Zaylee's Story

Case Study: VACTERL Association

The Brendan Tripp Elam Transplant Center at Children’s Mercy Kansas City specializes in pediatric heart, liver and kidney transplants as well as multi-organ transplants like heart-liver, heart-kidney and liver-kidney.

The Transplant Center is comprised of specialists built from three nationally recognized departments by U.S. News & World Report within Children’s Mercy. Our purpose is to be the destination for holistic, innovative, and unparalleled transplant care for children.

Built on collaboration, our transplant team works hand in hand with you to deliver thorough care from pre-transplant evaluation to post-transplant recovery.

Read how our transplant team at Children’s Mercy came together to help Zaylee:

Patient Background: Zaylee, a 4-year-old female, experienced her first ocean visit this spring, demonstrating a strong affinity for water. Despite her current vibrant health and active lifestyle, Zaylee’s early prognosis was dire.

Prenatal Concerns: At 20 weeks gestation, maternal ultrasound revealed oligohydramnios and an absence of visible renal structures. Subsequent referral to a specialist in Wichita identified additional anomalies, including cardiac, limb, and hand malformations. Continuous monitoring was conducted at the Elizabeth J. Ferrell Fetal Health Center, Children’s Mercy Kansas City.

Diagnosis: Zaylee was diagnosed with VACTERL association, presenting with a solitary dysplastic kidney. VACTERL encompasses vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities. The etiology remains idiopathic, with an incidence of 1 in 10,000 to 40,000 live births.

Clinical Management: Dr. Judith Sebestyen VanSickle, MD, MHPE, FAAP, FASN, Zaylee’s nephrologist, emphasized the importance of multidisciplinary collaboration in managing VACTERL. Initial renal function was preserved, allowing for necessary cardiac surgeries without dialysis. However, by March 2022, discussions regarding end-stage renal care commenced.

Acute Complications: Post-colostomy reversal, Zaylee developed severe constipation leading to sepsis and subsequent renal failure, necessitating hemodialysis. Following stabilization, she was listed for pediatric kidney transplantation.

Transplantation: On May 2, 2023, Zaylee underwent successful kidney transplantation performed by Dr. Bhargava Mullapudi and Dr. Richard Hendrickson. Postoperative recovery was rapid, with significant improvements in her overall health and renal function.

Ongoing Care: Zaylee’s post-transplant care involves strict adherence to immunosuppressive therapy and regular monitoring for potential rejection and infections. Her multidisciplinary team includes nephrology, colorectal, cardiology, orthopedics, developmental and behavioral health specialists, supported by social workers, pharmacists, and nutritionists.

Conclusion: Zaylee’s case underscores the critical role of comprehensive, multidisciplinary care in managing complex congenital conditions. Her progress highlights the importance of early intervention, continuous monitoring, and family involvement in achieving favorable outcomes.

Get to know the Brendan Tripp Elam Transplant Center at Children’s Mercy and how we can partner.