EDS Patients, Caregivers Support Multidisciplinary Care Teams: Survey

Treatment plans for patients suffer from poor integration, resulting in heavy burden

Lindsey Shapiro, PhD avatar

by Lindsey Shapiro, PhD |

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Most families affected by Ehlers-Danlos syndrome (EDS) feel their medical care needs are not being met, according to a survey of patients and caregivers in the midwestern U.S.

Survey respondents believe they or their child would benefit from an integrated and multidisciplinary team, with an ideal approach including pain management, physical therapy, rheumatology, and cardiology, among other specialties. And more than half said they were willing to travel at least an hour to receive such care, with 15% saying they’d be willing to fly.

The survey findings provide “a framework for a multidisciplinary clinic model that is endorsed by those with EDS,” the researchers wrote.

The study, “Resource utilization and multidisciplinary care needs for patients with Ehlers-Danlos syndrome,” was published in Molecular Genetics & Genomic Medicine.

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EDS encompasses a group of disorders broadly characterized by unusually mobile, or hypermobile, joints and soft, stretchy skin. Patients may also have a wide range of other symptoms, including, but not limited to, chronic pain, heart and digestive problems, musculoskeletal and skin issues, and neurological and skin problems.

These increase the disease burden and often require patients to see a number of different medical specialists to help them manage their condition.

Treatment plans from each specialist may be poorly integrated with one another and managing these various avenues of care falls on patients and families, however, increasing their overall burden.

“To address the many [coexisting health conditions] and high disease burden in those with EDS, multidisciplinary care has been proposed to help facilitate coordination of care, maximize treatment, and reduce medical visits,” the researchers wrote.

Multidisciplinary care is an approach wherein different types of doctors coordinate with one another to integrate components of a patient’s care.

Learning about the EDS patient spectrum

Still, “it is unclear which specialties are diagnosing EDS, being utilized by those with EDS and what those with EDS feel they need as part of a multidisciplinary care model that can address their needs,” wrote the researchers in Kansas, who developed and sent an electronic survey to adults with EDS or hypermobility spectrum disorder (HSD) and parents of minors with these conditions in order to learn more.

HSD is often the diagnosis for people with hypermobile joints, but who don’t fulfill other EDS criteria.

The survey was sent to people belonging to EDS support groups within 200 miles of a midwestern U.S. metropolitan area between March and June 2019.

Responses regarding 368 patients — 206 adults and 162 minors — were included in the analysis. Notably, 16% of the adults and 29% of the children were currently under evaluation for EDS or HSD and did not yet have a formal diagnosis.

Hypermobile EDS was the most common diagnosis among both adults (63%) and children (62%). HSD was the next most common diagnosis, accounting for 8% of the entire study group. While these were most common, “almost all EDS subtypes were represented,” the researchers wrote.

Nearly half (46%) of all diagnoses were made with genetic testing, but a diagnosis by a rheumatologist, who studies joints, muscles, and ligaments, was also relatively common, accounting for 23% of diagnoses.

Responses showed nearly half (48%) of patients attended one hospital or clinic for their care, while others attended two (23%), three (10%), four (8%) or more (11%).

Among the 155 adults with an EDS/HSD diagnosis, the most common types of visits included cardiology (12%), neurology (8%), gastroenterology (7%), and emergency (7%). For the 83 children with a diagnosis, the most common types were rheumatology (13%), cardiology, neurology, and genetics (11% each).

“The more commonly utilized subspecialty care may represent a place to implement education about EDS/HSD to improve care and outcomes, but also may be ideal specialties to target for multidisciplinary care,” the research team wrote.

Unmet medical care needs

Two-thirds of respondents (67%) reported their HSD/EDS medical care needs were not being met, while fewer answered maybe (23%) or yes (10%). Most (87%) said they’d benefit by receiving care from a multidisciplinary team. Respondents most often noted their ideal team would include pain management (84%), physical and occupational therapy (84%), rheumatology (82%), cardiology (81%), gastroenterology (76%), and neurology specialties (75%).

Many other specialties were also listed among the needs for multidisciplinary care, including genetics, orthopedics, immunology, gynecology, and psychiatry.

About 60% felt primary care (family medicine, internal medicine, and pediatrics) would be important in this multidisciplinary team.

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“However, primary care was poorly utilized for care by those with EDS/HSD and the emergency department had higher utilization than primary care based on responses,” the researchers wrote. “This paradigm may arise from poor awareness, insufficient knowledge, or discomfort from primary care clinicians in managing those with EDS/HSD.”

When asked how far they were willing to travel to obtain multidisciplinary care, the largest proportion of respondents said 30–60 minutes (34%), followed by one to two hours (22%) two or more hours (22%), and 15 minutes or less (8%). Another 15% said they’d be willing to fly to obtain care.

These findings emphasize that many patients “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,” the researchers wrote.

While multidisciplinary care offers many advantages for patients, there will undoubtedly be challenges to implementing it, such as “coordination of multiple services, interest and knowledge of specific conditions, and financial burdens,” they said, noting nevertheless that “this represents a unique opportunity to develop specialized multidisciplinary care for adults and minors with EDS/HSD …” that would “provide a foundation” to develop better coordinated care models and better understand patient needs.

The team also noted their findings may not be generalizable to adults and children with EDS/HSD in different regions.