With chubby cheeks and eyes that sparkle when he giggles, Bracken is the light of his parents’ lives. The 1-year-old loves Elmo, jumping, singing, and chasing his family’s two dogs.

“Looking at him, you’d never know what he’s gone through,” says his mom, Bridget.

Like most new parents, Bridget and Jason were looking forward to the birth of their first child. At their 20-week ultrasound, their obstetrician in York, PA, noticed something amiss. He sent them to The Children’s Hospital of Philadelphia (CHOP) for further tests.

The couple met with Nahla Khalek, MD, MPH, FACOG, an attending obstetrician at CHOP’s Center for Fetal Diagnosis and Treatment. After a day of extensive testing, Dr. Khalek confirmed what their obstetrician had suspected: their baby had bladder exstrophy.

Bladder exstrophy is a rare condition that occurs during fetal development. The bladder doesn’t develop normally and is exposed through an opening in the abdominal wall. Their son would be born with his bladder on the outside of his body.

The condition, which affects about 1 in every 30,000 births, requires delicate reconstructive surgery by urologic and orthopedic surgeons.

The couple was in shock. A million questions swirled in their minds: How did this happen? Why did it happen? Was it treatable? Could their baby have a normal life?

“I just couldn’t stop crying,” Bridget says. Her husband was more stoic as he waited for answers.

Coming up with a plan

They met with Douglas A. Canning, MD, an internationally recognized leader in pediatric urology and chief of CHOP’s Division of Urology. Dr. Canning thoroughly explained their son’s condition and answered their questions.

“We were so scared, so unsure; yet Dr. Canning made us feel better,” Bridget says. “He understood our fears and answered question after question about Bracken’s condition. He told us how he would treat it, about possible complications, and that most kids with this type of condition live happy, active lives.”

The couple decided CHOP offered their unborn baby the best chance at a normal life. For the next few months, they commuted from York, PA — more than a two-hour drive — to Philadelphia for prenatal appointments.

On Aug. 6, 2014, Bracken was born at CHOP’s Garbose Family Special Delivery Unit, the world’s first birth facility in a pediatric hospital designed for mothers carrying babies with known birth defects.

An experienced team of neonatologists, urologists and nurses began immediate care for Bracken and his exposed bladder. Initial treatment included covering the exposed bladder with gel and a special dressing to keep the area moist and protected.

After a day in the Newborn/Infant Intensive Care Unit, Bracken was stable enough to be moved to a private room where his parents could visit with him.

With the help of doctors and nurses, Bracken’s parents learned how to change the dressing covering his bladder and how to correctly position his diaper over the bladder. The goal was to keep Bracken's bladder from being irritated by his diaper before surgery.

Once Bracken’s parents felt comfortable caring for his covered bladder and related issues, they were allowed to take him home before returning for Bracken’s surgery.

Surgery

On Aug. 18 — just 12 days after his birth — a multidisciplinary team, including urologic and orthopedic surgeons, pediatric anesthesiologists and specially trained surgical nurses, performed the complex bladder exstrophy repair.

On the night before surgery, exstrophy specialists from Wisconsin and Boston arrived to help record important steps with the delicate surgery and to help coach the team during critical portions of the procedure. CHOP collaborates with the two institutions to help surgical outcomes in exstrophy.

First, orthopedic surgeon Wudbhav N. Sankar, MD, surgically separated Bracken’s pelvic bones that had formed improperly in utero. 

Then Dr. Canning performed the delicate repair of Bracken’s bladder, bladder neck and urethra and repositioned these structures back into the appropriate positions in the tiny baby’s body. For Bracken, this was particularly difficult because his bladder was extremely small.

Once the bladder repair was complete, Dr. Sankar placed Bracken into a special traction system to help splint the baby's hip bones into a more normal position to better protect Bracken’s internal organs and help improve his eventual urinary continence.

“Doctors and nurses kept coming out to tell us how each part of the surgery was going,” Bridget says. “We couldn’t be in the room but we knew he was in good hands.”

Once Bracken’s surgical recovery was complete, he was finally able to go home with his parents. But, as Bridget and Jason soon discovered, urologic issues don’t necessarily end with one surgery.

Ongoing care

At home, Bracken thrived in most ways almost immediately. He gained weight. He hit developmental milestones. He was a happy baby.

But his bladder was still very small. Because of this, he developed urinary reflux — where his urine would back into his kidneys — and frequent urinary tract infections that required hospitalization.

When Bracken was 9 months old, Dr. Canning told his parents that Bracken’s ureter and kidneys needed a break from the cycle of infections.

Dr. Canning gave the couple two options: He could implant a suprapubic catheter into the bladder through a small hole in Bracken’s belly — a semi-permanent solution — or they could learn how to catheterize him at home. Catheterization would give Bracken’s ureter and kidneys a break, and give his bladder time to grow, but would not require surgery. This would prove difficult because even though Bracken's urethra was in a good position, it was not easy to place the catheter.

“We wanted to try catheterization because it was less permanent,” Bridget says. “But I was having a really hard time learning how to catheterize him. Part of me worried I was hurting him.”

So Bracken and his parents came to CHOP for intensive education. “We worked with Dr. Canning, urology residents and nurses for four days until I learned how to catheterize Bracken and felt comfortable doing it by myself,” recalls Bridget.

Today and tomorrow

For the past three months, Bracken has been infection free. His parents catheterize him every two to three hours to drain his bladder, which is helping Bracken’s bladder grow. His ureter and kidneys are also no longer inflamed. And his family hopes his frequent catheterizations won’t be needed for much longer.

“We’re not sure what’s next for Bracken. He may need additional surgery or treatment as he grows. But we’re confident Dr. Canning and CHOP will be there for us — whatever we need,” Bridget says. “Right now, we’re just enjoying our happy little boy.”

“I’m so glad we went to CHOP,” Bridget says. “I don’t even want to imagine how Bracken would be doing if we had gone anywhere else.”