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Disclaimer: CME certification for these activities has expired. All information is pertinent to the timeframe in which it was released.


Optimizing Patient Outcomes: Clinical Use of the Cystic Fibrosis Pulmonary Guidelines


The severity of cystic fibrosis (CF) varies greatly from person to person and is determined largely by the degree in which the lungs are affected. Expression of CF transmembrane conductance regulator leads to abnormal ion conductance, resulting in dehydrated airway secretions and chronic, progressive airway infections, pancreatic insufficiency, nutrition absorption problems, hepatobiliary abnormalities, male infertility, female reproductive challenges, and eventually death. Colonization with pseudomonas aeruginosa and other organisms leads to impaired mucocilliary clearance and host defense mechanisms. The ensuing chronic infection and inflammation leads to bronchiectasis and progressive loss of lung function. Improvements in survival are associated with early diagnosis, specialty care, and primary treatments that can now postpone some of the damaging changes occurring in the lungs.

Goal
This educational activity will provide physicians with an update on the clinical use of cystic
fibrosis (CF) therapies as recommended in the CF Pulmonary Guidelines to optimize
patient care and develop strategies for improving treatment adherence and compliance for
emerging adults (aged 18-25 years old).

Intended Audience
This activity is designed for pediatric pulmonologists, pulmonologists, geneticists, and
primary care physicians. No prerequisites required.

Learning Objectives
Upon the conclusion of this activity, the participant should be able to:

  • Evaluate current and emerging therapies and the delivery mechanism of these therapies.
  • Recognize and identify adherence issues in patients with cystic fibrosis.
    The Johns Hopkins University School of Medicine takes responsibility for the content,
    quality, and scientific integrity of this CME activity.

Accreditation Statement
The Johns Hopkins University School of Medicine is accredited by the Accreditation Council
for Continuing Medical Education to provide continuing medical education for physicians.

Credit Designation Statement
The Johns Hopkins University School of Medicine designates this educational activity for
a maximum of 1 AMA PRA Category 1 Credit(s)TM. Physicians should only claim credit
commensurate with the extent of their participation in the activity.

Policy on Faculty and Provider Disclosure
It is the policy of The Johns Hopkins University School of Medicine that the faculty and provider disclose real or apparent conflicts of interest relating to the topics of this educational activity, and also disclose discussions of unlabeled/unapproved uses of drugs or devices during their presentation(s). The Johns Hopkins University School of Medicine OCME has established policies in place that will identify and resolve all conflicts of interest prior to this educational activity. Detailed disclosure will be made during the course of the activity.

Michael P. Boyle, MD, FCCP
Associate Professor of Medicine
Director, The Johns Hopkins Adult Cystic Fibrosis Program
The Johns Hopkins University School of Medicine
Baltimore, MD

John Paul Clancy, MD
Professor, Director, and Raymond K. Lyrene
Chair in Pediatric Pulmonology
The Children’s Hospital of Alabama
University of Alabama at Birmingham
Birmingham, AL

Kristin A. Riekert, PhD
Assistant Professor
Co-Director, The Johns Hopkins Adherence Research Center
Division of Pulmonary and Critical Care Medicine
The Johns Hopkins University
Baltimore, MD





Johns Hopkins Advanced Studies in Medicine (ISSN-1558-0334), is published by Galen Publishing, LLC, d/b/a ASiM, PO Box 340, Somerville, NJ 08876. (908) 253-9001. Copyright ©2012 by Galen Publishing. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, without first obtaining permission from the publisher. ASiM is a registered trademark of The Healthcare Media Group, LLC.