Objective:
To characterize the one-year bleeding pattern and assess the burden of bleeding among persons with hemophilia B, a subgroup of individuals with hemophilia that is frequently under-represented in population-based and health services surveillance.
Methods:
Hemophilia Utilization Group Study Part Vb (HUGS-Vb) collected prospective information about bleeding episodes, healthcare utilization, and burden of illness among persons with hemophilia B who obtained comprehensive care at one of ten hemophilia treatment centers in eleven geographically diverse states. Participants completed an initial interview and quarterly follow-up surveys. This analysis reports on baseline and one-year follow-up data from 93 participants. Wilcoxon-Mann-Whitney tests were used to determine the differences among subgroups. Spearman correlation coefficients (rho) were used to assess the relationship between bleeding episodes and utilization of health-related care.
Results:
Of the 93 participants, 50 (54%) were children. Forty-four (47%) participants (24 children) had severe hemophilia B, of whom 26 (59%) (16 children) reported using prophylactic therapy at initial interview. Fifty-eight (62%) participants reported having at least one bleeding episode within the one-year follow-up period. Mean number of bleeding episodes among participants with mild, moderate or severe hemophilia were 2.11±2.75, 3.47±6.25 and 6.53±7.66, respectively. Participants with severe hemophilia on prophylactic therapy had significantly (p=0.0203) fewer bleeding episodes (4.67±6.31), compared to those on episodic therapy (9.22±8.76). Significantly fewer outpatient visits (rho=0.2831, p=0.0060) and emergency room visits (rho=0.2810, p=0.0064) were reported among participants with fewer bleeding episodes. Mean absent days from work/school due to hemophilia among all participants was 2.24±4.58, and it was 0.98±2.16 days among parents of children under 18 years. Increased bleeding episodes were positively associated with increased days absent from work/school among both participants (rho=0.4598, p<0.0001) and parents of children (rho=0.3433, p=0.0147). Moreover, Positive relationship were found between increased bleeding episodes and increased time spent on the telephone with hemophilia centers (rho=0.5399, p<0.0001), pharmacists (rho=0.3373, p=0.0009) and employer/school personnel (rho=0.2230, p=0.0317).
Conclusions:
The HUGS Vb study documents the considerable burden of illness imposed by frequent bleeding episodes on persons with hemophilia B. These episodes increase patient and caregiver absenteeism from work/school, due to increased healthcare utilization and work/school coordination. As participants on prophylactic therapy have significantly fewer bleeding episodes than those on episodic therapy, prophylaxis should be encouraged among persons with hemophilia.