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Saturday
W22--APP&D-GMS-NT: *CF Airway Inflammation
10:30am - 11:50am Mountain - October 20, 2018
Track: Workshop

In this session, we will present data outlining new understanding of inflammatory pathways in CF. We will link inflammation to changes in cellular metabolism and ion channel transport in innate immune cells and bronchial epithelial cells. We will show how these pathways are dysregulated in CF and expand upon potential therapeutic options, which will modify their pro-inflammatory effects.

Learning Objectives:

  • Define how inflammation influences, and is influenced by, changes in cellular metabolism and ion channel transport in CF immune cells and epithelium.
  • Relate how these inflammatory pathways may be regulated therapeutically.
  • Summarize how immune cells and bronchial epithelial cells interact in the CF lung to provide a distinct inflammatory cycle.

Note: Speakers Valerie Urbach and Pauline Bardin's presentations were not recorded at their request.


Saturday
W23 - W23--CFTR: Structure Function & Dynamics of CFTR Ion Channel
10:30am - 11:50am Mountain - October 20, 2018 | Room: 207
Patrick Thibodeau, Ph.D., Workshop Chair; Ina Urbatsch, Ph.D., Workshop Chair; Diogo Poroca, M.Sc., Workshop Speaker; Carleen Mae Sabusap, Ph.D., Workshop Speaker; David Sheppard, Ph.D., Workshop Speaker; Han-I Yeh, B.A., Workshop Speaker
Track: Workshop

CFTR is a chloride channel related to the multi-domain ATP binding cassette (ABC) transporter family. Mutations compromising CFTR folding, such as the prevalent F508del, result in faulty maturation, poor localization to the cell surface, and reduced channel function. Mutations that alter the energetic equilibria and native state conformational dynamics of CFTR likely result in changes to channel gating and conductance. The first cryo-electron microscopy structures of CFTR provide snapshots of the protein in multiple conformational states. However, understanding channel gating and its regulation by both ATP binding and phosphorylation requires in-depth understanding of the dynamics and conformational plasticity of the protein. An integrated structural and functional model of CFTR gating will facilitate understanding of the impact of clinical mutations and the ways that small molecules may provide correction and potentiation.

Learning Objectives:

  • Evaluate the existing structures of CFTR and identify regions of CFTR critical for proper channel biosynthesis and function.
  • Identify conformational and dynamic processes that are critical to CFTR channel gating.
  • Describe structural and conformational states that facilitate CFTR correction and potentiation by small molecules.

Saturday
W24--CLIN: *Progress in CF Pulmonary Disease
10:30am - 12:05pm Mountain - October 20, 2018
Raksha Jain, M.D., M.Sc., Workshop Chair; Douglas Li, M.D., Workshop Chair; Zackary Cleveland, Ph.D., Workshop Speaker; Sonya Heltshe, Ph.D., Workshop Speaker; Annalisa Piccorelli, Ph.D., Workshop Speaker; Margaret Rosenfeld, M.D., M.P.H., Workshop Speaker; Don Sanders, M.D., M.S., Workshop Speaker; Jennifer L Taylor-Cousar, M.D., M.Sc., Workshop Speaker
Track: Workshop

Care of CF pulmonary disease is multifaceted, including various tools for characterization and treatment. This session will highlight recent major advances in the care of both pediatric and adult CF pulmonary disease. Topics covered will include radiologic, pharmacologic treatment, and lung transplant advances.

Learning Objectives:

  • Describe the early structural changes in the CF lung using 129Xe ADC and CT modalities.
  • Characterize variables that affect the probability of, and time to lung transplant in CF patients.
  • Describe the treatment effect of hypertonic saline in preschool children with CF, and variables affecting IV pulmonary exacerbation treatment course in adults with CF.

Saturday
W25 - W25--GMS: In Vivo Models of CF
10:30am - 11:50am Mountain - October 20, 2018 | Room: 203
Camille Ehre, Ph.D., Workshop Chair; Anthony Fischer, M.D., Ph.D., Workshop Chair; Craig A. Hodges, Ph.D., Workshop Speaker; Alessandra Livraghi-Butrico, Ph.D., Workshop Speaker; Jyoti Sharma, M.S., Workshop Speaker; Frédéric Velard, Ph.D., Workshop Speaker; Keyan Zarei, BSE, Workshop Speaker
Track: Workshop

This session will discuss recent advances in the study of animal models of CF.

Learning Objectives:

  • Compose insights into the pathogenesis of cystic fibrosis by examining different animal models.
  • Discuss how animal models are used to test novel therapies for cystic fibrosis.
  • Compare cystic fibrosis disease manifestations in different animal species.

Saturday
W26 - W26--INF/MIC: Microbial Ecology of CF Airways
10:30am - 11:50am Mountain - October 20, 2018 | Room: 107
Lucas Hoffman, M.D., Ph.D., Workshop Chair; Katrine Whiteson, Ph.D., Workshop Chair; Jennifer Bomberger, Ph.D., Workshop Speaker; Do Yeon Cho, M.D., Workshop Speaker; Sophie Darch, Ph.D., Workshop Speaker; Brian Kocak, B.S., Workshop Speaker; Sarah Lucas, B.S., Workshop Speaker; Geraint Rogers, Ph.D., Workshop Speaker
Track: Workshop

It has become increasingly clear that polymicrobial interactions, environmental conditions and evolutionary adaptations are critical to the progression of CF disease. Yet, a lack of ecological perspective has limited the translation of bench research into effective therapeutic strategies. For example, Pseudomonas does not colonize the airways alone - how do other community members and the infection environment impact Pseudomonas physiology? Recently, culture-independent approaches (genomics, transcriptomics and metabolomic) have shed light on CF infection communities, though many outstanding questions remain. In this workshop we will explore the ecology of CF airway infection communities, with the ultimate goal of devising more effective clinical therapies. Talks from scientists studying clinical samples and also recreating polymicrobial communities in the lab will educate the audience about new tools and hypotheses for relating the CF infection ecosystem to disease progression.

Learning Objectives:

  • Compare and contrast the presented approaches for studying polymicrobial communities in the CF airways that will help us identify evolutionary and ecological instabilities.
  • Identify missing datasets that are critical for managing CF as an ecosystem.
  • Exchange information about polymicrobial infection ecology amongst microbiologists, clinicians, and bioinformaticians.

Saturday
W27 - W27--NRS-SW/PSYCH: Innovative Approaches to Building a Successful Team
10:30am - 11:50am Mountain - October 20, 2018 | Room: Milehigh 4CDEF
Lynn Bonitz, R.N., M.S., Workshop Chair; Ashlee Harvey, B.S.N., R.N., Workshop Chair; Veronica Downer, R.N., Workshop Speaker; Colleen E. Dunn, M.S., R.R.T., CCRC, Workshop Speaker; Lynne M. Fries, P.A.-C., MPAS, D.P.T., Workshop Speaker; Katie Hall, LMSW, Workshop Speaker; Emily Muther, Ph.D., Workshop Speaker; Karen Siklosi Raraigh, M.G.C., Workshop Speaker
Track: Workshop

This session will focus on the integration of the different rolls of the Cystic Fibrosis care team members, and how they work with the entire team to provide high quality care to each patient.

Learning Objectives:

  • Discuss the importance of each team member's roll on the multidisciplinary team.
  • Identify characteristics of a high functioning CF team.
  • Describe strategies to integrate each individual discipline into CF clinic.

Saturday
W28 - W28--NT: Novel & Emerging Targets for Those in Need
10:30am - 11:50am Mountain - October 20, 2018 | Room: 106
Peter Haggie, Ph.D., Workshop Chair; Christoph Randak, M.D., Workshop Chair; Anna Ermund, M.Sc., Ph.D., Workshop Speaker; Kelly Martinovich, B.Sc., Workshop Speaker
Track: Workshop

During this session we will explore novel and emerging approaches to overcome the basic physiological consequences of CFTR deficiency that do not directly target mutant CFTR proteins. These approaches have the potential to reduce disease burden in people with cystic fibrosis who currently are not responsive to CFTR modulator therapies. Considered will be potential therapeutic effects of recently discovered compounds targeting the epithelial sodium channel, sodium-hydrogen exchanger 3, the intracellular scaffold protein phosphoinositide 3-kinase γ, and VPAC (vasoactive intestinal peptide and pituitary adenylate cyclase activating peptide) receptors. In addition, a new pre-mRNA editing technique to remove disease-associated mutations by exon 15 skipping will be presented.

Learning Objectives:

  • Analyze how compounds inhibiting the epithelial sodium channel and sodium-hydrogen exchanger 3 reverse the effects of CFTR deficiency on airway surface liquid hydration and mucus clearance.
  • Describe how polypeptides that either inhibit the intracellular scaffold protein phosphoinositide 3-kinase γ or have agonistic effects at VPAC (vasoactive intestinal peptide and pituitary adenylate cyclase activating peptide) receptors increase channel function of F508del CFTR and reduce cystic fibrosis disease manifestations in mouse models.
  • Discuss a novel pre-mRNA editing technique to induce exon 15 skipping to remove an array of deleterious CFTR mutations in order to restore CFTR channel function.

Note: Speakers Patrick Moore, Alessandra Murabito, Valerie Chappe's presentations were not recorded at their request.


Saturday
W29 - W29--PT&RT-CLIN: The Role of the PT/RT for Patients Who Are Pre & Post Lung Transplant
10:30am - 11:50am Mountain - October 20, 2018 | Room: 505
Track: Workshop

This session will look at current and evolving practices in the pre and post lung transplant patient with cystic fibrosis with an emphasis on physical therapy/pulmonary rehab and airway clearance. Exercise and ACTs have become effective treatment options for patients on the waiting list for lung transplantation, and can improve quality of life. Exercise is also necessary to ensure patients are strong enough to recover from surgery. Improving education on exercise and airway clearance is essential to improve the quality of life in these patients.

Learning Objectives:

  • Define the importance of exercise and airway clearance techniques in patients with cystic fibrosis pre-lung transplant and post lung transplant wanting to improve their quality of life and prior level of function.
  • Describe challenges before and after lung transplant to support patients with cystic fibrosis that are utilizing physical therapy/pulmonary rehab.
  • Discuss the impact that physical therapy/pulmonary rehab and airway clearance can have on recovery of patients with cystic fibrosis who have had a lung transplant.

Saturday
W30 - W30--SW/PSYCH-NTR-NRS: Feeding & Eating Disorders
10:30am - 11:50am Mountain - October 20, 2018 | Room: Milehigh 1CDEF
Sheehan Fisher, Ph.D., Workshop Chair; Lisa Opipari-Arrigan, Ph.D., Workshop Chair; Adrienne Borschuk, Ph.D., Workshop Speaker; Dixie Durham, CCRC, MHS, Workshop Speaker; Stephanie Filigno, Ph.D., Workshop Speaker; Jillian Mai, B.S., RD, LD, CNSC, Workshop Speaker; Elizabeth Reid, M.S., R.D.N., L.D.N., Workshop Speaker; Tiffany Robb, B.S., Workshop Speaker
Track: Workshop

One of the most concerning symptoms of cystic fibrosis for children are failure to thrive and malnutrition. Patients with cystic fibrosis must follow a strict diet that is implemented by their parents or self-implemented. The nutritional focus for survival establishes child feeding as a primary treatment goal for parents, and children with CF require 20 to 50% more calories per day and a higher fat diet than a healthy child. Treatment regimens that specify a therapeutic diet may place the child at increased risk for feeding problems or disordered eating behaviors. This session will focus on recent research on feeding and disordered eating in children and adults.

Learning Objectives:

  • Describe the unique risk factors for feeding problems in patients with cystic fibrosis.
  • Determine the impact of behavioral-nutrition intervention on families of children with cystic fibrosis.
  • Describe the relation between cystic fibrosis and the risk for negative body image.

Saturday
W31 - W31--U&C-CLIN: Hot Topics in Health Policy
10:30am - 11:50am Mountain - October 20, 2018 | Room: 711
Gregory Sawicki, M.D., M.P.H., Workshop Chair; Jeffery Zobell, Pharm.D., Workshop Chair; Mary Daniels, M.D., M.P.H., Workshop Speaker; Lisa Feng, DrPH, Workshop Speaker; Semret Seyoum, M.P.H., Workshop Speaker
Track: Workshop

The content of this workshop session with be based on abstracts submitted by researchers focusing on cost of care, access and insurance issues, and utilization of healthcare resources for CF patients in both the U.S. and abroad.

Learning Objectives:

  • Describe trends in cost of CF Care.
  • Identify patterns of health care utilization for CF patients with government-funded insurance (i.e. Medicaid, Medicare, Universal).
  • Discuss access to care challenges for CF patients.

Note: Speaker Kate Skolnik's presentation was not recorded at her request.


Saturday
S17 - S17--CFTR: Challenging CFTR Mutations: Meeting Unmet Needs
02:30pm - 03:55pm Mountain - October 20, 2018 | Room: 207
Jennifer S Guimbellot, M.D., Ph.D., Symposium Chair; Phil J. Thomas, Ph.D., Symposium Chair; Peter Haggie, Ph.D., Symposium Speaker; Nael A. McCarty, Ph.D., Symposium Speaker; Martin Mense, Ph.D., Symposium Speaker
Track: Symposium

The care of CF patients who possess mutations responsive to available CFTR modulators has changed dramatically in recent years. If new modulators being tested in the clinic perform as hoped, the majority of patients with CF could be treated with at least one drug targeted to the underlying cause of their disease. However, a subset of rare variants will not share the benefit of these developments. In particular, patients with premature stop codons, splice defects, conductance mutations, and refractory folding mutants, such as N1303K, will remain an unmet need. To expand novel treatments to all patients, a deeper understanding of these variants is needed. In this session, we will explore current knowledge of these challenging molecular pathologies.

Learning Objectives:

  • Discuss the molecular and functional phenotypes of rare disease-causing variants (including premature stop codons, splicing mutations, and conductance mutations) and outline potential therapeutic strategies for correcting them in cystic fibrosis.
  • Describe development of new tools to understand the molecular and functional features of rare mutations, including those that could enable screening to identify novel therapeutics.
  • Define why challenging folding mutants, such as N1303K, are refractory to currently approved CFTR modulators.

Note: Speaker Batsheva Kerem's presentation was not recorded at her request.


Saturday
S18 - S18--CLIN: Transplant & Advanced Lung Disease
02:30pm - 03:55pm Mountain - October 20, 2018 | Room: Milehigh 1CDEF
Christian Merlo, M.D., M.P.H., Symposium Chair; Kathleen J. Ramos, M.D., M.Sc., Symposium Chair; Siddhartha Kapnadak, M.D., Symposium Speaker; Dio Kavalieratos, Ph.D., Symposium Speaker
Track: Symposium

The care of individuals with CF and advanced lung disease can be challenging, especially as one considers the transition to lung transplantation. This session will give an overview of the care of patients with advanced lung disease, the timing of referral and listing for lung transplantation, the transition to transplant and palliative care.

Learning Objectives:

  • Identify important indications for lung transplant referral and describe comorbid conditions that require specific treatment among patients with reduced lung function.
  • Discuss the known barriers and facilitators to a smooth transition to lung transplant with a focus on lessons learned through the CF Lung Transplant LLC.
  • Summarize the available evidence for palliative care in CF and describe practices that can be incorporated into the care of patients with advanced lung disease.

Saturday
S19--CLIN-NRS: *Pain Management in CF
02:30pm - 03:55pm Mountain - October 20, 2018
Deborah Friedman, Ph.D., Symposium Chair; Noah Lechtzin, M.D., M.H.S., Symposium Chair; Sarah J. Allgood, B.S.N., Ph.D., Symposium Speaker; Daniel Clauw, M.D., Symposium Speaker; Terry Gebhardt, P.T., D.P.T., Symposium Speaker
Track: Symposium

Pain is increasingly recognized as a common and clinically important symptom in people with CF. Some estimate that up to 80% of adults with CF experience frequent pain that is at least moderately severe. However, there is poor understanding about what constitutes 'CF pain' and even less agreement on appropriate and effective treatment strategies. This session will feature four speakers who will address the following topics: 1. Parsing CF pain into nociceptive, neuropathic, and centralized pain with appropriate treatment targeting. 2. Managing misuse, abuse, and addiction to opioids. 3. Cognitive behavioral therapy for chronic pain. 4. Physical therapy and exercise therapy for pain management.

Learning Objectives:

  • Explain the different types of pain common in people with CF and understand how this shapes treatment.
  • Define strategies for preventing and managing misuse and addiction to opioids.
  • Discuss how cognitive behavioral therapy, physical therapy and exercise therapy can be utilized for CF pain management.

Saturday
S20 - S20--CLIN-NTR: Assessment of Function in GI, Endocrine, & Nutrition
02:30pm - 03:55pm Mountain - October 20, 2018 | Room: Milehigh 4CDEF
Michael Shane Stalvey, M.D., Symposium Chair; Michael Wilschanski, MBBS, Symposium Chair; Jessica Alvarez, Ph.D., R.D., Symposium Speaker; Abeer Anabtawi, M.D., Symposium Speaker; Jill Dorsey, M.D., Symposium Speaker; Baha Moshiree, M.D., Symposium Speaker
Track: Symposium

Assessment of clinical measures of disease are essential to both research and clinical care. Non-traditional aspects of CF, and the determination of health in that area, may be unfamiliar with providers and researchers. This symposium will provide an understanding to promote study and interpretation of these additional comorbidities.

Learning Objectives:

  • Describe the importance of body composition in clinical care and research.
  • Identify with measurements of bone health and metabolism in clinical research.
  • Discuss assessments of fecal/serum markers of intestinal inflammation and research & clinical care assessments of constipation in CF.

Saturday
S21 - S21--INF/MIC: Controversies in Airway Infection
02:30pm - 03:55pm Mountain - October 20, 2018 | Room: 107
Paul Planet, M.D., Ph.D., Symposium Chair; Natalie E. West, M.D., M.H.S., Symposium Chair; J. Stuart Elborn, M.D., FRCP, Symposium Speaker; Jordana Hoppe, M.D., Symposium Speaker; John J. LiPuma, M.D., Symposium Speaker; Jessica Pittman, M.D., M.P.H., Symposium Speaker
Track: Symposium

This session will involve two Pro/Con debates involving issues in airway infection in cystic fibrosis. The first talk will debate whether aggressive antimicrobial management of infants and young children is good or bad. The second debate will be a discussion on whether anaerobes are good or bad actors in the airways of cystic fibrosis individuals.

Learning Objectives:

  • Define the rationale behind aggressive early treatment of young children, and the reasons that treatment may not be beneficial or harmful.
  • Define what is known about the role of anaerobic bacteria in cystic fibrosis in the progression of lung disease and exacerbation.
  • Review treatment options for chronic therapy as well as treatment for pulmonary exacerbations.

Saturday
S22 - S22--NT-APP&D: Resolution of Inflammation in CF Airways: CFTR & Beyond
02:30pm - 03:55pm Mountain - October 20, 2018 | Room: 505
James F. Chmiel, M.D., M.P.H., Symposium Chair; Katie Hisert, M.D., Ph.D., Symposium Chair; Antonio Recchiuti, Ph.D., Symposium Speaker; Steven M. Rowe, M.D., M.S.P.H., Symposium Speaker; Abdullah Tarique, Ph.D., Symposium Speaker
Track: Symposium

Airway disease remains the leading cause of morbidity and mortality in individuals with cystic fibrosis. The absence of CFTR leads to multiple changes in airways surface secretions and host defenses that are associated with severe and sustained airway inflammation. Correction of CFTR function may decrease part of the inflammatory process and prevent tissue destruction. However inflammation independent of CFTR is likely to persist. This symposium will address both CFTR -dependent and -independent mechanisms of resolving inflammation.

Learning Objectives:

  • Describe the impact of CFTR modulators on the host inflammatory response in cystic fibrosis.
  • Define the roles of macrophages and pro-resolving mediators in the CF airway inflammatory response.
  • Describe the results of a recently completed clinical trial of an anti-inflammatory drug in cystic fibrosis.

Saturday
S23 - S23--PT&RT: Literature Review of Hot Topics in RT & PT Care
02:30pm - 03:55pm Mountain - October 20, 2018 | Room: 203
Colleen E. Dunn, M.S., R.R.T., CCRC, Symposium Chair; Lauren Lyons, MSPT, cNDT, Symposium Chair; Julie A. Christiansen, A.S., P.T., Symposium Speaker; Ryan A. Harris, Ph.D., Symposium Speaker; Mary K. Lester, R.R.T., Symposium Speaker; Carlos Milla, M.D., Symposium Speaker
Track: Symposium

This symposium is planned to highlight current literature as it pertains to PT and RT practice across the care spectrum from inpatient to outpatient care for adults and children with cystic fibrosis. Our physical therapy and exercise science session presenters will overview current evidence based practice around exercise prescription and musculoskeletal management. Our respiratory therapy session presenters will highlight the most up to date literature pertaining to airway clearance and pulmonary diagnostics. Collaborative care within the physical and respiratory care teams will be discussed as it pertains to each of these subjects.

Learning Objectives:

  • Demonstrate an understanding of current literature as it relates to respiratory care for airway clearance devices and pulmonary diagnostics.
  • Apply knowledge of current evidence surrounding exercise and musculoskeletal management.
  • Recall learning back to their care centers ideas for collaborative care between respiratory and physical therapy team members as it relates to current literature.

Saturday
S24--SW/PSYCH-NRS-CLIN: *'Adulting' With CF
02:30pm - 03:55pm Mountain - October 20, 2018
Christopher Drescher, Ph.D., Symposium Chair; Meg Dvorak, LCSW, Symposium Chair; Megan Barker, M.S., Symposium Speaker; Adrienne Borschuk, Ph.D., Symposium Speaker; Traci Kazmerski, M.D., M.S., Symposium Speaker; Pablo Saldana, Ph.D., LCSW, Symposium Speaker
Track: Symposium

As life expectancy increases among CF patients, the model of care is expanding to include more focus on adult responsibilities, co-morbidities, and challenges of daily living and quality of life. There are many unique and challenging concerns for aging CF patients and the families and communities that support them. This symposium will offer a wide range of interesting topics on 'adulting with CF' to enhance understanding of adult issues and engage persons with CF and their families more fully in care. Four expert speakers will discuss: disclosure related to CF diagnosis, sexual/reproductive health for women with CF, career and employment, and the CF spouse's journey.

Learning Objectives:

  • Identify that adult CF patients and their spouses have multiple challenges and stressors-- balancing lifestyle, career, health, and family.
  • Identify important care needs for females with CF from adolescence through adulthood, specifically related to sexual and reproductive health.
  • Define the complexities of disclosure of CF diagnosis at all stages of disease.