As a pediatric pulmonologist, I care for patients with a wide range of lung and airway disorders. I have a particular interest in using flexible bronchoscopy as a tool in the care of patients with cystic fibrosis (CF), congenital airway malformations and technology dependence.
My interest in medicine was sparked by working with children with chronic medical conditions. This population continues to be my primary focus. During my training as a pediatrician, I gravitated towards children with respiratory diseases, specifically cystic fibrosis and chronic respiratory failure, because of the complexity of these disorders and the long-term impact they have on these children.
Though I was interested in CF, I did not initially intend to pursue a research career. During my fellowship, the first cystic fibrosis transmembrane conductance regulator (CFTR) modulator, ivacaftor, was released but was only effective in less than 5 percent of the CF population. Having such an effective treatment limited to a small subset of my patients was frustrating. This frustration led me to the lab of J.P. Clancy, a CF expert at Cincinnati Children's. Through working with Dr. Clancy, I began my current research path with efforts to expand and optimize these therapies.
Mutations in the CFTR gene cause CF, and there are more than 2,000 such mutations described to date. Half of these are estimated to occur in five people in the world or less. Each mutation causes slightly different changes in the CFTR protein, which means various tools are needed to fix the protein cause of the disease. Some of these tools, called CFTR modulators, are available for people with certain CFTR gene mutations. My work seeks to identify people with rare CFTR gene mutations that would benefit from these therapies but otherwise do not have access due to their genotypes' rarity. We are also working to identify patient- and therapy-specific factors that may reduce the benefits these treatments offer and develop tools to optimize these lifelong medications.
My research goal is to provide personalized, precise care to patients with rare CF genetic variants. I specifically work with patient-derived models of respiratory epithelia to model different functions of the CF airway. Through this work, we seek to maximize the potential benefits of CFTR modulator drugs by expanding access and aiding in clinical therapeutic selection. We also seek to identify patient-specific factors that influence the severity of CF and the likelihood of a therapeutic response.
During residency, I received the Samuel Dalinsky Memorial Award (2012), a peer-selected award for a resident dedicated to academic excellence and the care of others. As a fellow and young faculty member, I received a Harry Shwachman clinical investigator award (2016-2019), a competitive grant from the CF Foundation aimed at developing young, promising researchers in the CF field.
In addition to my clinical and research efforts, I enjoy teaching learners across all aspects of medical education. I've been lucky to have many opportunities to teach, including work with our medical students and residents. I am also the coordinator of the international pediatric flexible bronchoscopy course held each year here in Cincinnati.
MD: Wake Forest University, Winston-Salem, NC, 2009.
Residency: Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 2012.
Chief Residency: Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2013.
Fellowship: Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2016.
Certification: Pediatrics, 2012; Pediatric Pulmonary Medicine, 2016.
Pulmonary medicine; bronchology; cystic fibrosis; congenital airway malformations
Pulmonary Medicine, Bronchoscopy, Rare Lung Diseases, Cystic Fibrosis Center
Cystic fibrosis; CFTR modulators; patient-derived model systems
Gradual increase in sweat chloride concentration is associated with a higher risk of CRMS/CFSPID to CF reclassification. Pediatric Pulmonology. 2023; 58:1074-1084.
Alpha-1 antitrypsin limits neutrophil extracellular trap disruption of airway epithelial barrier function. Frontiers in Immunology. 2023; 13:1023553.
Phenotypic Alteration of an Established Human Airway Cell Line by Media Selection. International Journal of Molecular Sciences. 2023; 24:1246.
Novel Applications of Biomarkers and Personalized Medicine in Cystic Fibrosis. Clinics in Chest Medicine. 2022; 43:617-630.
606 Focused clinical trials of modulator response for rare cystic fibrosis genotypes. Journal of Cystic Fibrosis. 2022; 21:s336.
380 Neutrophil extracellular traps disrupt bronchial epithelial barrier function: Alpha-1 antitrypsin to the rescue. Journal of Cystic Fibrosis. 2022; 21:s223.
683 Characteristics of cystic fibrosis transmembrane conductance regulator modulator response in nasal cell models derived from patients with rare cystic fibrosis transmembrane conductance regulator variants. Journal of Cystic Fibrosis. 2022; 21:s373.
CRISPRi links COVID-19 GWAS loci to LZTFL1 and RAVER1. EBioMedicine. 2022; 75:103806.
When CFSPID becomes CF. Journal of Cystic Fibrosis. 2022; 21:e23-e27.
Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation. Journal of Cystic Fibrosis. 2021; 20:e69-e71.