Advancing Clinical Protocols for EB Urologic Complications
Epidermolysis bullosa (EB) is a rare inherited condition that presents challenges when managing urologic complications. An error in a skin protein can cause varying degrees of skin fragility, causing internal and external blisters and scarring. From a urologic perspective this can affect the bladder, ureters and urethra. Damage from EB can lead to meatal stenosis, obstructive uropathies, ureteral strictures and urinary tract infections.
At the Epidermolysis Bullosa Center at Cincinnati Children’s, patients receive comprehensive care and the opportunity to live fulfilling and productive lives. Every patient “has lots of eyes on them,” says Beth Moeves, APRN-CNP. The team regularly reviews cases to provide the best care, using several modifications to manage EB-related urologic conditions, including:
- Circumcision protocols. Surgeons use Vaseline instead of Dermabond™ to prevent skin sticking to a diaper and to promote healing.
- Meatal stenosis. Boys are more likely to develop narrowing at the tip of the urethra when the penis rubs against underwear. This can cause infection and trouble urinating. Surgeons can widen the opening to provide relief and prevent backflow. Though less common, meatal stenosis also occurs in girls with EB.
- Kidney stones. Children with EB are prone to kidney stones. “One of the big lessons we learned is that stents are dangerous,” says Pramod Reddy, MD, director, Division of Pediatric Urology. “If it moves in the lower end of the ureter as it goes into the bladder it can cause trauma, scarring or a blockage of the ureter.”
“What sets us apart is the coordinated and compassionate care we provide while respecting the autonomy of the child and their parents,” says Reddy.
Each year, the center provides expert care for more than 200 children and adults from more than 25 states and several countries.
Innovations and Modifications
Most patients with EB are diagnosed shortly after birth. The severity of their condition depends on the layer of skin affected. The most aggressive type of EB impacts the skin’s inner base layer.
Novel medicines and salves now help protect the skin’s natural microbiome, preventing infections that, years ago, could have been fatal.
Anesthesia protocols ensure safe surgeries for these patients, Reddy says. Fragile skin cannot tolerate tape used to secure an IV or a breathing tube. Instead, surgeons use Bovie electrosurgical grounding pads to avoid electrical shock and burns. The pads are placed on the skin, allowing tape to be secured to the pads rather than directly on the skin.
Moeves says patients have appreciated the team’s anesthesia care protocols. “They found it very comforting that they didn’t have to teach us how to care for their skin.”
As one of the nation’s first EB centers, Cincinnati Children’s offers specialized care, including:
- Improved nutrition
- Novel antibiotics for infection prevention
- Safe anesthesia protocols
- Skin barriers to prevent infection and protect skin
- Urologic procedures for improved function and health
