Pulmonary Programs Update

The Center for Thoracic Insufficiency Syndrome is a multidisciplinary (Orthopedics, Radiology, and Pulmonology) center that, in addition to its clinical mission, performs research to describe the change in thoracospinal volume and motion longitudinally in healthy children. This knowledge will then be applied to children with thoracospinal disorders such as congenital scoliosis, Jarcho-Levin syndrome, and Jeune’s thoracic dystrophy. The goal is to show how such structural and functional imaging can better discern which children will benefit from VEPTR (vertical expandable prosthetic titanium rib) surgery, and assist surgeons in deciding the best surgical approach for these complex patients.

The Pulmonary Function Laboratory has opened in Voorhees to serve patients and families in Southern New Jersey. It’s the only pediatric PFT lab in that region and is staffed by clinicians experienced in testing children and adolescents. The PFT is located in the CHOP at Virtua Specialty Care Center adjacent to Virtua Voorhees Hospital.

The Cystic Fibrosis (CF) Center at CHOP cares for more than 350 children with CF and continues to maintain extremely high quality indicators when compared to other CF centers. CHOP ranks among the top centers nationally for FEV1 in children between the ages of 6 and 17 in the most recent CF Foundation Data Registry.

CHOP participates in multicenter clinical trials studying investigational inhaled and oral medications for cystic fibrosis. In addition, research has elucidated basic mechanisms of the molecular problems that lead to cystic fibrosis, as well as potential therapeutic agents that correct these problems.

CHOP’s Lung Transplantation Program was officially activated by the United Network for Organ Sharing (UNOS) in May 2006. Patients have undergone lung transplantation for such disorders as cystic fibrosis, pulmonary hypertension, and surfactant protein deficiency. The Lung Transplantation Program is a contributor to the Pediatric Lung Transplantation National Data Registry, and clinical multicenter research is under way, including studies of the late pulmonary effects of childhood cancer. The program is also joining a multicenter national network investigating interstitial lung disease in children.

The Technology Dependence Center follows more than 250 ventilator-assisted children with chronic respiratory failure due to premature birth, neuromuscular disease, and heart disease in their homes. Investigations are under way studying interventions to increase airway flow and cough effectiveness in infants and children with neuromuscular disease, and long-term cardiopulmonary consequences of congenital diaphragmatic hernia and gastroschisis.

The Annual Polgar Lectureship, sponsored by the Division of Pulmonology, invites a world-renowned expert in pediatric pulmonology to present at CHOP Grand Rounds, attend inpatient rounds on the Pulmonary Unit, critique research presentations from division members and fellows, give a noon conference for residents, and give a research talk to the division.

The 2012 visiting professor was Carolyn Kercsmar, MD, co-director of Pulmonary Medicine at Cincinnati Children’s Hospital, who spoke on asthma quality improvement initiatives, asthma in inner-city children, and diagnosis and management of the wheezing infant and toddler. The 2013 visiting professor will be Margaret Leigh, MD, chief of the Pediatric Pulmonary Division at the University of North Carolina Medical Center in Chapel Hill. She will be discussing primary ciliary dyskinesia (immotile cilia syndrome) at CHOP Grand Rounds on April 24.

About the Division of Pulmonary Medicine at CHOP

CHOP’s Division of Pulmonary Medicine provides multidisciplinary inpatient and outpatient services for infants and children with acute and chronic respiratory problems, including cystic fibrosis, asthma, bronchopulmonary dysplasia (BPD), congenital lung abnormalities, neuromuscular respiratory diseases, chronic respiratory failure/ insufficiency, chest wall disorders, pulmonary sleep disorders, and pediatric lung transplantation. Each year, the division sees more than 7,800 outpatients at The Children’s Hospital of Philadelphia’s Main Campus and CHOP Care Network locations, and treats 7,600 inpatients at the Hospital.

The division’s interdisciplinary model combines the expertise of physicians, nurses, nutritionists, respiratory and physical therapists, psychologists, and social workers — all working together to improve the health and well-being of our patients. CHOP Pulmonary Medicine was ranked No. 1 by U.S.News & World Report in 2008, 2009, 2010, 2011, and 2012 and was ranked No. 3 by Parents magazine in 2013.

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