Fourteen-year-old Evyn Weiss has memories of wearing an anesthesia mask and being connected to tubes as a small child at Johns Hopkins. But those memories are balanced by ones of playing bingo with Johns Hopkins Child Life team members and picking out something special from their big bag of toys.
“While I do sometimes feel anxious about the procedures, I have grown to know that I will ultimately have a positive experience because of the incredible members of the medical and child life teams,” says Evyn, who raised more than $14,000 to support the Johns Hopkins Division of Pediatric Urology and Child Life Department as part of her bat mitzvah last year. “I am braver because of them, and I am really proud of everything that I have overcome with their support.”
Evyn was born with bladder exstrophy (BE), a rare and major birth defect that occurs in one out of every 50,000 live births where the baby’s abdominal wall and pelvic bones do not close, leaving the bladder exposed. Though about 60% of bladder exstrophies are diagnosed prenatally, Evyn’s wasn’t discovered until birth. She underwent an initial bladder closure procedure in Los Angeles when she was just three days old.
A year later, the Weiss family traveled to Baltimore to meet John Gearhart, MD, the Robert D. Jeffs Professor of Urology and a leading expert in BE. Hopkins has been a world referral center for nearly four decades and is among the top centers for primary and reoperative surgery of BE. Gearhart determined the initial closure had failed and performed an 8-hour bladder reclosure surgery on Evyn.
“If the closure works, then later on, BE patients can have a procedure to make them continent of urine. This is why it’s so important to go to a center of excellence like Hopkins,” Gearhart explains, adding that while the average children’s hospital may see two BE patients a year, he and his team have been working together for three decades to provide 15-20 BE surgeries annually. “If the first closure fails, they have less than a 25% chance ever of urinating from below and being dry.”
Gearhart’s team, which treats patients from around the globe, includes Dr. Heather Di Carlo, Dr. Paul Sponseller, Dr. Isam Nasr, pediatric surgery experts, pediatric pain service professionals, and a group of senior nurses.
“So it is a well-established, well-oiled machine that takes care of these babies,” Gearhart says.
Evyn and her parents, Danny and Joni, spent six weeks at Hopkins after her reclosure surgery, and Joni says the family became well-acquainted with the hospital and its “remarkable” staff.
“We were told that her orthopedic condition could potentially impede her ability to walk totally normally,” Joni remembers. “We are happy to report that she not only walks perfectly but just finished running in her third cross-country meet and is also a beautiful dancer.”
Evyn also enjoys playing volleyball and piano, acting in dramatic musical performances, and creating beautifully designed snack boards. She returns to Hopkins annually, and she and her family have developed close relationships with the medical and child life teams, as well as other BE patients, thanks in part to Hopkins’ yearly BE picnic. Three BE friends even live nearby.
“It’s a really sweet group, and the four of them are inextricably connected by their shared experiences,” Joni says.
So, when Evyn chose to engage in a Tikkun — a Hebrew word that means to mend or fix — social justice project as part of her bat mitzvah, she says she “knew without question” that she wanted to support the work of Gearhart and the pediatric urology and child life teams.
The funds Evyn has raised are supporting the Johns Hopkins Division of Pediatric Urology’s BE program and have helped purchase copies of A Story About You and Your Special Bladder, a book made for kids with BE that explains how the bladder works, what exstrophy is, and the possibility of future surgery. Friends and family have also purchased items from the Johns Hopkins Child Life Department’s wish list.
Gearhart says Evyn’s advocacy, which includes speaking at the annual picnic, has also helped lower the barriers for other young BE patients.
“It helps especially the young parents who have an 18-month-old, or a three-year-old, or a two-year-old with this. They see these beautiful, sophisticated young people like Evyn standing up there and speaking and they think, ‘My kid’s going to be like that.’ And that’s exactly right. It’s wonderful. It’s reassuring to young parents that there’s nothing awkward or weird about these kids. They just are born with a major birth defect, which can be fixed,” Gearhart says, adding that three doctors who practice at Hopkins have bladder exstrophy, and they are his patients.
Though Evyn will continue to see Gearhart and his team throughout her adolescence as she makes decisions about future procedures, her parents say her journey with BE has not held her back in the slightest.
“She is a thriving 14-year-old with no limitations,” Joni says. “Something remarkable about BE patients — and we’ve heard and seen this among other families as well — is that it instills a certain resilience and drive that leads to endless potential. We see that so clearly in our Evyn.”
Topics: Friends of Johns Hopkins Medicine, Johns Hopkins Medicine, Strengthening Partnerships
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