Hemophilia a Carriers Experience Errors and Deficiencies in Care Leading to Emotional and Behavioral Responses That Could Further Compromise Their Care

Introduction Through previous studies and conferences, it was noted that medical errors perceived by haemophilia A (HA) carriers often resulted in poor relationships with health care providers (HCPs; principally physicians and nurses), and lead to reports of unfortunate and extreme emotional and behavioural responses. To explore the range medical experiences and responses of HA carriers, we undertook this qualitative study. M&M Interested members of the Canadian Haemophilia Society participated in semi-structured interviews. Nine adult interviewees (8 HA carriers, 1 non-carrier) discussed 11 carriers (7 adults, 4 children). A qualitative review exploring themes from anonymized interview transcripts was done using QSR NVIVO 8.0. Transcripts were coded for carrier symptoms, medical care experiences, perceived correctness of care, attitude of the caregiver, and emotional and behavioural responses.

Results Nine of 11 carriers had experienced serious bleeds such as spontaneous bleeds, excessive post-operative or post-partum bleeding, or joint bleeds following mild to moderate trauma. Ten carriers had experienced symptoms of excess bleeding. All adult carriers reported heavy or prolonged menstrual bleeding. However, only 3 were reported as registered in a haemophilia treatment centre, and only one was regularly followed. Three carried factor first cards and four wore medic alert bracelets. 153 medical care experiences were discussed including 78 described as errors or deficiencies in care. HCP attitude was described as negative in 38 of 81 experiences. Interviewees felt that their concerns were trivialized, symptoms down-played, and they were not believed. In response, interviewees relayed deep feelings of disappointment, frustration, desperation, and complete loss of respect for the medical system. Other symptomatic carriers expressed self-doubt. Most often expressed was mistrust, disappointment, or loss of confidence in HCPs (8 of 9 interviewees, 46 instances), or anger (8 of 9 interviewees, 33 instances). Fear or anxiety was also commonly relayed (7 of 9 interviewees, 32 instances). Self-advocacy was the most common behavioural response (9 of 9 interviewees, 49 instances). Extreme behaviour included complete avoidance of the healthcare system (14 instances), and diagnosing and treating serious bleeds without medical supervision. Interviewees believed there was insufficient knowledge about bleeding disorders in women among HCPs (5 of 9 interviewees), insufficient knowledge about gynaecological norms among HCPs and patients (3 of 9), reticence of carriers to discuss personal gynaecological problems (2 of 9), and down-playing by carriers of their own symptoms as compared to more severely affected males (4 of 9). Interviewees often related errors in the care of family members and non-family members. Experiences shared within and among families affirmed their concepts of appropriate care and reinforced perceptions of inadequate care.

Conclusions The bleeding disorder that affects HA carriers is a unique issue of women and children. HA carriers can experience serious bleeds and report numerous errors in medical management and negative attitudes toward their concerns. Their experiences often result in strong emotional responses, mistrust of the medical system, and some extreme and potentially detrimental behaviours. Solutions to these problems could include widely circulated management principles endorsed by both HCP and HA carrier groups, improved communication and education, registration of carriers in haemophilia treatment centres or women’s bleeding disorder clinics, and improved communication with the wider haemophilia community to rebuild a positive relationship of trust with the medical care system.