Best Practices in the Treatment and Management of Cystic Fibrosis (CF): Recent Updates and Advances in CFTR Therapy

A continuing medical education activity provided by NAMCP and AAMCN

This activity is an archive from the live session from the 2020 Spring Managed Care Forum. If you participated in the live session, you are not eligible for continuing education credits from this archive.

This activity is valid from July 1, 2020 to July 1, 2021

Instructions for CME/NCPD: Complete the pre-test, listen to the audio and view the slides, complete the post test, complete the evaluation form and hit submit. You will be asked to enter your name and email address on the pre-test, evaluation and post-test. If you close your internet browser without completing the post test, you will have ONE more opportunity to complete. A score of 70% must be achieved on the post test to receive continuing education credits. If you do not pass the post test after two attempts, you will not be eligible to try again. Once you complete the evaluation form and score 70% or higher on your post test, you will automatically be given your certificate.

To print or save your certificate, you will need to click on the “download” button and either print or save.


Audience: This activity is intended for healthcare professionals practicing in managed care environments.

This activity is supported by an educational grant from
Vertex Pharmaceuticals

Cystic fibrosis (CF), also known as mucoviscidosis, is a genetic, autosomal recessive disorder that affects mostly the lungs but also the pancreas, liver, kidneys and intestine. It is caused by the presence of mutations in both copies of the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus. When CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Long-term issues include difficulty breathing and coughing up sputum as a result of frequent lung infections. Other symptoms include sinus infections, poor growth, fatty stool, clubbing of the finger and toes, and infertility in males among others. According to the Cystic Fibrosis Foundation, about 30,000 people are currently living with cystic fibrosis, and approximately 1,000 new cases of CF are diagnosed each year. There is no cure for cystic fibrosis, but recent treatment breakthroughs have the ability to provide a greater quality of life and improved outcomes for patients with CF, especially in combination therapy.

Upon completion of this activity, participants will be able to:

  • Compare and contrast current and emerging CFTR-based therapeutic options, including combination therapies, in terms of efficacy, safety, and clinical potential to improve pulmonary and non-pulmonary outcomes of patients with cystic fibrosis

  • Explore clinical trial data and the role of newer combination CFTR modulator therapies, including triple combination regimens, and discuss ways to integrate them into the evolving treatment paradigm

  • Identify cystic fibrosis patients who would potentially benefit from a CFTR modulator therapy

  • Describe challenges and opportunities in CF disease management, including patient adherence, quality of life and total cost of care

  • Examine the importance of newborn screening, accurate diagnosis, genomic evaluation, and early pharmacologic intervention in the management of cystic fibrosis

  • Assess the managed care considerations on CFTR modulator therapies, including combination options, and explore where these agents fit in the cystic fibrosis management paradigm


Faculty: Patrick A. Flume, MD, FCCP
Professor of Medicine and Pediatrics
Director, Adult Cystic Fibrosis Program
Powers-Huggins Endowed Chair for Cystic Fibrosis
Medical University of South Carolina


Dr. Flume serves as a consultant for Aureus Clinical, Corbus Pharmaceuticals, Eloxx Pharmaceuticals, Insmed, and Janssen. He has received grant/research support from AbbVie, Corbus Pharmaceuticals, the Cystic Fibrosis Foundation Therapeutics, Insmed, and the National Institute of Health. His presentation has been peer reviewed for any bias.
  Planning Committee:
Bill Williams, MD has no real or perceived financial relationships to disclose.
Jeremy Williams has no real or perceived financial relationships to disclose.
Jacqueline Cole, RN, MS, CMCN has no real or perceived financial relationships to disclose.

NAMCP and/or the presenter has copyright or has received permissions for use of materials provided in this activity.

Accreditation & Designation
This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of the National Association of Managed Care Physicians (NAMCP) and American Association of Managed Care Nurses (AAMCN). The National Association of Managed Care Physicians is accredited by the ACCME to provide continuing medical education for physicians.

NAMCP designates this enduring material for a maximum of 1 AMA PRA Category 1 credit(s)TM. Each
physician should claim credit commensurate with the extent of their participation in the activity.

The American Association of Managed Care Nurses is accredited as a provider of nursing continuing professional development by the American Nurses Credentialing Center's Commission on Accreditation.

Nurses who complete this activity and achieve a passing score will receive 1 hour in nursing continuing professional development.

This activity has been approved by the American Board of Managed Care Nursing for 1.0 contact hours toward CMCN recertification requirements.

This activity is supported by an educational grant from
Vertex Pharmaceuticals

NAMCP and/or this website does not provide medical advice, diagnosis or treatment. NAMCP does not endorse or imply endorsement of the content on any linked website. This website is to be used as an informational resource. With any health related concern, consult with your physician or healthcare professional.

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