In 2013, the Cystic Fibrosis Foundation (CFF) revised the evidence-based infection prevention and control guidelines. Many embrace the increased measures, others feel they are restrictive, not justified based on evidence, create challenges and have a negative psychosocial impact. It is our responsibility to provide the best care in the safest environment and to find solutions to these challenges so patients do not experience a lack of psychological support and social isolation. This symposium will review the key changes in the recommendations, discuss the evidence showing reduction in CF pathogens when guidelines are implemented, review the psychosocial impact and solutions will be shared.
Summarize the 2013 CFF Infection, Prevention and Control Guidelines to understand the key changes in recommendations that impact on the CF teams in the hospital and clinic setting and to understand how changes in infection control can reduce prevalence of CF pathogens.
Identify practical solutions to the challenges of implementing these new guidelines.
Examine the psychosocial impact of these guidelines on patients and families and develop strategies to minimize social isolation.
This session will provide an update on endocrine issues in cystic fibrosis (CF). Animal models will be presented to provide insight into the role of CFTR in CF bone disease. The applications DXA and new technologies in the assessment of bone in the clinical and research settings will be discussed. The role of diabetes in inflammation, lung disease and mortality will be reviewed.
Describe to describe the role of CFTR in bone.
Discuss to discuss the techniques to assess bone health in cystic fibrosis
Discuss to discuss the relationship of cystic fibrosis related diabetes to inflammation and mortality in cystic fibrosis
Andrea Kelly, M.D., MSCE, Associate Professor, Children's Hospital of Philadelphia
This session will focus on concepts regarding pH modulation in cystic fibrosis. We will examine this in regards to maintenance of a healthy immune system, normal healthy gut flora and the microbiome; nutrition and enzyme utilization; and the effects of pharmacological management. Within the CF population and their frequent need for medications and antibiotics, as well as PERT, we now understand the significance of pH modulation for minimizing the effects of malabsorption and subsequent malnutrition. It is imperative that we strive for interventions that will maximize multi-system functions to minimize long-term concerns and maximize overall health.
Discuss the significance of pH modulation for maximizing general overall health
Describe both the benefits, as well as drawbacks, of acid suppression in Cystic Fibrosis
Identify appropriate pharmacological and non pharmacological practices to ensure appropriate nutritional benefits
This session will present the evidence for making exercise testing and exercise recommendations part of clinical practice for treating CF. The positive effects of regular exercise on the pulmonary and musculoskeletal systems will be described. The evidence for regular exercise evaluations will be presented. The elements of various successful programs will be described, allowing attendees to select those practices that will best fit their context.
Summarize the physiological and clinical effects of regular exercise in patients with CF
Describe the importance of regular exercise testing in clinical practice
Identify elements of exercise programming that will allow successful implementation of exercise recommendations for CF patients
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.
Describe new pathways and signaling molecules (e.g. tyrosine kinases, TGFbeta1, ERK1/2) that regulate CFTR expression and/or function.
Identify signaling molecules and pathways that are altered/affected by lack of CFTR in CF cells and tissues (e.g. TLR4, TPPP and SLC26A9).
Summarize possible ways that manipulation of cell signaling can translate into improved therapies for cystic fibrosis.
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.
Examine host-pathogen interactions in CF disease.
Discuss the outcome of altered immunology of CFTR-deficient cells
Evaluate aberrant mechanisms of repair & remodeling in CF tissue
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.
Define the current genetic and genomic research in CF
Describe the methodology and recent advances to identify modifiers of CF disease severity
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.
Describe current status of clinical trials investigating mutation-specific therapies
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.
Define the microbiota at different locations of the airways in pediatric & adult individuals with cystic fibrosis.
Describe the impact of novel diagnostics & transcriptional profiling on our understanding of the cystic fibrosis airways microbiota.
Discuss the insights gained from metabolic analysis of the airways microbiota & the new hypotheses emerging based on these insights.
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.
Summarize new research in CF liver disease focusing on the role of serum biomarkers in CF & the correlation between fecal microbiome & CF cirrhosis.
Discuss the role of vitamin D in cystic fibrosis intestinal inflammation & understand the impact of lubiprostone in bicarbonate secretion.
Review abnormalities in mitochondrial oxidation of linoleic acid among CF newborns as well as understand the impact of a novel point of care lipase on total fat & long chain fatty acids.
Molly A. Bozic, M.D., Assistant Professor of Clinical Pediatrics, Indiana Univ. School of Medicine