Noah, 10, was diagnosed as a baby with caudal regression syndrome, a genetic condition that affected her lower body.
The condition sometimes comes with a neurogenic bladder, which includes symptoms such as urinary tract infections, loss of feeling that the bladder is full and urinary incontinence. By her second-grade year, Noah struggled with these issues, a stressful time for the family.
“Noah was having so much trouble,” said Leah, Noah’s mother, “and we were sad she wasn’t able to be a ‘normal’ kid.”
After talking with the school nurse, Leah and Saul, Noah’s dad, made an appointment with the Division of Urology at Lurie Children’s.
Ranked 7th in the nation by U.S. News & World Report, the Division of Urology at Lurie Children’s, is the largest pediatric urology department in the region with more than 18,000 pediatric visits each year for both common conditions, such as bedwetting, and more complex ones, like bladder exstrophy.
At Lurie Children’s, the family met with Elizabeth Yerkes, MD, a urologist and surgeon who specializes in neurogenic bladder, to start catherization training. This meant Noah’s school nurse, parents or grandmother would empty her bladder using a traditional catheter every three or four hours. While effective in keeping the girl dry and comfortable throughout the day, it also meant Noah was limited in some of her activities.
About a year later, Dr. Yerkes discussed with the family the possibility of what’s known as a Mitrofanoff procedure, in which a surgeon creates a small tunnel connecting a small hole (stoma) in the skin of the torso to the bladder using the appendix or part of the intestines. Once recovered, the patient can empty his or her own bladder without much disruption of their daily activities by sliding the catheter through this small hole.
In most cases, this procedure allows for greater independence for children with neurogenic bladder because, after some training and practice, it allows them comfortably to empty their bladder on their own privately wherever they are during the day. Sometimes the patient will benefit from additional procedures at the same time, such as making the bladder larger with another piece of intestine (bladder augmentation) to enhance continence. These decisions are individualized based upon patient and family goals and the patient’s bladder situation. Noah and her parents chose to have bladder augmentation with the goal of continence without medications.
“Dr. Yerkes understood that it was a priority for our family to give Noah the most ‘normal’ life we could, despite her challenges,” Leah said. “She always takes the time to go through and talk to not only me but also Noah. She speaks to Noah, which to me is such a small but monumentally meaningful gesture. She doesn’t speak at or over her.”
Trusting Dr. Yerkes, the family chose to pursue the procedure in November 2019.
Thanks to a process that Lurie Children’s urologists implemented at the hospital to help children recover more quickly from invasive urological procedures, Noah was up walking right away and eating takeout sushi just a few days after surgery. On the fourth day after surgery, Noah was able to return home.
This spring, Noah finished fourth grade. She feels great, her mom said, and isn’t limited in doing her favorite activities such as swimming, scuba diving and riding horses.
“Everyone told us going into Mitrofanoff surgery that this would be a game-changer for Noah and we couldn’t have possibly understood the magnitude of this for her,” Leah said. “It has allowed Noah so much independence and self-reliance -- more than we could have ever known.”