Background
Patient-centered research requires active engagement of patients. The vascular Ehlers-Danlos
Syndrome (vEDS) research collaborative was established to ascertain patient-centered
vEDS research priorities and to engage affected individuals as research partners.
Evaluation of access to information and interest in research among individuals with
vEDS was the first step undertaken as part of this work.
Methods
A 28-question survey was created to evaluate 4 domains of interest: diagnostic and
clinical care history, vEDS experience, information resources, and willingness to
collaborate with researchers. The survey was created in REDCap™ and disseminated between
January and April 2018 via the vEDS social media pages, blogs, and advocacy Web sites.
Results were collated and described. A single open-ended question yielded additional
narrative data, which were analyzed qualitatively.
Results
Of the 300 responses, 228 (76%) were completed on behalf of oneself. The vEDS diagnosis
was confirmed by genetic testing for 85% of respondents. When asked “Did a physician
explain vEDS to you and how to manage it?” 25% answered no. Most had a primary care provider (65%), cardiologist (56%), and vascular surgeon
(52%). Only 32% had a local vascular surgeon. The most commonly reported frustration
was no cure/treatment available and the emergency rooms do not know what VEDS is (64.5% and 61.8%, respectively). The Internet was the most useful information source
(62.3%) followed by a geneticist (18.4%). Most (87.7%) are willing to share their
medical records for research studies (87.7%) and wished to be contacted about future
studies (83.8%); however, only 65.4% would be willing to upload medical records via
a secure confidential Web application. The most common reason for interest in research
partnership was to advance research for a treatment/cure (83.8%) and helping others
learn from their experiences (82.9%). The qualitative analysis provided additional
insights into the patient experience living with vEDS.
Conclusions
Among individuals with vEDS, there is substantial frustration with the lack of treatment,
lack of knowledge among health care providers, and a high degree of interest in research
involvement. The survey highlights an opportunity to discuss the optimal modality
for research participation and methodologies for building trust in the research teams.
The methodology lessons learned can also be applied to other rare vascular diseases.
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References
- Crossing the Quality Chasm: A New Health System for the 21st Century. National Academies Press, Washington, DC2001
- Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome.Am J Med Genet C Semin Med Genet. 2017; 175: 40-47
- The challenge of rare diseases.Chest. 2018; 153: 1309-1314
- Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type.N Engl J Med. 2000; 342: 673-680
- Haploinsufficiency for one COL3A1 allele of type III procollagen results in a phenotype similar to the vascular form of Ehlers-Danlos syndrome, Ehlers-Danlos syndrome type IV.Am J Hum Genet. 2001; 69: 989-1001
- COL3A1 haploinsufficiency results in a variety of Ehlers-Danlos syndrome type IV with delayed onset of complications and longer life expectancy.Genet Med. 2011; 13: 717-722
- Survival is affected by mutation type and molecular mechanism in vascular Ehlers-Danlos syndrome (EDS type IV).Genet Med. 2014; 16: 881-888
- Molecular diagnosis in vascular Ehlers-Danlos syndrome predicts pattern of arterial involvement and outcomes.J Vasc Surg. 2014; 60: 160-169
- The 2017 international classification of the Ehlers-Danlos syndromes.Am J Med Genet C Semin Med Genet. 2017; 175: 8-26
Vascular Ehlers-Danlos Syndrome (vEDS) Collaborative. Available at: http://www.becertain.org/projects/patient-engagement/vascular-ehlers-danlos-syndrome-veds-collaborative. Accessed May 1, 2018.
- Three approaches to qualitative content analysis.Qual Health Res. 2005; 15: 1277-1288
- The Medicare Electronic Health Record Incentive Program: provider performance on core and menu measures.Health Serv Res. 2014; 49: 325-346
- Personal health record: new opportunity for patient education.Orthop Nurs. 2007; 26 (quiz 92–93): 185-191
- Patient portals and patient engagement: a state of the science review.J Med Internet Res. 2015; 17: e148
Article info
Publication history
Published online: August 23, 2019
Accepted:
June 8,
2019
Received:
March 25,
2019
Footnotes
Funding: Research was funded through a Patient-Centered Outcomes Research Institute (PCORI) Award (7718436).
Disclosures: None to report.
Conflicts of interest: None to report.
Identification
Copyright
© 2019 Elsevier Inc. All rights reserved.
