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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


Goal
To provide pediatric pulmonologists, pulmonologists, geneticists, and primary care physicians with up-to-date information on the treatment and management of emerging adult patients with cystic fibrosis.

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 2 AMA PRA Category 1 Credit(s)TM. Physicians should only claim credit commensurate with the extent of their participation in the activity.

The estimated time to complete this educational activity: 2 hours.

After reading this monograph, participants may receive credit by completing the CME test, evaluation, and receiving a score of 70% or higher.

Release date: March 15, 2009. Expiration date: March 15, 2011.

Disclaimer Statement
The opinions and recommendations expressed by faculty and other experts whose input is included in this activity are their own. This enduring material is produced for educational purposes only. Use of The Johns Hopkins University School of Medicine name implies review of educational format, design, and approach. Please review the complete prescribing information of specific drugs or combinations of drugs, including indications, contraindications, warnings, and adverse effects, before administering pharmacologic therapy to patients.

This activity is supported by an educational grant from Novartis Pharmaceuticals Corporation.

Johns Hopkins Advanced Studies in Medicine (ISSN-1530-3004) is published by Galen Publishing, LLC, d/b/a Advanced Studies in Medicine, an HMG Company, PO Box 340, Somerville, NJ 08876. (908) 253-9001. Copyright ©2009 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. Advanced Studies in Medicine is a registered trademark of The Healthcare Media Group, LLC.

Full Disclosure Policy Affecting CME Activities:
As a provider accredited by the Accreditation Council for Continuing Medical Education (ACCME), it is the policy of The Johns Hopkins University School of Medicine to require the disclosure of the existence of any relevant financial interest or any other relationship a faculty member or a sponsor has with the manufacturer(s) of any commercial product(s) discussed in an educational presentation. The Course Director and Participating Faculty reported the following:

COURSE DIRECTOR

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

Dr Boyle reports having no relevant financial or advisory relationships with corporate organizations related to this activity.

PARTICIPATING FACULTY

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

Dr Clancy reports having no relevant financial or advisory relationships with corporate organizations related to this activity.

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

Dr Riekert reports receiving grants/research support from Genentech, Inc and Novartis Pharmaceuticals Corporation.

Notice: The audience is advised that no faculty member in this CME activity references unlabeled or unapproved uses of drugs or devices.

Johns Hopkins Advanced Studies in Medicine provides disclosure information from contributing authors, lead presenters, and participating faculty. Johns Hopkins Advanced Studies in Medicine does not provide disclosure information from authors of abstracts and poster presentations. The reader shall be advised that these contributors may or may not maintain financial relationships with pharmaceutical companies.

Optimizing Patient Outcomes: Clinical Use of the Cystic Fibrosis Pulmonary Guidelines
Michael P. Boyle, MD, FCCP*

"Woe is the child kissed on the forehead who tastes salty, for it is cursed and soon must die." With much time having passed since this well-known excerpt from German literature echoed the earliest prognosis related to cystic fibrosis (CF), clinicians and researchers have achieved what is perhaps the most relevant of all milestones: more birthdays. In contrast to just a few decades ago when the outlook for children with CF was often regarded as hopeless, today's patients with CF can be expected to reach adulthood, surviving to nearly 40 years of age. Although this disease remains life shortening, newly introduced therapies (eg, mucolytics and inhaled antibiotics) and aggressive management of CF lung disease have resulted in significant improvements in length and quality of life. Reports describing the benefits of antibiotics in CF surfaced as early as the 1940s, when oral sulfonamides and nebulized penicillin were shown to be effective against what was then considered the main pathogen (Staphylococcus aureus). Since then, the role of intravenous and inhaled antibiotics in suppressing Pseudomonas aeruginosa infection has been recognized as having a tremendous impact on patient outcomes.

Institution of aggressive Pseudomonas eradication strategies targeting patients from the beginning of life may aid in the future emergence of a young adult CF population in which chronic infection with P aeruginosa is not so prevalent. Such a group would have a favorable pulmonary prognosis, as it is known that infection with mucoid Pseudomonas is associated with worse pulmonary outcomes. Indeed, Pseudomonas infections exhibiting a mucoid and/or biofilm phenotype represent a growing challenge in CF because bacteria in a biofilm state exhibit increased resistance to antibiotics and host defense factors. As a result, antibiotic concentrations in the airways may be insufficient in eradicating biofilm infections, allowing the bacteria to persist and cause a gradual loss of lung function.

Because lung disease accounts for more than 85% of mortality in CF, pulmonary care is clearly the main focus of current therapy, with inhaled treatments a key component of recently released CF pulmonary guidelines.1 Specifically, agents that aid in mucociliary clearance (dornase alfa) and antimicrobials targeting P aeruginosa (inhaled tobramycin) are mainstays in the management of CF lung disease, and are backed by "good" quality of evidence substantiating benefits in moderate-to-severe disease.1 Comprehensive CF treatment centers offer most patients the best means of maintaining good overall health, provided that they follow the treatment regimen advised. Unfortunately, adherence with treatment is becoming increasingly difficult, ironically as a result of progress. With expanding treatment options, medical regimens have become complex and burdensome in regard to the required time, money, and health resources. And, as more patients with CF survive longer and reach adulthood, they strive to lead independent, unrestricted lives that do not always leave room for time-consuming treatments. It is not uncommon for individuals in the turbulent stages of emerging adulthood to dismiss progressive symptoms and skip clinic visits. Left undermanaged, these individuals may spiral into progressive pulmonary and nutritional deterioration and develop a state of chronic lung disease where infection and inflammation lead to more rapid pulmonary decline. It is thus critical for clinicians to provide continuity of care as these patients age and to place emphasis on both clinical and adherence management.

This issue of Johns Hopkins Advanced Studies in Medicine is dedicated to providing physicians with an update on the clinical use of CF therapies (as recommended in the CF pulmonary guidelines) and strategies for optimizing adherence among patients transitioning into adulthood.

John Paul Clancy, MD, reviews current, evidence-based treatment strategies, with a focus on recommendations from recently developed CF pulmonary guidelines and emerging trends in Pseudomonas infections. In his discussion on the natural course of Pseudomonas infection in CF, Dr Clancy, from The Children's Hospital of Alabama and University of Alabama at Birmingham, highlights the importance of early, aggressive therapy to optimize and maintain lung function. He also underscores the critical need to monitor for and eradicate P aeruginosa infections in young adult patients with CF who are not chronically infected with this organism. In addition, Dr Clancy discusses future strategies, including new classes of nebulized antibiotics, dry powder inhalation delivery systems, and use of antibiotics based on biofilm susceptibility testing.

Kristin Riekert, PhD, explores the various factors that are known to impact adherence, focusing on the contribution of emerging adulthood on the major nonadherence typologies (erratic adherence, unwitting nonadherence, and intelligent nonadherence). Patients who fall into one of these nonadherence categories have a higher risk of nonadherence as they enter adulthood because a major aspect of social support (parental involvement) begins to diminish. Dr Riekert, who is from the Johns Hopkins Adherence Research Center, emphasizes that although it is critical to understand and utilize treatment guidelines in clinical practice, it is essential to think about the type of counseling strategies that are the right fit for convincing patients to follow these guidelines. Dr Riekert discusses reliable ways of measuring adherence (eg, medication refill history and patient reports of nonadherence) and utilizing treatment strategies specifically for the type of nonadherence.

Finally, we present a case study of a young man who considers himself to have "mild" CF and has become increasingly dismissive of progressive symptoms and the need to aggressively manage his condition. The case reviews the patient's clinical history and reviews the findings contributing to his decreasing adherence. We finally discuss the strategy utilized to both address his declining pulmonary function and his particular nonadherence typology.

This case highlights that combining knowledge of current treatment guidelines with strategies for optimizing patient adherence offers the best possibility of improving patient outcomes in the steadily growing population of adolescents and emerging adults with CF.

REFERENCE

1. Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176:957-969.

*Associate Professor of Medicine, Director, The Johns Hopkins Adult Cystic Fibrosis Program, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Address correspondence to: Michael P. Boyle, MD, FCCP, Associate Professor of Medicine, Director, The Johns Hopkins Adult Cystic Fibrosis Program, The Johns Hopkins University School of Medicine, 1830 E. Monument Street, 5th Floor, Baltimore, MD 21205. E-mail: mboyle@jhmi.edu.

The content in this monograph was developed with the assistance of a medical writer. Each author had final approval of his or her article and all its contents.





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.