Haemophilia A carriers experience reduced health-related quality of life.
BACKGROUND
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.


OBJECTIVE
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 18-60 years. Control subjects were mothers of children with cancer who receive care at the Vanderbilt paediatric haematology-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.


RESULTS
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 vs. 90; P = 0.02) and 'General health' (75 vs. 85; P = 0.01) compared to control subjects.


CONCLUSIONS
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.
Vanderbilt University, Nashville, TN, USA.
L
L
Gilbert
Vanderbilt University, Nashville, TN, USA.
A
A
Paroskie
Vanderbilt University, Nashville, TN, USA.
D
D
Gailani
Vanderbilt University, Nashville, TN, USA.
M R
MR
Debaun
Children's Healthcare of Atlanta/Emory University, Atlanta, GA, USA.
R F
RF
Sidonio
D000293:Adolescent; D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006262:Health; D006467:Hemophilia A / Q000150:complications / Q000235:genetics*; D006470:Hemorrhage / Q000150:complications; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D055815:Young Adult
Rand 36-item Health Survey 1.0; factor VIII deficiency; haemophilia carrier; health-related quality of life; short form 36 (SF-36); women with bleeding disorders