Improving Patient Outcomes in the
Treatment and Management of Cystic Fibrosis: What's New in CFTR
A continuing medical education activity sponsored by NAMCP and AAMCN.
This activity is an archive from the
live session from the 2019 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 June 1, 2019 to June 1, 2020
Instructions for CME/CNE: Complete the pre-test, listen to the audio
and view the slides, complete the post test, complete the evaluation
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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
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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
Cystic fibrosis (CF), also known as mucoviscidosis, is an autosomal
recessive, genetic disorder that affects mostly the lungs but also
the pancreas, liver, kidneys, and intestine. 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. 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. 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.
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. The degrees of
symptoms vary among patients and fortunately, for about half of CF
patients, new treatments have become available for those who have
two copies of the F508del mutation in their CFTR gene. This newer
CFTR modulation therapeutic focuses on counteracting the damage
caused by the disease, enabling patients to breath more easily and
stay out of the hospital, improving outcomes and quality of life.
This option is a major breakthrough in CF management, because it is
the first therapy that specifically targets an important genetic
mutation. Additionally, There has been new data presented in recent
months in the cystic fibrosis arena, especially as it relates to
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
Examine the role of newer
combination CFTR modulator therapies and discuss ways to
integrate them into the evolving treatment paradigm
Describe challenges and
opportunities in CF disease management, including quality of
life and total cost of care
Explore recent clinical trial data
on emerging triple combination therapies and where they may fit
in cystic fibrosis management
Analyze the importance of newborn
screening, accurate diagnosis, genomic evaluation, and early
pharmacologic intervention in the management of cystic fibrosis
Assess the managed care
considerations of CFTR modulator therapies by exploring where
these agents fit into an evolving cystic fibrosis management
||Gregory Sawicki, MD, MPH
Director, Cystic Fibrosis Center
Boston Children's Hospital
Assistant Professor of Pediatrics
Harvard Medical School
serves on an advisory board for Gilead and Vertex. His
presentation has been peer reviewed for any bias.
MD has no relevant financial relationships to disclose.
Jeremy Williams has no relevant financial relationships
Will Williams has no relevant financial relationships to
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 (ACCME) to provide continuing medical education for
NAMCP designates this enduring material for a maximum of 1 AMA PRA
Category I creditsTM.
The American Association of Managed Care Nurses (AAMCN) is
accredited as a provider of continuing nursing education by the
American Nurses Credentialing Center’s Commission on Accreditation (ANCC).
Nurses who complete this activity and achieve a passing score will
receive 1 hour in continuing
This activity has been approved by the American Board of Managed
Care Nursing for 1.0 contact hour toward CMCN recertification
This presentation is supported by
an educational grant from
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
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