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dc.contributor.authorYoung, Jane
dc.contributor.otherQueen's University (Kingston, Ont.). Theses (Queen's University (Kingston, Ont.))en
dc.date2015-11-17 14:21:27.992en
dc.date.accessioned2015-11-18T20:25:29Z
dc.date.available2015-11-18T20:25:29Z
dc.date.issued2015-11-18
dc.identifier.urihttp://hdl.handle.net/1974/13835
dc.descriptionThesis (Master, Pathology & Molecular Medicine) -- Queen's University, 2015-11-17 14:21:27.992en
dc.description.abstractHemophilia A and B are X-linked deficiencies of coagulation factor VIII and IX respectively. Males are affected, while females are carriers of the disease. Recently, it has become clear that more than a third of hemophilia carriers (HC) are symptomatic. Carriers may experience prolonged bleeding postpartum, following trauma and surgery as well as menorrhagia. This is the first research to incorporate both a bleeding assessment tool, to assist in objectively quantifying severity of bleeding symptoms, as well as a validated quality of life (QoL) questionnaire to facilitate an understanding of the relationship between bleeding symptoms and QoL. The objectives for this study are to validate a self-administered bleeding assessment tool (Self-BAT) as a screening tool to accurately identify HC with low factor levels (FL) and abnormal bleeding symptoms, to measure QoL of HC using the SF-36v2 and describe its relationship to bleeding severity and FL, and to investigate variability in FL. To date, 59 HC have been enrolled. A positive or abnormal Self-BAT bleeding score (≥6) has a sensitivity of 83%, specificity of 42%, a positive predictive value (PPV) of 0.48 and a negative predictive value (NPV) of 0.79 for the identification of HC with low FL. Analysis of QoL data shows an inverse correlation between QoL and BS (r=-0.52, p<0.0001). No significant relationship is observed between QoL and FL. This study found skewed X-inactivation ratio (XIR) to be significantly more prevalent in this population than has been observed in the general population. This suggests that determination of XIR may be useful in predicting risk of deficient FL. This study validates the Self-BAT as an effective tool to identify HC with low FL and/or abnormal bleeding symptoms. For HC, Self-BAT BS is a more accurate measure of bleeding severity and resultant QoL than FL. HC will benefit from treatment strategies aimed at controlling bleeding symptoms which will facilitate improvements in the management of these patients overall.en_US
dc.languageenen
dc.language.isoenen_US
dc.relation.ispartofseriesCanadian thesesen
dc.rightsQueen's University's Thesis/Dissertation Non-Exclusive License for Deposit to QSpace and Library and Archives Canadaen
dc.rightsProQuest PhD and Master's Theses International Dissemination Agreementen
dc.rightsIntellectual Property Guidelines at Queen's Universityen
dc.rightsCopying and Preserving Your Thesisen
dc.rightsCreative Commons - Attribution - CC BYen
dc.rightsThis publication is made available by the authority of the copyright owner solely for the purpose of private study and research and may not be copied or reproduced except as permitted by the copyright laws without written authority from the copyright owner.en
dc.subjectHemophilia Carriers, Quality of Life, X-inactivation, FVIII, FIX, Bleeding Assessment Toolen_US
dc.titleVALIDATION OF THE SELF-BAT (SELF-ADMINISTERED BLEEDING ASSESSMENT TOOL) AND EVALUATION OF DETERMINANTS OF HEALTH IN HEMOPHILIA CARRIERSen_US
dc.typethesisen_US
dc.description.degreeMasteren
dc.contributor.supervisorJames, Paulaen
dc.contributor.departmentPathology and Molecular Medicineen


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