The Role of the Fetal Spina Bifida APN Program Coordinator

Expectant mothers and partners who are given the diagnosis of myelomeningocele or myeloschisis — the most severe forms of spina bifida — meet with many care providers at the Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment (CFDT). During the initial visit at our Center, mothers will undergo a detailed, high-resolution fetal ultrasound as well as an ultrafast fetal MRI. Imaging is performed and if the diagnosis is confirmed, a consultation with the maternal-fetal medicine specialist and neurosurgeon will follow. The diagnosis is explained at length, as are options for pregnancy management. If the mother and fetus meet eligibility criteria and the parents are interested in fetal surgery for the closure of the spinal defect, parents meet with a fetal surgeon the following day to discuss the details of fetal surgery. Other pregnancy options include planned traditional closure of the spina bifida after birth.

The Fetal Spina Bifida APN Program Coordinator participates in these consultations, as it is important to know the family’s level of understanding when receiving the diagnosis and what parents hear and subsequently take away from these discussions. The prospective parents need to discuss and decide management options presented for their pregnancy and start to make choices for their baby.

Beginning in 2019, as a result of a generous gift by the Garbose Family Endowed Chair in Fetal Family Care, the role of supporting families from prenatal diagnosis through their stay in our Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU) and discharge was created. As the inaugural chair holder, the CFDT’s Executive Director, Lori Howell, DNP, MS, RN, established the role recognizing the amazing support and coordination of care these families need.

Parents who choose to have fetal surgery will have met with the entire fetal surgery team to ensure maternal safety the day prior. During this meeting, the procedure is again explained at length and consent is obtained. The family’s current state and their level of understanding are gauged. At this time, we also review the family’s arrangement to relocate closer to our Center to follow the postoperative recommendations. Knowing the families and their circumstances prenatally allows us to personally and efficiently address any needs that may arise at the time of delivery or while the infant is in the N/IICU.

Delivering at CHOP

CHOP’s Special Delivery Unit is the world’s first birth facility in a pediatric hospital specifically designed for healthy mothers carrying babies with known birth defects.

Following fetal surgery, mothers have weekly prenatal appointments at our Center. Mothers carrying a fetus with spina bifida who will have closure after birth are usually followed monthly until later into gestation, when frequency may increase. During one of these appointments, the parents meet with the Fetal Spina Bifida APN Program Coordinator to review what to expect at the time of delivery, what will happen in the N/IICU, discharge planning and follow-up. This provides anticipatory guidance to these parents so they can better prepare for what lies ahead.

Topics covered before delivery include:

• The physical set up of the resuscitation room and the common interventions needed in the delivery room for infants with spina bifida who have undergone fetal surgery as well as those with planned postnatal closure of spina bifida.
• The routine consults for infants with spina bifida, including Neurosurgery, Urology, Orthopaedics, Physical Therapy, and the Spina Bifida Clinic.
• The various testing and imaging that is done — such as ultrasounds and MRIs — during the infant’s hospital stay.
• The extensive evaluations by Urology and Neurosurgery, including monitoring for the need for a shunt.
• Milestones the infant must meet prior to discharge from the hospital such as stable vital signs, tolerating feedings and maintaining body temperature.
• The expected timeline of follow-up visits with the spina bifida specialists who will follow the child at CHOP. If the family lives geographically distant from our hospital, we discuss local follow-up options and occasional visits to CHOP if the family is able.
• Selecting a pediatrician who will provide routine pediatric care before discharge and reaching out to their local spina bifida clinic to get connected with care providers in their area.

By doing this prior to delivery, parents are better prepared and have plans in place before they inevitably experience the whirlwind after the birth of their beautiful baby. Any questions parents may have about their infant after birth are always addressed. While the infant is in the N/IICU, continuity of care is still in place, helping facilitate consultations with all services, ensuring the required testing is done, and continuing extensive discharge planning.

Follow-up at CHOP is critical for continued care and evaluation for those who have had fetal surgery as well as those with postnatal spina bifida closure. This follow-up includes visits within our multidisciplinary spina bifida clinic as well as visits with the surgical subspecialties of Neurosurgery, Urology and Orthopaedics. Neurodevelopmental evaluations are also performed by our neuropsychologist at specific time frames. All of these appointments are on a standardized schedule to ensure comprehensive follow-up and monitoring. As discharge approaches, all follow-up appointments are made. If the infant is following up at an outside spina bifida clinic, we communicate all clinical details with the spina bifida clinic to ensure a smooth transition of care.

Parent education regarding the infant and the diagnosis of spina bifida continues into the N/IICU. Our Neonatal Myelomeningocele/Myeloschisis Clinical Pathway contains educational materials that are given to all parents in one comprehensive folder prior to discharge. Some of this material pertains to their current circumstances, while other documents may be needed in the future should issues develop. This extensive education allows the parents to be aware of potential complications after discharge — most importantly signs of increased intracranial pressure — that can develop in infants with spina bifida. Contact information for all the specialists is given to the parents so they are able to reach us 24 hours a day, seven days a week should any issues arise.

This extensive relationship enables us to discharge families from our care knowing they understand their child’s condition, are confident to care for their child, and know when to call one of their care providers should a concern arise.

Contributed by: Katie M. Schmidt, MSN, CRNP