Recent Advances in CFTR Modulator Therapy in the Management of Cystic Fibrosis: Best Practices for Improved Patient Outcomes

A continuing medical education activity sponsored by NAMCP and AAMCN.

This activity is an archive from the live session from the 2018 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 August 8, 2018 to August 8, 2019

Instructions for CME/CNE: 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.

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

This presentation is supported by an educational grant from
Vertex Pharmaceuticals

Cystic fibrosis (CF) is a genetic, autosomal recessive disorder that affect the lungs, pancreas, liver, kidneys and intestine. Long-term issues include difficulty breathing and coughing up sputum, sinus infections, poor growth, fatty stool, clubbing of the finger and toes, and infertility in males. CF is caused by the presence of mutations in both copies of the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR) which is involved in the production of sweat, digestive fluids, and mucus. The condition is diagnosed by a sweat test and genetic testing and screening of infants at birth takes place in some areas of the world. There is no cure for cystic fibrosis but fortunately a new treatment is available for those who have two copies of the F508del mutation in their CFTR gene (about half of patients). This new CFTR modulation therapeutic focuses on counteracting the damage caused by the disease, enabling patients to breath more easily and stay out of the hospital, providing a greater quality of life and improved outcomes.

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

  • Analyze approved 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

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

  • Discuss current challenges with medication and treatment adherence in the cystic fibrosis population

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

  • Develop optimal strategies for the CFTR modulated treated patient including accurate life-long assessment, monitoring, and management

  • Review and analyze the current and emerging clinical trial data on CFTR modulator therapeutics, including combination therapies, to develop effective individualized treatment plans

Faculty: Peter J. Mogayzel, Jr., MD, PhD, MBA
Professor of Pediatrics
Director, Cystic Fibrosis Center
Medical Director, Pediatric Specialty Clinic
Johns Hopkins Cystic Fibrosis Center


Dr. Mogayzel has received research/grant support from Vertex Pharmaceuticals and the Cystic Fibrosis Foundation. His presentation has been peer reviewed for any bias.
  Planning Committee:
Bill Williams, MD has no relevant financial relationships to disclose.
Jeremy Williams has no relevant financial relationships to disclose.
Jacqueline Cole, RN, MS, CMCN has no relevant 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
The National Association of Managed Care Physicians (NAMCP) is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

NAMCP designates this enduring material for a maximum of 1 AMA PRA Category I creditsTM.

The American Association of Managed Care Nurses is accredited as a provider of continuing nursing
education 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 continuing
nursing credit.

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

This presentation 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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