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Author Notes:

Correspondence Robert F. Sidonio Jr. MD, MSc; Division of Pediatric Hematology-Oncology, Department of Pediatrics; Emory University School of Medicine; 1760 Haygood Drive, Health Sciences Research Building, Suite 340, Atlanta GA 30322; 404-727-2846 (office) robert.sidonio.jr@emory.edu.

Authors 1, 2, and 5 designed the study.

Author 1 analyzed the data.

Authors 1 and 5 wrote the manuscript.

Author 4 oversaw the design and study completion.

Authors 2, 3, and 4 contributed to editing and revision of the manuscript.

Conflicts of Interest: Robert Sidonio, Jr. has participated in advisory boards for Grifols, CSL Behring, Baxter and Kedrion. Dr. Sidonio has an investigator-initiated study with Grifols. The authors stated that they had no interests, which might be perceived as posing a conflict or bias.


Research Funding:

Funding partially supported by The Vanderbilt Clinical & Translational Research Scholars (VCTRS) KL2 Program, the Vanderbilt CTSA grant UL1 RR024975 from NCRR/NIH, by a Conducting Research in Pediatric Hematology-Oncology training grant NIH 5T32CA154267-03, and by an investigator-initiated grant from Grifols.


  • Science & Technology
  • Life Sciences & Biomedicine
  • Hematology
  • factor VIII deficiency
  • haemophilia carrier
  • health-related quality of life
  • Rand 36-item Health Survey 1.0
  • short form 36 (SF-36)
  • women with bleeding disorders

Haemophilia A carriers experience reduced health-related quality of life


Journal Title:



Volume 21, Number 6


, Pages 761-765

Type of Work:

Article | Post-print: After Peer Review


Introduction: Haemophilia A is an X-linked recessive bleeding disorder that primarily affects males. Emerging data support evidence for increased bleeding in female haemophilia A carriers despite factor VIII activity within the normal range. Aim: Data regarding the effect of increased bleeding on health-related quality of life (HR-QOL) in haemophilia A carriers is sparse. We tested the hypothesis that haemophilia A carriers have reduced HR-QOL related to bleeding symptoms. Methods: We conducted a cross-sectional study at Vanderbilt University. Case subjects were obligate or genetically verified haemophilia A carriers age 18 to 60 years. Control subjects were mothers of children with cancer who receive care at the Vanderbilt pediatric hematology-oncology clinic. Trained interviewers administered the Rand 36-Item Health Survey 1.0, a validated questionnaire evaluating eight health concepts that may affect HR-QOL, to each study participant. Mann-Whitney U tests were used to compare median scores for the eight health domains between the case and control groups. Result: Forty-two haemophilia A carriers and 36 control subjects were included in analyses. Haemophilia A carriers had significantly lower median scores for the domains of “Pain” (73.75 versus 90; p= 0.02) and “General health” (75 versus 85; p= 0.01) compared to control subjects. Conclusion: Haemophilia A carriers in our study demonstrated significantly lower median scores on the Rand 36-item Health Survey 1.0 in the domains of “Pain” and “General Health” compared to women in the control group. Our findings highlight the need for further investigation of the effect of bleeding on HR-QOL in this population.

Copyright information:

© 2015 John Wiley & Sons Ltd.

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