This workshop will review clinical research on the evaluation and treatment of pulmonary manifestations of cystic fibrosis. The research presented will include predictors of disease severity and response to treatment as well as a novel imaging technique utilizing magnetic resonance. The session will also explore how the addition of therapies such as hypertonic saline and ivacaftor may benefit cystic fibrosis patients.
Rebekah F. Brown, M.D., Assistant Professor of Pediatrics, Vanderbilt University Medical Center
Caralee Forseen, M.D., Director, Adult CF and Co-Director, GRU CF Center, Georgia Regents Univ
The presentation of advanced lung disease patients in the cystic fibrosis population is becoming more common due to improved treatment strategies which extend life. Caregivers are required to be well versed in the care of this population including introduction to palliative care, lung transplant, and advanced directives. Special considerations in the administration and compliance with immunosuppression, pain therapy, and antibiotic treatments is critical in the advanced lung disease patient.
This session will explore the multiple facets of patient education, as well as collaboration and communication between the CF Care Center and in-patient staff when implementing behavioral interventions for pediatric patients in the hospital setting.
Lisa C. Greene, M.A., CFLE, Executive Director, Parenting Children With Health Issues
This workshop will focus on clinical scenarios and novel methods of delivery and enhancement of airway clearance and invite discussion from a mixed audience of physical and respiratory therapists, primary providers and researchers. Invited speakers will define difficult situations, especially in the adult CF patient, as well as pulmonary and extra-pulmonary physical barriers to effective airway clearance. The second portion of the workshop will explore research into novel methods of approaching the current standard of care of airway clearance and means to improve and augment current standard practice.
Many signaling molecules and pathways affect CFTR expression and/or function, thus directly impacting CFTR deficiency in CF. Conversely, lack of CFTR in CF cells and tissues alters cell functions other than anion conductance, such as inflammatory responses, that contribute to health outcomes. This session is devoted to presentations that highlight interplays between CFTR and its cellular environment that affect disease progression and/or the efficacy of therapeutic approaches for CF.
Wito Richter, Ph.D., Assistant Professor, University of South Alabama College of Medicine
This session will evaluate mechanisms underpinning the interplay between infection, inflammation and repair/remodeling in CF lung disease. Presentations will focus on host-pathogen interaction in the setting of dysfunctional CFTR.
This session will allow the participant to (1) Define the current genetic and genomic research in CF; (2) Describe the methodology and recent advances to identify modifiers of CF disease severity; and (3) Summarize the current state of genetic modifiers in CF.
Hara Levy, M.D., Staff Physician, Children's Hospital of Wisconsin
J. Michael Collaco, M.D., M.B.A., M.P.H., Assistant Professor, Johns Hopkins Medical Institutions
In this session the most recent data of clinical trials evaluating CFTR mutation-specific pharmacological therapeutics will be presented. Also, promising novel molecular therapies targeting CFTR mutations will be discussed.
The focus of this Workshop is on the microbiome and emerging pathogens of the airways in cystic fibrosis. This combines advances from basic science through to new clinically-important insights on the airways microbiota.
This workshop session will provide an overview of current research in the field of gastroenterology, hepatology and nutrition for cystic fibrosis patients. The workshop will cover both clinical care topics as well as basic science research. Attendees will learn about biomarkers for CF related cirrhosis, the role of the fecal microbiome in CF liver disease and the anti-inflammatory properties of vitamin D in CF intestinal disease. Research on a novel point of care lipase and long chain polyunsaturated fatty acids, impact of lubriprostone in bicarbonate secretion and abnormalities in mitochondrial oxidation of linoleic acids in newborns will be presented.
Molly A. Bozic, M.D., Assistant Professor of Clinical Pediatrics, Indiana Univ. School of Medicine