Assesment of Quality of Life by Functional Independance Score in Hemophilia Patients

Hemophilia is a blood-clotting disorder caused by a deficiency in factor VIII (FVIII) or factor IX (FIX), which manifests itself through bleeding in the muscles and joints [1].

There are three categories, depending on the percentage of the blood-clotting factor: severe hemophilia (<1% FVIII/IX), characterised by spontaneous bleeding; moderate hemophilia (1-5% FVIII/FIX), with bleeding from slight injuries; and mild hemophilia (>5-40% FVIII/FIX) with bleeding during surgical procedures or from severe injuries(2).(3) Hemophilia C (a deficiency of Factor XI) is encountered, but its effect on clotting is far less pronounced than A or B Hemophilic arthropathy of the ankle causes pain and deterioration in gait, causing disability. Although some physiotherapy modalities are effective in the management of acute bleeding, the results are unknown in chronic arthropathy Due to the progression of hemophilic arthropathy and the resulting deterioration of the joint, deformities such as the estriction of motion (4). Physiotherapy through the RICE method (Rest, Ice, Compression, and Elevation) has been described for the improvement of acute joint injuries (5)as well as in the management of hemarthrosis in patients with hemophilia (6) Treatment of the ankle with physical therapy is complicated because it involves a small joint surface that bears significant ranges of body weight and the joint limitation alters biomechanical movement during walking(7) Prevention of musculoskeletal morbidity is an important objective in the treatment of hemophilia. Efficacy of such therapy has classically been measured by quantifying the extent of arthropathy using a joint scoring (8)(9)

Design of the Functional Independence Score in Hemophilia

Patients with hemophilia and their therapists were asked to list activities that could be affected by hemophilia. These were categorized according to the International Classification of Functioning, Disability and Health (ICF) (10)Activities that were considered unsafe to perform were excluded from the assessment. Other activities such as the use of transport, education and employment - that could not be assessed objectively in the clinic were also excluded. We finally included seven activities under three categories - self-care, transfers and locomotion (Table 1). Each activity was graded from 1 to 4 according to the amount of assistance required to perform the activity. Each activity and level of independence was clearly defined to reduce interobserver variation.core from 1 to 4 in each area:

1. = the subject is unable to perform the activity, or needs complete assistance to perform the activity;
2. = the subject needs partial assistance/aids/modified (9) (10) instruments/modified environment to perform the activity;
3. = the subject is able to perform the activity without aids or assistance, but with slight discomfort. He is unable to perform the activity like his healthy peers 11 4= the subject is able to perform the activity without any difficulty like other healthy pears