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


OBJECTIVE
The aim of this study was to assess HRQOL in carriers of severe and moderate haemophilia with and without increased bleeding tendency.


METHODS
One 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.


RESULTS
There 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).


CONCLUSIONS
We 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.
Department of Haematology and Coagulation Disorders, Sahlgrenska University Hospital, Gothenburg, Sweden.
A
A
Olsson
Department of Antenatal Care, Närhälsan, Primary Care, Västra Götaland, Sweden.|Institute of Clinical Science, Department of Obstetrics and Gynaecology, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
M
M
Hellgren
Lund University, Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden.
E
E
Berntorp
Department of Haematology and Coagulation Disorders, Sahlgrenska University Hospital, Gothenburg, Sweden.
F
F
Baghaei
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016022:Case-Control Studies; D003430:Cross-Sectional Studies; D005260:Female; D006262:Health; D006467:Hemophilia A / Q000150:complications* / Q000235:genetics*; D002836:Hemophilia B / Q000150:complications* / Q000235:genetics*; D006470:Hemorrhage / Q000150:complications*; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D055815:Young Adult
bleeding score; bleeding tendency; carrier of haemophilia; haemophilia A; health-related quality of life; short form 36