Published on in Urology Update
Ninety-two percent of the global burden of congenital anomalies occurs in low-and-middle-income countries. Worldwide, one in 10,000 to 50,000 children are born with bladder exstrophy-epispadias complex (BEEC), a congenital disorder in which the lower abdominal wall fails to develop and the bladder develops outside the body. As a result, a child born with BEEC is unable to store urine and is therefore incontinent.
A little over 50 years ago, medical consensus was that bladder functionality could not be restored in patients with BEEC. Since then, through surgical advances, bladder functionality can be restored using surgical intervention, bringing hope to these children and their families. Often, however, this requires multiple operations and hospitalizations to close the bladder, urethra and abdominal wall to restore urinary continence while protecting renal function and allowing for the creation of cosmetically acceptable genitalia.
In India, given that there are 25 million live births each year, it is estimated that 500 to 2,500 children are born with BEEC. Without surgical intervention, children with BEEC face a life of disability and being stigmatized and ostracized in their communities. These challenges often affect a patient’s entire family due to the socially isolating issues of foul-smelling urine, lack of urinary absorptive clothing, and a lack of access to clean laundry facilities.
Children with significant physical disabilities like BEEC also have an increased risk of psychosocial problems; thus, in addition to the surgery, they also need comprehensive psychological evaluation, counseling, and follow-up care to support their well-being. However, like in many low-and-middle-income countries (LMIC), pediatric surgeons most often manage pediatric urology given a shortage of urologists, and most have not had exposure to this complex condition. Without expertise in this area, the surgeries may fail, or the babies will have to live with an open bladder.
Expanding access to care for BEEC
Since 2009, pediatric urologists at Children’s Hospital of Philadelphia (CHOP) led by Aseem Shukla, MD, have provided care and helped build capacity in India to provide surgical interventions to children with BEEC and advance scientific knowledge about BEEC through research. Our team has collaborated with doctors from Cincinnati Children’s Hospital and Seattle Children’s Hospital, as well as the host surgical team at Civil Hospital Amdavad and B.J. Medical College in India. Watch the following video to learn more about this collaborative and how it's impacting the lives of children and families around the world:
Since this partnership began, we have used a three-pronged approach to improve pediatric urologic services:
- Surgical/clinical care — The same CHOP pediatric urology team has traveled to India and has performed surgery on nearly 150 children over 10 years
- Training/capacity building — During each surgical clinic, our team provides training to Indian and U.S. physician and advanced nursing trainees, as well as to trainees from other countries.
- Research — There is rigorous follow-up of patients to monitor and evaluate patient outcomes and their needs post-surgery, and to understand the patient experience of BEEC before and after surgery.
Research indicates that in addition to the physical challenges of having BEEC, the stigma and related psychosocial problems that result from living with a health condition like BEEC can affect how patients access care, their adherence to care instructions, and their willingness to engage with the community around them. Additionally, parents and caregivers of children with BEEC, especially in their child’s first year of life, struggle to cope, and this can hurt the parent-child relationship. Through this partnership, we are working to transform the lives of children with BEE and their families, and give children with this condition the opportunity to grow into healthy adults who can participate fully in society.
Dr. Shukla and his team have also been collaborating with physicians from military children’s hospitals in Beijing, China, to develop a center of excellence in pediatric urology and one of the first pediatric urology robotic surgery programs in the country. The team’s goal is to improve safety and quality and share best practices.