Neurogenic bladder is the norm for the vast majority of children with spina bifida (SB) because of damage to the nerves that control both bowel and bladder function. Advances in treatment mean that most children with the disease are now living long into adulthood, but potential kidney problems make lifelong bladder management a critical factor in their care. With one of the largest SB clinics in the United States, the Duke Section of Pediatric Urology uses an
array of treatment modalities and ongoing research to
focus on 3 primary goals for these patients:
- To grow up with normal kidney function
- To experience fewer urinary tract infections throughout their lives
- To become continent and avoid wearing diapers
“Not many hospitals in the country have the breadth and depth of experience in managing bladder disorders that we have at Duke—from simple cases to the most complex,” says John Wiener, MD, a Duke pediatric urologist and Duke’s Principal Investigator for the Centers for Disease Control and Prevention (CDC) National Spina Bifida Patient Registry. “We work with patients from the prenatal stage to well into their 50s and beyond, and we really provide lifelong, state-of-the-art care to them,” says Wiener.
Duke is 1 of 26 centers in the United States that are part of the National Spina Bifida Patient Registry, which collects data to evaluate existing medical services for SB patients and provides a framework for improving quality of care at SB clinics nationwide. Duke University Hospital is also 1 of 9 hospitals selected by the CDC to evaluate the optimal bladder management of children with SB from birth through age 5. Duke pediatric urologist Jonathan Routh, MD, leads the study nationally, and Wiener is a local Principal Investigator.
Clean intermittent catheterization (CIC) to regularly empty the bladder is sometimes the only treatment patients need, says Wiener. Others will need medications, and oxybutynin is currently the only approved medication for children. “That’s why we’re engaged in studies with industry to get FDA approval for medication options for children that are now only available for adults,” he says.
Surgical options include injection of onabotulinumtoxinA into the bladder muscle, bladder augmentation to enlarge the bladder and lower its pressure using a section of the bowel, and reconstructing the bladder neck or placing an artificial urinary sphincter for patients who have weak sphincter muscles. For patients who have difficulty with catheterization, appendicovesicostomy is an option, in which the appendix is detached from the intestine and used as a channel between the bladder and the skin, enabling patients to catheterize their bladder through the skin without leakage.
“When we do surgery to get them dry, we also frequently do bowel procedures so patients simultaneously gain fecal continence,” says Wiener. These surgeries are also performed on children with other neurologic issues, such as spinal cord injury, spinal cord tumors, and cerebral palsy.
Wiener notes that surgery is typically not performed until children are school-aged and can understand that the operation is being done for them and not to them, and, at that age, they’re also mature enough to realize the benefits. “Helping children start wearing underwear instead of diapers for the first time is a very gratifying part of what we do,” he says.
For children without neurologic disorders who have bladder dysfunction, surgery is rare. Behavioral therapies often help these children become continent, including:
- Simple technology, such as vibrating watches, to remind them to go to the restroom
- Enuresis alarms to cure bedwetting
- Exercises to help children “find” their sphincter muscle and learn to relax it
- Animated biofeedback, in which children play video games by controlling characters on the screen by contracting and relaxing their sphincter muscle
As highly experienced clinicians and researchers, Duke pediatric urologists and nurse practitioners are heavily involved in ongoing federally funded research to further explore bladder dysfunction and treatments in specialized populations, such as children with Pompe disease and diabetes.
Most people don’t realize that the bladder is affected by diabetes, says Wiener, and no one has investigated how and when diabetes affects bladder function in children and adolescents despite its increasing prevalence. Duke pediatric researchers include Maryellen Kelly, DNP, who has a grant to study this at Duke and around North America, and J. Todd Purves, MD, PhD, who is investigating this connection in the laboratory.