Haemophilia Carriers Experience Study (CARES): A mixed method exploration into the experience of women who are carriers of Haemophilia

Abstract

Introduction Haemophilia research has traditionally focused on patients diagnosed with haemophilia and although research priorities are rapidly changing, there is still a lot more we need to understand about the experiences and psychosocial issues facing women who are diagnosed as carriers of haemophilia (Haemophilia, https://doi.org/10.1111/hae.14043). One study noted that the understanding of carriers’ experience of bleeding by healthcare professionals is limited and that many women have had negative experiences of healthcare (Haemophilia, 17, 2011, 237). The carrier population does not typically receive much support for themselves as individuals as they are often not registered at Haemophilia Centres in their own right. Aim This study aimed to look at the emotional wellbeing of carriers in more detail. Method This was initially investigated through the use of focus groups and individual interviews with 16 participants (Stage 1) and then widening the study using an online questionnaire battery developed from the themes identified from these interviews (Stage 2). The questionnaire battery was completed by 226 participants. Results Descriptive statistics are reported on the endorsement of themes identified in Stage 1 and around participants’ experiences of their carriership and healthcare. Results demonstrated that the participants have had a number of difficulties with accessing helpful information and support during key times in their lives, for example, at diagnosis and when deciding whether to start a family. They also showed that although participants endorsing a higher number of bleeding symptoms scored statistically significantly higher in measures of depression, anxiety and negative affect, this difference was not clinically significant. Conclusion These results lend support to the growing evidence base that women who are carriers of haemophilia have a distinct set of (currently unmet) needs that need assessing and treating.

Challenges and knowledge gaps facing hemophilia carriers today: Perspectives from patients and health care providers

“…Although the number of hemophilia carrier participants in focus groups was small (11 carriers in two groups), this sample size is in line with other similar qualitative studies that had 11–15 participants 16,22 . Similarly, our study design of in‐depth qualitative work via focus groups, supplemented by questionnaires for both carriers and health care providers, has been used in many similar studies 14,15,22,26 . Another strength of our study is the wide age range of hemophilia carrier participants, 21–69 years of age, as women's experiences of being a hemophilia carrier has been documented as a process that evolves over time 30 .…”

“…16,22 Similarly, our study design of in-depth qualitative work via focus groups, supplemented by questionnaires for both carriers and health care providers, has been used in many similar studies. 14,15,22,26 Another strength of our study is the wide age range of hemophilia carrier participants, 21-69 years of age, as women's experiences of being a hemophilia carrier has been documented as a process that evolves over time. 30 An important consideration to note is that our results relate to the situation in a high resource setting with a specific HTC organization model, publicly funded health care system and represents the Canadian point of view.…”

“…6,12,13 There is a growing evidence base that women who are carriers of hemophilia have a distinct set of unmet needs that require targeted interventions and initiatives. [14][15][16] Although our knowledge of abnormal bleeding in carriers has greatly evolved, the remaining barriers result in suboptimal care, missed and untreated bleeding symptoms, and many unidentified hemophilia carriers. The true prevalence of hemophilia carriers is not known but is estimated that for every male with hemophilia there are three to five hemophilia carriers.…”

Preoperative diagnosis and management of inherited bleeding disorders in female adolescents and adults

“…The clinical issues of bleeding severity, determination of the patient’s individual risk related to surgery and pregnancy, disease transmission, testing and reproductive decisions contribute to health-related quality of life. 4 – 17 Compared to females without IBDs, those with IBDs have lower values for bleeding control, vitality, and physical and social functioning, and increased pain (joint bleeding/heavy menstrual bleeding) and psychosocial problems (guilt, anxiety, depression, work and school absenteeism, isolation and difficult social relationships). 4 – 17 Women’s occupational opportunities are substantially affected, related to the person’s disease severity and, in the case of familial hemophilia, the potential need to undertake a role as caregiver.…”

“… 4 – 17 Compared to females without IBDs, those with IBDs have lower values for bleeding control, vitality, and physical and social functioning, and increased pain (joint bleeding/heavy menstrual bleeding) and psychosocial problems (guilt, anxiety, depression, work and school absenteeism, isolation and difficult social relationships). 4 – 17 Women’s occupational opportunities are substantially affected, related to the person’s disease severity and, in the case of familial hemophilia, the potential need to undertake a role as caregiver. 4 – 17 Patient access to specialist care is commonly reported as an obstacle.…”