Lack of EDS educational materials cited by patients, parents in survey
Newly diagnosed patients say this hindered them in managing disease
Within three months of an Ehlers-Danlos syndrome (EDS) diagnosis, many patients and their parents felt there was a lack of related educational materials, which was a barrier to their knowledge and comfort in managing the condition, according to a new survey study conducted by U.S. researchers.
The respondents also expressed uncertainty about symptoms related to EDS and lacked confidence that medical providers were familiar with them, the survey found.
“The results of this survey show that there is a continued need to develop educational interventions for patients and families, especially shortly after diagnosis of EDS,” the researchers wrote.
According to the team, “educational efforts aimed at youths with EDS may help improve knowledge and understanding of EDS earlier in their medical journey and help guide long-term management of symptoms and ultimately improve outcomes and [quality of life].”
The study, “Patient and parent knowledge, understanding, and concerns after a new diagnosis of Ehlers Danlos syndrome,” was published in the Orphanet Journal of Rare Diseases.
Researchers call study first to assess patient, family knowledge after diagnosis
EDS is a group of rare conditions that affect connective tissue in the body, which provides structure and support to the joints, skin, and organs. Hallmark symptoms include joint hypermobility — marked by movement past the normal range of motion — and soft and fragile skin that can be easily damaged.
Because of its rarity, few medical providers are familiar with EDS, and this can contribute to long delays in diagnosis and treatment. Even after diagnosis, despite patients and families being provided disease-focused education, standard recommendations on the best timing for EDS education to start or what it should include are still lacking.
According to the researchers, it thus is essential to figure out what patients and their families initially know about the disease and its management, both before and after a diagnosis is made.
To that end, a team of scientists from Kansas and Ohio asked EDS patients and their parents to complete questionnaires assessing their knowledge and awareness of the condition before diagnosis and within three months thereafter.
“The timeframe between diagnosis and initiation of active management may be a ‘window of opportunity’ to appropriately educate families,” the researchers wrote. “It is vital to understand what patients and families initially understand about the disease process, what general or age-specific needs they require due to the disease, and what questions they would most like to have answered as part of their care to better tailor the education provided to patients and families.”
The team noted that “this is the first study to assess patient and family knowledge of EDS after a diagnosis.”
Responses were received from 72 pairs of patients and parents or legal guardians. Nearly all of the parents/legal guardians (96%) were parents. Most were married (81%), were white individuals (85%), and were women (93%).
Among the parents/legal guardians, 1 in 5 or 20% had been diagnosed with EDS, while nearly 1 in 3, or 30%, suspected they had the condition, although no diagnosis had been made. A total of 51% of the parents/legal guardians reported 1-3 three family members with EDS.
All of the patients had hypermobile EDS, known as hEDS, which is the most common type of EDS. Most were female at birth (81%), and about half were ages 16 to 18.
Both patients, parents want more available educational materials on EDS
According to the questionnaires, 57% of parents and patients had heard of EDS before the diagnosis. The most common sources of knowledge for parents were medical providers or family members, while for patients they were a parent or another individual with the condition. More patients than parents heard about EDS from social media.
About half the patients and two-thirds of parents were “somewhat comfortable” with their knowledge of EDS. Barriers to parent comfort included a lack of knowledge (47%), EDS-related educational materials (42%), and confidence that medical providers knew about EDS (42%). For patients, barriers were a lack of knowledge (57%) and educational materials (32%).
Overall, 72% of parents and 62% of patients reported that they were “somewhat comfortable” with their ability to identify and manage symptoms of EDS.
In particular, more parents than patients said they were uncertain about which symptoms were associated with EDS and which were not (51% vs. 32%), and lacked confidence that medical providers were familiar with EDS symptoms (45% vs. 18%). Both said there were limited educational materials related to EDS symptoms (38% vs. 32%).
Parents and patients look for information about EDS differently. … [But] both want well-constructed, empirically supported educational materials delivered via multiple modalities.
Regarding such materials, more parents than patients accessed online educational materials about EDS, both before diagnosis (43% vs. 30%) and after (76% vs. 43%). After diagnosis, more patients than parents said they were given educational materials about EDS at a medical visit (77% vs. 58%).
Both before and after the EDS diagnosis, many parents expressed concern about the need for musculoskeletal care (66%), primary care for EDS (58%), education about EDS (45%), and gastrointestinal care (41%). Additional concerns reported by 46% of parents were focused on mental health and multidisciplinary care.
The results were similar among patients, with 66% expressing concern about the need for musculoskeletal care, 55% for primary care for EDS, 60% for education about EDS, and 45% for gastrointestinal care. About one-third of patients worried about mental health care before diagnosis, while long-term outcomes were concerning for 37% after the diagnosis.
Overall, the researchers concluded, “parents and patients look for information about EDS differently,” with “a higher proportion of patients [obtaining] additional information from social media.”
However, the team noted that “both want well-constructed, empirically supported educational materials delivered via multiple modalities.”
According to the team, “more research is needed to confirm these findings as well as develop and pilot optimal age-appropriate educational modalities for patients and their parents.”
About the Author
