Beyond Therapy: Long-term Follow-up Facilitates Early Recognition and Support for CDH Complications

Published on in In Utero Insights

Structured neurodevelopmental surveillance facilitates early recognition and treatment of complications, providing parents anticipatory guidance to assist them in mitigating the impact of these impairments on their child’s quality of life.

Neonates with congenital diaphragmatic hernia (CDH) often require intensive treatment after birth and have prolonged hospitalizations. After discharge from the hospital, infants may suffer respiratory insufficiency, gastroesophageal reflux, poor growth, neurodevelopmental delay, behavior problems, hearing loss, hernia recurrence and orthopedic deformities.

At CHOP, a highly specialized team of maternal-fetal medicine doctors, pediatric surgeons, neonatologists and advanced practice nurses provides comprehensive care for babies with CDH starting from prenatal diagnosis. Babies with CDH are born in the Garbose Family Special Delivery Unit and transferred to our Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU), which is top-ranked in the nation for neonatal care.

For infants with severe respiratory failure and pulmonary hypertension, our Extracorporeal Membrane Oxygenation (ECMO) Program — which was founded in 1990 and has done more than 1,169 runs on 1,098 patients — is one of the most experienced programs of its kind in the country. It has been a designated Center of Excellence by the Extracorporeal Life Support Organization since 2008.

Comprehensive interdisciplinary follow-up care is provided through our Pulmonary Hypoplasia Program (PHP), which was founded in 2004 and cares for more than 500 children from infancy well into adolescence. Structured neurodevelopmental surveillance facilitates early recognition and treatment of complications, providing parents anticipatory guidance to assist them in mitigating the impact of these impairments on their child’s quality of life.

Children enrolled in the PHP receive coordinated care from a multidisciplinary team of pediatric surgeons, pulmonologists, cardiologists, advanced practice nurses, developmental pediatricians, child psychologists, dieticians, audiologists and social workers. Assessments are focused on improving cardiopulmonary health, evaluating neurodevelopmental growth, monitoring and treating any surgical issues that arise as children grow, meeting nutritional needs, monitoring for hearing loss, and connecting families to resources close to home.

“The uniqueness of this program is its multidisciplinary nature and the continuity of care,” says Lisa Herkert, MSN, CRNP, coordinator of the PHP. “Patients see providers who are specialized in taking care of children with CDH and see the same providers for all of their appointments.”

PHP team members first meet families while they are in the N/IICU to discuss the plan for follow-up. Appointments are tailored to each child’s needs, but typically occur at 6 months, 12 months, 2 years, 4.5 years and 6 years, and then every two years thereafter, as needed, and are designed for visits to multiple specialists in one day. This schedule and approach to care was created based on the American Academy of Pediatrics recommendations for post-discharge follow-up of infants with CDH, published in 2008. After each day of assessments, team members regroup to develop the most comprehensive care plan for each child.

“Everyone has their specific areas they are focused on,” says Herkert. “At the end of the day, we put our heads together and make recommendations for how families should proceed.”

Pulmonary Hypoplasia Program Enrollment

Total patients enrolled in PHP Research Study as of May 21, 2018: 740

 
Patients

 

 
Patients