ORIGINAL ARTICLE Carriers of haemophilia Qualitative assessment of the emotional and behavioural responses of haemophilia A carriers to negative experiences in their medical care N. K. RENAULT,* R. E. HOWELL,  K. S. ROBINSONà and W. L. GREER* *Department of Pathology, QEII Health Sciences Centre and Dalhousie University;  Department of Oral and Maxillofacial Sciences, Dalhousie University; and àDivision of Hematology, QEII Health Sciences Centre, Halifax, NS, Canada Summary. Previous discussions with haemophilia A (HA) carriers suggested that carriers may experience inappropriate care, resulting in poor relationships with healthcare providers (HCPs; principally physicians and nurses), and unfortunate and extreme emotional and behavioural responses. This was a qualitative study to explore medical experiences of HA carriers and their emotional and behavioural responses. Eleven HA carriers and five Haemophilia Treatment Centre nurses were interviewed. Themes were identified using QSR NVivo 8.0. Carriers and nurses reported HA-related bleeding symptoms in carriers, including life-threatening haemorrhage following injury or medical intervention. Menorrhagia was common and distressing. Negative carrier experiences were related in the determination of genotypic and phenotypic status, management, pre- cautions and HCP attitude, including dismissing carriers’ symptoms, concerns or requests for care. Carriers res- ponded with mistrust, lost confidence, disappoint- ment, fear, anxiety, doubt of self or child, discussing experiences, avoidance of healthcare and self-treatment. Dismissive HCP attitudes, ignorance about bleeding disorders in women and unique aspects of the carrier population appear to make errors more likely. This study indicates that carriers experience inappropriate care and encounter dismissive attitudes, and respond emotionally and behaviourally. Our model suggests that systematic medical errors aggravate a negative feedback loop leading to negative emotional and behavioural responses and worsening carrier care. Improved carrier care poli- cies and increased awareness of women’s bleeding disor- ders may improve this situation. Further research is needed to determine whether the themes identified in this study accurately reflect the experiences of carriers in general. Keywords: behavioural responses, emotional responses, healthcare provider attitudes, quality of life, symptomatic haemophilia carriers, women’s bleeding disorders Introduction Haemophilia A (HA) is an X-linked disorder caused by mutations in the factor VIII (FVIII) gene (F8) that prolong clotting times. It is often viewed as a classical recessive disorder affecting males’ hemizygous for the mutation and females only when they have inherited two mutated F8 genes. Heterozygous, or carrier, females are expected to have FVIII activities of about half normal with normal blood coagulation. However, carriers can exhibit a range of FVIII activities [1] and disease severity [2]. We have previously shown that FVIII activities in carriers are directly related to X-chromosome inactiva- tion ratios [3], and can range from severely low (<2 IU dL )1 ) to high normal (>100 IU dL )1 ) among carriers within the same family. Carriers expressing severe HA (FVIII <2 IU dL )1 ; normal range 50– 200 IU dL )1 ) exhibit bruising [4], spontaneous bleeding [1], haemarthroses resulting in chronic haemophiliac arthropathy [4] and internal bleeding problems such as intra-abdominal haemorrhage [2], as well as female- specific symptoms such as menorrhagia [5] and excessive postpartum bleeding [6]. Carriers with FVIII activities ranging from 5 to 60 IU dL )1 can also experience bleeding symptoms [1]. Some may not bleed excessively with minor injury, but are at increased risk for menor- rhagia, postpartum haemorrhage and excessive bleeding after serious injury or medical intervention [1,6–8]. Postpartum haemorrhage has been reported in carriers with FVIII levels as high as 71 IU dL )1 [7]. Unfortunately, some healthcare professionals appear to maintain the narrow view of haemophilia as a classical X-linked recessive disease in which excessive Correspondence: Dr. Wenda L. Greer, Division of Hematology, QEII Health Sciences Centre, 5788 University Avenue, Mackenzie Building, Rm 223B, Halifax, NS, B3H 1V8, Canada. Tel.: +1 902 473 6691; fax: +1 902 473 4113; e-mail: w.greer@dal.ca Accepted after revision 3 September 2010 Haemophilia (2011), 17, 237–245 DOI: 10.1111/j.1365-2516.2010.02424.x Ó 2010 Blackwell Publishing Ltd 237