| Description |
Problem Ehlers-Danlos Syndrome (EDS) is an underrecognized heritable connective tissue disorder (HCTD) that presents with joint hypermobility, hyperextensible skin, tissue fragility, and can also have widespread systemic manifestations. A knowledge gap among providers leads to lengthy delays in diagnosis and misdiagnosis leading to psychological and physical consequences for patients. This project aimed to educate providers on the presentation and evaluation of EDS in order to decrease the delay in diagnosis for patients. Methods An educational program was given to providers at the Veterans Administration Salt Lake City Healthcare System (VASLCHS) which included a presentation of the clinical features, evaluation, and a critical physical exam technique for assessing joint hypermobility called the "Beighton score." Change in knowledge was measured through three pretest-posttest knowledge-based questions on the presentation and evaluation of EDS. Change in confidence was measured through three Likert scale pretest-posttest questions on the presentation, evaluation and identification of resources for EDS. Participants included physicians (15.6%), nurse practitioners (31.3%), physicians' assistants (9.4%), registered nurses (21.9%), pharmacists (3.1%), and licensed practical nurses (18.8%). Key Findings Results showed a statistically significant improvement (p<0.05) in confidence and knowledge of the presentation and evaluation of EDS. Participant knowledge of presenting symptoms (pre-median:1, post-median:3, p=1.36 e-6), assessment (X2=21.989, df=1, p=2.742e--6), and a physical exam finding (X2=51.897, df=2, p=5.379e-12) improved. Measures of confidence in the presentation (pre-median=1, post-median=3, p=3.91e-6), evaluation (pre-median=1, post-median=3, p=1.18e-6), and identifying clinical resources for EDS (pre-median=1, post-median=4. p=3.19e-6) also improved. Most participants reported an intent to utilize the Beighton score and refer patients for evaluation by the Genomic Medicine Service. A tool was created for the electronic medical record which was utilized 11 times after the initial educational presentation, reflecting a willingness of participants to utilize the Beighton score. The project data were not able to demonstrate an increase in referrals or diagnosis of EDS, possibly related to a short follow-up period and the low prevalence of the condition. Conclusion This study proved effective in teaching providers about this underrecognized genetic disorder. Future studies should include an extended follow-up period to monitor knowledge use and impacts on patient outcomes. The Beighton score for joint hypermobility should be included in musculoskeletal assessment courses in order to identify patients with EDS and other HCTD that present with joint hypermobility. |
