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Despite the proliferation of over 45 000 smartphone mobile health applications (MHAs), as far as we know, there is no MHA for those living with rare diseases such as Bladder Exstrophy-Epispadias-Cloacal Exstrophy complex (BEEC). We hypothesized that an MHA could provide similar “on-demand” information and connectivity within health communities for patients with BEEC as they do for more common diseases. Thus, our primary goal was to create an MHA for patients and families affected by BEEC to provide them with important information about the condition and a format for them to connect with other affected patients and families. A secondary goal was to develop an adaptable MHA template for other rare diseases in the future.
Methods
We began our app development by examining existing common-disease MHAs for thematic structure. We conducted an extensive literature search of PubMed and Google scholar for MHA development and existing MHAs related to BEEC, utilizing these search terms: mobile health applications, rare diseases, bladder exstrophy, and online health communities. Our app development team began with our clinical multidisciplinary team of pediatric urologists; a child psychiatrist; a patient/family mental health therapist; and a certified nurse practitioner. We hired a website engineer and a production team. All clinical members have extensive experience caring for children and families affected by BEEC.
Additionally, clinical team members compiled lists of themes deemed relevant from these reviews and themes gleaned from their clinical experience that appear with some frequency or urgency and from the myriad of themes discussed within the literature for MHAs.
Results
We found no existing rare disease MHAs in the literature or our search of app stores online. However, we derived basic app categories from existing MHA formats and the thematic content of all sources reviewed. These categories aligned with the groupings of our lists of clinical themes. Thus, we could subsume diverse themes within a broad categorical format: for example, child development (as “Psychological Development” in the app) or various clinical care options (as “Treatment”). This app structure became nine sections, as shown in. This format allows diverse information to be retrieved efficiently from broader categories. This app is being offered to affected families, healthcare providers, and individuals unrelated to where care is offered.
Conclusion
“We the BE” is the first MHA developed for a rare disease, BEEC. It has been published in a downloadable format for the general public at no cost. Further research is required to determine its efficacy for the BEEC community members; preliminary, unsolicited feedback from multiple users has been positive.
Bladder Exstrophy-Epispadias-Cloacal Exstrophy complex (BEEC) is a range of midline developmental anomalies affecting genital, urinary, intestines (rarely), and a variety of musculoskeletal structures [
]. BEEC can present with varying degrees of severity, from isolated epispadias to cloacal exstrophy, each resulting from some idiopathic embryonic failure of the cloacal membrane to develop appropriately [
]. Bladder exstrophy is the most common abnormality. It affects 1:10,000 to 1:50,000 live births, with males having 2 to 3 times the incidence of females [
]. Therefore, people with BEEC typically have difficulty obtaining appropriate disease and treatment information. Nearby medical care is often lacking. Thus, the child and the family can have trouble adapting to a clear path toward typical development and relationships. Affected children frequently express fear that peers will discover their abnormalities or urinary incontinence; such fears can evolve developmentally into feelings of loneliness and isolation. Parents often decry their difficulty obtaining appropriate information, including, for example, different treatment options or management of the course of the disorder [
]. Additionally, parents frequently search for other BEEC parents experiencing similar struggles. These issues can be addressed through online health communities (OHCs) through MHAs.
Applications (apps) for mobile devices such as smartphones and tablets utilize their advanced functions and embed vast information [
] demonstrates patients' desire for rapid access to health information. Indeed, several studies showed MHAs' ability to significantly improve patient outcomes for common diseases, such as diabetes mellitus, chronic lung disease, and cardiovascular disease [
]. There is an absence, however, of MHAs for rare diseases such as BEEC. We hypothesized that BEEC individuals and their families would benefit from a specific MHA, which we called “We the BE.”
Material and methods
We convened a multidisciplinary team composed of two pediatric urologists, a child psychiatrist, a patient/family mental health therapist, and a certified registered nurse practitioner and urodynamics technician; each has extensive experience caring for BEEC individuals and their families in multiple health care facilities. We searched the literature using PubMed and Google scholar using the search terms “mobile health applications,” “rare diseases,” “bladder exstrophy,” and “online health communities.” We searched the Apple App Store for existing MHAs. We added a computer software engineer and a video production team. The Black Family Foundation funded the app development. The software engineer and the video production team received compensation.
We compiled a list of diverse clinical themes within the literature for existing MHAs and examined their typical formats. The clinical team then individually provided lists of themes deemed relevant from clinical experience or frequently appearing in clinical encounters with affected individuals and their families. We called common-disease MHA themes. Common themes were identified; redundant themes were eliminated.
Results
We found about 45 000 extent MHAs but none for BEEC or other rare diseases. Themes from the MHA literature and our clinical experience seemed to fall into broad categories, such as treatment options, parent questions, patient compliance, etc. These broad categories pointed to patient needs for summaries of basic disease/condition information at various stages of child development and parents’ needs for interaction with other affected families. Thus, we had a rational thematic categorization covering fairly detailed and extensive information possibilities for an organized, information-oriented smart device app.
We ascertained a broad array of themes in extant MHAs for the various disease entities covered. After adding our compilation of discussed themes to those within the literature for existing MHAs, the material aligned with categories based on the nature of the accrued themes. Thus, our reorganized material formed into coherent lists of themes. Utilizing these lists, such as “various treatment options,” “parents’ questions,” “patient compliance,” and so forth, a need for summaries of basic disease/condition information at various clinical stages of affected children, discussions of available treatment options, communication with other affected children and parents, and so on, became apparent.
Our thematic categories fit nicely into an app format such that the many common topics and concerns could be located relatively efficiently within broader, more generalized headings, including, for example, child development (as “Psychological Development” in the app), frequently asked questions (as “FAQ”), and clinical care options (as “Treatment”).
Our MHA headings are outlined as nine sections, as demonstrated in Fig. 1a. They are About Bladder Exstrophy, What to Expect, FAQ, Psychological Development, Treatment, Videos, Medical Products, Forum, and Contact (Fig. 1a). Each section contains sub-sections for coherent information organization and allows users to find information on specific topics and concerns more efficiently.
Fig. 1(1a) depicts the nine sections included in the application. (1b) shows a sub-section (ei, “Epispadias”) contained within the “About” section, which includes a summary/overview of epispadias.
Thus far, anecdotal data from our clinic patients and families who have used “We the BE,” plus online comments from other users, indicate that this MHA is well received with a positive impact on their BEEC community. However, further research and analytical data collection are ongoing to measure the effect of “We the BE.” Phases of development, as well as updates and new content, are continuing.
Discussion
With over 45 000 medical/health-related applications available for download in Apple's App Store in 2019 [
], it is becoming increasingly apparent that there is a market for MHAs and demand from patients to access MHAs. This is likely due to MHAs' many advantages to patients and physicians. For patients, MHAs offer convenience by eliminating traditional barriers such as cost, distance, and time [
]. In addition, by providing patients with a contact page, MHAs save the patient time in scheduling appointments and allows patients to have their questions answered that would otherwise require an appointment, saving both time and cost for the patient and eliminating unnecessary appointments for the physician.
Additionally, it has been well described in many aspects of medicine that providing adequate patient education leads to better clinical outcomes. MHAs can educate in a manner that is the most convenient for the patient. Procedure preparation is one benefit of patient education that is well established, which allows the patient to optimize their preparation, thus improving clinical outcomes [
]. This type of education is commonly achieved by physician illustrations or educational videos, which can be readily accessed on an MHA. A similar result can be achieved through the same educational techniques for patient adherence to improve the management of their condition, whether it be medication usage or adherence to patient-performed procedures, such as placement and use of L-stents to prevent stomal stenosis for BEEC patients.
While we assume our MHA will provide BEEC patients with the same benefits prior MHAs have supplied to their target population, as we implemented many similar aspects in “We the BE,”; we will be conducting further research to analyze the impact our MHA has on the BEEC community.
Online health communities (OHCs) have been shown to play an important role in electronic health services provided in today's digital age [
]. OHC allows users, usually patients or caregivers, to ask others coping with a similar disease/condition questions. Additionally, the users may share experiences, recommendations, and emotional support with other users within the community. Users can also observe others' interactions without having to participate within the community directly while still having their questions answered and benefiting from the shared experiences provided by the other users [
Impact of social processes in online health communities on patient empowerment in relationship with the physician: emergence of functional and dysfunctional empowerment.
Furthermore, OHCs have been shown to provide their members with significant improvements in empowerment. This is depicted through higher quality management of health-related issues and increased self-esteem, self-efficacy, and finding satisfaction through aiding others with similar conditions. In addition, members experience increased confidence while interacting with their physicians, improved competency while using other health services, and improved overall social welfare and quality of life [
Our “About BE” section contains four sub-sections (Bladder Exstrophy, Cloacal Exstrophy, Male Epispadias, and Female Epispadias). Each sub-section provides a brief description of the respective condition, including the physical manifestations of the disease, as well as diagnostics and treatment summaries which are expanded upon in other sections of the MHA (Fig. 1b).
The “About” section is the first section that appears when opening the MHA to summarize information on the typical manifestations of BEEC. The intention is to display the basic background information on BEEC while indicating to the user the information provided throughout the rest of the application.
The “What to Expect” section contains three sub-sections (Bladder Exstrophy, Cloacal Exstrophy, Epispadias) (Fig. 2a) that are broken down into four tabs (Introduction, In Utero, Operation Day, Age-Specific Tips) (Fig. 2b).
Fig. 2(2a) Image demonstrates the three sub-sections contained within the “What to Expect” section. The sub-sections are further broken down into informational tabs correlating to their respected sub-sections (2b).
The “Introduction” tabs contain advice for parents that have recently learned of their child's diagnosis, usually before the child is born. In addition, users are provided information on ways to prepare for the birth of their child, encouragement to research further outside the information provided within the MHA, and questions that concerned parents should discuss with their provider during the planning process.
The “In Utero” tabs expand upon the importance of connecting with a physician experienced in treating BEEC. Additionally, the tab encourages parents to reach out to organizations that will support parents and their children through the birthing process and throughout the child's life.
The “Operation Day” tabs explain the timing at which the parents can expect the reconstructive surgeries to occur and detail standard techniques used during the reconstructive surgeries. Additionally, parents are encouraged to maintain relationships with a trusted pediatric urologist to avoid complications in the future.
The “Age-Specific Tips'' tabs discuss with parents the developmental process children experience as they age with BEEC. In addition, the tabs urge parents to have reasonable expectations for their children as they age and establish appropriate expectations at different stages of development. Furthermore, the tabs provide an idea of potential setbacks during their child's development.
Our frequently asked questions (FAQ) section contains ten questions commonly asked by parents (Fig. 3). The section includes a range of questions - from the reproductive capability of people with BEEC to the ability to have functional and active lives. Additionally, the section further elaborates on common questions that are also addressed in other sections of the MHA.
Fig. 3It shows five examples of the questions contained within the “Frequently Asked Questions” section.
The goal of the section is to address the most common questions parents have when they first hear of the diagnosis and further inform parents of their child's diagnosis while reassuring parents concerned about the long-term outcomes of BEEC. In addition, addressing these questions early on can lead to more productive conversations with their pediatric urologist and allow for greater focus on managing issues that the child will have to face throughout their life, such as long-term bladder and kidney management, as well as treatment adherence.
The “psychological development” section contains six sub-sections within it (“Psychological Development of Children with Bladder Exstrophy,” “Year 1”, “Year 2”, “Early Childhood,” “Middle Childhood,” and “Adolescence”) (Fig. 4a). The first contains an overview of the information that will be included within the section, while the rest of the sub-sections have information specific to their respective age groups. The sub-sections provide an overview of normal psychological, cognitive and social development and potential challenges in their child's development as it relates to living with BEEC (Fig. 4b).
Fig. 4(4a) shows five of the six sub-sections contained within the “Psychological Development” section. These sub-sections, when clicked upon, are further broken down into different types of development that may be impacted at that given developmental age (4b).
The section aims to provide parents with a basic understanding of the psychological developmental process their child will experience while identifying potential setbacks. A basic understanding of their child's psychological development can avoid many of the challenges children with BEEC may experience. In addition, it allows parents to identify challenges earlier so they can be addressed and managed before they have detrimental effects on the child's development.
The “Treatment” section contains three sub-sections (“Bladder Exstrophy,” “Epispadias,” and “Cloacal Exstrophy”). The sub-sections take the user to a short slide show that provides information and infographics on treatment options (Fig. 5a). In addition, each sub-section details the goals of each surgical treatment option and outlines a timeframe for the necessary surgical interventions (Fig. 5b).
Fig. 5(5a,b) demonstrate the slideshows contain within the sub-sections (ei, “Bladder Exstrophy”) of the “Treatment” section, which covers different treatment/surgical modalities (5b). (5c) screenshot of the “Video” section, showing examples of the educational videos contained within the section.
The goal is to allow parents to plan accordingly throughout the treatment process and provide the information needed to make informed decisions on their child's treatment. In addition, this can save both parents and providers time as it pertains to explaining complex surgical techniques and the goals of the techniques that will be performed.
Our “Video” section contains an array of educational videos from the providers at OKC Kids Urology (Fig. 5c). The videos address many issues patients and families with BEEC face, from treatment adherence to trauma therapy, in a personal and easy-to-understand format that written information can fall short of. In addition, the video format allows a more efficient way of absorbing information that usually cannot be done by reading long descriptions of the complex information covered within these topics.
Additionally, the section contains testimonials from parents and patients treated at OKC Kids Urology. The testimonials demonstrate the impact that BEEC had on these families and patients while providing inspirational accounts of living with BEEC. The video testimonials give a more intimate form of communication that allows the user to see and share the emotional experiences that these patients and families have endured, thus adding to the sense of community that the MHA is attempting to build.
The “Medical Products'' section contains two sub-sections (“Stents” and “Catheters”) (Fig. 6a). The two sub-sections detail the different types of products that are commonly used and the purpose of the products and provide step-by-step instructions on proper procedural uses of the products via infographics and visual examples (Fig. 6b).
Fig. 6(6a) depicts the categorization of information contained within the “Catheter” sub-section of the “Medical Products” section. These tabs expand when clicked upon to reveal information related to their respective tabs (6b).
The goal is to provide proper user techniques to reduce complications associated with improper techniques. Providing the knowledge of the products and their appropriate uses, the hope is that this will increase patient adherence, thus resulting in improved patient outcomes.
The “Forum” section requires the user to create an account by providing an email and establishing a username (Fig. 7). Once an account is made, the user cannot only ask questions but respond to other users' questions or comments within the forum.
Fig. 7It is a screenshot from the “Forum” section where users are instructed to login or register to join the forum.
For safety, all questions and comments must pass through an admin, an OKC Kids Urology team member, for approval. The admin receives a notification on their mobile device when a user posts a question or replies to another user's message. All messages posted within the forum must receive approval from an admin before other users can view them. They can also delete any comments within the forum containing false or misleading information. These safeguards are meant to ensure that accurate information is disseminated and that no harassment or inappropriate comments will be seen or received by other users in the forum.
The forum is designed to encourage patients to exchange helpful information promptly. It provides the users a platform to share their personal experiences and support other users experiencing difficult times. With these abilities, the users can form an online health community (OHC) that will encourage and support one another while expanding their understanding of living and coping with BEEC.
The “Contact” section allows the patients to reach out to the OKC Kids professionals directly via email (see Fig. 8). This feature enables patients to ask specific questions to experts in the field and have the questions addressed in a fast, convenient fashion. This provides the advantage of surpassing the need to schedule an appointment to acquire specific information, thus saving time for both patients and physicians.
This app is now available to any affected family, healthcare provider, or interested parties through Apple App, Android, and Google Play Stores by searching “Bladder Exstrophy.”
Objective #2
Our secondary goal was to provide a template that other physicians or interested parties could apply to any rare disease. With over 400 million people managing more than 7000 rare diseases worldwide [
], the need for rare disease MHAs is becoming clear. Despite technological growth for MHAs, rare diseases have remained MHA orphans. Our “We the BE” app provides a format for other rare disease MHA development using our plug-in-play template. The code used to develop “We the BE” will be available for anyone interested in creating an application for any rare disease community but lacking the technological fronts. An MHA can be created by simply inputting the information specific to a rare disease and uploading specific educational videos where desired.
Additionally, our MHA presents a forum section that can be adapted to any rare disease, supplying the groundwork for building OHCs. This allows any patient or family member to be connected to others in similar situations. Such connections can provide emotional and social support.
Limitations
There is a lack of research available on rare disease MHAs. Thus, it is difficult to evaluate the efficacy of our MHA. While awareness of the need for such an MHA is valuable, the small size of the BEEC communities leads to methodological difficulties in designing outcomes research. Only further research can identify either the benefits or potential risks of our MHA.
Conclusion
“We the BE” is the first MHA developed for a rare disease, BEEC. It has been published in a downloadable format for the general public at no cost. While we assume that this app will be beneficial to the BEEC community and preliminary feedback from multiple users has been positive, further research is required to determine its efficacy and safety.
Funding
This work was supported by the Black Family Foundation.
Conflicts of interest
We have no conflicts of interest.
Acknowledgements
Funding was provided by the Black Foundation.
References
Gearhart J.P.
Tadros Y.E.
The bladder exstrophy-epispadias-cloacal exstrophy complex.
in: The NORD guide to rare disorders. Lippincott, Williams and Wilkins,
Philadelphia2003: 686-687
Impact of social processes in online health communities on patient empowerment in relationship with the physician: emergence of functional and dysfunctional empowerment.
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