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Our patients’ stories: overcoming bladder exstrophy

  • Kipaya Kapiga
  • 10/7/2013 12:00:00 AM
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On a warm June morning, a District of Columbia tour guide stops in front of the Korean War Memorial. Pointing out the 19 statues erected in tribute to soldiers who gave their lives in the conflict, he explains that the impressive seven-foot sculptures are meant to represent the 38th parallel, the demilitarized zone separating North and South Korea. When he turns to ask the group why a reference to the 38th parallel would only contain 19 statues, 16 year-old Thomas Vincent immediately gets to work and soon has a theory.

Thomas, who is in the nation’s capital to meet his senators and congressional representative as a patient representative of Boston Children’s Hospital, is the portrait of an over-achieving student. When not busy with homework, chances are you can find Thomas practicing for any of the three sports he plays for his school. If he’s not on the field or the court, you might find him tutoring other students or at Spanish club. At first, second and even third glance, he is healthy and vital—the last person you might believe required extensive surgery only hours after he was born.

Dreary start to a bright future

Complete primary repair of exstrophy (CPRE) to treat Bladder Exstrophy When Thomas was born early Thanksgiving morning in 1996, the obstetrician and pediatrician-on-call at the Vincent’s local hospital immediately knew something was wrong. “I just heard them say, ‘We have a problem here’ and they took him away,” Thomas’ mother Jean explains. "It was terrifying." 

Looking at the newborn Thomas, doctors could see part of his bladder was exposed, and they soon diagnosed him with bladder exstrophy, a rare congenital condition where part of a newborn’s bladder protrudes out of the body. (Or, as Thomas has gotten used to explaining, “it basically means my insides were on the outside.”)

Although bladder exstrophy can sometimes be diagnosed in utero through an ultrasound, not all technicians know what to look for. So, when Thomas was delivered with his bladder exposed, his doctors were as surprised as his parents.

Within hours, a transport team whisked the newborn to Boston Children’s—with his father following in his car—where Thomas would become only the second patient at Boston Children’s to undergo a complete primary repair of exstrophy (CPRE).

The procedure—developed by former Boston Children’s physician Michael Mitchell, MD, in 1989—not only placed Thomas's bladder back inside his abdomen, but also repaired anomalies to his urethra and reset his hips. By performing CPRE, Thomas’ surgeon, W. Hardy Hendren, MD, and Joseph G. Borer, MD, were able to address all of these issues in a single procedure, rather than spreading the operation across three separate surgeries as other treatment methods require.

It took Hendren and Borer more than 12 hours to complete the CPRE, lasting from Thanksgiving afternoon to 4 a.m. the next morning. In the end, the procedure was a success. A day later, Jean was able to join her husband in Boston and set eyes on her son for the very first time. To give his organs time to heal, Thomas’ doctors placed the newborn in traction and it would be another three and a half weeks before Jean was able to hold her son.

No sign of slowing

Complete primary repair of exstrophy (CPRE) to treat Bladder Exstrophy Fast-forward 16 years, and unless you knew him well, you would never know Thomas is still living with a congenital bladder condition. Like most bladder exstrophy patients, he’s had other operations, but none have slowed his drive or spirit. Some have been planned, including surgery to enlarge his bladder and install a stoma to help drain it. Others were unanticipated, including surgery to repair a bladder rupture from a sports injury.

Regardless of the time he’s spent in hospitals, Thomas has barely skipped a beat in his busy life. At school, he is a star pupil and has played for his school's basketball, soccer and tennis teams since his freshman year. He is also involved with Amnesty International and even helps backstage at school theatre productions. He works with disabled students in an equine therapeutic program. “When you throw in homework, it’s a lot,” he admits.

Thomas also isn’t shy about speaking up and sharing his experiences with other patients. When he first attended Youth Rally, a weeklong summer camp for teenagers with bladder and bowel conditions, he was so engaged with other campers he was mistaken for a counselor—a position he hopes to hold one day. “Growing up, everyone says this is a rare condition and a lot of kids think no one knows what they’re going through,” he says. "But Youth Rally’s motto is 'you are not alone', which is something that really resonates with me. I take it to heart."

The missing 19 statues

Back in Washington, D.C., Thomas is sure that the answer has something to do with the soldier's reflections being visible in memorial’s reflective wall—and when he voices his answer, the tour guide confirms his theory.  Later that afternoon when Thomas speaks with Senators Kelly Ayotte and Jeanne Shaheen and Congresswoman Carol Shea-Porter, he’s enthusiastic and engaged, displaying a seriousness of thought not normally associated with boys his age.  Watching him in this scenario it's easy to see why Boston Children’s invited Thomas and his parents to DC to represent the hospital at Family Advocacy Day, an annual event that brings families and legislators together to discuss healthcare reform.

Looking ahead, Thomas is focused on his last two years of high school and wants to attend college in Boston. “My absolute dream would be going to MIT, because of their biochemistry program. Also, they’re a Division 3 school, so I could play soccer,” he says.

He may face more surgery in the future, but given his track record it’s unlikely to slow him down. As his father Maurice says, “there are different levels of severity for bladder exstrophy, but having a condition like this doesn’t mean a child can’t lead a full, active life. Thomas is living proof of that.”

Visit Boston Children's Center for Bladder Exstrophy website for more information on their unique approach to treatment.

 

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