In this podcast Dr. John P. Clancy reviews CFTR modulators, and discusses insights presented at the 2017 NACFC.

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In this podcast Andrea Granados, MD describes the clinical impact of the early diagnosis of glucose abnormalities in people with CF, summarizes the limitations of alternative modalities for screening and diagnosing CFRD, and and identifies the current recommendations for the management of CFRD.

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In this podcast Dr. George Solomon discusses new directions in CFTR modification in the context of case studies.

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Our guest author is Amanda Leonard, MPH, RD, CDE, from the Johns Hopkins Children's Hospital in Baltimore, MD.

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Our guest author is Gregory Sawicki, MD, MPH from Boston Children's Hospital, and Harvard Medical School in Boston, MA.

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Our guest authors are Mark T. Jennings, MD, MHS and Rebecca Dezube, MD from the Johns Hopkins University School of Medicine in Baltimore, MD.

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Claire Wainwright, FRACP, MD and Tonia Douglas, MD cover the important topic of the Future Landscape of CFTR Modulators in the format of case-study scenarios for the clinical practice.

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Christopher Oermann, MD covers the important topic of the Pseudomonas Eradication in the format of case-study scenarios for the clinical practice.

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Dr. Deepak Agrawal, MD covers the important topic of Nutritional Issues in Cystic Fibrosis in the format of case-study scenarios for the clinical practice.

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Daniel Heintz, MD and Karen McCoy, MD cover the important topic of Trends in Inhaled Antibiotic Therapy in the format of case-study scenarios for the clinical practice.

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Our guest author is John J. LiPuma from the University of Michigan in Ann Arbor, Michigan.

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In this issue we are joined by one of our Program Directors, Dr. Noah Lechtzin from the Johns Hopkins University. Dr. Lechtzin will address the current state of CFTR modification and how it can be applied in practice.

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Join us as Dr. Patrick Flume of the Medical University of South Carolina interviews Dr. Stuart Elborn of Queen's University in Belfast, Ireland. Dr. Elborn discusses the implications of the CAT study and how it effects his practice.

Join us as Dr. Patrick Flume of the Medical University of South Carolina interviews Dr. JP Clancy of Cincinnati Children's Hospital. Dr. Clancy discusses the implications of the CAT study and how it effect his practice.

Join us as Dr. Patrick Flume of the Medical University of South Carolina interviews Dr. Scott Bell of the QIMR Berghofer Medical Research Institute. Dr. Bell discusses the implications of the CAT study and how it will effect his practice.

Join us as Dr. Peter Mogayzel of the Johns Hopkins University discusses the design and findings for the CAT study with Dr. Patrick Flume of the Medical University of South Carolina. Learn about the importance of the study and how it may effect your practice.

In this issue we are joined by Dr. Shawn Aaron of University of Ottawa in Ontario, Canada. Dr. Aaron will discuss infection in patients with optimal therapy of chronic Pseudomonas aeruginosa cystic fibrosis, describe the pathophysiology of pulmonary exacerbations associated with Pseudomonas infection in patients with cystic fibrosis, and evaluate the optimal choice of antibiotics to treat Pseudomonas-associated pulmonary exacerbations in patients with cystic fibrosis.

In this issue of eCysticfibrosis Review Dr. Chris Goss of the University of Washington Medical Center. Dr. Goss discusses the benefits of CFTR modification beyond Fev1 improvement through the use of patient-cases.

In this issue of eCystifibrosis Review we will be join by Dr. Stuart Elborn of Queens University in Belfast, Northern Ireland. Dr. Elborn discusses the effects of CFTR-Modifying Therapies. He will present three patient-case scenarios and identify appropriate treatment options.

In this issue of eCysticFibrosis Review Dr. John Pohl discuses how to optimize nutrition in individuals with CF. Through patient case-scenarios he applies best practices to achieve optimal nutrition using PERT dosing.

In this issue Dr. Steven Freedman, Professor of Medicine from Harvard Medical School discusses digestive capabilities in nutritionally compromised patients with CF. Listen in as Dr. Freedman explains the importance of pancreatic enzyme replacement and present patient scenarios and best practices of treatment.

In this issue of eCysticfibrosis Review Dr. Ratjen discusses the challenges of P. aeruginosa eradication. He explains the importance to treat early and available treatment options. He also addresses whether treatment should be repeated in patients who develop recurrence of infection and how to handle patients failing eradication therapy.

In this podcast Dr. Patrick Flume, Professor of Medicine and Pediatrics at the Medical University of South Carolina in Charleston, discusses pulmonary exacerbation. He provides patient case scenarios to understand the importance of pulmonary exacerbation and the ability to identify when it occurs.

In this issue our guest author is Dr. Patrick Sosnay, Assistant Professor at the Johns Hopkins Cystic Fibrosis Center. Dr. Sosnay will illustrate how the genetics of CF can be used to make a diagnosis or identify a CF carrier, predict how individuals with a given mutation will do clinically and allow clinicians to select currently available and hopefully future therapies that directly address the defect caused by their individual CFTR mutations.

In this podcast we have guest author Lori Stark, PhD, ABPP, Professor of Pediatrics at the University of Cincinnati College of Medicine. Dr. Stark, discusses how to use “shaping” to encourage a child to eat a non-preferred food, describes the role of “attention” in maintaining behaviors incompatible with eating, and discusses the use of caloric goal-setting and dietary tracking applications to monitor treatment progress.

In this special edition of eCysticFibrosis Review, Dr. Peter Mogayzel discusses data findings from Dr. Steven Rowe results of the G551D observational study presented at the 27th Annual North American Cystic Fibrosis Conference in Salt Lake City, Utah.

In this special edition of eCysticFibrosis Review, Dr. Peter Mogayzel discusses data findings from Dr. Steven Rowe results of the G551D observational study presented at the 27th Annual North American Cystic Fibrosis Conference in Salt Lake City, Utah.

In this special edition of eCysticFibrosis Review, Dr. Peter Mogayzel discusses findings from Dr. Scott Powers clinical trial on Behavioral & Nutrition Treatment for Preschoolers With CF which he presented at the 27th Annual North American Cystic Fibrosis Conference in Salt Lake City, Utah.

In this special edition of eCysticFibrosis Review, Dr. Peter Mogayzel discusses outcomes presented by Dr. Elizabeth during her presentation on Nutritional Status and Outcomes in Pediatrics CF presented at the 27th Annual North American Cystic Fibrosis Conference in Salt Lake City, Utah.

In this special edition of eCystic Fibrosis review, Dr. Peter Mogayzel discusses highlights from Dr. Kenneth Olivier's talk on Nontuberculosis Mycobacteria: Updates in CF presented at the 27th Annual North American Cystic Fibrosis Conference in Salt Lake City, Utah.

In this podcast guest author Steven Rowe, MD, Associate Professor of Medicine from the University of Alabama at Birmingham, describes how CFTR modulator therapy fits into the clinical armamentarium of CF lung disease; identifies which patients are most appropriate for CF modulator treatment; and describes current research efforts and how to identify patients suitable for referral for consideration in CFTR modulator clinical trials.

In this podcast guest author Dr. Margaret Rosenfeld from the University of Washington School of Medicine, discusses the rationale for early Pseudomonas aeruginosa eradication, Identifies the inhaled and oral antibiotics that have been compared in clinical trials for early Pseudomonas eradication, and describes the potential adverse effects of early Pseudomonas eradication therapy.

In this podcast guest author Amanda Radmer Leonard, Pediatric Nutrition Practitioner, of the Johns Hopkins Children’s Center, describes approaches to the treatment of low vitamin D levels in patients with cystic fibrosis, discuss the variability of fecal elastase result during the first year of life, and summarizes the importance of tailoring nutritional interventions to specific patient and family situations.

Nonadherence to any medical regimen is common particularly among adolescents and young adults, and cystic fibrosis (CF) is no exception. There are many barriers to following the complex daily regimen that CF management requires, and researchers and clinicians have not yet found the ideal approach for counseling and supporting patients to improve and maintain their level of adherence. In this podcast Dr. Kristin Riekert, of The Johns Hopkins Adherence Research Center discusses best practices for determining which clinic patients are nonadherent and Identifies risk factors for nonadherence.

Case studies offer opportunity to differentiate the needs of each patient when managing respiratory infections. Dr. Elliott Dasenbrook will review the prevalence of important CF organisms over the last 10 years, the impact of respiratory tract MRSA on survival, the association between a virulent strain of P. aeruginosa that can spread among patients and lung transplant or death, as well as the impact of chronic non-tuberculous Mycobacterial lung disease on lung function.

Dr. Elizabeth Yen offers a clinical perspective to her review on how new research that highlights the strong positive impact of early behavioral and nutritional education and predictors of long-term response to such interventions. In this podcast she will expand on that information by discussing case scenarios.

In this issue, Dr. Elizabeth Yen reviews new research highlighting the strong positive impact of early behavioral and nutritional education. Exploring predictors of long-term response to such interventions she discusss how body image may affect compliance with nutritional recommendations in adolescents; and, then present data describing positive outcomes with gastrostomy tube placement for supplemental enteral intake.

Drs. Cutting and Collaco will help expand our understanding of the genotypic and phenotypic modifiers in the treatment of cystic fibrosis, with the discussion some typical case scenarios.

This podcast has been developed for clinicians caring for patients with issues related to cystic fibrosis. You can also read the companion newsletter. In this edition Dr. Chris Goss will explain the use of new inhalation therapies for the treatment of cystic fibrosis, with the discussion some typical case scenarios.

The eCysticFibrosis Review podcast is a clinical discussion between our November authors, Michael Boyle, MD, Elliott Dasenbrook, MD and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is Emerging Pathogens in Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on the website page, eCysticFibrosisReview.org.

Clinical practice guidelines have become a regular component of health care. The Cystic Fibrosis Foundation has supported the development of several recent clinical practice guidelines to review the evidence that endorses many of the therapies currently in use.

Although numerous clinical manifestations of cystic fibrosis (CF) exist, the principal causes of disease-related morbidity and mortality are due to pancreatic insufficiency and chronic airway infection. Recommendations are now available on the assessment and treatment of nutritional disease, use of airway clearance therapies, and use of chronic medications to maintain lung health. Acute complications of CF lung disease include pulmonary exacerbations, hemoptysis, and pneumothorax; guidelines on the management of these complications are currently available as well. Finally, as newborn screening for CF has become standard in all states, we can expect that most patients will be diagnosed in the absence of symptoms. Infant care guidelines should provide primary care physicians with the information needed for appropriate assessment and treatment of newly diagnosed patients.

In this podcast recording, Dr. Patrick Flume utilizes patient cases to summarize the clinical practice guidelines for individuals with CF, to aid clinicians in improving the quality and consistency of the care they provide.

In 2002, the CF Foundation convened a consensus conference. This consensus conference developed specific recommendations for the prevention of bone disease and the optimal supplementation of vitamin D for patients with cystic fibrosis. The original consensus guidelines did not have any actual demonstration in cystic fibrosis patients, and most of this was expert opinion. And at this time we are actually trying to test these guidelines to see if they hold true in those with cystic fibrosis. Since patients with cystic fibrosis are living longer, they are developing more complications, such as bone disease, and insuring that vitamin D supplementation is adequate and figuring out new ways to prevent bone disease is becoming more and more important for CF patients. In the discussion today, Peter Mogayzel, MD and Dianna Green, MD, of the Johns Hopkins University School of Medicine, will focus on the basis of the current vitamin D recommendation for patients with cystic fibrosis, describe the potential for success of various strategies for treatment of vitamin D insufficiency, and compare the potential benefits and risks of using bisphosphonates for the treatment of osteoporosis in patients with cystic fibrosis.

Improvements in nutrition have been a key factor in reducing morbidity and mortality in patients with cystic fibrosis (CF.) In this issue, Dr. Sarah Jane Schwarzenberg will summarize current progress in the definition and epidemiology of 2 CF GI-related issues—small intestinal bowel overgrowth (SIBO) and distal intestinal obstruction syndrome. The discussion continues with case studies to illustrate causes of nonpancreatic enzyme–related defects in fat absorption, including the use of omega-3 supplements.

In this edition Dr. Richard Moss will help expand our understanding of allergic bronchopulmonary aspergillosis (ABPA) with the discussion some typical case scenarios. ABPA is a major pulmonary complication in patients with cystic fibrosis (CF). The impact of ABPA on the course of CF lung disease poses special challenges, with chronic Aspergillus respiratory infection, even in the absence of ABPA, playing a significant role, according to data from 2 recent retrospective cohort studies. Dr. Moss' use of current diagnostic criteria provides perspective also on the limitations.

In September of 2009, the Cystic Fibrosis Foundation held a third CFRD consensus conference, co-sponsored by the American Diabetes Association and the Lawson Wilkins Pediatric Endocrine Society, providing a new set of recommendations for treating cystic fibrosis-related diabetes. Johns Hopkins provided evidence review so there can be evidence-based guidelines. Diagnostic & treatment criteria are presented in this podcast.

The eCysticFibrosis Review podcast is a clinical discussion between our January authors, Stephanie D. Davis, MD, Jessica Pittman, MD and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is Measurement of Early Lung Disease in Children With Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on this page via eCysticFibrosisReview.org.

The eCysticFibrosis Review podcast is a clinical discussion between our November authors, Michael Boyle, MD, Elliott Dasenbrook, MD and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is Emerging Pathogens in Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on the website page, eCysticFibrosisReview.org.

The eCysticFibrosis Review podcast is a clinical discussion between our September authors, Garry Cutting, MD, Barbara Karczeski, MS and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is How to Interpret Genetic Testing for Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on the website page, eCysticFibrosisReview.org