They’ve been BITTEN: reports of institutional and provider betrayal and links with Ehlers-Danlos Syndrome patients’ current symptoms, unmet needs and healthcare expectations

Abstract

Introduction: Patients with rare and/or care-intensive conditions, such as Ehlers-Danlos Syndrome (EDS), can pose challenges to their healthcare providers (HCPs). The current study used the BITTEN framework1 to code EDS patients’ open-ended written responses to a needs survey to determine their self-reported prevalence of healthcare institutional betrayal and its link with their expressed symptoms, provider perceptions, unmet needs, and on-going healthcare-related expectations. Methods: Patients with EDS ( n = 234) were recruited via a rare disease electronic mailing list and snowball sampling. A total of one-hundred and six respondents (45.3%) endorsed having unmet healthcare-related needs; of these, 104 (99%) completed an open-ended prompt about these needs. Responses were coded for components of BITTEN, a framework designed to link patients’ past, current, and future healthcare-related experiences in a trauma informed manner. Results: Many respondents with ongoing needs endorsed experiencing past institutional and provider betrayal (43%; n = 45), current mental health symptoms (91.4%; n = 95), negative expectations for future healthcare (40.4%; n = 62), and a lack of trust in their healthcare provider (22.1%; n = 23). There were no significant differences in post-traumatic stress disorder (PTSD)/anxiety, depression/sadness, or isolative symptoms between respondents coded for institutional betrayal ( n = 45) compared with those not ( n = 59). However, EDS respondents reporting institutional betrayal were significantly more likely to self-report anger and irritability symptoms, a lack of trust in their HCPs, and more negative expectations for future healthcare than those not reporting institutional betrayal. Discussion/conclusions: The frequent spontaneous reporting of past healthcare betrayals among patients with EDS implies the need for trauma-informed care and provider education. Given that experiences of institutional betrayal are associated with increased anger and irritability, as well as with negative expectations for future healthcare interactions, efforts to repair healthcare provider and system-wide relationship ruptures might have positive healthcare consequences. Plain language summary Reports of Institutional and Provider Betrayal and Links with Ehlers-Danlos Syndrome Patients’ Current Symptoms, Unmet Needs and Future Healthcare Expectations What is EDS? Ehlers-Danlos Syndrome (EDS) refers to a group of rare genetic connective tissue disorders that are primarily characterized by skin hyperelasticity, joint hypermobility, and tissue fragility. Connective tissue is largely responsible for the structural integrity of our bodies, and there are several EDS subtypes which each describe a specific connective tissue problem. In addition, there is significant overlap between EDS types and other kinds of connective tissue disorders. As a result, recognizing, diagnosing, and treating EDS is often challenging. What is Institutional betrayal? Institutional Betrayal here refers to a harmful action (i.e. commission) or lack of action (i.e. omission) on the part of a healthcare institution, individual provider/healthcare team, or insurance company. When a patient trusts that the healthcare system will act in their best interest, and trust is violated, institutional betrayal occurs. What is BITTEN? BITTEN is an acronym for Betrayal, Indicator, Trauma symptoms, Trust, Expectations, and Needs. It is a framework meant to capture previous problematic healthcare-related experiences in EDS patients, then to consider how those experiences influence a patient’s current symptoms, provider trust, future expectations in healthcare encounters, and on-going needs. Why was this done? EDS, like many rare diseases, is hard to recognize and manage. We aim to: Give voice to EDS patients and their common unmet needs and healthcare-related expectations. Highlight how healthcare providers can apply BITTEN to improve care practices in rare disease patient encounters. What did we do? Using a newly articulated applied model of healthcare, BITTEN, we analyzed the open-ended responses of EDS patients describing their unmet emotional and mental health needs. What did we find? Nearly half of EDS patients who indicated they had unmet needs reported experiencing institutional betrayal. EDS patients who reported institutional betrayal also expressed anger, a lack of trust in healthcare providers, negative expectations for future healthcare, and more unmet needs more frequently than EDS patients who did not report institutional betrayal. What does this mean? The EDS patients in this sample were not directly asked if they had experienced institutional betrayal, so the exact prevalence is not known. Furthermore, responses were obtained voluntarily via the internet, so caution should be taken when generalizing these findings. However, results indicate that too many patients with EDS have experienced healthcare betrayals; these experiences are associated with current anger and negative expectations for future healthcare interactions. The prevalence of past negative healthcare experiences, along with current unmet needs and future negative healthcare expectations in EDS patients who have experienced institutional betrayal, highlights the need for healthcare providers to tend to these experiences, mend patient-provider barriers, and provide higher quality healthcare.

Healthcare experiences among adults with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder in the United States

“…The second most common theme was negative attitudes from healthcare professionals, including being dismissed, ignored, belittled, and blamed. Others have described this as "healthcare-related institutional betrayal" because it involves a violation of the patient's trust that healthcare providers will act in the patient's best interest [17]. Accessibility/convenience and financial aspects, two themes derived from the PSQ-18, followed negative attitudes as the most reported themes.…”

“…In one small mixed-methods study of 28 patients with hEDS and HSD, participants reported poor quality physical therapy for joint hypermobility issues [16]. In another mixed-methods study (N = 234), almost half of the participants (45%) reported having unmet medical needs, and nearly half of those reported experiencing institutional betrayal, including professionals' lack of knowledge or willingness to learn about EDS [17]. Finally, in a recent study examining satisfaction with chronic illness care among HSD and EDS patients from both the US and Canada, participants had scores below the midpoint on almost all scales assessing chronic illness care (e.g., goal setting, follow-up/ coordination) [18].…”

“…Responses were coded using directed content analysis [28]. An initial set of six codes (i.e., professional awareness/education, diagnostic process, negative attitudes, coordination of care, treatment, given psychological explanations) were developed using themes found in a systematic review of nine studies [29] and three additional qualitative studies not in the review [2,15,17]. Codes for the accessibility/convenience and financial aspects themes from the PSQ-18 and an "other" code were also included.…”

Resource utilization and multidisciplinary care needs for patients with Ehlers–Danlos syndrome

“…Findings from this study suggest that there are several specialties that diagnose EDS/HSD, and similar to previous reports, health care utilization among adults and minors with EDS/HSD is high due to the many comorbidities that occur. Many respondents obtain care from several subspecialties across many medical institutions which may lead to fractured, poorly coordinated care that does not meet the patient's needs, and leads to lack of trust in healthcare providers and negative expectations for healthcare needs (Langhinrichsen-Rohling et al, 2021). This represents a unique opportunity to develop specialized multidisciplinary care for adults and minors with EDS/HSD, that can address their specific needs and provide a foundation to better understand the patients' needs and develop better, coordinated care to meet the needs of those with EDS/ HSD.…”

Healthcare utilization among youth with Ehlers–Danlos syndrome hypermobile type

“…In line with management of pediatric chronic conditions, the HCU pattern observed in the current study supports a shift from diagnostic to rehabilitative care over time; HCU became more treatment focused from prediagnosis to postdiagnosis, following current clinical recommendations for this population. Patients with EDS-HT often report experiencing a lack of awareness from healthcare professionals and difficulty accessing appropriate treatment in the healthcare system (Langhinrichsen-Rohling et al, 2021;Terry et al, 2015). Thus, creating efficient processes for referral, diagnostic evaluation, education for families, and prioritization of treatment services remains an ambitious but necessary goal.…”