Review highlights impact of uncertainty in hypermobile EDS

Patients with chronic pain say they feel dismissed by healthcare professionals

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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People with hypermobile Ehlers-Danlos syndrome (hEDS) often face uncertainty and feelings of dismissal from healthcare providers, a new review paper highlights.

The findings underscore how important it is for clinicians to have open and honest conversations with patients, building relationships to foster collaboration, and make patients feel empowered to take control of their own healthcare journey, researchers said.

The study, “A narrative review of the literature on illness uncertainty in hypermobile ehlers-danlos syndrome: implications for research and clinical practice,” was published in Pediatric Rheumatology.

hEDS is the most common type of EDS, marked by symptoms such as abnormally mobile joints and stretchy skin. Unlike with other types of EDS, the exact cause of hEDS isn’t clear, and the diagnosis of the disease is made clinically based on the symptoms a patient experiences. Chronic pain is common in hEDS.

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Examining impact of illness uncertainty

A team of scientists in the U.S. conducted a review of the scientific literature aiming to examine the impact of illness uncertainty (IU) in hEDS. The scientists defined IU as the “perceived loss of control regarding one’s illness and its treatment.”

The review found IU can be an issue for people with all sorts of conditions that are characterized by chronic pain. In many cases, these conditions are identified as diagnoses of exclusion, meaning the condition is diagnosed only by ruling out other potential explanations for symptoms.

People with chronic pain often report feeling dismissed or not taken seriously by medical personnel, and it’s also common for people experiencing chronic pain to be recommended nonmedication interventions, “which may elicit feelings of being dismissed by the medical community,” the researchers said.

People with hEDS commonly face many of these same issues, but the review also highlighted some aspects of IU that are more unique to hEDS. For example, patients may face uncertainty or misdiagnoses about exactly what type of EDS they have.

The fact that hEDS is diagnosed based on symptoms also causes complications, especially since the precise definitions and terminology used to describe hEDS and related disorders has shifted over the years. This can make clinicians wary to offer a definitive diagnosis, especially in children — which causes increased uncertainty for patients.

The researchers said having more standardized procedures for diagnosing hEDS might go a long way toward reducing uncertainty for patients, especially children.

Data suggest the physical changes in the bodies of people with hEDS may set the stage for anxiety by changing interoceptive cues (a person’s sensation of their own body), which may contribute to further complications with mental health and IU.

Overall, the review highlights how important it is for clinicians to have open, constructive dialogues with patients to minimize uncertainty and make patients feel empowered. The researchers stressed that this is a key part of providing care, since IU can have serious consequences for patients’ health.

“Due to their long journeys to diagnosis, perception of dismissal from health-care providers, and misattribution of symptoms, patients with hEDS have often experienced delays in access to appropriate care and, as a result, may be experiencing diminished psychosocial well-being,” the researchers wrote.

The scientists added they encourage clinicians to “recognize and validate any challenges in reaching diagnosis, including consideration of frequently co-occurring diagnoses … assess for negative experiences with healthcare providers which may influence the current therapeutic relationship and undermine treatment engagement, and … collaboratively create a clear treatment plan with the patient and their families.”