Development of a Multidisciplinary Clinic for Patients with Ehlers Danlos Syndromes: Considerations and Strategies

Abstract

Ehlers-Danlos Syndromes (EDS) is a group of connective tissue disorders often encountered within rheumatology clinics and is associated with several overlapping symptoms, which may require attention from other medical subspecialities. Barriers exist to implementing multidisciplinary care for EDS, including a lack of knowledge, comfort, and time managing EDS. In the absence of multidisciplinary care, patients are often forced to self-coordinate care. This can lead to gaps in care and a lack of clarity of medical ownership over the patient’s care. Integrated multidisciplinary clinics are sorely needed, but the development and implementation of such clinics is limited by resources and personnel. As such, the development of a multidisciplinary clinic can be daunting and may serve to discourage providers with competencies in EDS from attempting to develop this service. In this editorial, we share our experiences in developing a multidisciplinary clinic for EDS at a moderately-sized children’s hospital, relying on several core disciplines with established EDS clinical loads (ie, rheumatology, cardiology, genetics, and psychology). We discuss considerations for the expansion of this service, pitfalls, and barriers throughout the development of the clinic, and our rationale underlying our process-related decisions. Development of a greater number of multidisciplinary EDS clinics, even in the potential absence of larger institutional support, is both possible and imperative for improving EDS care globally.

Barriers to the Diagnosis, Care, and Management of Pediatric Patients With Ehlers-Danlos Syndrome in the United States: A Qualitative Analysis

“…Further, this study was conducted as an institution which has a multidisciplinary EDS clinic that serves the region; the availability of this resources may bias providers in both increased awareness and potentially a greater reliance on subspecialty care as a referral source. 31 We expect the themes reported in this study to be present and potentially more profound in areas without a multidisciplinary EDS clinic. Also, this sample includes a large number of trainees, which are earlier in their career, and they may be more interested to participate in EDS care compared to established clinicians that may have less availability or interest.…”

“…Multidisciplinary care has been recommended in the management of patients with EDS due to the complexity and number of comorbidities that can be associated with EDS. 30 Additionally, there are multiple reports of how to development a multidisciplinary clinic and provide care for patients with EDS 31,32 ; however, these options are not available in all areas, which may lead to diagnostic and management delays. Patients and families with EDS do report a willingness to travel to obtain subspecialty or EDS-specific care, 24 but this comes at a significant time and cost-burden to families and is not a sustainable solution to improve consistent and available care and management of patients with EDS.…”

Patient and Parent Knowledge, Understanding, and Concerns After a New Diagnosis of Ehlers Danlos Syndrome

“…Previous studies have shown that many providers are uncomfortable with diagnosis, care, and management of patients with EDS and note the existence of many barriers that prevent adequate care 11 , 12 . For this reason, multidisciplinary teams with EDS expertise have been developed 13 , 14 to aid in diagnosis, care and management with appropriate referrals and education to prevent new or worse symptoms that may lead to poor quality of life (QoL). Despite the increase in multidisciplinary EDS clinics there is still a limited number of medical providers familiar with EDS to service the population needs, which further contributes to long delays in diagnosis and treatment and may limit the transmission of empirical medical information to patients and families.…”

“…It regularly involves referrals to many specialists, but patients report being provided with few definitive answers, which leads to negative outcomes such as mistrust in medical providers 16 . After diagnosis, patients and families are typically provided EDS focused education on diagnosis and symptoms 13 , however, there are no standard recommendations on when EDS education should occur or what it should include. The timeframe between diagnosis and initiation of active management may be a “window of opportunity” to appropriately educate families, identify age-specific needs and concerns, build trust and confidence, and develop age-appropriate care strategies that could lead to improved outcomes and QoL.…”