Summer Medical Student Respiratory Research Fellowship

Raouf Amin, MD

Dr. Amin is a pediatric pulmonologist whose research program focuses on three areas of investigations in pediatric sleep including upper airway function in children with sleep apnea, mechanisms of resolution of sleep apnea after surgical interventions such as adenotonsillectomy and bariatric surgeries, and the cardiovascular and neurocognitive outcomes of sleep apnea in children. Medical students will receive specific training in the methods of hypothesis-driven clinical research. Students will also be exposed to other sleep research projects and receive mentorship on abstract preparation and will be invited to collaborate on manuscripts, as appropriate.

Katherine Auger, MD, MSc

Dr. Auger is a pediatric hospitalist and physician scientist who works to support families with both the stress and strain of hospitalization as well as the challenges taking a sick child home after hospitalization. In particular, we focus on improving the care of provided to children hospitalized with common conditions including bronchiolitis, asthma and pneumonia. Additionally, some of our recent work has focused on the effect of the COVID-19 pandemic on the health and well-being of children.

Alister Bates, PhD

Dr. Bates research focuses on human airways and how they change with various disease conditions. He is specifically interested in airway behavior in children with obstructive sleep apnea (OSA) and premature babies born with tracheomalacia (TM) and congenital abnormalities. Research goals are to identify how airway problems affect patients’ symptoms, inform and evaluate surgical and therapeutic interventions, and differentiate the effects of airway abnormalities versus lung disease.

Dean Beebe, PhD

Dr. Beebe’s lab focuses on the influence of sleep disorders and inadequate sleep on adolescents’ health and health-related behaviors. During the summer we will be gathering data for two NIH-funded projects. The first uses a driving simulator and neuropsychological measures to assess driving-related risk associated with obstructive sleep apnea in licensed adolescent drivers. The second uses an experimental paradigm to assess the impact of circadian-aligned versus circadian-misaligned sleep on adolescents’ physical activity, dietary intake, and mood and cognition.

Tom Boat, MD

Dr. Boat is a pediatric pulmonologist and former chair of pediatrics at Cincinnati Children’s and dean of the University of Cincinnati College of Medicine. Dr. Boat oversees the CF WELL, which is a program that is focused to fostering family wellness and enhancing health outcomes for both patients with disabling or life-threatening chronic diseases and their families. Program components have targeted improved sleep, more family focused exercise, better nutrition, a higher level of school success for chronic disorder-disadvantaged children, and ability of the family to better manage stress that is a part of living with chronic disease. Research projects span a spectrum from assessing needs of families in their quest to improve wellness, a better quality of life, and meet the care needs of their child with extraordinary health care needs; to designing and implementing interventions that can be tested using an array of clinical research designs, and if beneficial inserted into the chronic care model. Extension of learnings with families of children with CF in Cincinnati will be shared with other CF Centers and extended to other chronic disease care programs, starting with those at Cincinnati Children's. Research projects in progress or planning that offer SMURFF awardees opportunities to engage a research project include:

1. Ascertaining the extent and nature of sleep insufficiency in families of children with CF, led by Dr. Barbara Chini, and piloting an intervention designed to improve sleep by sleep psychologist, Dr. Kelly Byars.
2. Working with Dr. Stephanie Filigno and Ms. Sarah Strong on the assessment of challenges presented by school for CF children and families, and identifying their risk factors for academic success.
3. Ascertaining the relationships among nutrition, exercise, weight gain, body composition, muscle strength and pulmonary function for patients with CF, working with CF team experts in exercise physiology and nutrition.
4. Working with Dr. Chris Siracusa and the pulmonary function technicians to assess the impact of several potential mind-body interventions on stress reduction interventions at the time of pulmonary function testing, a situation that is identified by one third of CF patients as a source of anxiety and considerable stress.
5. Other research opportunities will undoubtedly emerge as the CF WELL program advances its initiatives.

Pat Brady, MD

Dr. Brady is a hospital-based physician-scientist with training in epidemiology, biostatistics, and advanced improvement science. His primary area of research focuses on improving patient safety specifically situation awareness, a high reliability strategy, to improve the prediction, recognition, and treatment of deteriorating hospitalized patients, the vast majority of which have respiratory disease such as pneumonia or bronchiolitis. Summer students have helped our research team in both prospective and retrospective studies of acute pediatric respiratory disease and its complications. His lab aims to ensure these projects are right-sized for the 8-10 week stretch and provide a rich and mentored experience in clinical research.

John Brewington, MD

Dr. Brewington is a pediatric pulmonologist who focuses primarily on epithelial biology in cystic fibrosis (CF). His research aims to maximize the individual benefit from “modulator” drugs, a relatively new group of treatments that directly improve the dysfunctional protein that causes CF. These drugs are only available for certain subgroups of patients, based on their specific genotype, and the largest group receives the smallest benefits. To improve this benefit, there is ongoing work to understand the negative interactions between these modulators and classical CF therapies (such as inhaled beta-agonists). Additionally, the lab is working on generation of patient-derived, individualized model systems to predict who will benefit from these new drugs, and to potentially expand them to new genotypes. A variety of techniques are used in these studies, with a focus on cell-culture models and assays (both traditional and novel) of ion/fluid transport.

Cole Brokamp, PhD

Dr. Brokamp studies causal inference for machine learning, environmental and population health, fairness in precision medicine and geoinformatics. My research studies the impacts of environmental exposures and community characteristics on child and adolescent health.

Zack Cleveland, PhD

Dr. Cleveland’s research interest is centered on developing magnetic resonance-based imaging techniques to visualize lung structure and function in humans and in small animal models of disease and injury. A particular focus of his research has been on visualizing ventilation and diffusive gas uptake using hyperpolarized (HP) 129Xe MRI. His focus will be to develop novel magnetic resonance imaging methods to assess regional lung function in preclinical animal models, as well as adult and pediatric populations. His emphasis will be directed toward developing techniques to quantitatively image pathophysiology in poorly understood disorders such as acute lung injury (ALI) and idiopathic pulmonary fibrosis.

Hitesh Deshmukh, MD 

Dr. Deshmukh is an attending neonatologist, who studies how current clinical practices, such as increased antibiotic use and increasing rate of cesarean deliveries extract a ‘significant penalty’ in the form of altered microbiota and increased morbidity to respiratory pathogens. Straddling the disciplines of pulmonary epithelial biology and developmental immunity, his laboratory investigates how events in critical developmental window of early life, patterns lifelong pulmonary resistance.

Thomas Dye, MD

Dr. Dye’s research focuses on understanding the causes of sleep-related movement disorders in children, such as RLS, and identifying the best way to treat them. He studies the role certain therapies for epilepsy may play in sleep. In addition, his research effort is also working to bridge the divide between circadian research and the clinical treatment of circadian sleep disorders.

Theresa Guilbert, MD

Dr. Guilbert is a pediatric pulmonologist performing clinical research focusing on identifying the roles of early life risk factors, exposures, and environment interactions that lead to early childhood asthma and recurrent wheezing. Much of Dr. Guilbert’s research experience has been with multicenter trials and networks. Another area of research interest is using clinical electronic medical data and subsequent linking of this data to public and databases on environmental, socioeconomic, and demographic profiles in order to identify asthma phenotypes. Specific interventions will be targeted to these phenotype populations and clinical outcomes will then be followed in the electronic medical record. Dr. Guilbert utilizes these research interests to provide research training opportunities for future physician-scientists.

William Hardie, MD

Dr. Hardie is a pediatric pulmonologist with both clinical and laboratory projects available for students. Clinical projects include a study in patients with pectus excavatum where students will examine the relationship of patient symptoms of chest pain and dyspnea with exercise testing outcomes, body composition analysis, cardiac MRI and EKG findings while collaborating with physicians in pediatric surgery and cardiology. Laboratory-based projects are focused on examining blood biomarkers associated with the progression of pulmonary fibrosis in idiopathic pulmonary fibrosis, and mechanisms of elastosis in the rare progressive pulmonary fibrosis entity of pleuroparenchymal fibroelastosis

Don Hayes, MD

Dr. Hayes is both an adult and pediatric-trained pulmonologist who oversees the lung transplant program at Cincinnati Children's. His research focuses on the role of the immune system in the lungs and how it contributes to the progression of lung disease and affects lung allografts in patients who have undergone a lung transplant. Dr. Hayes also studies biomarkers to help diagnose lung diseases and pulmonary vascular disorders.

Gurjit (Neeru) Hershey MD, PhD

Dr. Hershey is a pediatric allergist whose research centers on identifying the genes and environmental factors important in promoting asthma and allergy, especially at the epithelial surfaces, and dissecting the molecular mechanisms underlying their contributions. Current projects in the laboratory include:  (1) Elucidation of epithelial genes and exposures critical to allergic disease; (2) Genomic biomarkers of treatment response in childhood asthma; (3) Gene: environment interactions in asthma and allergic diseases; (4) Mechanistic basis of the adverse effect of traffic pollution exposure in early life; (5) pathogenesis of atopic dermatitis; and (6) Identification of genetic and biologic markers of allergic diseases.

Kristin Hudock, MD

Dr. Hudock is an adult and critical care pulmonologist whose research laboratory is at Cincinnati Children's. Her research seeks to understand how an individual’s innate immune response contributes to the pathogenesis of cystic fibrosis (CF), acute respiratory distress syndrome (ARDS) and pneumonia. Her laboratory utilizes primary human cells, murine models, and patient samples to generate new knowledge regarding disease mechanisms with the goal of uncovering unique therapeutic targets.

Erik Hysinger, MD

Dr. Hysinger is developing an imaging techniques that evaluates airway motion without the need for sedation or radiation in children of all ages. This novel magnetic resonance imaging (MRI) provides a unique opportunity to evaluate the impact of airway motion on pediatric health and understand the response to therapeutic interventions.

Paul Kingma MD, PhD

Dr. Kingma is a neonatologist and co-director of the Fetal Care Center of Cincinnati. His clinical expertise in neonatology includes managing children with congenital diaphragmatic hernias and neonatal infections. Dr. Kingma’s lab investigates the role of the innate immune system in pulmonary infections in children. Specific projects currently focus on the role of surfactant protein D in neonatal sepsis and acute respiratory distress syndrome and on lung injury in congenital diaphragmatic hernia patients. Students will work directly with Dr. Kingma and his associates on any of these projects during their summer tenure.

Elizabeth Kramer, MD, PhD

Dr. Kramer is a pediatric pulmonologist interested in genetic modifiers of cystic fibrosis (CF) related lung disease. Her overall aim is to identify novel therapeutic targets in CF. One of the best studied CF lung disease modifiers and biomarkers is transforming growth factor beta (TGF-β), a cytokine that drives CF related pulmonary abnormalities including epithelial ion channel dysregulation, goblet cell hyperplasia, and inflammation. Her research focuses on the impact of TGF-β on CF mice and patient-derived CF airway cell models, with a focus on the unique susceptibility of CF models to the detrimental effects of TGF-β.

Tim LeCras, PhD

The LeCras lab studies the mechanisms that regulate and coordinate lung development, and particularly blood vessel development in the lungs. The lab integrates transgenic mouse models, cell culture studies, and gene arrays to identify new genes and pathways that play a role in newborn lung development, and lung disease in newborns and also children and adults who develop chronic lung diseases and pulmonary hypertension. The long-term goal of these studies is to develop new therapeutic targets for chronic lung diseases, including lung disease in premature infants, pulmonary hypertension, and asthma.

Ian Lewkowich, PhD

Dr. Lewkowich’s laboratory is focused on the immunologic mechanisms responsible for the development of severe allergic asthma. Using a mouse model of allergic asthma in which one strain develops a phenotype characteristic of mild asthma and others develop a phenotype characteristic of severe disease, his group has identified several novel mechanisms through which asthma severity is regulated. Utilizing these mouse models his group is specifically investigating the role of T-cell regulatory immune cells and dendritic cells which influence the inflammatory response to asthmagenic antigens. Students would have the opportunity to work directly using these experimental models of asthma developed in the laboratory.

Tesfaye B. Mersha, PhD

Dr. Mersha's research combines quantitative, ancestry and statistical genomics approaches to unravel genetic and non-genetic contributions to complex diseases and racial disparities in human population, particularly asthma and asthma-related allergic disorders. Current research in his laboratory include: 1) admixture and association analysis; 2) transcriptome profiling studies; 3) microbiome/epigenome analysis among allergic immigrants, and 4) developing web-based bioinformatics tools specifically designed to integrate omics resources from public databases (e.g., 1000 Genomes Project, ENCODE and Epigenome Roadmap). Dr. Mersha's long-term career goals are to develop a program that will lead to an in-depth understanding of the complex interplay between genomic variations and environmental exposure risk factors in the etiology of complex diseases, including asthma.

Dr. Pajor is a pediatric pulmonologist, with a focus on rare lung diseases and those with chronic respiratory failure who are dependent on a ventilator. His research focuses on data science and informatics to improve care and outcomes for children on chronic ventilator support.

Anne-Karina Perl, PhD

Dr. Perl lab utilizes conditional transgenic mice to learn about lung development and to study lung regeneration, and more recently, to study aberrant epithelial repair and lung fibrosis. The Perl lab is integrating lessons learned from lung development with lung repair and successfully established novel conditional mouse models to study re-alveolarization and bronchiolar repair after acute and chronic injury. Current research projects are focused toward understanding the cellular and molecular mechanisms related to the cure of lung diseases including bronchopulmonary dysplasia, emphysema, chronic obstructive pulmonary disease (COPD) and bronchiolitis obliterans.

Adam Powell, MD

Dr. Powell is a pediatric sports cardiologist with a clinical focus on young athletes with complaints of chest pain, fatigue, and shortness of breath during exercise sports. He has expertise in exercise physiology and part of his research focuses includes the evaluation and treatment of children with chest pain and dyspnea with exercise.

Marc Rothenberg MD, PhD

Dr. Rothenberg is a pediatric allergist whose lab has several areas of concentration. The main objective is to elucidate the processes involved in allergic responses in the gastrointestinal tract and lung using cutting-edge approaches of molecular biology, genetics, biochemistry, translational medicine, and computer computation. The importance of key molecules in the body that perpetuate the allergic responses by examination of genetically induced experimental mouse models are employed. Dr. Rothenberg’s lab characterizes the regulation and pathways responsible for eosinophil development and activation and tests the importance and dysfunction of these pathways in patients with inflammatory diseases such as eosinophilic esophagitis and hypereosinophilic syndromes. A direct clinical project includes identifying genetic variations that may predispose patients to eosinophilic disorders.

Patrick H. Ryan, PhD, MS

Dr. Ryan is an environmental epidemiologist whose research interests include air pollution epidemiology and exposure assessment. Currently he is the principal investigator of the Cincinnati Childhood Allergy and Air Pollution Study (CCAAPS), a longitudinal birth cohort study of the effects of diesel exhaust particles on children’s respiratory health and neurodevelopment. His research with the CCAAPS study includes the development of land-use regression models for air pollution exposure assessment and the association between air pollution, respiratory health, and neurobehavioral development. His research also includes the application of novel sensor technology for personal sampling of air pollution, the effectiveness of anti-idling efforts to reduce air pollution exposures at schools, and the use of HEPA filtration to reduce indoor air pollution exposure.

Amanda Schondelmeyer, MD, MSc

Dr. Schondelmeyer is a pediatric hospitalist and physician scientist who uses participatory qualitative and implementation science-informed methods to understand how to implement evidence-based practices across disciplines and health systems and improve the quality and value of care provided to young children hospitalized with common conditions, including bronchiolitis, asthma, and pneumonia.

Grant Schulert, MD, PhD

Dr. Schulert’s laboratory studies inflammatory disease in juvenile idiopathic arthritis (JIA) and complications of systemic JIA such as macrophage activation syndrome and systemic JIA-associated lung disease.

Justin Schwartz, MD, PhD

The Schwartz laboratory is focused on developing less invasive testing for pediatric patients with eosinophilic esophagitis (EoE) including trying to identify blood-based biomarkers that could reduce the need for many endoscopy procedures. In addition, his lab is trying to characterize early immunologic responses to oral immunotherapy for food allergy to determine if these responses are associated with therapy outcomes and could be leveraged as biomarkers for predicting responses to therapy.

Dr Seid is professor of pediatrics in the University of Cincinnati School of Medicine, director of Health Outcomes and Quality of Care Research in the Division of Pulmonary Medicine and holds a joint appointment with the James M Anderson Center for Health Systems Excellence at Cincinnati Children's Hospital Medical Center. Dr. Seid applies behavioral and social science to answer the question ‘What does it take to make sure the right treatment gets to the right child in the right way at the right time, every time?’ In 2009 Seid and Peter Margolis, MD, PhD, received a Transformative Research Award from the NIH to design and test a new system for transforming chronic care, with ImproveCareNow as the prototype. Since then, more than a dozen such Learning Networks have been developed and shown to improve outcomes. Dr. Seid’s focus is on developing the science and practice of collaborative learning health systems to ensure that they benefit the greatest number of people. Opportunities include being involved in the Cystic Fibrosis Learning Network and the development of an Asthma Learning Health System.

Narong Simakajornboon MD

Dr. Simakajornboon is a pediatric pulmonologist who researches the correlation between periodic limb movement disorder (PLMD) in children and low iron storage in a larger patient population. The effect of iron therapy on PLMD is prospectively investigated in a double blinded, placebo-controlled approach. All pediatric patients with PLMD at Cincinnati Children's are being recruited into the study. An overnight polysomnographic evaluation is performed in all patients to confirm the presence and severity of disease. Complete blood count, serum levels of iron, total iron-binding capacity, percentage iron saturation and ferritin is obtained in all patients and compared with age and sex-matched control. Patients with low iron storage are randomly assigned to receive treatment with either iron sulfate or placebo. The treatment response will be measured by an improvement in clinical symptoms, actigraphy monitoring and overnight polysomnographic study. Medical students will participate in the recruitment and data analysis of this ongoing project.

Deborah Sinner, PhD

Dr. Sinner’s lab studies the molecular mechanisms underlying the patterning of the mammalian respiratory tract during normal development and congenital disease. We focus on the interactions between two major components of the developing lung: the pulmonary epithelium - that gives rise to the conducting airways and the respiratory surface of the lung - and the mesenchyme - that gives rise to cartilage, muscle, connective tissue. Experimental approaches include genetic studies using transgenic mice and in vitro cell and organ culture.

David Smith, MD, PhD

Dr. Smith studies how disruption of the circadian clock occurs in the setting of obstructive sleep apnea. Using animal models, his efforts is aimed at better understanding this disease process and creating new treatments for obstructive sleep apnea. Dr. Smith’s long-term goal is aimed at improving diagnostic procedures for children with circadian disorders, standardizing treatments for children with sleep disorders and determining how sleep disruption impacts other diseases.

Rhonda Szczesniak, PhD

Dr. Szczesniak is a statistician who specializes in the clinical translation of dynamic prediction models for child health and care. Her developments have included flexible nonlinear analyses for ambulatory blood pressure monitoring, glucose monitoring and lung function monitoring. Her current work focuses on cystic fibrosis research, specifically on developing forecasting models of rapid lung function decline, to be used for personalized medicine. She has mentored students in biostatistics and pulmonary medicine, and teaches longitudinal data analysis to students on the UC campus.

Joanna Thomson, MD 

Dr. Thomson is a pediatric hospitalist whose research focuses on the hospital management of acute respiratory infections in children with neurologic impairment. Her current work seeks to identify hospital management practices associated with better outcomes in this population through comparative effectiveness research with an eventual goal to develop and implement care recommendations.

Chris Towe, MD

Dr. Towe is a pediatric pulmonologist with a research focus on improving outcomes for pediatric patients with rare lung diseases, lung injury secondary to immunologic insults and lung transplants. This is accomplished by understanding all aspects of these rare lung diseases including: pathogenesis, early detection, diagnosis, prevention, management and treatment. One primary disease of interest is bronchiolitis obliterans, a non-reversible and frequently progressive lung disease that occurs idiopathically, post-viral infection, post-bone marrow transplant and post-lung transplant. Medical students will get exposure to both clinical and translational research efforts with collaborators in pulmonary biology, pulmonary imaging, radiology, pathology, rheumatology, bone marrow transplant and lung transplant.

Brian Varisco MD

Dr. Varisco is a pediatric intensive care physician and his laboratory focuses on understanding the role of stretch in regulating postnatal lung morphogenesis. Specific areas of focus are the role of stretch in the transcriptional and non-transcriptional regulation of lung matrix remodeling, the role of matrix remodeling in inducing microvascular expansion, and defining how lung stretch influences epithelial and mesenchymal signaling pathways. The ultimate goal of the Varisco laboratory is to understand how the postnatal lung grows in a ventilation-perfusion matched manner and to leverage this knowledge to develop novel therapies to improve lung health in patients with congenital and acquired disorders of alveolar simplification.

Laura Walkup, PhD

Dr. Walkup’s research focuses on the translational application of magnetic resonance imaging (MRI) to assess adult and pediatric lung disease. One area of interest is hyperpolarized 129Xe gas as an inhaled MRI contrast agent to quantify regional lung ventilation, alveolar air-space microstructure, and diffusion/perfusion relationships. Dr. Walkup’s research spans the spectrum of rare-lung disease; biomarkers from MRI can impact individual patients directly in “n=1” studies of new therapeutics but also can reveal interesting, basic, regional pathophysiological information on rare diseases which can be assessed longitudinally as MRI is free from ionizing radiation. One project focuses on 129Xe MRI for the early detection of pulmonary complications such as bronchiolitis obliterans following bone-marrow transplantation, where MRI can provide a sensitive, alternative means to assessing lung disease and response to therapy in children too young to perform traditional clinical pulmonary-function testing.

Jeff Whitsett MD

Dr. Whitsett is a neonatologist who leads a well-established laboratory that has focused its attention to the elucidation of cellular and molecular mechanisms regulating lung formation and function.  The laboratory has a long-standing interest in the roles of surfactant proteins SP-A, SP-B, SP-C, SP-D, and GM-CSF in innate host defense and pulmonary function.  The mechanisms controlling lung epithelial specific gene transcription are an active area of study, seeking to determine the functions of a number of transcription factors, including the members of the NKX, FOX, SOX, GATA, and ETS families of transcription factors that regulate lung cell differentiation.  Genetic pathways mediating lung morphogenesis, maturation, and repair are actively studied, seeking to determine the roles of cell signaling and gene transcription in the pathogenesis of lung disease.  The studies seek to understand the molecular pathways causing chronic lung diseases including asthma, cystic fibrosis, emphysema, and lung cancer.  The laboratory makes extensive use of conditional, lung specific gene targeting in transgenic mice.  Systems biology, with an emphasis on bioinformatics of genomic and expression data, are applied to the study of lung biology.  In vivo and in vitro studies are designed to elucidate the cellular and molecular processes regulating lung function.  There are many opportunities for the study of both established and novel pathways critical for lung formation and disease pathogenesis within the laboratory.

Kathryn Wikenheiser-Brokamp MD, PhD

Dr. Wikenheiser-Brokamp is a clinical pathologist with research programs in lung development and cancer. Her laboratory studies the genetic and developmental basis of lung disease, with specific interest in the molecular mechanisms controlling epithelial cell growth, differentiation and signaling.  Dr. Wikenheiser-Brokamp is specifically interested in mechanisms underlying retinoblastoma (Rb), p16 and p53 tumor suppressor control of epithelial progenitor/stem cell growth and lung carcinogenesis. She also studies the role of Dicer1, and the miRNAs it generates, in lung disease. With mentorship by Dr. Wikenheiser-Brokamp and other laboratory members, the students design controlled experiments, organize and interpret data, and present their findings in oral and/or poster format at laboratory meetings and institutional symposia.

Jason Woods, PhD

Dr. Woods is one of the world’s leading experts on hyperpolarized-gas MRI and the use of such gas MRI to measure regional lung function, microstructure and physiology. Dr. Woods directs the Center for Pulmonary Imaging Research—a multidisciplinary research and training program between Pulmonary Medicine and Radiology. His current ongoing research projects include development of MR-imaging biomarkers to quantify i) alveolar size and morphometry during development and as a result of premature birth, ii) efficacy of treatment after regional or endobronchial interventions, iii) imaging after lung transplantation, and iv) the use of murine models of disease, all ultimately to perform longitudinal monitoring without ionizing radiation in pediatric patients. Possible student projects include studies related to murine lung transplantation, morphometric validation of in-vivo MRI, and quantitative image analysis.

Assem Ziady, PhD

The Ziady laboratory focuses on the differential regulation of Nrf2 signaling pathways in the inflammatory lung disease observed cystic fibrosis (CF). The lab has unique expertise in developing and characterizing DNA nanoparticles for nonviral gene delivery to the lung, liver, and brain. In addition, the lab has extensive experience with proteomic analyses for biomarker discovery and the examination of systems biology of various tissues. Potential summer student projects include: 1) Analyzing the database of serum protein differences in mild versus severe lung disease in cystic fibrosis. 2) Examination of how gene transfer enhancing compounds impact the cellular distribution of gene delivery in the lung. 3) Examining the impact of CFTR modulators on inflammatory signaling by primary airway epithelial cells and serum biomarkers of progression of pulmonary fibrosis.