Epidemiological survey of the orthopaedic status of severe haemophilia A and B patients in France

One hundred and 16 patients contributed to an analysis of the impact of the consequences of severe haemophilia A or B (factor levels < 2%) on orthopae dic status, resources consumed in relation to this status and resultant cos t, and quality of life as perceived by the patient, using the MOS 36-Item-S hort-Form Health Survey (SF-36). This French cross-sectional study involved outpatients regularly attending a haemophilia treatment centre. Data were collected retrospectively over a period of 1 year by the physician of the h aemophilia treatment centre. Patients had a mean age of 23, and consisted o f 50% students, 25% salaried workers, 17.2% with no professional activity a nd 7.8% physically impaired; 82.8% of them had type A haemophilia. Mean pai n score was 2.5 per patient for the six main joints; 7.7 for the clinical s core and 18.8 for the radiological score, with a mean number of bleeds of 1 6.3 per year per patient. During the year prior to inclusion, and because o f their orthopaedic status, 22.4% of patients were hospitalized, 76.7% atte nded for an outpatient visit and 76.7% required at least one special invest igation; 97.4% received replacement therapy, 41.4% required treatment for j oint pain and 42.2% orthopaedic equipment. The less affected dimensions wer e the physical function (74.8 +/- 22.2) and the social relations (76.1 +/- 23.1). Least good quality of life scores concerned the pain (60.2 +/- 25.2) , perception of general health (59.3 +/- 23.1) and vitality (57.8 +/- 19.5) dimensions. The age was a discriminant criterion since quality of life was better in patients of the 18-23 age group for five dimensions. Mean annual treatment costs of a patient with severe haemophilia were determined as 42 5 762 French francs ($73 029). Loss of production was estimated at a mean o f 4609 French francs ($791) per active patient over the course of the year. Results showed indirect evidence of the usefulness of early home treatment .