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.
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.
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.
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.
This session focuses on recent discoveries relating to (1) the molecular defects of CFTR mutants; (2) the identification, development, and characterization of novel CFTR modulators; and (3) therapeutic implications.
S. Vamsee Raju, B.Pharm., Ph.D., Post Doctoral Fellow, Gregory Fleming Cystic Fibrosis Research Center
This session will highlight the recent and important advances in understanding the mechanisms underlying altered mucus clearance in the CF airway. These talks will focus on three aspects of mucus clearance; fluid secretion, ion transport, and mucus properties and composition. Additionally, the information presented will focus on inter-relationships between these facets of the mucus clearance apparatus.
This workshop will focus on emerging research models for investigating the pathogenesis and treatment of cystic fibrosis. In addition to new animal models including the CF rat and ferret, cellular models to be presented range from cell lines expressing multiple CFTR mutations to primary culture methods expanding human airway tissue, generating organotypic structures and investigating male reproductive tract biology. Emphasis is placed on the advantages these research methods offer for investigating the multiple manifestations of CF disease.
This workshop will review the latest trends in respiratory microbiology based on US CF Registry data. This session will also focus on the long-term impact of successful Pseudomonas aeruginosa eradication on health outcomes and the chronic use of inhaled liposomal amikacin in the management of chronic Pseudomonas aeruginosa infection. The results of a large survey will also be presented examining antibiotic utilization to treat Mycobacterium abscessus infection. Lastly, the usefulness of biofilm antimicrobial susceptibility testing and microbiome analysis in the management of CF pulmonary exacerbations will be presented.
Bradley S. Quon, M.D., M.Sc, M.B.A., Clinical Research Fellow, University of British Columbia
Jane L. Burns, M.D., Professor, Pediatrics/Infectious Diseases, Univ. of Washington School of Medicine
This workshop will help you understand the importance of having a Crisis Communication Plan; as well as give you examples of what other sites have experienced and learned while creating their plan. You will come away with tools, ideas and motivation to make your own Crisis Communication Plan. If a CF patient has a life-threatening event while enrolled in a clinical trial, having a Crisis Communication Plan can prevent a catastrophe.
This workshop is intended to address the management of CF patients with advanced lung disease. The session will include a discussion of CFTR modulation in patients with advanced lung disease. A broad array of topics in lung transplantation will be reviewed. Transplantation will be discussed from pediatric and adult viewpoints as well as from an international perspective.