The Newborn and Infant Chronic Lung Disease Program at The Children’s Hospital of Philadelphia (CHOP) is one of the largest programs of its kind in the world. Since its inception 3 years ago, the program has followed 165 patients with severe chronic lung disease (CLD), with a steady increase in referrals year over year.

newborn infant sleepingThis large patient volume has driven the team to develop a comprehensive and individualized approach to these fragile infants. For example, while the common practice in neonatology is to try to wean ventilation and extubate as soon as possible, experience has taught the team that for many older patients with established lung disease, especially those with evolving pulmonary hypertension, that approach may not be appropriate and sometimes can even be detrimental.

“Infants with established chronic lung disease are very different from fresh premature newborns with respiratory distress syndrome,” says Medical Director Huayan Zhang, MD. “Simply weaning their respiratory support may not be the right treatment for these patients. This is a chronic and complicated disease that needs an individualized approach and comprehensive long-term management.”

Patients are evaluated to determine the extent of CLD, the presence and degree of pulmonary hypertension, and the degree to which other factors, such as airway malacia or reflux and aspiration, contribute to the lung disease. Evaluation may include ultra-low dose CT angiogram interpreted by a team of expert radiologists, pulmonary function testing, echocardiogram, developmental assessment, nutritional/ bone health testing, speech/physical/ occupational therapy tests, bronchoscopy and airway assessment, testing for gastroesophogeal reflux (GER) and aspiration, ventilation/perfusion scan, and cardiac catheterization. For babies who are atypical, a lung biopsy is performed to determine the severity of CLD or an alternative disease process.

As a multisystem disease, CLD requires a multidisciplinary approach to care. The NeoCLD Program’s team consists of 7 attending neonatologists— including Zhang and Program Director Haresh Kirpalani, MD — specialized nursing teams, respiratory therapists, neonatal clinical pharmacists, neonatal nutritionists, physical, occupational, and speech therapists, case managers, and a psychosocial support team made up of social workers, psychologists, and child life specialists. This core team collaborates with specialties throughout CHOP — including Cardiology, Otolaryngology, Endocrinology, the Neonatal Airway Program, and the Center for Bone Health.

A CLD nursing committee developed frequent unit-wide educational activities to train nurses in providing appropriate care to patients with the condition. These highly trained nurses provide important clinical observations that aid in management and deliver developmentally appropriate care to patients.

After thorough evaluation, each team member contributes their expertise and input to create an individualized treatment plan. For many patients, an important component of therapy is time. Providing adequate yet gentle ventilatory support, controlling pulmonary hypertension, preventing the deleterious effects of gastroesophageal reflux and aspiration on the developing lungs and airways, and providing optimum nutrition allows for growth and healing of the immature and damaged lungs.

Weekly walk rounds and monthly discussion meetings allow the team to review progress, discuss medical, social, nutritional, and developmental issues, identify problems, and make plans for further management. These plans are then communicated directly to the bedside nurse and entire medical team.

Each patient has a primary CLD attending, who follows the child closely and communicates with the parents frequently throughout the hospitalization. Currently the team is developing a visual decision aid that will serve as a helpful, multidimensional tool in counseling parents so they can make the most informed decisions about their child’s care.

Long-term care is coordinated with CHOP’s Neonatal Follow-up Program, Division of Pulmonary Medicine, Pulmonary Hypertension Program, and, in the case of infants with tracheostomies, the Technology Dependence Center to monitor disease progression, growth, and development throughout childhood. And when treatment in the intensive care setting is no longer needed, the team facilitates seamless transition to home, the referring hospital, or a chronic care facility.

The NeoCLD Program is at the forefront of evidence-based and collaborative research. The program is 1 of the founders of a new national collaborative group consisting of 8 specialized CLD units throughout the country. The group’s first work meeting was hosted at CHOP in May 2013. The NeoCLD Program team has been instrumental in setting up a unified REDCap database and participated in the collaborative group’s first “snapshot” survey to quantify the disease burden and management needs of babies in the 8 units.