eCysticFibrosis Review

Studies have shown that adolescent and young adult women with cystic fibrosis often turn to their CF care team for answers to their questions about their sexual and reproductive health. But how prepared are CF clinicians to initiate conversations? Do they have the most current data about pregnancy decisions for women taking CFTR modulators? When should modulator therapy be stopped? When is it safe to continue?

In this eCysticFibrosis Review podcast, Dr. Traci Kazmerski from UPMC Children's Hospital of Pittsburgh and Dr. Natalie West from Johns Hopkins University take us into the clinic to address these important questions.

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Airway infections are a hallmark of cystic fibrosis, making antibiotic therapies a mainstay of CF treatment. While the fight against ABX resistance is ongoing, newer data are showing that commonly used dosing regimens may have to be altered to account for the particular pharmacodynamics in people with CF. How should ABX dosing be altered, and what additional effect might CFTR modulation have on dosing decisions?

That’s the topic Dr. Andrea Hahn, Assistant Professor of Pediatrics and an Infectious Disease Specialist at Children’s National Medical Center and George Washington University School of Medicine and Health Sciences, addresses in this eCysticFibrosis Review podcast. 

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No-contact telemedicine to better protect CF clinic patients and staff during this pandemic may sound like a great idea, but is it even possible to institute?

In this eCysticFibrosis Review Special Edition podcast, we find out how pulmonary and critical care specialists Dr. Dana Albon and Dr. Lindsay Somerville successfully brought telemedicine to their Adult Cystic Fibrosis Clinic at University of Virginia Health.

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Where, beyond the lungs, do CFTR modulators provide beneficial effects? Can treatment promote growth in height and BMI? What’s known about CFTR modulator effects on cystic fibrosis-associated liver disease?  

In this eCysticFibrosis Review podcast, Drs. Darla Shores and Anna Reed from the THRIVE Pediatric Intestinal Rehabilitation Center in the Division of Gastroenterology, Hepatology, and Nutrition at the Johns Hopkins Children's Center take us to the clinic to answer these and other questions crucial to the health of our patients with cystic fibrosis.

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Elexacaftor/tezacaftor/ivacaftor — the newly approved triple combination CFTR modulator therapy.  What are the benefits?  For which patients?  What are the potential adverse effects?  Who’s most likely to receive them?  

In this issue, Dr. Scott Sagel from the Breathing Institute at the Children’s Hospital Colorado, part of the University of Colorado’s Anschutz Medical Campus, takes us to the clinic to discuss some answers.

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Anxiety and depression in cystic fibrosis — who do these conditions affect? How much do they influence patient health? How can clinicians identify and manage them? What evidence-based interventions have been shown to work?

In this issue, Dr. Anna Georgiopoulos, Assistant Professor of Psychiatry, Part-Time, Harvard Medical School and Consulting Psychiatrist, Massachusetts General Hospital Cystic Fibrosis Program, takes us to the clinic to answer these questions that can be key to the health and well-being of children, adults, and families living with CF.

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New CFTR modulators for F508del mutations? New research into reducing inflammation in the CF lung?  In this issue, Dr. Jennifer Taylor-Cousar from National Jewish Health in Denver explains the potential clinical impact of new and emerging CF disease modifying therapies.

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Inhaled antibiotics have become the foundation of treating chronic respiratory tract Pseudomonas infection in individuals with cystic fibrosis. But what happens when inhaled monotherapy doesn’t sufficiently manage the symptoms? Which patients are appropriate candidates for treatment with multiple inhaled agents?  Which medications are appropriate for continuous alternating therapy (CAT)?

In this Issue, Dr. Elliott Dasenbrook from the Cleveland Clinic brings the evidence he analyzed in his recent eCysticFibrosis Review Newsletter issue (Vol 8; Issue 10) into the clinic to help guide clinical decision-making.

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The effects of CFTR modifiers on improving lung function in people with cystic fibrosis are well known. But what about their effects on the GI tract that directly affect nutrition?

In this issue, Dr. Michael Wilschanski — Director of Hadassah Hospital’s Pediatric Gastroenterology Unit at Hebrew University in Jerusalem — explains how new findings about CFTR modification can impact clinical practice.

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