Patients with severe haemophilia had lower FISH scores compared to those with moderate and mild haemophilia (20.5 ± 3.97 vs. 24.7 ± 2.4 in moderate haemophilia and 27.7 ± 0.48 in mild haemophilia). These results are matched with a recent Mexican study [7] where mean FISH score was significantly higher in patients with mild haemophilia (28 ± 0) than in patients with moderate (26.27 ± 2.54; P = 0.004) or severe haemophilia (24.08 ± 4.74; P = 0.0006). Similarly, step climbing, squatting and walking were the most affected activities.…”

“…haemophilia). These results are matched with a recent Mexican study [7] where mean FISH score was significantly higher in patients with mild haemophilia (28 ± 0) than in patients with moderate (26.27 ± 2.54; P = 0.004) or severe haemophilia (24.08 ± 4.74; P = 0.0006). Similarly, step climbing, squatting and walking were the most affected activities.…”

“…Traditional clinical and radiological joint scores do not consider the impact of arthropathy on overall musculoskeletal function [7]. The FISH has been developed as a performance-based assessment tool to address this need [6].…”

Validation of the Haemophilia & Exercise Project‐Test‐Questionnaire (HEP‐Test‐Q)—An instrument for the assessment of subjective physical functioning in children with haemophilia

“…The self‐reported PedHAL focuses on activities of daily living such as use of transport, self‐care and household tasks, whereas the HEP‐Test‐Q assesses aspects of physical functioning based on motor ability (mobility, strength, coordination and endurance). PedHAL is an internationally recognized instrument with use in clinical trials, yet there is limited published evidence of its use . FISH has also been used internationally in children and adults and has been shown to be a reliable tool for assessing functional ability in CWH compared to normal controls revealing lower limb functional abnormalities (squatting, walking, and step climbing) and functional “independence” in adolescents .…”

Outcome Assessment in Hemophilia

“…The FISH was found to be highly reliable, with a pooled intraclass correlation coefficient of 0.98. In studies from Mexico [46] and Egypt [47], the FISH was found to discriminate the severity of hemophilia, as well as between patients and healthy controls. The FISH had a good correlation with the WFH clinical score (r = −0.61).…”