High school freshman Hope has had enough surgeries to know exactly what to do when she wakes up from a procedure. She focuses on the people around her, slowly taking in one and then another. She concentrates on her surroundings and reminds herself where she is: safe in a Children's Health℠ recovery room. And that helps her push away any pain or fear.
"She has always been so positive in facing everything she's up against," says Hope's mom Kat. "She always tells me ‘I was born ready.' When I ask her if she's ready for her next appointment, ready to get up after surgery, ready to leave the hospital - it's always ‘I was born ready.'"
Hope was born with cloacal exstrophy. This extremely rare condition happens when a baby is born with abdominal organs, like the bladder and intestines, outside of their body. It requires a series of surgeries throughout childhood and adolescence to get the digestive, urinary and reproductive organs in the right place and working as well as possible.
"I've treated fewer than five patients with this condition in the 20 years I’ve been at Children’s Health. That’s how rare it is," says Joseph T. Murphy, M.D., pediatric general and thoracic surgeon at Children's Health. "It happens when the abdominal wall and pelvic organs don't form fully or close correctly in the womb."
The family's journey started in Louisiana more than 15 years ago, before Hope was born.
Expert care, far from home
Kat's local doctor in Louisiana knew something wasn't right on her five-month pregnancy ultrasound. They sent her to the nearest specialist in Shreveport, LA. But that was still 180 miles away from where Kat would find the care her baby needed.
"That doctor saw a lot of anomalies on the ultrasound, but wasn't sure how they all correlated into one diagnosis," Kat says. "When Hope was born in Shreveport, doctors there realized they could not care for her complex needs and life-flighted her to Children's Medical Center Dallas."
Hope's dad, Michael, went with her, while Kat recovered from her C-section. Three days later, Kat and a friend set off on the four-and-a-half-hour drive to meet Hope and Michael in Dallas. Hope had already undergone one 10-hour surgery to close her abdominal wall and move her bladder inside of her body.
"Her doctors were extremely thorough in trying to explain everything. It was complicated," Erin says. "They really took the time to make sure we understood all that they had to do, why it was important for Hope's health and what her life was going to look like."
Hope's care team was made up of specialists from departments across Children's Health - urology, gastroenterology, pediatric surgery and NICU - who knew how vital her first surgeries were for her future. Finally, after five weeks in the hospital, their family got to go home. But their journey with Children's Health was just beginning.
An expert care team and strong support system
Cloacal exstrophy affects children in different ways. But all of them need several surgeries early in life to help them go to the bathroom, move their organs into the right place and get them working as well as possible.
"Among the many reasons these children often require repeat operations is because their bodies grow and change, so their surgical repairs and anatomical corrections need to be revised over time," Dr. Murphy says.
In addition to surgeries to get her bladder working, Hope needed surgery on her spine and hips. She needed assistance to walk as a small child, but thanks to her expert surgeons and her hard work in physical therapy, she now walks independently. Over the years, Hope and Kat learned the ins and outs of Children's Health and built a support system for their family.
"I always appreciated how Diane Becker, RN, in the pediatric surgery clinic would call and make sure Hope was doing okay and was getting everything she needed," Kat says. "That attentiveness was so important, especially since we live four-and-a-half hours away. They really worked with us on scheduling so we didn't have to make extra trips."
This expert care has helped Hope grow into a resilient teenager.
"I wouldn't say this affects me every day necessarily. It's just part of my life and there are certain things I need to do to take care of myself," Hope says.
High school and beyond
As Hope takes on high school, she's reaching a milestone in her care: the end of more than 25 surgeries. Her providers generally focus on keeping her organs working as well as possible and maintaining her overall health.
Hope now receives ongoing, multidisciplinary care within the Colorectal and Pelvic Center at Children's Health, the first program in North Texas established to care for patients, like Hope, with congenital conditions that affect the pelvis and bowels. The program is made up of a team of experts who work together to provide expert care tailored to each child's needs for the best long-term outcome.
"The relationships we've built with these doctors over the years is something we've both appreciated," Kat says. "Especially when you have a child that has so many doctors, it can feel daunting if you are having to go over a history with a new doctor all the time, so to have one that knows your history, that has built a relationship with your child is so valuable."
While Dr. Murphy understands why Hope hasn't needed an appointment with him lately, he's grateful to have been part of her care team since she was a baby.
"Hope is one of my favorite long-term patients because of her infectious attitude and upbeat optimistic outlook. She has been through so many things that would defeat most kids. She just bounces back with a great attitude and undefeatable resilience," Dr. Murphy says.
Hope looks forward to spending less time at the hospital, and more time pursuing her many interests. She loves reading and drawing - especially getting ideas of things to draw on Pinterest and then reimagining them and drawing them from her own perspective.
"I really want to keep doing art," she says. "I might eventually go into baking or try web development or programming. There are a lot of things I want to do."
Hope has a bright future thanks to her perseverance, her family's support and the longstanding support and expertise of her care team.
"Her name is Hope for a reason," Kat says. "I really didn't know what to expect when I walked through the doors of Children's Health that first day. But I've learned that every time, we leave a little better than when we came. That's a triumph for us as a family. And it's a triumph just knowing how it feels to walk with her through this and see her overcome."
Her name is Hope for a reason. I really didn’t know what to expect when I walked through the doors of Children's Health that first day. But I’ve learned that every time, we leave a little better than when we came. That’s a triumph for us as a family. And it's a triumph just knowing how it feels to walk with her through this and see her overcome.
Learn more
The Colorectal and Pelvic Center at Children's Health is home to a team of experts with deep experience caring for babies, children and teens who were born with conditions that affect their pelvis and bowels. The team creates a custom care plan for each child to meet their unique needs. Learn more about the Colorectal and Pelvic Center at Children's Health.
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