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Exstrophy asaGlobal Health Concern: Strategies forAddressing theBurden
19
ofDisease
KatelynA.Spencer, AseemR.Shukla, andRakeshS.Joshi
Global Health, Global Surgery, andTheir Role Within Treating Bladder Exstrophy
The World Health Organization (WHO) has dened health to be “a state of com­plete physical, mental, and social well-being, and not merely the absence of disease or inrmity” since the advent of the organization in 1948 [1]. This denition pushed health beyond the binary of solely sick versus well and enshrined the idea that health is a state which can be promoted. This opened the door for global health advocates to work to improve the health of people around the world and across dened country borders.
Global health is a concept that has changed in its denition and scope over the years. It is most commonly dened as “an area for study, research, and practice that places a priority on improving health and achieving equity in health for all people worldwide” [2]. Of note, global health is distinctly different from that of public health or international health according to current denitions. Public health has traditionally been constrained by national borders, while global health focuses on issues that transcend borders. Additionally, conversely to international health,
K. A. Spencer . A. R. Shukla Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA e-mail: ShuklaA@chop.edu
R. S. Joshi ( Department of Paediatric Surgery, Ahmedabad Civil Hospital, Ahmedabad, Gujarat, India
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 A. R. Shukla, R. S. Joshi (eds.), Bladder Exstrophy and Epispadias,
https://doi.org/10.1007/978-3-031-91238-2_19
*)
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which was initially developed through the lens of colonialism with knowledge, services, and interventions exclusively coming from high-income countries (HICs) to low- and middle-income countries (LMICs), global health attempts to shift the power imbalance. It aims to promote partnership or pooling of expertise between high-, middle-, and low-income countries in addition to reducing health inequity by advocating for social equity. Global health also works to include pop­ulation-level prevention without neglecting the importance of individual-level clinical care through multidisciplinary strategies that draw on health and social sciences in addition to elds like engineering, urban planning, climate science, and others.
Despite global health being a large eld that encompasses many domains, sur­gery has not always been considered a primary element [3]. As the eld has tradi­tionally been dened by specic populations (i.e., mothers, children, etc.) and diagnoses (i.e., HIV), surgery is a hard entity to fund, as it intersects all patient populations and includes a variety of disease pathologies. There has been a more recent push to dene global surgery and include it within the umbrella of global health [4]. This includes supporters of the addition such as Jim Kim, the 12th President of the World Bank, who has stated that “surgery is an indivisible, indis­pensable part of health care and of progress toward universal health coverage” [5]. The addition of surgery within the eld is especially important since common surgical procedures are cost-effective and comparable to other widely accepted global health interventions (i.e., antiretroviral therapy) when performed in LMICs [6].
There is a large burden of surgical disease worldwide and a disproportionate allocation of resources currently. Three-fourths of all surgeries take place in the wealthiest third of the world [7]. As a result, there are two billion people who do not have access to surgical care [8]. This is all due to differing levels of resource avail­ability and capacity. For example, HICs have 10 times as many operating rooms [8] and 11 times as many trained surgeons [9] per person as LMICs.
Conversely, the burden of disability-adjusted life-years from surgical conditions is concentrated within LMICs [10]. Approximately 94% of complex congenital anomalies that require surgical intervention occur in LMICs [11]. When specically examining genitourinary conditions, they are incredibly common [12]. They are ve to ten times more likely than cleft lip and/or palate and are the third most common non-chromosomal anomaly behind congenital heart and limb defects. While rare compared to other genitourinary anomalies, bladder exstrophy epispadias complex (BE) occurs in 1in 50,000 live births [13]. As such, it can account for a signicant number of cases, especially in places with higher birthrates. It is a common trend within LMICs to have higher birth rates than HICs, and this can contribute to an increased burden of disease [14]. Global surgery endeavors to treat conditions like BE and should not be viewed as too costly [6] or complex because the burden of disease within these countries is signicant. Outside of cost, from a moral stand­point, there is an obligation that exists to provide the highest level of care possible for these children and their families.
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Unique Challenges ofBladder Exstrophy inLMICs
Outside of the standard challenges of surgical conditions within LMICs, including lack of access to care, decreased resource availability, etc., BE provides unique challenges to patients and providers designing interventions to treat this population.
Delay inDiagnosis andClosure
BE is primarily diagnosed prenatally via ultrasound in HICs. As a result, parents can be counseled on what to expect, and surgical planning about closure can be done before delivery. However, access to and equity of prenatal care within and between countries impacts early detection of BE [15, 16]. In LMICs there is less access to prenatal ultrasounds, and so, less of these children are diagnosed prior to delivery. This then creates a delay in closure because surgical planning is delayed. Additionally, due to the rare nature of BE, diagnosis can be further delayed as par­ents are sent through the referral network until they nally reach a physician with knowledge of the condition. Then, there is further time required for parents to reach a physician who can perform the closure operation.
Delayed closure for BE also comes with its own challenges. Closing children with BE at older ages, which is common in LMICs because of all of the reasons listed above, is associated with a higher failure rate [17], greater challenges with continence, and the need for repeat closures and other operations throughout the lifetime of the child [18]. All of these challenges contribute to worsening the sequelae of mental health challenges that come with BE.
Complex Level ofMedical andSurgical Care Required forBladder Exstrophy
Treating BE successfully requires a high degree of technical skill and experience. While the timing and type of closure for BE remain a debated topic among pediatric urologists [19], there is denitive support that well-performed initial closures are more successful for patients than redo closures after a failed initial attempt [18]. In order to achieve this, the initial closure should be performed by well-trained urolo­gists with the assistance of orthopedic surgeons. This creates a challenge in LMICs, where the access to and availability of highly trained surgeons is low in general. There is a high rate of failure when less experienced surgeons attempt to perform the initial closure, and this creates future challenges if the child needs a repeat pro­cedure due to the presence of scar tissue and altered anatomy.
Additionally, these children need dedicated and specic postoperative care and long-term follow-up to facilitate a successful closure and good quality of life. This includes having well-trained nurses postoperatively who are familiar with the unique needs of these children. These children will also need continued follow-up
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care to monitor their long-term outcomes, including continence, renal function, fer­tility, and sexual function. All of this is complicated in the LMIC setting, where access to care and medical specialties is limited.

Mental Health Concerns

Children with BE, irrespective of their location, are at a dramatically increased risk of psychosocial adjustment issues, including suicidal ideation and behaviors, depression, and anxiety compared to their anatomically intact peers [2025]. This is complicated by the fact that children in LMICs have less access to mental health services [26].
The mental health concerns of this population also extend to the caregivers of these children. It has been documented that parents of children with BE have stress levels comparable to the parents of children with other long-term diagnoses such as type one diabetes [27]. Parents, in general, are concerned about the long-term impacts of BE, including helping with hygiene and uncertainty about their child’s future [28]. Primarily these parents are concerned about their child’s ability to get married, have children, achieve independence, and obtain complete continence, and these worries increase with subsequent surgeries.
Mental health within LMICs for this patient population is uniquely complicated by the cultural traditions and beliefs surrounding religion within those countries. Specically, in many LMICs around the world, there is an increased rate of multi­generational housing [29], and living within close quarters creates challenges for families with children with BE due to the constant leakage of urine. This can con­tribute to perceived and experienced stigma from family, school classmates, and community members [28]. All of this is compounded by lower nancial resources for disposable diapers, mental health services, etc., While some of these challenges are not unique to the LMIC setting, they can compound to create different chal­lenges for patients and their caregivers that must be understood and addressed within interventions created to address this need.
Strategies toDesign aGlobal Surgery Collaboration
In order for a global surgery collaboration to be successful, it must address the chal­lenges the specic population of interest has holistically and treat all aspects of the disease [4]. The focus cannot just be on creating anatomic integrity and then leav­ing, as might be seen within many “short-term surgical missions.” These programs, which have become common within the eld of global surgery, are typically focused on increasing surgical volume over a short timeframe and do not always lead to improved surgical capacity for host institutions [30]. They are not designed to culti­vate long-term follow-up for patients and have been known to result in higher com­plication rates that are outside of the local health system’s capacity to address [31]. Furthermore, when volunteer teams are not well integrated with the local hosts, they
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can burden local providers, stress hospital resources, and create negative opinions of HIC volunteers [32]. While conceived and executed with the best of intentions, these global surgery programs can cause more harm than good for local populations.
In response to these programs, the American Surgical Association Working Group (ASAWG) on Academic Global Surgery created a set of guiding principles for ethical engagement within global surgery. They are as follows [10]:
1. Local priority: The interests of the local partners and patients should take prece-
dence in all decision-making.
2. Resources: Programs should not hinder existing infrastructure, and obtained
HIC resources should be focused on the host site and partners.
3. Clinical care: HIC surgeons and staff should create a plan for complication man-
agement and long-term follow-up.
4. Capacity building: Training should be applicable to the LMIC setting in which
host partners will function, focus on local as opposed to visiting surgeons, and not contribute to out-migration.
5. Research: Research should be mutually collaborative and equitably attribute
authorship.
6. General: All HIC partners should practice cultural sensitivity and uphold the
standards of care and practice that exist in their HIC.
The Ahmedabad Model ofExcellence
Successful global surgery collaborations employ the above strategies and put an emphasis on capacity building. An example of a robust global surgery collabora­tion, irrespective of disease of interest, is the International Bladder Exstrophy Consortium (IBEC) based in Ahmedabad, Gujarat, India. The program began in 2009 when surgeons from two academic research centers in the United States joined with the Department of Pediatric Surgery at the Civil Hospital and BJ Medical College in Ahmedabad, Gujarat, India, to form a multi-institutional col­laboration. Since then, the partnership has grown to include the Children’s Hospital of Philadelphia, Cincinnati Children’s Hospital, Seattle Children’s Hospital, sur­geons from the Hospital for Sick Children in Toronto, Canada, and a pediatric anesthesiologist specialized in pediatric pain management. The Civil Hospital, a government-nanced public hospital offering free medical care to a primary catch­ment area of 60 million people, serves as the host institution. The collaboration includes at least one annual visit from the same team of surgeons, during which new patients are evaluated and care plans are made, while all previous patients are recalled for long-term follow-up. Patients undergo yearly urodynamic evaluation, cystogram, ultrasound, DMSA scan, and bloodwork. The collaboration also evolved toward a more holistic approach to care, including mental health screen­ings, educational sessions with family members, and community-building activi­ties for the entire cohort.
318
The IBEC is an example of successfully putting the criteria outlined by the ASAWG into practice in order to exact long-term benets for patients. The follow­ing sections will outline the unique benets of the collaboration and how they have been implemented according to the ASAWG principles.
K. A. Spencer et al.

Local Priority

The IBEC has a conscious focus on maintaining local priority throughout all aspects of the collaboration. The coordinators from both the host and visiting teams fre­quently discuss the priorities of local partners throughout the planning process. This includes focusing on the host team’s goals and adjusting the collaboration so it best serves the local patient cohort. For example, the rst few years of the collaboration identied a greater need for patient education, so the collaboration added novel education initiatives that included a doll representative of a BE patient to better explain to patients, their caregivers, and nursing staff what they should expect after surgery [33].
A gap in care and support for the caregivers was also identied, and research endeavors were added to focus on the patients’ quality of life and strategies to best support them [28]. Patient- and family-centered educational lectures were strength­ened based on the gathered data, and social workers and psychologists were added to the group to offer support. Patients and families who attend the collaboration annually can gain several ancillary psychosocial benets, including group support through a family-coordinated WhatsApp group, one-on-one counseling, and social resources.

Resources

The collaboration has focused on leveraging the existing infrastructure to provide more care to a greater number of patients as the geographic catchment area of the collaboration has increased [34]. Collaboration funding is prioritized for the host institution’s needs, including purchasing necessary resources for the collaboration and providing services to patients. Importantly, funds are not overwhelmingly dedi­cated to travel for the visiting team.

Clinical Care

The clinical care plan for complication management for the collaboration was devised together with the host and visiting providers and is based on pathways pro­duced over several years [17]. The clinical care aspect of the collaboration is cen­tered on an annual commitment of the primary surgeons from HICs to return for patient follow-up. Additionally, all interval follow-up care is completed by the host
19 Exstrophy as a Global Health Concern: Strategies for Addressing the Burden…
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team with support from the teams from HIC through regular teleconference communication.
Long-term follow-up is an essential element of the collaboration to create the continuity of care required to treat such a complex condition. Specically for BE, consistent follow-up is required to ensure that the long-term consequences of even successful BE closures are captured within data and addressed either through medi­cal or surgical management. For many global surgery programs, including short­term surgical missions, there is little data on long-term outcomes because there is not a large emphasis placed on coordinating follow-up [35]. As a result, these pro­grams are hard to evaluate. Previous work has found that the postoperative follow­up for many of these programs typically stops after 6months. For the small number of programs that do follow patients for longer than 6 months, their follow-up rate is on average 56% with a 22.3% complication rate [35]. Conversely, the IBEC has continued to follow patients years after their original procedure and has a consistent follow-up rate of 76% [17]. This is despite the various challenges within the LMIC setting.
This is a testament to the original inception of the IBEC.It was prospectively constructed to include a long-term commitment between the host and visiting sur­geons. The collaboration includes rigorous preoperative counseling and setting expectations of care with patients to ensure that they not only return for follow-up but also undergo a comprehensive examination.

Capacity Building

All aspects of the collaboration are committed to capacity building. All repairs are performed with at least one resident of the host institution participating in the sur­gery. Emphasis is placed on knowledge sharing between host and visiting stake­holders, with a focus on training local residents and attending physicians to create autonomous surgeons that have the capacity to perform the CPRE with the same outcomes as the teams from the HIC academic research centers. As a result, the host team has increased experience and greater competency in performing a CPRE.Over the last 5years, almost as many closures have been performed outside of the time of annual collaboration as during the set window, with comparable results. This is the ultimate showcasing of capacity building, as the host institution is now able to independently provide an extremely high level of care for this spe­cic patient population.
Also, essential to capacity building for the IBEC was the selection of the host institution that had the ability to house the collaboration and expand with it as it grew. The IBEC purposefully selected the second-largest hospital in Asia to partner with. Civil Hospital has a pediatric surgery department that oversees 100 beds and performs 2500–3000 surgical procedures per year. When selecting a host institu­tion, Civil Hospital had demonstrated through these parameters that it possessed the capacity to care for postoperative patients with BE.
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Research

The collaboration has been focused on research and, as such, has produced several collaborative manuscripts [17, 28, 34, 3638]. Each of these projects has equitably credited host partners with rst, co-rst, or corresponding authorship.

General Principles

Cultural sensitivity is of primary importance for the collaboration, so local partners lead all patient interactions, and all visiting partners are educated on cultural sensi­tivity prior to attending the collaboration.
Equally as important is the commitment to providing care to the IBEC cohort of patients that is equivalent to the standard of care within the visiting surgeons’ home institutions. The IBEC has reported outcomes that are comparable with those seen in HICs [17].
Overall, the IBEC offers a model for a sustainable, long-term global surgery col­laboration. It has generated positive, long-term benets for local partners and patients with surgical outcomes on par with HICs. By focusing on surgical capacity over volume, the collaboration’s scope has widened to increase the geographic catchment area for patients [34]. But, like all things in the eld of global health, there is always more work to be done to address gaps in care. Even well-designed interventions, like the IBEC, are still trying to address the unique challenges of BE to bring earlier referrals, operate on children at younger ages, and provide strategies to make the collaboration even more equitable for individuals of all social classes within the region [34].
Despite the continued work that is ongoing, the IBEC offers a template for global surgery collaboration when focused on addressing the burden of BE.Such projects must embody the principles of the ASAWG and should push the eld forward by maintaining its commitment to a sustainable, long-term collaboration. While this specic program deploys a large volume of resources for a rare disease such as BE, it serves as a template for other pathologies. BE, being a global genitourinary defor­mity, mastery of the procedure enables downstream expertise in other genitourinary reconstructive procedures such as hernia repairs, reimplantation of ureters, hypo­spadias, bladder neck reconstruction, and more. By using BE and the IBEC as a model for global collaboration, we submit that there are a multitude of benets for many more children with less rare conditions.

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