Comprehensive Care for Infants with Severe Chronic Lung Disease

Kailey was born premature and suffered from CLD after birth. She was the first patient evaluated by our NeoCLD Program. Today, she is a healthy, active 6-year-old. The Newborn and Infant Chronic Lung Disease (NeoCLD) Program at Children’s Hospital of Philadelphia was established in 2010 to meet the growing clinical and research needs for infants with severe chronic lung disease. Over the past six years, this program has evolved into a well-established and nationally recognized clinical program. Having cared for about 300 infants with severe CLD in the past few years, this program has become one of the largest specialty care programs in the world focusing on infants with severe CLD.

This program’s emphasis is on providing comprehensive yet individualized care based on the pathophysiology of each patient, enhancing family well-being, and maximizing infant developmental potential. Our multidisciplinary team includes experienced complex care physicians from not only Neonatology, but also Pulmonary Medicine, Cardiology, ENT, and General Surgery. Crucially, this team also includes a broader set of healthcare workers specializing in the care of CLD babies including nurses, frontline clinicians, dietitians, respiratory therapists, pharmacists, physical/occupational therapists, speech-language pathologists, psychologists, and social workers.

Close collaboration with specialists throughout the Hospital — including other specialty teams like the Pulmonary Hypertension Program, Neonatal Airway Program, Bone Health Team, Technology Dependence Center, Jill and Mark Fishman Center for Lymphatic Disorders, psychosocial team, and developmental team — means that patients in the NeoCLD Program receive the most comprehensive care, all in one place.

“Direct communication between multiple specialists across different disciplines, as well as regular group discussions, really helps us understand the baby better as a whole person and promotes joint decision making based on the needs of each individual patient,” says Huayan Zhang, MD, neonatologist and medical director of the NeoCLD Program.

Individualized care for infants with severe CLD

The program uses a systematic approach toward the management of infants with severe CLD. Upon admission to the program, each infant receives an initial evaluation including careful assessment of cardiopulmonary status, pulmonary mechanics and pulmonary function, growth and nutritional status, and presence of metabolic bone disease, as well as a developmental assessment. Treatment plans and further testing are based on this initial evaluation and ongoing close observations from our multidisciplinary team.

“Although all of these infants are diagnosed with severe CLD, there are often very different reasons causing the persistent respiratory insufficiency. Finding the underlying pathophysiology in order to tailor the treatment to target the fundamental problem(s) is therefore extremely important,” says Zhang.

The program utilizes the advanced diagnostic and treatment modalities and expertise of Children’s Hospital’s extremely experienced specialists. If needed, additional testing such as ultra-low dose CT angiograms with controlled ventilation protocol, bedside or operating room bronchoscopy, echocardiograms and cardiac catheterizations, lung biopsies, dynamic contrast MR lymphangiographies, and genetic testing will be carried out in a stepwise and highly coordinated manner.

Individualized treatments will be based on patient needs. These include pulmonary vasodilators and balloon occlusions of hyperinflated lung fields followed by lobectomies, bronchoalveolar lavages, catherization closures of patent ductus arteriosus, lymphatic embolizations, anti-GE reflux surgeries, and tracheostomies. In some patients, the specific treatment may just be time. In those cases, treatment will be geared toward supporting growth and development with the slow weaning of respiratory support.

Long-term care is coordinated through our specialty clinics, Progressive Care Unit, Integrated Care Service, and primary care pediatricians to ensure these infants continue to thrive after being discharged. A specific CLD clinic that combines pulmonary follow-up and neurodevelopmental testing is currently being planned and is expected to begin seeing patients early next year.

Seeking evidence-based treatments

Besides extraordinary clinical care, the other focus of the NeoCLD Program has been to establish translational research to advance management of CLD and promote evidence-based treatments. Starting from retrospective clinical studies, the team has had 15 abstracts presented at national/regional scientific meetings, 11 manuscripts published, and six other manuscripts submitted or under revision over the past few years. Now the team has moved into prospective studies with the following three current research protocols actively recruiting patients:

• Intrinsic positive end-expiratory pressure measurement in infants ventilated for severe CLD
• N-of-1 trial comparing hypoxemia events with gastric vs. post-pyloric feeding
• Nitric oxide responsivity in infants with severe CLD

In addition, a clinical trial led by William Fox, MD, evaluating the safety and effectiveness of liquid ventilation using perflubron in infants with severe CLD is set to start enrollment later this year. The program is also a founding and active member of the National BPD Collaborative.

These research activities provide important data that help guide the immediate treatment of some patients. More importantly, data obtained from these studies greatly enriches the currently lacking scientific evidence regarding this condition and will aid the development of management guidelines specific for infants with severe CLD.

Members of our NeoCLD Program are dedicated to providing the best, most comprehensive care. We thrive in being at the forefront of pediatric medical care and leading the advancement of clinical care and medical knowledge for this unique patient population.