Pilewski J, Higgins M, Cooke J, et al. A phase 3 extension study evaluating the safety and efficacy of long-term ivacaftor (IVA) in patients with cystic fibrosis (CF) and phenotypic or molecular evidence of residual CFTR function. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 632.
Fidler MC, Sullivan JC, Boj SF, et al. Evaluation of the contributions of splicing and gating defects to dysfunction of G970R-CFTR. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 817.
Ratjen F, Tian S, Marigowda G, et al. Efficacy and safety of lumacaftor/ivacaftor (LUM/IVA) in patients (pts) aged 6-11 years (yrs) with cystic fibrosis (CF) homozygous for F508del-CFTR: a randomised placebo (PBO)-controlled phase 3 trial. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 601.
Davies J, Bell S, van Koningsbruggen-Rietschel S, et al. GLPG1837 in subjects with cystic fibrosis (CF) and the G551D mutation: results from a phase II study (SAPHIRA1). Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 585.
Romanowska E, Dmenska H, Semczuk K, et al, Five year observational study of fungal colonisation in CF paediatric patients. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 972.
Carrasco Hernández L, Quintana Gallego E, Delgado Pecellín I, et al. Fungal microbiota in patients with cystic fibrosis. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 613.
Felton IC, James P, Benson S, et al. The distinct airways mycobiome in adult cystic fibrosis and bronchiectasis. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 900.
Bricca R, Cavalli Z, Durupt S, et al. Risk of non tuberculous mycobacteria isolation according to different risk factors in adult CF patients: a systematic review and meta-analysis. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 367.
Gooch J, Turnbull L, Fullwood C, et al. Non-tuberculous mycobacteria infection in children with cystic fibrosis -- experience from a large tertiary centre in the United Kingdom. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 899.
Moran A, Dunitz J, Nathan B, et al. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009;32:1626-1631. Abstract
Bellin MD, Laguna T, Leschyshyn J, et al. Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study. Pediatr Diabetes. 2013;14:417-421. Abstract
Zangen D. Changes in glucose metabolism in the presence of CFTR modifiers. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Symposium S08.
Davies JC, Cunningham S, Harris WT, et al. Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. Lancet Respir Med. 2016;4:107-115. Abstract
Borowitz D. Management of pancreatic insufficiency in the presence of CFTR modifiers. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Symposium S08.
Prosser A, Speight L, Byrne A, et al. A retrospective review of deaths of our patients with CF under the care of the All Wales Adult CF Centre (AWACFC) from 1999 to 2016. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 494.
Eschenhagen P, Albrecht N, Staab D, et al. End-of-life-issues -- a single-centre retrospective analysis. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 852.
McIntosh L, MacDonald K. Supporting end-of-life care in CF through the introduction of a collaborative palliative care support group. Presented at 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 331.
Faulkner R, Reid N, Brennan A, et al. An evaluation of CF team views and experiences of end of life "debrief" sessions. Presented at: 40th European Cystic Fibrosis Conference; June 7-10, 2017; Seville, Spain. Abstract 723.

CME / ABIM MOC / CE

Advances in Cystic Fibrosis Management: Perspectives From Seville, Spain, 2017

Authors: J. Stuart Elborn, MD; Kris De Boeck, MD, PhD; Patrick A. Flume, MD; Marcus A. Mall, MD; Susan Madge, PhD, RN, RSCN; Natalie E. West, MD, MHS
CME / ABIM MOC / CE Released: 7/25/2017
THIS ACTIVITY HAS EXPIRED FOR CREDIT
Valid for credit through: 7/25/2018

Start Activity

Target Audience and Goal Statement

This activity is intended for pulmonologists, pediatricians, nurses, and gastroenterologists.

The goal of this activity is to provide CF clinicians with insight on new data related to CF management.

Upon completion of this activity, participants will:

have increased knowledge regarding the
- Latest clinical data on agents targeting pulmonary and nonpulmonary symptoms of cystic fibrosis (CF)
- Potential impact of clinical advances in CF on treatment options and strategies

Disclosures

As an organization accredited by the ACCME, Medscape, LLC, requires everyone who is in a position to control the content of an education activity to disclose all relevant financial relationships with any commercial interest. The ACCME defines "relevant financial relationships" as financial relationships in any amount, occurring within the past 12 months, including financial relationships of a spouse or life partner, that could create a conflict of interest.

Medscape, LLC, encourages Authors to identify investigational products or off-label uses of products regulated by the US Food and Drug Administration, at first mention and where appropriate in the content.

Moderator

J. Stuart Elborn, MD

Clinical Chair in Respiratory Medicine; Director, Center for Specialist Adult Cystic Fibrosis

Disclosures

Disclosure: Royal Brompton Hospital, Imperial College London, London, United Kingdom J. Stuart Elborn, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Gilead Sciences, Inc.; Galápagos NV; Novartis Pharmaceuticals Corporation; Vertex Pharmaceuticals Incorporated
Served as a speaker or a member of a speakers bureau for: Gilead Sciences, Inc.; Novartis Pharmaceuticals Corporation; Vertex Pharmaceuticals Incorporated
Received grants for clinical research from: Novartis Pharmaceuticals Corporation

Dr Elborn does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Elborn does intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Faculty

Kris De Boeck, MD, PhD

Clinic Chair, Pediatric Pulmonology, University of Leuven, Belgium

Disclosures

Disclosure: Kris De Boeck, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Boehringer Ingelheim Pharmaceuticals, Inc.; Galapogos NV; Proteostasis Therapeutics, Inc.; Vertex Pharmaceuticals Incorporated Pharmaceuticals Incorporated
Served as a speaker or a member of a speakers bureau for: PTC Therapeutics.

Dr De Boeck does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr De Boeck does intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Marcus A. Mall, MD

Professor of Pediatrics; Director, Division of Pediatric Pulmonology and CF Center, University of Heidelberg, Heidelberg, Germany

Disclosures

Disclosure: Marcus A Mall, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Arrowhead Pharmaceuticals; Bayer HealthCare Pharmaceuticals; Boehringer Ingelheim Pharmaceuticals, Inc.; ProQR Therapeutics; PTC Therapeutics; Spyrix Biosciences; Vertex Pharmaceuticals Incorporated
Served as a speaker or a member of a speakers bureau for: PARI GmbH; Vertex Pharmaceuticals Incorporated

Dr Mall does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Mall does intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Patrick A. Flume, MD

Professor of Medicine and Pediatrics; Director, Cystic Fibrosis Center, Medical University of South Carolina, Charleston, South Carolina

Disclosures

Disclosure: Patrick A. Flume, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Bayer HealthCare Pharmaceuticals; Gilead Sciences, Inc.; Insmed Incorporated; Vertex Pharmaceuticals Incorporated
Received grants for clinical research from: Bayer HealthCare Pharmaceuticals; Corbus Pharmaceuticals; Cystic Fibrosis Foundation Therapeutics; Gilead Sciences, Inc.

Dr Flume does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Flume does intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Natalie E. West, MD, MHS

Assistant Professor of Medicine; Assistant Program Director, Pulmonary and Critical Care Fellowship, Johns Hopkins University Pulmonary and Critical Care Medicine, Baltimore, Maryland

Disclosures

Disclosure: Natalie E West, MD, MHS, has disclosed the following relevant financial relationships:
Served as a speaker or a member of a speakers bureau for: Genentech, Inc.; Gilead Sciences, Inc.; Novartis Pharmaceuticals Corporation

Dr West does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr West does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Susan Madge, PhD, RN, RSCN

Doctor, Royal Brompton Hospital, Imperial College, London, United Kingdom

Disclosures

Disclosure: Susan Madge, PhD, RN, RSCN, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Gilead Sciences, Inc.; Vertex Pharmaceuticals Incorporated
Served as a speaker or a member of a speakers bureau for: Gilead Sciences, Inc.

Dr Madge does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Madge does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Editor

Julia Muino

Scientific Director, Medscape, LLC

Disclosures

Disclosure: Julia Muino has disclosed no relevant financial relationships.

CME Reviewer/Nurse Planner

Amy Bernard, MS, BSN, RN-BC

Lead Nurse Planner, Medscape, LLC

Disclosures

Disclosure: Amy Bernard, MS, BSN, RN-BC, has disclosed no relevant financial relationships.

Peer Reviewer

This activity has been peer reviewed and the reviewer has disclosed no relevant financial relationships.

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Medscape, LLC designates this enduring material for a maximum of 0.75 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Medscape, LLC staff have disclosed that they have no relevant financial relationships.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 0.75 MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program. Participants will earn MOC points equivalent to the amount of CME credits claimed for the activity. It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit. Aggregate participant data will be shared with commercial supporters of this activity.

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This activity is designed to be completed within the time designated on the title page; physicians should claim only those credits that reflect the time actually spent in the activity. To successfully earn credit, participants must complete the activity online during the valid credit period that is noted on the title page. To receive AMA PRA Category 1 Credit™, you must receive a minimum score of 70% on the post-test.

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From Medscape Education Pulmonary Medicine

CME / ABIM MOC / CE

Advances in Cystic Fibrosis Management: Perspectives From Seville, Spain, 2017

Authors: J. Stuart Elborn, MD; Kris De Boeck, MD, PhD; Patrick A. Flume, MD; Marcus A. Mall, MD; Susan Madge, PhD, RN, RSCN; Natalie E. West, MD, MHSFaculty and Disclosures

THIS ACTIVITY HAS EXPIRED FOR CREDIT

CME / ABIM MOC / CE Released: 7/25/2017

Valid for credit through: 7/25/2018

processing....

Contents of This CME Activity

All sections of this activity are required for credit.

Section 1. Update on Mutations and Residual Function

A summary of key results on mutation-targeting therapies.
Kris De Boeck, MD, PhD; J. Stuart Elborn, MD

Section 2. New Research on CFTR Modulator Therapies

Findings on current agents and those in development.
Marcus A. Mall, MD

Section 3. Fungal and NTM Infection in CF

Insights into research on aspects of fungal infection and NTM in patients with CF.
Patrick A. Flume, MD; J. Stuart Elborn, MD

Section 4. Considering the Pancreatic Effects of CFTR Modulators

What effect do CFTR modulators have on other CF medications?
Natalie E. West, MD, MHS; J. Stuart Elborn, MD

Section 5. Advanced Care Planning and End-of-Life Care

Research on approaches to end-of-life care.
Susan Madge, PhD, RN, RSCN; J. Stuart Elborn, MD

Page 1 of 6

Update on Mutations and Residual Function

CME / ABIM MOC / CE Information Download Slides

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Disclaimer

The educational activity presented above may involve simulated case-based scenarios. The patients depicted in these scenarios are fictitious and no association with any actual patient is intended or should be inferred.

The material presented here does not necessarily reflect the views of Medscape, LLC, or companies that support educational programming on medscape.org. These materials may discuss therapeutic products that have not been approved by the US Food and Drug Administration and off-label uses of approved products. A qualified healthcare professional should be consulted before using any therapeutic product discussed. Readers should verify all information and data before treating patients or employing any therapies described in this educational activity.

References

Faculty and Disclosures

Moderator

J. Stuart Elborn, MD

Faculty

Kris De Boeck, MD, PhD

Marcus A. Mall, MD

Patrick A. Flume, MD

Natalie E. West, MD, MHS

Susan Madge, PhD, RN, RSCN

Editor

Julia Muino

CME Reviewer/Nurse Planner

Amy Bernard, MS, BSN, RN-BC

Peer Reviewer

CME / ABIM MOC / CE

Advances in Cystic Fibrosis Management: Perspectives From Seville, Spain, 2017

Target Audience and Goal Statement

Disclosures

Moderator

J. Stuart Elborn, MD

Faculty

Kris De Boeck, MD, PhD

Marcus A. Mall, MD

Patrick A. Flume, MD

Natalie E. West, MD, MHS

Susan Madge, PhD, RN, RSCN

Editor

Julia Muino

CME Reviewer/Nurse Planner

Amy Bernard, MS, BSN, RN-BC

Peer Reviewer

Accreditation Statements

For Physicians

For Nurses

Instructions for Participation and Credit

Advances in Cystic Fibrosis Management: Perspectives From Seville, Spain, 2017

Contents of This CME Activity

Section 1. Update on Mutations and Residual Function

Section 2. New Research on CFTR Modulator Therapies

Section 3. Fungal and NTM Infection in CF

Section 4. Considering the Pancreatic Effects of CFTR Modulators

Section 5. Advanced Care Planning and End-of-Life Care

Expert Commentary