Abstract
BackgroundThis study was undertaken to explore the longitudinal patterns of health-related quality of life (HRQoL) among youth and young adults with Hemophilia A (HA) over a 3-year period. This report presents the baseline characteristics of the study cohort.MethodsMales, 14 to 29 years of age, with predominantly severe HA were recruited from six treatment centres in Canada. Subjects completed a comprehensive survey. HRQoL was measured using: the CHO-KLAT2.0 (youth), Haemo-QoL-A (young adults) and the SF-36v2 (all).Results13 youth (mean age = 15.7, range = 12.9-17.9 years) and 33 young adults (mean age = 23.6; range = 18.4 -28.7 years) with moderate (7 %) and severe (93 %) HA were enrolled. All were on a prophylactic regimen with antihemophilic factor (Helixate FS®) during the study. The youth had minimal joint damage (mean HJHS = 5.2) compared to young adults (mean HJHS = 13.3). The mean HRQoL scores for youth were: 79.2 (SD = 11.9) for the CHO-KLAT, and 53.0 (5.5) and 52.3 (6.8) for the SF-36 Physical Component Summary (PCS) and Mental Component Summary (MCS) scores respectively. The mean HRQoL scores for young adults were: 85.8 (9.5) for the Haemo-Qol-A, and 50.8 (6.4) and 50.9 (8.8) for PCS and MCS respectively. PCS and MCS scores were comparable to published Canadian norms, however significant differences were found for the domains of Physical Functioning and Bodily Pain. The disease-specific HRQoL scores were weakly correlated with the PCS for youth (CHO-KLAT vs. PCS r = 0.28, p = 0.35); and moderately correlated for the MCS (r = 0.39, p = 0.19). Haemo-QoL-A scores for young adults were strongly correlated with the PCS (r = 0.53, p = 0.001); and weakly correlated with the MCS (r = 0.26, p = 0.13). Joint status as assessed by HJHS was correlated with PCS scores. A history of lifelong prophylaxis resulted in better PCS but worse MCS scores.ConclusionDespite having hemophilia, the youth in this cohort have minimal joint disease and good HRQoL. The young adults demonstrated more joint disease and slightly worse HRQoL in the domains of physical functioning and pain. The data presented here provide new information to inform the selection of Health Related Quality of Life (HRQoL) instruments for use in future clinical trials involving persons with hemophilia.Trial registrationClinicalTrials.gov : NCT01034904. Study funded by CSL Behring Canada.Electronic supplementary materialThe online version of this article (doi:10.1186/s12878-016-0052-x) contains supplementary material, which is available to authorized users.
Highlights
This study was undertaken to explore the longitudinal patterns of health-related quality of life (HRQoL) among youth and young adults with Hemophilia A (HA) over a 3-year period
Hemophilia Joint Health Score (HJHS) scores ranged from 0 to 17 in the youth and 0 to 34 in the young adults, where no joint damage is indicated by a score of 0
These results should be interpreted with caution given the small number of subjects involved and the difference in age between the two categories of subjects. They are intriguing given that we identified Role Emotional and Mental Health domains as areas of strength in our hemophilia cohort compared to Canadian norms
Summary
This study was undertaken to explore the longitudinal patterns of health-related quality of life (HRQoL) among youth and young adults with Hemophilia A (HA) over a 3-year period. Health-related quality of life (HRQoL) tools are self-administered questionnaires developed in order to measure the perceived impact of a medical condition and its treatment on a person’s physical, emotional and social well-being. In hemophilia both generic and disease-specific HRQoL questionnaires have been used in recent years seldom simultaneously in the same subjects. Significant variations in HRQoL may not be determined only by change in physical status but may be influenced by common social events such as educational, vocational or relational changes associated with transition from adolescence to adulthood
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