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Friday
S09 - S09--APP&D-NT-CLIN: The Spectrum of CF Muco-obstructive Airway Disease
10:30am - 11:55am Mountain - October 19, 2018 | Room: Four Seasons 2
Jennifer Goralski, M.D., Symposium Chair; Marcus Mall, M.D., Symposium Chair; Christopher M. Evans, Ph.D., Symposium Speaker; Charles Esther Jr., M.D., Ph.D., Symposium Speaker
Track: Symposium

One of the fundamental pathophysiologic defects in CF is abnormal mucociliary clearance (MCC), a basic host defense mechanism. This deficiency in the CF lung leads to mucostasis, chronic bacterial infection and progressive inflammation, ultimately resulting in diminished lung function and shortened lifespan in patients with CF. As defective MCC is common among all patients with CF, this pathway represents a target for treatment that would be beneficial for all patients, regardless of genotype. Moreover, accumulating data suggests that mucus obstruction alone results in inflammation, regardless of the presence of bacteria. This symposium session will provide an overview of the mechanisms involved in mucociliary clearance and the therapeutic targets associated with them. 

Learning Objectives: 

  • Distinguish the pathophysiologic phenotypes of CF mucus obstruction, and compare and contrast with other muco-obstructive disorders. 
  • Discuss important therapeutic approaches that address abnormal mucociliary clearance in CF. 
  • Examine novel targets for therapy to slow the progression of CF lung disease.

Note: Speaker David Stoltz's presentation was not recorded at his request. 


Friday
S10 - S10--CFTR: Say Hello to CFTRs Little Friends
10:30am - 11:55am Mountain - October 19, 2018 | Room: 207
Track: Symposium

Recent research has identified that both normal and mutant CFTR function can be modulated by endogenous and exogenous elements. This session will describe several studies focusing on how to understand current CFTR modulator compound interactions with CFTR as well as endogenous cellular mechanisms that regulate CFTR trafficking and function.

Learning Objectives:

  • Summarize the mechanism of action of existing small molecule therapies on CFTR and how they physically interact with CFTR.
  • Summarize how post-translation modification of CFTR (e.g., sumoylation) can significantly regulate CFTR maturation or degradation.
  • Describe how other endogenous proteins may alter CFTR trafficking and function through direct interactions.

Note: Speakers Christine Bear and Jue Chen's presentations were not recorded at their request. 


Friday
S11 - S11--CLIN: CF Liver Disease: Advances & Challenges
10:30am - 11:55am Mountain - October 19, 2018 | Room: Milehigh 1CDEF
Daniel H Leung, M.D., Symposium Chair; Michael R. Narkewicz, M.D., Symposium Chair; David N. Assis, M.D., Symposium Speaker; Simon Ling, MBChB, Symposium Speaker; Jean P. Molleston, M.D., Symposium Speaker
Track: Symposium

The session is designed to provide insight into the current state of liver disease in CF. The session will include up to date information on the evaluation and management of CF associated liver disease. The symposium will include information relevant for clinicians, care team members and CF scientists. The focus of this symposium will be on the identification and evaluation of liver disease in CF, including unique aspects for adults with CF. Discussion of the management of advanced liver disease and therapeutic options including liver transplantation will be addressed.

Learning Objectives:

  • Define/describe the classification of liver involvement in CF and various theories of CF liver disease pathogenesis.
  • Discuss the implications of abnormal imaging findings and the role of elastography in CF.
  • Review the management of complications of portal hypertension in CF and role of liver transplantation.

Friday
S12 - S12--INF/MIC: Microbial Adaptation in the CF Airway
10:30am - 11:55am Mountain - October 19, 2018 | Room: Milehigh 4CDEF
Amal Amer, M.D., Ph.D., Symposium Chair; Helle Krogh Johansen, M.D., Symposium Chair; Silvia Cardona, Ph.D., Symposium Speaker; Lucas Hoffman, M.D., Ph.D., Symposium Speaker; Barbara Kahl, M.D., Symposium Speaker; Daniel J. Wozniak, Ph.D., Symposium Speaker
Track: Symposium

This session focuses on the mechanism by which CF-related pathogens establish infection and persist in the host. The session will start with Staphylococcus aureus as one of the earliest and most prevalent microorganism in CF, which can persist in the airways for years or even decades. The second talk will focus on the fitness traits exhibited by Pseudomonas aeruginosa colony variants and the selective forces that underlie conversion and patho-adaptation during chronic infection. This will be followed by a talk describing how Burkholderia cenocepacia senses the amino acid-rich environment of the cystic fibrosis lung and elicits an adaptive response that is niche-specific according to nutritional differences. The final talk will describe microbiome methods and how they differ and limitations of each. This session will offer new advances in microbial pathogenesis and how the microbiome alters lung disease pathogenesis in CF.

Learning Objectives:

  • Describe microbial adaptation for Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cenocepacia in CF.
  • Evaluate the microbiome approaches and how the study of microbiome in CF changed the way we think about pathogenesis in CF.
  • Characterize the most recent updates in the field of microbial pathogenesis in CF.

Friday
S13--NRS-CLIN: *The Changing Face of Pulmonary Exacerbation Treatment
10:30am - 11:55am Mountain - October 19, 2018
Stacy Bichl, A.P.N., CPNP, AE-C, Symposium Chair; Connie Richless, M.N., R.N., Symposium Chair; Debbie Benitez, ACNP-BC, Symposium Speaker; Alex Burchill, Pharm.D., Symposium Speaker; Elliott Dasenbrook, M.D., M.H.S., Symposium Speaker; Don Sanders, M.D., M.S., Symposium Speaker; Joshua Wang, Pharm.D., Symposium Speaker
Track: Symposium

This session will provide an overview of the current trends in pulmonary exacerbation management highlighting the pros and cons of home versus hospital exacerbation treatment. In addition, models for both inpatient and outpatient exacerbation management from onset to resolution will be discussed.

Learning Objectives:

  • Describe the benefits, risks and outcome variation of treating pulmonary exacerbations in the home versus hospital setting, addressing the medical, social and economic implications of each.
  • Identify trends of home intravenous management, discussing a model of monitoring, and issues related to delivery devices, safety and drug stability.
  • Review the importance of following exacerbation to resolution and present a sample algorithm of standardized exacerbation identification and treatment.

Friday
S14--NT: *Progress & Promise of the CFTR Modulator Pipeline
10:30am - 11:55am Mountain - October 19, 2018
Track: Symposium

The drug discovery and development pipeline for CF has never been more active. In this session, we will begin by reviewing the state of play with small molecule CFTR modulators, currently clinically available in single and dual agent form and in trials as triple agents. Recognizing that patients with certain, rarer mutations are more difficult to treat, presenters will then look to the future, considering best ways of testing drugs for these groups and reviewing progress with splice and nonsense mutations.

Learning Objectives:

  • Review the current landscape of CFTR modulating agents and other drugs targeting the basic CFTR defect.
  • Illustrate the challenges inherent in drug development for patients with rare gene mutations.
  • Use two examples of rare mutation groups (splice mutations and premature truncation codons) to describe preclinical progress and anticipate routes to the clinic.

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


Friday
S15 - S15--NTR-CLIN-SW/PSYCH: Multidisciplinary Approach to Nutrition
10:30am - 11:55am Mountain - October 19, 2018 | Room: Four Seasons 1
Karen M. Maguiness, M.S., R.D., C.S.P., Symposium Chair; Ala Shaikhkhalil, M.D., Symposium Chair; Tanja Gonska, M.D., Symposium Speaker; Daina B. Kalnins, M.Sc., R.D., Symposium Speaker; Marina Litvin, M.D., Symposium Speaker; Abby Orkis, M.S.W., L.S.W., Symposium Speaker
Track: Symposium

This session will promote disciplines working together to care for patients with CF, especially those patients with complicated or complex needs. There will be one adult case presentation and one pediatric case presentation.

Learning Objectives:

  • Express the complicated and multifaceted nature of nutrition problems in children and adults with CF.
  • Categorize some of the main barriers to improving nutrition including medical and non medical.
  • Identify and summarize the roles of different professionals and members of the CF team in improving nutrition in CF patients: including dietitians, physicians, mental and behavioral health professional, social workers, and pharmacists.

Friday
S16 - S16--PT&RT-CLIN: The Multidisciplinary Care of the CF Patient With NTM
10:30am - 11:55am Mountain - October 19, 2018 | Room: Four Seasons 4
Jerry A. Nick, M.D., Symposium Chair; David Young, Pharm.D., Symposium Chair; Laney Brennan, Pharm.D., Symposium Speaker; Sarah Ellington, B.S.N., Symposium Speaker; Cara-lynn Gomez, C.R.T., R.R.T., Symposium Speaker; Stacey L Martiniano, M.D., Symposium Speaker
Track: Symposium

Infection with nontuberculous mycobacteria is one of the most feared and poorly understood complications of CF lung disease. There is a good deal of uncertainty in identifying which patients will benefit from treatment, which antibiotics to use, proper dosing and monitoring, specific aspects of respiratory care, and how long to continue treatment. This Symposium will review the central aspects of the diagnosis and treatment of NTM in individuals with CF, based on the 2016 CFF/ECFC Guidelines. In particular, the role and coordination of the multidisciplinary team will be emphasized.

Learning Objectives:

  • Review the criteria for treatment of NTM in the context of CF lung disease.
  • Discuss the considerations for the selection and administration of antibiotics for the treatment of NTM disease.
  • Describe specific consideration for airway clearance, respiratory therapy and coordination of the multidisciplinary team in optimizing response to NTM treatment.

Friday
W11 - W11--APP&D-CFTR-INF/MIC: Airway Innate Defense Defects in CF
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: Milehigh 1CDEF
Paul McCray Jr., M.D., Workshop Chair; Dao Nguyen, M.D., FRCPC, Workshop Chair; Bijean Ford, B.A., Workshop Speaker; Kaitlin Hamilton, B.S., Workshop Speaker
Track: Workshop

This session will highlight recent findings on the airway innate defense defects in CF, including immune, inflammatory and functional abnormalities that contribute to CF lung disease.

Learning Objectives:

  • Define alterations in monocyte and macrophage function in CF.
  • Describe the microanatomy of CF airways.
  • Examine the role of basal cell dysfunction and Resolvin in CF airway repair and inflammation.

Note: Speakers Hui Min Leung, Eszter Vladar and Domenico Mattoscio's presentations were not recorded at their request. 


Friday
W12--CFTR: *Novel Approaches to Modulate CFTR
02:00pm - 03:20pm Mountain - October 19, 2018
Marie E. Egan, M.D., Workshop Chair; Dusan Garic, M.Sc., Workshop Chair; Christina Barone, B.S., M.S., Workshop Speaker; Xiaoyan Gong, M.D., Workshop Speaker; Giulia Mancini, Ph.D., Workshop Speaker; Agnieszka Swiatecka-Urban, M.D., FASN, Workshop Speaker; Mark Turner, Ph.D., Workshop Speaker
Track: Workshop

This session will examine a number of novel pathways and approaches that alter CFTR expression or activity and may be potential therapeutic targets.

Learning Objectives:

  • Discuss the potential role of phosphodiesterase inhibition on mutant CFTR function. 
  • Identify unique cellular pathways that may rescue mutant CFTR. 
  • Describe unique molecules that may stabilize and/or rescue CFTR. 

Friday
W13 - W13--CLIN: Lung Transplantation in CF
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: Four Seasons 1
Alice Gray, M.D., Workshop Chair; David Sayah, M.D., Ph.D., Workshop Chair; Elisabeth Potts Dellon, M.D., M.P.H., Workshop Speaker; Andres Floto, M.D.,Ph.D.,FRCP, Workshop Speaker; Geneviève Mailhot, Ph.D., Workshop Speaker; Alyssa Perez, M.D., Workshop Speaker; Kathleen J. Ramos, M.D., M.Sc., Workshop Speaker
Track: Workshop

This session will present some of the latest data highlighting the unique considerations of lung transplant in the context of cystic fibrosis. The session will explore more precise ways of assessing who is at risk of needing a transplant, ways to optimize decision-making for those considering transplant, and what cystic fibrosis lung transplant recipients can expect after transplant. This session is designed to help CF care teams increase understanding of significant issues in lung transplant and better care for those preparing for or living life after lung transplantation.

Learning Objectives: 

  • Analyze new methods for assessing potential need for lung transplantation.
  • Summarize how systemic manifestations of cystic fibrosis affect transplant candidacy and outcomes.
  • Define how lung transplantation impacts quality of life in cystic fibrosis.

Friday
W14 - W14--CLIN: Endocrine
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: Four Seasons 4
Track: Workshop

Clinical and epidemiologic studies of cystic fibrosis related endocrine disease.

Learning Objectives:

  • Discuss data from large national registries regarding screening for diabetes and patterns of diabetes treatment.
  • Evaluate the risk factors for development of diabetes.
  • Evaluate the utility of novel assessments of bone health in predicting lung function.

Note: Speaker Quitterie Reynaud's presentation was not recorded at her request. 


Friday
W15 - W15--CLIN-NRS: Quality Improvement Innovations
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: Four Seasons 2
Michelle Prickett, M.D., Workshop Chair; Christopher M. Siracusa, M.D., Workshop Chair; Christina Baldwin, M.D., Workshop Speaker; David Josey Jr., M.D., Workshop Speaker; Thida Ong, M.D., Workshop Speaker; Michael Seid, Ph.D., Workshop Speaker; Joshua Wang, Pharm.D., Workshop Speaker
Track: Workshop

This session will highlight the varied work being done at pediatric and adult programs across North America. Work will be presented that showcases quality improvement projects that influence lung function, nutrition, co-production of care and patient/parent partners.

Learning Objectives:

  • Illustrate the work of pediatric and adult CF programs in quality improvement.
  • Discuss the use of quality improvement methods in improving metrics in nutrition, lung function and other factors affecting people with CF.
  • Apply quality improvement techniques at the local level.

Friday
W16 - W16--GMS: In Vitro Models of CF
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: 203
George M. Solomon, M.D., Workshop Chair; Mirza Ali, M.D., Workshop Speaker; Theo Moraes, M.D., Ph.D., Workshop Speaker; Aleksander Szymaniak, Ph.D., Workshop Speaker; Hillary Valley, Ph.D., Workshop Speaker
Track: Workshop

In vitro models have played a key role in the development of new therapeutics for CF. The gold standard model, human bronchial (HBE) epithelial cells is but one of the many available pre-clinical models that demonstrate pathogenesis and are being used to advance therapeutics. In this session, use of in vitro models will be described for clinical prediction of CFTR modulator response, developmental systems that model untreated mutations, and in the development of personalized pathobiology and therapies.

Learning Objectives:

  • Appraise the evolving landscape of cell-based model that aim to model human disease in the airway.
  • Define the relative advantage of each model for studying the cellular consequences of different mutations.
  • Define the relative advantage of each model for therapy development.

Friday
W17 - W17--INF/MIC: Epidemiology & Management of Infection
02:00pm - 03:35pm Mountain - October 19, 2018 | Room: 505
Jane E. Gross, M.D., Ph.D., Workshop Chair; Giovanni Taccetti, M.D., Workshop Chair; Ana Blanchard, M.D., C.M., Workshop Speaker; Christopher Goss, M.D., M.Sc., Workshop Speaker; John J. LiPuma, M.D., Workshop Speaker; Rebecca Stockwell, Workshop Speaker
Track: Workshop

This session highlights the importance of understanding the significance of epidemiologic impact of pulmonary infection with clinically relevant CF pathogens.

Learning Objectives:

  • Identify potential environmental sources of clinically relevant CF pathogens.
  • Describe how epidemiologic investigation can be used to evaluate acquisition of clinically relevant CF pathogens.
  • Apply epidemiologic approaches to investigation of highly related strains of clinically relevant CF pathogens.

Note: Speaker Ranjani Somayaji's presentation was not recorded at her request. 


Friday
W18 - W18--NRS: Unique Challenges of a CF Diagnosis Later in Life
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: Milehigh 4CDEF
Track: Workshop

This session will address issues affecting a unique part of the CF population: those diagnosed later in life. There are many issues exclusive to this population that present challenges to the patient and to the healthcare team. During this workshop we will discuss issues such as the impact of peer support, hear about important medical management concerns, learn about the impact and realities of Medicare & CF, as well as a unique opportunity to hear firsthand from a patient regarding their experience as an adult.

Learning Objectives:

  • Describe the evolving role of the CF provider as a PCP in the routine care of the Adult CF patient.
  • Describe the impact of social support on both the mental and physical health of Adult CF patients.
  • Identify challenges for Adults with Cystic Fibrosis who are on Medicare.

Friday
W19--NTR: *Nutrition Research
02:00pm - 03:20pm Mountain - October 19, 2018
Track: Workshop

This session will explore current topics in nutrition related to cystic fibrosis care. With new medications on the market, new research on the impact on nutrition needs to be examined. This session will also look at the limitations of current practices and discuss areas of opportunity for future nutrition research.

Learning Objectives:

  • Identify alternative measures for assessing nutritional status.
  • Explore effect of modulators on nutritional plans.
  • Discuss the inconsistencies in iron dosing practices.

Friday
W20 - W20--PT&RT: Novel Quality Improvement Initiatives
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: 207
Track: Workshop

This session will present five abstracts highlighting methodology used in Quality Improvement (QI) Initiatives in cystic fibrosis. The abstracts offer a variety of QI projects and include improving pulmonary function, nebulizer care, airway clearance, CF clinic flow and screening in CF clinic by PT for developmental delay. The initiatives are novel and the topics are of particular interest to the PT and RT caregiver.

Learning Objectives:

  • Describe and summarize methodology used in Quality Improvement Initiatives.
  • List current QI projects and their impact on CF care/outcomes.
  • Discuss the role RT and PT can play in QI projects.

Friday
W21 - W21--SW/PSYCH: Psychosocial Research Showcase
02:00pm - 03:20pm Mountain - October 19, 2018 | Room: 711
Beth Smith, M.D., Workshop Chair; Chelsea Toth, D.S.W., M.S.W., L.S.W., Workshop Chair; Deborah Friedman, Ph.D., Workshop Speaker; Dio Kavalieratos, Ph.D., Workshop Speaker; Jamie L. Miller, LICSW, M.S.W., Workshop Speaker; Susan Moore, M.S.W., LISW, Workshop Speaker; Christopher Richards, M.D., Workshop Speaker; Kristin A. Riekert, Ph.D., Workshop Speaker
Track: Workshop

This workshop focuses on new psychosocial research findings. There is a breadth of knowledge and diversity in the arena of psychosocial research for Cystic Fibrosis. This workshop will provide participants an understanding of: 1. the development of a CF-specific Cognitive Behavioral Therapy intervention to prevent depression and anxiety in adults with CF, 2. the prevalence of alcohol and opioid misuse among adults with CF, 3. barriers to adherence identified by adolescents and adults with CF utilizing the Barriers Assessment Screener, 4. implementation of primary palliative care skills for CF care team members, and 5. the implementation of automated psychosocial reports.

Learning Objectives:

  • Review the latest developments in psychosocial research for individuals living with CF.
  • Describe the data associated with psychosocial research in the fields of mental health and substance abuse.
  • Identify the benefits of psychosocial research and implementation of interventions for patients and their families.

Saturday
P3--*Partnering: The Oldest New Idea to Improve CF Care
09:00am - 10:00am Mountain - October 20, 2018
Maren Batalden, M.D., Plenary Leader; Melanie Abdelnour, Plenary Panelist; Cynthia George, R.N., M.S.N., F.N.P., Plenary Panelist; Kathryn Sabadosa, M.P.H., Plenary Panelist
Track: Plenary

Central to the management of cystic fibrosis are the relationships that people with CF and their families build with their clinical care teams. As people with CF live longer, their disease increases in complexity, making coordination and individualized care ever more dependent upon these foundational relationships. Actively partnering in health care is a learning process and although, highly regarded, realizing and maintaining full partnership can be challenging. This plenary will walk through the evolution of partnering, specifically in CF care and more broadly in the context of health care and society, further thinking by exploring the tensions impacting partnership, and highlight new strategies supported by the CF Foundation, so that all people living with CF can better balance their daily care while attaining personal goals. The plenary will end with a panel discussion between an adult living with CF, a parent, and a healthcare professional from the CF Foundation.

Learning Objectives:

  • Describe the evolution of partnerships in the context of society, health care and CF care.
  • Explore the tensions underlying the shift away from a traditional, clinician-driven approach toward a more collaborative partnership with patients.
  • Apply innovative ways to approach partnering in CF care.