Following an easy pregnancy, Kathryn and Zach were ready, and thrilled, in 2018 to meet their first child: a baby boy they named Liam.
But as soon as he was born, doctors and nurses at the Rockford, Illinois, hospital were surprised to find an opening larger than a quarter around his bladder.
“They had only heard of the condition, bladder exstrophy, in textbooks,” said Kathryn.
A rare condition, bladder exstrophy occurs when the bladder or bowel does not form properly in utero. The condition requires complex surgical reconstruction and other treatments. Sometimes it can be diagnosed following ultrasounds before a baby is born, but in other cases, like Liam’s, health care providers see the opening and make the diagnosis only after birth.
Just hours after he was born, Liam was taken by ambulance to a hospital in Madison, Wisconsin, where specialists confirmed he had bladder exstrophy.
“There was so much going on and so quickly. It felt overwhelming,” Kathryn said, but added that she and Zach found comfort in knowing the condition was not life-threatening.
More common in males than females, the condition can lead to issues later in life with urination and other functions, but in most cases, surgical treatment reduces these complications.
The surgeon in Madison referred Liam’s family to Earl Cheng, MD, at Ann & Robert H. Lurie Children’s Hospital in Chicago.
“He was retiring and told us ‘my good friend Earl Cheng knows everything about this condition,’” Kathryn recalled.
The parents headed to Chicago with their newborn.
Finding the Right Care Team
Dr. Cheng, and Lurie Children’s urologist and surgeon Elizabeth Yerkes, MD, direct the hospital’s bladder exstrophy program, along with exstrophy team member Diana Bowen, MD. Kathryn said Dr. Cheng’s expertise in the condition was obvious to her and Zach almost as soon as they met with him.
“There’s something very calming about Dr. Cheng. He was just on top of everything, telling us exactly what the condition is, the treatment process and how it would work,” Kathryn said.
In addition to co-leading the exstrophy team, Dr. Cheng is the division head of urology at Lurie Children’s and also a professor of urology at the Northwestern University Feinberg School of Medicine in Chicago. He helms a team of researchers and a Midwestern consortium of children’s hospitals, which aims to identify advances and best practices in bladder exstrophy treatment.
Furthermore, earlier this year, Lurie Children’s was recognized as a premier provider of treatment for bladder exstrophy. It is among nine children’s hospitals in the U.S. on the Association for the Bladder Exstrophy Community’s Centers of Excellence list, designating it as a program that meets the highest standards of treatment for the condition.
“We knew we were in amazing hands,” Kathryn said.
The First Step in a Long Journey
Bladder exstrophy appears differently in each patient and presents a wide range of potential complications and abnormalities. Necessary surgeries and treatment, therefore, vary by patient.
With more surgeries and perhaps medication, some bladder exstrophy patients can achieve bladder control without needing regular assistance from a catheter. Others may need intermittent assistance from a catheter to urinate.
Liam’s exposed bladder and urethra meant his body was unable to hold urine, so Dr. Cheng and his team needed to close the exposure and rebuild Liam’s genitals in two separate surgeries.
Kathryn said Dr. Cheng explained to her family every step of the process for Liam, who would endure the first at age four months.
After the first surgery, Kathryn, a kindergarten teacher, and her husband, an accountant, had to learn how to care for their son while he was recovering, including managing a drainage bag for his urine and keeping his surgical incision clean.
“When we went home, it was hard not being steps away from the doctors and nurses, but they made sure we were confident to care of Liam for the rest of his recovery,” Kathryn said.
He had the second surgery at thirteen months. Liam had a catheter for about two weeks while in recovery, with which he had some issues.
“But Dr. Cheng and the urology team made sure that he was comfortable,” Kathryn said. “Liam recovered well from this procedure.”
‘Excited to See who He Grows Up to Be’
Now Liam, who is 15 months old, spends his time at home meeting milestones, playing with his toy cars and trucks and chasing the family’s cocker spaniel, Ruby.
Like many patients with bladder exstrophy, he will need more surgeries later in his life. Dr. Cheng said the best plan for Liam is to wait at least two more years to see whether and how much Liam’s bladder grows, which will help determine the next steps.
In the meantime, Liam’s appointments with Dr. Cheng for scans and check-ups are growing further and further apart.
“We know Liam has a long road ahead of him, but we feel confident that Dr. Cheng and the Lurie Children’s team is ready to help,” Kathryn said. “We are so excited to see Liam continue to grow and develop and see who he grows up to be!”