Association between bleeding tendency and health-related quality of life in carriers of moderate and severe haemophilia

IntroductionCarriers of severe and moderate haemophilia A and B are expected to have approximately 50% of the normal level of factors VIII and IX. However, due to X chromosome inactivation in early embryonic life, factor levels can vary considerably. This can lead to increased bleeding tendency, which may in turn impact on health-related quality of life (HRQOL). AimThe aim of this study was to assess HRQOL in carriers of severe and moderate haemophilia with and without increased bleeding tendency. MethodsOne hundred and twenty-four adult carriers and 90 controls were recruited. Bleeding tendency was evaluated using a structured bleeding assessment tool. HRQOL was measured by the short form 36 (SF-36) questionnaire. The SF-36 scores were compared with Swedish normative age-matched data and reported as Z scores. ResultsThere was no significant difference between the whole groups of carriers and controls in the Z scores of the eight SF-36 domains. The mental component summary (MCS) was lower in carriers, compared with controls (P=0.048). The subgroup of carriers with an increased bleeding tendency had significantly lower Z scores compared to controls regarding the General Health (P=0.008), the Social Functioning (P=0.040) and the Mental Health (P=0.048) domains. The MCS was significantly lower in this carrier subgroup than in controls (P=0.033). ConclusionWe conclude that the subgroup of carriers of haemophilia with increased bleeding tendency have impaired HRQOL. The SF-36 results indicate that this condition affects mental rather than physical health.