31st Annual North American Cystic Fibrosis Conference

Nov 2, 2017 ‐ Nov 4, 2017


The North American Cystic Fibrosis Conference (NACFC) provides a collaborative and educational forum for CF professionals to help advance CF research and care. This annual meeting brings together scientists, clinicians, and caregivers from around the world to discuss and share ideas on the latest advances in CF research, care, and drug development and to exchange ideas about ways to improve the health and quality of life for people with CF.


Sessions

S07: SW/PSYCH: Coping With the Invisible Disease

Nov 2, 2017 2:00pm ‐ Nov 2, 2017 3:25pm

Identification: S07

Cystic Fibrosis has sometimes been called an 'invisible illness' because signs or symptoms of the disease may not always be outwardly apparent. This symposium will discuss some of the challenges that accompany living with an invisible illness, including navigating disclosure of illness status, normalizing the experience, and managing a common 'invisible' symptom - CF-related pain. The symposium will also provide information about the development of a CF-specific Community Partnership program to support individuals in coping with these challenges. Participants will deepen their understanding of living with an invisible illness, and utilize this knowledge to identify practical strategies to help patients and their families.

Learning Objectives:

  1. Discuss the challenges of living with an 'invisible illness' and normalizing the experience.
  2. Describe research on topics related to managing 'invisible' aspects of the disease, including disclosure of illness status and CF-related pain.
  3. Identify current strategies utilized in the development of a CF-specific community program to support individuals in coping with these challenges.

Symposium Chair(s): Symposium Speaker(s):

S08: APP&D: New Treatment Strategies Focused on Abnormal Mucus

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S08

This symposium aims to provide insights into the abnormal properties of CF mucus and explore different therapeutic approaches for CF lung disease. New data from recent clinical studies offer unique perspectives on the pathological changes in CF mucus related to hydration, pH and viscoelasticity. This symposium will focus on basic findings and recent efforts towards translating these advances into novel therapies.

Learning Objectives:

  1. Identify the basic defects of CF mucus responsible for impaired mucociliary clearance.
  2. Discuss the different therapeutic approaches that address mucus clearance defects in CF.
  3. Evaluate drug efficacy and tolerability tested in experimental models and in CF patients.

Symposium Chair(s): Symposium Speaker(s):
  • Jane Davies, M.D., MBChB, MRCP, Prof - Paed Respirology & Experimental Medicine
  • John Fahy, M.Sc., M.D., UCSF Medical Center
  • Marcus Mall, M.D., Professor
  • David Stoltz, M.D., Ph.D., Professor, Univ. of Iowa Hospital & Clinics

S09: CFTR: CFTR's Neighborhood: It's All About Location

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S09

Ion and fluid transport mediated by CFTR does not happen in isolation; it happens as a multi-protein complex on the plasma membrane. CFTR is activated by its phosphorylation by various kinases (e.g., cAMP-/cGMP-activated protein kinases), by interacting with these kinases. It has been demonstrated that other proteins also regulate CFTR activity such as other ion channels, transporters, and scaffolding proteins. Since very little is known about the CFTR-interacting partners and the role they play in coupling CFTR to other regulatory or ion-conducting proteins in CFTR's neighborhood, the theme of this symposium will be 'CFTR's Neighborhood: It's All About Location'.

Learning Objectives:

  1. Define how CFTR and its interacting partners behave in microdomains of the plasma membrane.
  2. Define how CFTR is regulated by secondary messengers at or near the plasma membrane.
  3. Define how airway pH is regulated by CFTR interacting partner ATP12A and how SLC26A9 function is regulated by the mutant CFTR protein.

Symposium Chair(s): Symposium Speaker(s):

S10: CLIN-INF/MIC: Beyond ABPA: Understanding the Fungal Jungle in CF

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S10

The symposium will review the current epidemiology of fungal disease in cystic fibrosis, explore current important factors in fungal virulence and innate immune responses. The role of antimicrobial stewardship and therapeutic options will be outlined.

Learning Objectives:

  1. Distinguish the syndromes that form the scope of fungal disease in CF.
  2. Identify the complex fungal virulence factors and pulmonary host defense system responses.
  3. Select amongst treatment options for various fungal disease manifestations.

Symposium Chair(s):
  • J. Stuart Elborn, M.D., FRCP, Professor of Respiratory Medicine
  • Richard B Moss, M.D., Professor Emeritus, Pediatrics, Stanford University Medical Center
Symposium Speaker(s):

S11: CLIN: Medications, CF & the Gastrointestinal Tract

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S11

This session will provide an overview of the use of probiotics, acid suppression and pancreatic enzyme replacement therapy in cystic fibrosis. In addition, drug-induced liver injury and the management of elevated liver enzymes with commonly used cystic fibrosis medications will be discussed.

Learning Objectives:

  1. Define drug-induced liver injury, list commonly implicated agents in cystic fibrosis and discuss management of elevated liver enzymes with commonly used cystic fibrosis medications.
  2. Review use of acid suppression in cystic fibrosis, discuss indications for use and use of alternative agents, and appraise the risks vs. benefits of long-term acid suppression.
  3. Describe the pharmacology of pancreatic enzyme replacement therapy and probiotics and appraise the evidence regarding their use for the gastrointestinal manifestations of cystic fibrosis.

Symposium Chair(s): Symposium Speaker(s):

S12: INF/MIC: Microbial Interactions

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S12

CF lung infections are complex and dynamic communities of bacteria, fungi, and viruses that interact with each other and with the host. The purpose of this symposium is to explore important inter-species and niche interactions that will improve our understanding of airway infection in CF and explore new targets for antimicrobial agents and antimicrobial adjuvants.

Learning Objectives:

  1. Review the current understanding of microbial interactions in CF lung infections.
  2. Describe important bacterial, fungal, viral, and host interactions in the CF lung environment.
  3. Identify opportunities for therapeutic intervention to treat or manage CF lung infections.

Symposium Chair(s): Symposium Speaker(s):
  • Jane Davies, M.D., MBChB, MRCP, Prof - Paed Respirology & Experimental Medicine
  • Lucas Hoffman, M.D., Ph.D., Associate Professor
  • Deborah A. Hogan, Ph.D., Dartmouth College
  • Marvin Whiteley, Ph.D., EPIC Clinical Study Reception, Georgia Institute of Technology/Emory Medical School

S13: NRS: Non Pharmacologic Management of Pain in CF

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S13

Pain in CF is common and reported to affect quality of life and survival (Havermans, 2013). Furthermore, treatment of pain in CF may be challenging and complicated by concerns regarding comorbidities, adverse effects of medications, implications for transplant, and influence of depression and anxiety. This symposium will discuss integration of opioids within the pain management regimen, as a bridge to non-pharmacological treatments, including when to wean or when to add opioid therapy. A comprehensive overview of non-pharmacologic management of pain in CF will also be presented. The session also explores the co-occurrence of depression, anxiety and PTSD with chronic pain and psychological treatment of chronic pain. Finally, an individual with CF will describe their experience with pain. Participants will deepen their understanding of management of pain in CF and utilize this knowledge to identify opportunities to integrate non-pharmacologic strategies in the treatment regimen.

Learning Objectives:

  1. Discuss the decision points for choosing pharmacologic vs non-pharmacologic interventions.
  2. Describe the experience of pain in CF from a patient's perspective.
  3. Discuss the co-occurrence of depression and anxiety with pain and psychological treatment for pain in CF.

Symposium Chair(s): Symposium Speaker(s):

S14: NT: CF Interventions Advancing Through the Clinical Testing Phase

Nov 3, 2017 10:30am ‐ Nov 3, 2017 11:55am

Identification: S14

This symposium will review some of the latest trials of CF therapies including both CFTR modulators and anti-inflammatory therapy. Clinical trail data will be presented and put into context with previous work in the field as well as an outlook where the field will be moving towards in the near future.

Learning Objectives:

  1. Define the latest developments in CF therapies.
  2. Express the challenges for clinical trials addressing specific aspects of the disease.
  3. Define how we learn from both successful trials and those that have failed.

Symposium Chair(s): Symposium Speaker(s):

S15: APP&D: Inflammation, Infection & CFTR: Breaking the Vicious Cycle

Nov 4, 2017 2:30pm ‐ Nov 4, 2017 3:55pm

Identification: S15

Emerging evidence suggests that several virulence factors and proteases released from Pseudomonas aeruginosa and inflammatory cells in CF airways have profound adverse effects on CFTR & ENaC function and may thus impede benefits of CFTR modulator therapies. On the other hand, restoration of mutant CFTR function may improve airway defenses and reduce inflammation and infection in CF. This symposium will review and integrate the latest data in this field, allowing the participant to better understand the interrelation between CF ion transport defects, infection & inflammation and potential implications for CFTR modulator therapies as well as strategies targeting alternative targets such as ENaC

Learning Objectives:

  1. Describe unwanted effects of proteases released in CF airways' infection & inflammation on CFTR & ENaC and discuss consequences for CF ion transport defects.
  2. Describe impact of Pseudomonas aeruginosa infection on wild-type CFTR function and on pharmacological rescue of F508del-CFTR.
  3. Summarize latest data on effects of CFTR modulator therapy on infection & inflammation in CF airways.

Symposium Chair(s): Symposium Speaker(s):

S16: CLIN: CF-Related Diabetes & CF-Related Bone Disease

Nov 4, 2017 2:30pm ‐ Nov 4, 2017 3:55pm

Identification: S16

This symposium will address two major endocrine complications of cystic fibrosis, specifically CF-related diabetes (CFRD) and CF-related bone disease (CFBD). The emphasis will be on diagnostic and therapeutic approaches.

Learning Objectives:

  1. Evaluate the available modalities for CFRD screening.
  2. Describe the management strategies for CFRD.
  3. Describe the therapeutic options for CFBD.

Symposium Chair(s): Symposium Speaker(s):
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