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

Neonates with congenital diaphragmatic hernia (CDH) often require intensive treatment after birth and have prolonged hospitalizations. After discharge from the hospital, infants may suffer pulmonary hypoplasia, respiratory insufficiency, gastroesophageal reflux, poor growth, neurodevelopmental delay, behavior problems, hearing loss, hernia recurrence and orthopaedic deformities. Many patients need supplemental tube feedings and other therapies and services that require coordination to optimize outcomes.

At CHOP, our specialized Pulmonary Hypoplasia Program (PHP) provides ongoing developmental and medical assessments for more than 650 children from infancy through adolescence. The purpose of the PHP is to look beyond short-term surgical survival and provide long-term clinical care to children who have complex medical needs that affect quality of life.

“You won’t know that there are problems unless you look for them,” says Holly L. Hedrick, MD, Attending Pediatric and Fetal Surgeon and Director of the PHP. “Just getting out of the hospital isn’t the end of the story. It’s our responsibility to get everybody across the finish line.”

Establishing continuity of care from the start

After babies with CDH are born in our Garbose Family Special Delivery Unit they are transferred to our Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU). There, the PHP’s core team of pediatric surgeons, neonatologists and advanced practice nurses meet with the family to discuss the plan for follow-up.

Appointments in the clinic 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, with cohesive plans and recommendations coming from the team as a whole to avoid fragmented care. Children receive coordinated care from pediatric surgeons, pulmonologists, cardiologists, advanced practice nurses, developmental pediatricians, child psychologists, dietitians, audiologists and social workers. These specialists work together and also partner with primary care physicians to provide the most comprehensive care for patients.

Early recognition and treatment of complications

Assessments are focused on early recognition and treatment of complications. The team monitors cardiopulmonary health, neurodevelopmental progress, surgical issues that may arise as children grow, nutritional needs and psychosocial stability. The structured, collaborative nature of the PHP optimizes every dimension of care for patients and their families and helps provide parents anticipatory guidance to assist them in mitigating the impact of these impairments on their child’s quality of life.

“The PHP team’s multidisciplinary approach breeds both collaboration and coordination of care between providers and caregivers,” says Social Worker, Annie Markovits, MSS, LSW. “It allows me to gain a global understanding of the patient and family’s strengths and needs as well as afford the opportunity to assess and support families wherever and whenever needed, oftentimes at home and in the community in addition to the clinic setting.”

For infants with severe respiratory failure and pulmonary hypertension, our Extracorporeal Membrane Oxygenation (ECMO) Program — which has done more than 1,220 runs on 1,218 patients — is one of the most experienced programs of its kind in the country. It has been designated a Center of Excellence by the Extracorporeal Life Support Organization since 2008, and recently was named a Platinum Level Center of Excellence.

The uniqueness of this program is its multidisciplinary nature and the continuity of care. Patients see providers who are specialized in taking care of children with CDH and see the same providers for all of their appointments throughout the journey — prenatal, neonatal, infancy, childhood and adolescence.

Bridging the gap with telehealth

When the COVID-19 pandemic hit and put a pause on all nonessential hospital visits, the PHP team took immediate action, transitioning the multidisciplinary program to virtual visits in order to provide continual care to these fragile patients.

While stay-at-home orders helped to protect many children and their families from contracting or spreading COVID-19, the reality is that PHP patients have complex medical needs that require close attention. Telehealth allowed us to bridge this gap. While CHOP has since reintroduced in-person outpatient visits, the PHP team plans to continue using telemedicine as a component of future patient care.

“We improved accessibility, especially for those patients living at a distance,” adds Howard Panitch, MD, Attending Pulmonologist with the PHP and Director of CHOP’s Technology Dependence Center. “In addition, for some children, the ability to see them in their home environments was very helpful in understanding their abilities and limitations.”

For more information about this unique program, visit chop.edu/php.

By Lisa Herkert, MSN, CRNP, Coordinator, Pulmonary Hypoplasia Program