It didn’t take a urologist to notice the abnormality on Luke’s 20-week anatomy ultrasound images. “I’ll never be able to unsee the large, black bladder on the screen, knowing in my gut something was wrong,” says Katie Gingras, Luke’s mother.

Luke was diagnosed with congenital hydroureteronephrosis secondary to bladder outlet obstruction and had a lifesaving stent placement in utero a week later. Soon after, Katie and her husband, Matt, met with the Fetal Care Center team at Cincinnati Children’s. In attendance were David Hooper, MD, medical director of kidney transplantation at Cincinnati Children’s, and William (Bob) DeFoor Jr., MD, MPH. DeFoor is one of several pediatric urologists at Cincinnati Children’s who co-manages severe bladder outlet obstruction patients with nephrologists at the medical center.

“The meeting was overwhelming, but it helped us prepare mentally and emotionally for the challenges ahead,” recalls Gingras, a physical therapist specializing in neurorehabilitation. “The team provided realistic expectations of what Luke’s life would look like short- and long-term, in a way that made us feel incredibly supported and comforted.”

Luke was born in kidney failure in April 2022 and required ureterostomies in the first few days of his life. His kidneys were severely impacted by the blockage, presenting with bilateral cystic renal dysplasia. Broadly speaking, he would need a gastronomy tube to assist with his intake needs, surgeries to dilate his narrowed urethra, two revisions of his catheter line due to obstructions, months of bladder rehabilitation and dialysis—all in his first 16 months of life— before a decision could be made about listing him for transplant.

Near-Daily Interactions

“It is safe to say that very few pediatric urology teams have the experience and expertise ours does in co-managing patients with severe, prenatally detected bladder outlet obstruction,” DeFoor says. “Interpreting test results, knowing when to do the ureterostomy and when to reverse it, managing the bladder cycling process, doing the surgical reconstruction, working closely with nephrology to coordinate everything—all of that requires disparate skill sets we’ve acquired by caring for patients with these complex conditions year after year.”

Co-managing complex patients like Luke can involve near-daily interactions with nephrology in the clinic and inpatient setting, on the phone and by text. The physicians often coordinate care with other specialists, such as pulmonology and otolaryngology. They participate in weekly care conferences to discuss patients who are getting closer to transplant.

“We have built a lot of trust over the years, which gives us the freedom to share contrasting perspectives as treatment decisions arise,” DeFoor says. “We’re working in different lanes but going in the same direction.”

A Joint Decision to Do Post-Ureterostomy Reversal

Luke’s journey demonstrates how beneficial this collaboration can be, especially as families navigate difficult decision points and cope with setbacks and uncertainty. Gingras recalls how DeFoor and Hooper guided her family through one painful turning point. At that time, the parents had been cycling Luke’s bladder for five months.

“When we started the process, his bladder could capture 3 mL of urine,” says Gingras. “Progress was slow, and Drs. DeFoor and Hooper had been discussing whether to schedule the post-ureterostomy reversal to stretch his bladder to an appropriate, viable size for a kidney transplant. They told us that the reversal would likely stress his poorly working kidneys into complete failure.

“Both doctors took time for a long Zoom call with my husband and me to discuss the pros and cons, additional ideas and possibilities,” Gingras continues. “We ultimately decided that the possibility of improving Luke’s bladder viability outweighed the impact of pushing him into end-stage renal failure resulting in dialysis. The collaboration that these doctors provided our family at that time was invaluable. We truly felt listened to, as if we were colleagues working together for Luke’s best interests.”

Awaiting a Kidney Transplant

Three months after the post-ureterostomy reversal, a single straight catheterization of Luke’s bladder could capture >150mL of urine. He is on hemodialysis and the national kidney transplant waiting list in hopes of receiving his perfect match by early 2024.

It has been a long road for the Gingras family, and Luke’s future is uncertain. But DeFoor speaks for the entire care team when he says the intense effort is worthwhile. “Luke is such a sweet little boy, and when he smiles at you, you just know—you’ll do everything in your power to give him a great life.”