Follow-up study of patients with haemophilia A receiving factor VIII

Background: Haemophilia A is an inherited bleeding disorder resulting from factor VIII (FVIII) deficiency and FVIII replacement therapy is the foundation of treatment.  
Objectives: To know the clinical profile and physical quality assessment in children with haemophilia A using Functional Independence Scoring in Haemophilia (FISH) scoring as assessment tool.  
Method: This observational follow-up study was conducted in the paediatrics department of a tertiary care centre. A total of 24 haemophilia A patients was enrolled.
Results: Mean age of patients was 10.5 years; 83% were from urban area and 50% had a positive family history; 4% were mild, 38% moderate and 58% severe; 54% had 1st bleeding manifestation at <1 year of age and cumulatively 92% had bleeding manifestation before age of 5 years. Haemarthrosis (71%) was the most common manifestation. Among severe cases 93% and among moderate cases 56% of patients had joint bleeding. Knee joint was affected in 44% and elbow in 32%; 55% patients had post traumatic bleed. Severe haemophilia patients had prolonged duration of hospital stay; 80% of severe cases had limitation of joint movements; 33% patients developed FVIII inhibitors. At start of study mean FISH score was 30.1±2.54 in severe group, 31.55±1.33 in moderate cases and 32.0 in mild cases (p=0.0557). At end of study FISH score remained almost the same except in moderate cases. Average FVIII consumption in 6 months was 105IU/kg in severe haemophilia, 75 IU/kg in moderate haemophilia and 35 IU/kg in mild haemophilia.  
Conclusions: Cumulatively 92% of cases had bleeding manifestation before the age of 5 years. Haemarthrosis (71%) was the most common manifestation. Common joints involved were knee (44%) and elbow (32%); 55% patients had post traumatic bleeds.