Functional independence score in hemophilia: a cross-sectional study assessment of Mexican children.

BACKGROUND

The 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.

PROCEDURE

In 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.

RESULTS

As 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.

CONCLUSIONS

A 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.