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CME: Cystic fibrosis: Therapeutic updates and optimizing treatment


Activity Description / Statement of Need:

In this online, self-learning activity:

Cystic Fibrosis (CF) is a genetic disease that affects nearly 70,000 people worldwide with more than 90% of patients diagnosed of Caucasian descent and a median lifetime survival remains a mere 43.6 years. CF is caused by an autosomal recessive mutation in the CF transmembrane regulator (CFTR) gene, which controls the other chloride and sodium channels at the cell surface and is found in the lungs, liver, pancreas, intestine, sweat duct, and epididymis. The primary organs in which the disease manifests clinically are the pancreas, leading to malabsorption of nutrients, and the lungs due to the accumulation of thick, sticky mucous that contributes to airway obstruction. CF causes several clinical complications, including recurrent pulmonary infections, nasal polyps, CF-related diabetes, fat-soluble vitamin deficiencies, acid reflux, and liver failure.

Target Audience:

The following HCPs: pulmonologists, pediatricians, gastroenterologists and primary care physicians; physician assistants, nurse practitioners, nurses, and pharmacists who practice in the aforementioned areas of specialty; and any other healthcare professionals with an interest in or who clinically encounter patients with CF.

This program is supported by an educational grant from Vertex.

Release Date: July 22, 2020 -- Expiration Date: July 22, 2022

Faculty: Rebecca Dezube, M.D.


Faculty introduction, disclosures

Introduction content: Review of CF

  • Epidemiology: Statistics of CF by age and race and median survival
  • Causes of CF

            · Genetic changes in CFTR

  • Pathogenesis, clinical features, presentation
  • Diagnosis

Treatment of CF with a focus on emerging, new therapies

  • Brief review of treatment approaches
  • Treatment of gastrointestinal disease
  • Treatment of infectious complications and lung disease
  • Newer, approved therapies: CFTR modulators
  • Investigational therapy

           · Novel CFTR modulators

           · CB2 agonists

  • Best practice: Putting it all together
  • Patient case(s)

Summary, conclusions, and best practice recap

Learning Objectives

By the end of the session the participant will be able to:

  • Describe the pathophysiology of CF such that it might inform treatment mechanisms.
  • Identify the currently available and emerging pharmacotherapeutic treatments for the management of CF and apply them to patient cases using evidence-based medicine.
  • Describe newly approved and investigational therapies in development for CF.
  • Evaluate an ongoing treatment plan for a specific patient with CF to optimize safety and efficacy, suggesting modifications for improvement, including the management of complications.


ACCME Activity #201861272


Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review ScientiaCME.

Faculty Disclosure: Rebecca Dezube, MD MHS, Assistant Professor of Medicine, John Hopkins University School of Medicine, has received financial compensation for consulting work from Proteostasis.

Disclosures of Educational Planners: Charles Turck, PharmD, BCPS, BCCCP, CEO of ScientiaCME, has no relevant conflicts of interest to disclose.

Commercial Support Disclosure: This program is supported by an educational grant from Vertex.


  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowledge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Minimum participation threshold: Take the post-test. A score of 70% or higher is required to pass and proceed to the activity evaluation.
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural/Linguistic Competence & Health Disparities

System Requirements

Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version

Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.

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