The orthopaedic status of a selected severe haemophilia group

The aim of the present study was to describe the orthopaedic status of patients with severe haemophilia, and to relate this status to the type of replacement therapy received by patients prior to the study. Ninety two haemophiliacs with median age 26 were included. Six joints--knees, elbows and ankles were evaluated clinically using the Gilbert scale. The evaluation included physical status (0-12 points/joint) and pain score (0-3 points/joint). X-ray examinations were evaluated according to the Pettersson scale (0-13 points/joint). On all scales, normality was represented by 0 score. Knees were the most affected joints. Eighty four patients (91.3%) reported pain. Only one patient scored 0 on the Gilbert scale, and another on the Pettersson scale. Thirty seven percent of patients used orthopaedic equipment occasionally or constantly. Twenty five percent of patients had a history of orthopaedic surgery. Thirty eight percent were unemployed and received some form of social subvention. On demand treatment was applied. None of the patients received primary prophylaxis. The mean consuption of clotting factor concentrates was 68,054 IU per patient during the 12 months period prior to the current study. The results of this study indicate that vast majority of severe haemophilia patients in Poland above 20 are affected by haemophilic arthropathy. This disabling complication of severe haemophilia can be prevented only by introducing primary prophylaxis from the first years of life.