Lexi Isler got a kiss from her father, Luther, before the kidney transplant surgery in January.
In 2009, when a prenatal ultrasound showed an abnormal kidney, Luther and Ashley Isler knew that their daughter, Lexi, would be born with a urinary problem. What they did not expect, however, was that Lexi would also be born without a bladder, a condition known as bladder agenesis that has only 20 known survivors worldwide.
Today, 8-year-old Lexi is among those remarkable survivors as she continues an extraordinary medical journey supported by her loving family and an interdisciplinary team of Mount Sinai physicians that has included pediatric urologists and nephrologists, and transplant surgeons, along with nurses and countless other caring staff over the years.
When Lexi was 2 years old, doctors at Mount Sinai Kravis Children’s Hospital confirmed by magnetic resonance imaging that urine had been backing up into her kidney—because she did not have a bladder. While she was in diapers, not having a bladder was not immediately obvious, but as she grew older, Lexi would need a way to both hold and pass urine. Meanwhile, her kidneys—damaged by her urinary outflow anatomy—had been steadily deteriorating and doctors realized that she would eventually need a kidney transplant.
Lexi’s urology and pediatric doctors formed a close team to strategize about her case from the earliest stages. “Long before a transplant, our team first had to think about how to make a bladder and how to get it to work,” says Jeffrey M. Saland, MD, Chief of Pediatric Nephrology and Hypertension at Mount Sinai Kravis Children’s Hospital and Medical Director of the Pediatric Kidney Transplant Service.
When Lexi was 5 years old, Jeffrey A. Stock, MD, Associate Professor of Urology, and Pediatrics, Icahn School of Medicine at Mount Sinai, and Chief of the Division of Pediatric Urology at Mount Sinai Kravis Children’s Hospital, performed the complex, six-hour surgery to create an artificial bladder.
“We removed part of her colon and connected the ends to create a bowel pouch that functioned as a bladder,” says Dr. Stock. “Then, we used her appendix to create a urethra to drain urine from the neobladder.” The neobladder can store, but not empty, urine so Lexi has learned to use a catheter in the bathroom to empty her new bladder. The neobladder procedure is rare. Dr. Stock, who specializes in the surgical reconstruction of genitourinary birth defects, has done only four such procedures in his 25 years of practice.
By January 2018, Lexi’s kidney function was so compromised that at one point, she was unresponsive for days. The situation remained critical all year with very little improvement, and in December 2018, the need for a kidney transplant could not be deferred. Fortunately for Lexi, when she was 3, her father had been identified as a match for her. “It was an easy decision,” says Mr. Isler. “I felt blessed that I was a match.”
Victoria Caselnova, RN, BSN, left, and Stacey Brooks, RN, BSN, with a smiling Lexi Isler on the day she was discharged from the hospital.
In January 2019, as Lexi was about to go into kidney failure, surgeons from the Recanati/Miller Transplantation Institute at Mount Sinai performed the transplant using her dad’s kidney. “We had all of our firepower available,” says Ron Shapiro, MD, Professor of Surgery, and Surgical Director of the Kidney and Pancreas Transplant Program, referring to the expert team of surgeons involved with the transplant and the bladder creation. A smiling Lexi was discharged after spending nine days in the hospital for the transplant surgery.
Still, says Dr. Saland, “This is a work in progress. Lexi remains at higher risk for urinary tract infections and we’ll need to make ongoing adjustments for drainage as well as in her anti-rejection medications. But at Mount Sinai, we believe we have all the expertise and resources we need to treat all aspects of Lexi’s condition.”
“I have the utmost respect for the doctors and nurses at Mount Sinai,” says Ms. Isler. “They all treated Lexi with so much respect and love. The nurses even came to her bedside when their shifts were over to paint with her.”
Despite periodic hospital stays, Lexi is happy and back at school, facing her health challenges with grace and an amazingly positive attitude for a girl her age, says Ms. Isler. Lexi recently found the courage to stand before her classmates and tell them about the condition she was born with and how it was treated. “Her classmates have been very supportive, and they don’t treat her like she’s different,” says Ms. Isler.
At home, the child who had been taking dance classes since the age of 2 has newfound energy and dances around the house with a lack of inhibition, Mr. Isler says. “With a lot of support, our goal is to enable Lexi to live a long, happy, and healthy life,” says Dr. Stock.
To learn more about organ donation, visit www.LiveOnNY.org.