Introduction and Objectives

Bladder exstrophy (BE) is a severe congenital birth defect in which the bladder and external genitalia are born inside-out, and adjacent tissues are not joined together. This rare condition (approximately 3.3/100,000 live births) is more prevalent in boys and requires many major surgeries throughout life to protect renal function and achieve continence and reproduction. Adult urologists familiar with these procedures and their complications are essential for adulthood as urinary and reproductive function remains a lifelong struggle.

Methods

There is a lack of adult research, support and resources. The majority of BE research is focused on clinician-derived research priorities and surgical outcomes with little patient-generated research on what BE adults need. In 2018, the Association for the Bladder Exstrophy Community (A-BE-C) surveyed and interviewed our adult population. A parent stated: “Over the last several years, we have had a very difficult time in the proper management of his condition. There seems to be no support or specialists for him once he became an adult and could no longer see the pediatric specialists. He has become overwhelmed and disheartened and spent the last few years ignoring the fact he really needs a specialist and cannot rely on our general practitioner or a generic urologist at the local hospital for proper care.” Many adult urologists are not prepared to treat BE adults, and often pediatric urologists must care for them. If pediatric urologists are unable to provide care, adult patients have nowhere to go for treatment.

Results

With proper treatment, many children have survived to adulthood. Lifelong followup is mandatory and transitional care must be achieved for successful long-term care and quality of life. A-BE-C’s Center of Excellence transition models support early introduction to the adult provider. Comprehensive care of adult patients fell into 6 major categories: teens and puberty, women’s and men’s health, adult BE health maintenance, maintenance of childhood revisions/diversions, psychosocial concerns and medical self-advocacy

Conclusions

There is a thriving and growing population of adults with exstrophy in the United States and worldwide, with needs that both mirror and differ significantly from the pediatric population. A-BE-C is meeting the growing needs of adults with exstrophy globally through conferences, collaboration with exstrophy-specific organizations, surgical workshops, articles, webinars and research participation with exstrophy-designated Centers of Excellence.