Data Analyses. All analyses were stratified by the severity of hemophilia, and often by age category as well. As the clinical characteristics of hemophilia A and hemophilia B do not differ, we present combined results for hemophilia A and B. Data on the treatment modality, the number of bleeding episodes, the use of hospital facilities, and absence from school or work referred to the year that preceded the questionnaire surveys (2000). Children were defined as patients younger than 16, adolescents as patients between 16 and 25 and adults as patients older than 25 years. The use of prophylaxis refers to patients who received prophylaxis as their main treatment modality, excluding patients who received a combination of on demand treatment and prophylaxis during risk periods. Absence from school was calculated only for that part of the population that followed a full-time education. Absence from work was calculated for patients aged 16 to 65 who had a paid job (full-time or part-time). The inactivity ratio was calculated as the ratio of inactivity in the study population and inactivity in Dutch men. Patients that did not have a full-time or part-time paid job were defined as inactive. Descriptive statistics for age, the use of hospital facilities, absence from work and employment were compared to national figures for the general male population that were provided by the Central Bureau of Statistics Netherlands Statline database20. Self reported measures on joint impairment were obtained for a series of 16 joints which are, the neck, the left and right shoulder, the back, the left and right elbow, the left and right wrist, the left and right hand and fingers, the left and right hip, the left and right knee and the left and right ankle. The possible scores were 0 (no impairment), 1 (some impairment without daily problems), 2 (some impairment with daily problems), and a maximum of 3 (severe impairment with complete loss of function). From scores of the 16 separate joints a joint score was calculated with a minimum score of 0 and a maximum score of 48 points. As joint impairment was reported most frequently in the ankles, elbow and knees these were analyzed separately. Response was 70% in 2001, compared to 84% in 197219, 70% in 197821, 81% in 198522 and 78% in 199218. One hundred and ninety eight patients participated in all 5 surveys. Table 1 shows the characteristics of participants in each of the 5 surveys. The mean age of participants increased from 21 years (median 19; range, 0-74 years) in 1972 to 35 years (median 36; range, 0-90 years) in 2001. Table 1. Overview of characteristics of participants to the Hemophilia in the Netherlands studies obtained from self-reported data 1972 1978 1985 1992 2001 N 447 560 935 980 1066 age (years) 19 (0-74) 23 (0-70) 28 (0-85) 31 (0-84) 36 (0-90) Severity of hemophilia severe (<0.01 IU ml) 159 (36)* 245 (44) 384 (41) 387 (39) 420 (39) moderate (0.01-0.05 IU ml) 83 (19) 106 (19) 175 (19) 173 (18) 176 (17) mild (>0.05-0.40 IU ml) 172 (38) 138 (25) 376 (40) 420 (43) 470 (44) Type of hemophilia type A† 377 (84) 481 (86) 801 (86) 853 (87) 925 (87) Hereditary pattern sporadic hemophilia 112 (25) 128 (23) 237 (25) 195 (20) 246 (23) Values presented are medians( range) or number(%) *Of 33 patients severity was missing in 1972 †All other patients are patients with hemophilia B This was still somewhat lower than the mean age of Dutch men, which increased from 32 to 37 years over the same period. Of all participants in 2001, 39% had severe hemophilia, 17% had moderate hemophilia, and 44% had mild hemophilia. In 23% of patients, the genetic inheritance pattern of the disease was that of isolated hemophilia: they had no other family members with hemophilia. This proportion had remained constant over the 30 years spanned by the surveys. Thirty-eight percent of patients with severe hemophilia were isolated patients, and 13% of patients with moderate hemophilia were isolated patients.
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Sources: N/A, Doctoral Thesis