Abstract
Introduction:
People with differences of sex development (DSD) and their families need education about these conditions while receiving emotional and peer support to participate in shared decision-making, reduce social isolation, and optimize physical and psychosocial outcomes. Barriers to education and support include limited knowledge and awareness by healthcare providers, tension among patient and medical communities, varied quality of educational resources, and the sensitive nature of DSD. We aimed to create an electronic repository of vetted quality online resources about DSD.
Methods:
The electronic resource repository (e-RR) was a collaboration between affected individuals and advocates, and healthcare providers in the DSD-Translational Research Network (DSD-TRN), an NIH-supported consortium of US teams committed to standardizing and optimizing care in DSD. The e-RR development and ongoing growth involved: 1) identification of resources by the project team (three advocates and one physician); 2) evaluation and feedback by DSD-TRN clinical teams; 3) creation of the e-RR; and 4) review and revision. Twitter-like descriptions accompanied each entry; resources were categorized by target age, audience, and condition.
Results:
Thirty-seven web-based educational, peer and advocacy support, and clinician-oriented resources were reviewed. Eight of 10 DSD-TRN teams responded to a survey regarding resource inclusion. Awareness of individual resources varied widely. Consensus was achieved when opinions differed; 30 resources were included. The e-RR is available online and as a downloadable booklet: http://www.accordalliance.org/resource-guide/.
Conclusion:
The e-RR increases awareness of and access to vetted educational and support resources for those with DSD and healthcare providers. It represents important collaboration between advocates and providers.
Keywords: Disorders of sex development, DSD, advocacy, education, intersex
Introduction
Differences of sex development (DSD), sometimes referred to as disorders of sex development or intersex conditions, is a collective term that encompasses many congenital conditions affecting the development of genital and/or reproductive organs [Hughes et al., 2006]. Care for people with DSD is challenging due to the sensitive and potentially stigmatizing nature of these conditions, and due to limited outcome data to inform important, and frequently controversial, management decisions.
People with DSD and their families need information and education about their conditions, as well as emotional and peer support, to participate in shared decision-making, build resilience and reduce social isolation, and optimize physical and psychosocial outcomes [Crissman et al., 2011; Magritte, 2012; Baratz et al., 2014; Engberg et al., 2017; Wisniewski, 2017]. The landmark Chicago Consensus conference in 2005 laid the foundation for a shift from paternalistic to patient-centered care [Hughes et al., 2006; Hughes, 2015]. It recognized the fundamental importance of open communication with patients and families and shared decision-making, the need for information for families that is “balanced and sound”, and the role of peer and parent support to decrease isolation and stigma and improve care [Hughes et al., 2006]. For health professionals in the field, it underscored the importance of standardizing care, improving training and education about DSD, and integrating support groups to complement the healthcare team [Hughes et al., 2006].
More than a decade after the Chicago conference, despite the progress that has been made, many of the same clinical and research priorities for patients and parents prevail, including the need to improve information management and parental support [Cools et al., 2018]. A report entitled “Listen to us” by the UK charity dsdfamilies in 2019, generated from consultations with 194 individuals living with DSD and their families, echoed an ongoing plea for “information, peer support and psychological support”, as well as a wish to partner with healthcare providers (available at https://www.dsdfamilies.org/application/files/8015/5447/3715/reportdsdf-2019.pdf). Healthcare providers, who were also consulted, reaffirmed a need for information resources to give to families, more knowledge for non-specialist healthcare providers, and access to support for families.
In an age in which internet resources abound and offer great potential for education and support for patients and families, barriers include limited knowledge and awareness of informational resources by both families and healthcare providers, varied quality of educational and online resources, as well as tensions that may exist among patient advocacy and medical communities. There is a need to improve accessibility and awareness of existing quality resources, identify and address gaps, and integrate shared goals of healthcare providers, advocacy groups, and families and individuals living with DSD [Muscarella et al., 2014].
We aimed to create an electronic repository of vetted online resources about DSD. This project was a collaborative effort between advocates / individuals with DSD and healthcare providers who were members of the DSD-Translational Research Network (DSD-TRN) (https://dsdtrn.org/). The DSD-TRN is a consortium of interdisciplinary clinical teams in the US committed to extending discovery of genetic causes and standardizing and optimizing care for patients with DSD [Sandberg et al., 2015]. In keeping with the goals and mission of the DSD-TRN, an overarching goal of this project was to establish an accessible library of web-based educational material and support resources that both met a standard and enabled standardized information that teams could share with patients and families.
Materials and Methods
The e-RR was a collaborative effort of advocates and healthcare providers in the DSD-TRN Standard of Information work group (Fig. 1). Ethics approval was not required as this project did not involve patient participation or clinical data.
Fig. 1.
The electronic resource repository: The product and the process and what they represent
Creation of the e-RR
The creation of the e-RR is outlined in Figure 2. The e-RR was created in three phases:
Fig. 2.
Development of the electronic resource repository (e-RR)
1. Creation of an initial resource list:
The initial project team comprised three advocates and one physician (M.M., J.G., G.I., M.M.R.). They collectively compiled a repository of online resources through personal familiarity with many of the resources, as well as by searching the internet. Although strict inclusion and exclusion criteria were not defined, the intent was to include resources that were educational, informative, and supportive, while reflecting important and balanced viewpoints. Thirty-seven resources were reviewed and categorized. Broad groups and sources included: 1) educational and informational (articles, informational web pages, handbooks, online PDF documents and pamphlets); 2) media (films, videos, television programs); and 3) peer support and advocacy groups and DSD-related organizations. Resources were categorized according to the target audience, appropriate age group and medical condition. Brief Twitter-like descriptions were written for each resource to enable the prospective user to make a quick and informed decision as to its relevance for their needs.
2. Evaluation, critique and feedback of the initial repository:
The psychosocial workgroup of the DSD-TRN created a survey for teams to evaluate, critique and provide feedback on the e-RR. The psychosocial workgroup comprised psychologists, mental health providers and allied health providers representing each of the 10 participating DSD-TRN clinical sites at the time, and an advocacy representative from the e-RR project team (M.M.). The survey was then disseminated to the teams in the DSD-TRN, who were asked to review the resources and provide feedback. The survey involved: 1) a feedback form for teams to respond to a few questions about each resource (questions shown in Table 1), 2) a draft of the e-RR that included the descriptions of the resources with links to the relevant websites, and 3) a table listing the categories (intended age and audience, and DSD condition) for each resource. Teams were asked to provide yes/no answers to the questions and requested to provide additional feedback, if indicated. Teams were also asked for their members to discuss their opinions on the resources and offer consolidated feedback as a team, where possible.
Table 1.
Evaluation of items nominated for the electronic resource repository (e-RR)
| Topic surveyed | Questions asked about each resource |
|---|---|
|
| |
| Familiarity and use | Are you familiar with the resource and use it? If no, why not? |
| Comfort with inclusion | Do you feel comfortable with its inclusion in the repository? |
| Accurate and appropriate description | Do you find that the description below the resource is an accurate summary of resource content? If not, what information is missing or should be removed? |
| Target age and audience | Do you find the categorization of target age, diagnosis, and parent stage groups accurate for the resource? |
| Link works | Does the link to the resource function? |
| Missing resources | Are there any major resources that are missing from this list? |
3. Consensus and creation of the e-RR:
Feedback from the survey was collated (A.vK., M.M., G.I., J.G., M.M.R., D.E.S.). Twelve of the original resources were removed and 5 new ones were added. Consensus was achieved when opinions differed; 30 resources were eventually included in the e-RR. Resources were re-categorized as: 1) Educational and informational; 2) Living with DSD: young adult and adult voices; 3) Peer support and advocacy groups; 4) Clinician resources; and 5) Additional resources for purchase. The e-RR was subjected to health literacy review to ensure that it was at an acceptable reading level (K.K.), and finalized for publication.
Continuing evolution of the e-RR
The ongoing growth and development of the e-RR has involved recurrent review and revision (M.B., M.M.R., E.M.W., D.E.S.). Links were updated, and outdated and unavailable resources and links were removed. Several newer resources were added, for example, ‘Top Tips for Talking’ (available at https://www.dsdfamilies.org/application/files/4115/3780/1476/Top_Tips_for_Talking.pdf), research / registry opportunities (https://dsdtrn.org/; https://home.i-dsd.org/), and viewpoints (and debate) on current care for patients with DSD (available at https://www.accordalliance.org/resources/viewpoints-on-dsd-care/). Twitter-like descriptions were written for the new resources, and revised as needed for the original resources.
Results
Initial repository and response to survey
Of the 10 teams in the DSD-TRN at the time, 8 teams provided responses (Table 2 and Supplementary Table). Some teams divided up the resources between their members to enable more in-depth feedback. Familiarity with the listed resources by each team varied from 8 to 100% (averaging 49%) of resources, with most teams being familiar with (and comfortable with including) peer support groups and organizations, and often unfamiliar with (and uncomfortable with including) resources from media (Table 2). There were 8 resources that had involved healthcare professionals (not from the DSD-TRN) in their development; these were met with higher levels of agreement in terms of teams’ familiarity – and in particular, teams’ comfort for inclusion after review – and were retained for the e-RR. Teams agreed in general with the descriptions and target audiences and ages for each resource.
Table 2.
Familiarity with and comfort with inclusion of resources for electronic resource repository (e-RR) by DSD-TRN teams.
| Resource category in survey | Resources reviewed | HCP involvement in resource development | Team familiarity with resources | Team comfort with inclusion of resources | Original resources included in e-RR† |
|---|---|---|---|---|---|
| N | N | median N (range) | median N (range) | N | |
|
| |||||
| Educational / informational | 18 | ||||
| •Articles, informational web pages | 9 | 2 | 2 (0–9 | 7.5 (0–9) | 5 |
| •Handbooks, PDF documents, pamphlets | 9 | 6 | 5 (0–9) | 7.5 (6–9) | 9 |
| Media | |||||
| • Television, films | 10 | 0 | 2 (0–10) | 6 (0–10) | 2 |
| Peer support / advocacy / organizations | 9 | Not applicable | 9 (0–9) | 9 (8–9) | 9 |
|
| |||||
| All | 37 | 18 (3–37) | 29.5 (15–37) | 25* | |
After removing 12 resources, 5 additional resources were included in the original version of the e-RR, making a total of 30 resources.
The e-RR included all 8 resources that had involved healthcare professionals in their development.
Abbreviations: HCP, healthcare professional; N, number of resources
Additional feedback and comments from several teams involved:
Exclude blog sites, chat groups or personal webpages, as content from these could change and may not be moderated;
Use vetted medical (such as How Sex Development Works, available at https://pie.med.utoronto.ca/htbw/module.html?module=sex-development) and lay public sites (such as dsdfamiles, available at https://www.dsdfamilies.org/ and Accord Alliance, available at http://www.accordalliance.org/) for factual information, and support group sites for psychosocial issues;
Limit resources that need to be purchased;
Limit resources from media to those that are of high quality, professional, non-fictional and free;
Include resources that promote adaptive patient and family coping and exclude those potentially detrimental to relationship-building between parents, patients and the healthcare team.
After reviewing feedback and assessing comfort levels of teams, consensus was achieved. Twelve of the original resources were removed and 5 new ones were added: 30 resources were included in the original e-RR.
Continuing evolution of the e-RR
A summary of resources in the updated version (Version 2.0) of the e-RR is shown in Table 3. The e-RR, also known as ‘Lend a Helping Hand,’ is currently available online and free of charge through Accord Alliance (a non-profit organization focused on DSD) and the DSD-TRN websites. It can also be downloaded as a booklet (available at http://www.accordalliance.org/resource-guide/).
Table 3.
Summary of resources in the updated electronic resource repository (e-RR)
Healthcare providers: ✚; Parents and caregivers: ⚫; Adults (18+): ★; Teens (13–18): ▲; Pre-teens (9–12): ♥; Children (5+): ⬛
Abbreviations:
DSD: Differences / Disorders of Sex Development
CAH: Congenital adrenal hyperplasia
PAIS: Partial androgen insensitivity syndrome
AIS: Androgen insensitivity syndrome
5-ARD: 5-Alpha-Reductase deficiency
MRKH: Mayer-Rokitansky-Küster-Hauser syndrome
Discussion/Conclusion
The e-RR is the product of a novel collaboration between US-based healthcare professionals from interdisciplinary teams in the DSD-TRN and people living with DSD. The resources have been “peer reviewed” to meet a quality standard, and collated as a repository to allow standardization of information that can be used and disseminated by teams, endorsing the mission of the DSD-TRN to both optimize and standardize care for those with DSD (https://dsdtrn.org/) [Sandberg et al., 2015; Delot et al., 2017; Ernst et al., 2019].
The list of informational resources in the e-RR is comprehensive, although not exhaustive, and there is overlap with some of the resources found on the websites of major support organizations. In addition, the resources in e-RR span support organizations to media to medical and scientific (including DSD registries and viewpoints on surgery in the updated version), informative to all stakeholders, including families and healthcare providers. One of its distinctive features is that it serves as a hub in which vetted resources are systematically organized and directed to the prospective user, by target audience, age and DSD condition, with short descriptions that help guide the user as to the relevance for their needs. The resources have been made easily accessible: the e-RR is free, available online, contains quick links to each resource, and can also be printed and used as a reference booklet or handout.
The process by which the e-RR was generated was unique in that it involved not only collaboration between advocates and healthcare professionals, but also “peer review” and consensus, both within interdisciplinary teams and between teams across the national network. The feedback process faced some limitations: although teams were asked to consolidate feedback as a team, several teams adhered to this, while some teams divided the large number of resources amongst their members, making it more challenging to discern if the feedback represented that of an individual or the team. That said, review was undertaken at several levels and involved strong representation of the psychologists in the DSD-TRN, and collective input from specialists of all disciplines. In terms of quality - accuracy or veracity - of included resources, there were higher levels of agreement amongst evaluators for inclusion of educational and informational resources that had involved healthcare professionals in their creation, as well as links to support and advocacy organizations. For resources that were debated, consensus was achieved in an effort to include those of higher quality, representing important viewpoints, and promoting adaptive coping and support and relationship-building between patients and the healthcare team.
Although the e-RR is primarily directed towards patients and families, a key target audience is healthcare professionals and teams who often serve as “gatekeepers”, facilitating access to information and support resources [Fox and Duggan, 2013]. Our survey of DSD-TRN teams showed familiarity with the resources at the time ranged widely, with several teams being unfamiliar with many informational and media resources. Health professionals need to be aware of resources and support organizations that may benefit their patients and families. Families not only require a lay understanding of the complex concepts of typical and different sex development, they also need to understand the range of treatment options and associated considerations in order to actively engage in decision-making around clinical care for their child [Sandberg et al., 2019]. Support and advocacy groups, and information resources produced by these stakeholders, help incorporate the patient, rather than just the physician, perspective in decision-making and enhance patient-centered care, which ultimately improves psychological and health outcomes [Magritte, 2012; von Klan, 2015; Addario et al., 2018]. Many families are hesitant to join peer support organizations or may not proactively seek out information about their condition or clinical care. The role of the DSD team in identifying and supporting these families is all the more important, and provider familiarity with the resources in the e-RR may help facilitate the incorporation of these important aspects of care.
Health providers themselves (specialists and non-specialists) need education in caring for and communicating with patients and families with DSD that extends beyond the realm of that offered in medical textbooks and most training programs. Many medical schools do not provide sufficient education on this topic [Neff and Kingery, 2016; Zelin et al., 2018]. Amongst specialist trainees, pediatric endocrinology fellows internationally were found to have variable and limited knowledge regarding DSD management, as well as variable communication skills (with many rated as poor or insufficient) for information-sharing with patients with DSD and their parents [Kranenburg et al., 2016; Kranenburg et al., 2017]. The need for better education and communication skills for pediatric endocrinologists in the field of DSD has been identified as a gap and priority focus area in Europe [Kyriakou et al., 2016; Hiort et al., 2019] and the USA (Pediatric Endocrine Society DSD Special Interest Group poll, 2020; personal communication). While the e-RR directs health providers to clinician-focused resources that may inform educational curricula for medical students and fellows [Eckstrand et al., 2014], the non-clinician-focused resources offer important insights and perspectives that integrate patient values and voices, and a common language, providing invaluable learning for clinicians regarding communication of DSD-related topics with patients and their families [Sanders et al., 2018].
The e-RR underscores gaps and potential next steps. A lack of resources for sharing information about DSD with children is evident, and there is a need for developmentally-appropriate educational materials. There is scope for its use as a continuing education tool, or quality improvement or maintenance-of-certification activity, to improve knowledge and communication skills of healthcare providers. There needs to be ongoing assessment of its use, by electronic methods such as Google Analytics and by direct feedback from its users, with updating of its resources to ensure it continues to evolve and remain current. Finally, the e-RR represents a shared enterprise of collaboration and communication between people who have a DSD and interdisciplinary healthcare providers, a vital model to aspire to for delivering, informing, and transforming care for patients with DSD and their families (Fig. 1) [Muscarella et al., 2014].
Supplementary Material
Acknowledgements
We would like to thank teams in the DSD-TRN who provided feedback in the original survey. We are grateful to Lindsey Hornung for help with preparing Figure 2. We are grateful to Grace Kavanaugh and Savanna Weaver for help with updating and revising the current version of the e-RR, and for help with Table 3.
Funding Sources
This work was supported, in part, by grants from the Eunice Kennedy Shriver National Institute of Child Health and Human Development (R01-HD068138, R01-HD093450, DSD-TRN).
Footnotes
Conflict of Interest Statement
Ms. Weidler is a paid Executive Director of Accord Alliance. Dr. Rutter and Dr. Sandberg serve as unpaid members of the Board of Accord Alliance. The authors have no other conflicts of interest to declare.
Statement of Ethics
Ethics approval was not required as this project did not involve patient participation or clinical data.
Data Availability Statement
All data generated or analyzed during this study are included in this article. Further enquiries can be directed to the corresponding author.
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Data Availability Statement
All data generated or analyzed during this study are included in this article. Further enquiries can be directed to the corresponding author.