The roles and experiences of adolescents with cystic fibrosis and their parents during transition: A qualitative interview study
Cystic Fibrosis (CF) is a multi-organ disease which affects the respiratory, digestive and reproductive system and has also other clinical features. CF used to be a rare chronic disease exclusively concerning paediatric care services. Because of the increasing survival of children with CF into adulthood (median survival age of 51.7 years), CF has become an exemplary disease to illustrate the added value of healthcare transition programs [1,2]. Such programs facilitate the transfer from paediatric care to adult care services and include a planned and structured transfer of care [3]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 13, 2023 Category: Respiratory Medicine Authors: Karsten Vanden Wyngaert, Sara Debulpaep, Wim Van Biesen, Sabine Van Daele, Sue Braun, Kenneth Chambaere, Kim Beernaert Tags: Original Article Source Type: research
Elexacaftor/Tezacaftor/Ivacaftor therapy in cystic fibrosis children previously CFSPID: Is it over-medicalization?
To the Editor: (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 13, 2023 Category: Respiratory Medicine Authors: V. Terlizzi Source Type: research
Alterations in lipids after initiation of highly effective modulators in people with cystic fibrosis
Historically, cardiovascular disease (CVD) has been rare in people with cystic fibrosis (pwCF) [1 –3]. However, with advances in CF treatments, pwCF are living longer, experiencing increased rates of being overweight or obese, and facing a higher lifetime risk of cystic fibrosis related diabetes (CFRD) related to aging [4,5]. Recent case reports of myocardial infarction, combined with prior ev idence of pre-clinical atherosclerosis, suggest that the risk of CVD in pwCF may be changing [6–8]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 12, 2023 Category: Respiratory Medicine Authors: Katherine A. Despotes, Agathe S. Ceppe, Scott H. Donaldson Source Type: research
Frequent microbiological surveillance during inpatient cystic fibrosis pulmonary exacerbations has limited clinical value
In children with cystic fibrosis (CF), pulmonary exacerbations (PEx) requiring intravenous (IV) antibiotics are sentinel events. They are associated with negative outcomes, such as a greater decline in forced expiratory volume in one second (FEV1) [1] per year, profound negative impacts on quality of life [2], and considerable healthcare costs [3]. Despite their prevalence and impact, there are still aspects of the pathogenesis, diagnostic criteria and treatment of PEx events that remain elusive and unstandardized. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 12, 2023 Category: Respiratory Medicine Authors: Wendy HC Song, Kendrew SK Wong, David M. Goldfarb, Jeffrey N Bone, Jonathan H Rayment Tags: Original Article Source Type: research
Feasibility Testing of a Web-Based Reproductive Decision Support Tool for Cystic Fibrosis
Due to the positive impact of highly effective modulator therapies (HEMT) on the health of those with cystic fibrosis (CF), more people with CF are contemplating their reproductive futures and considering parenthood. Prior to widespread HEMT use, 309 pregnancies were reported among United States (US) people with CF in 2019. After approval of elexacaftor-tezacaftor-ivacaftor in October 2019, this number rose to 618 and 675 pregnancies annually in 2020 and 2021, respectively [1]. Even prior to HEMT, four out of five adolescent and young adult women with CF reported a desire to become a future parent [2]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 11, 2023 Category: Respiratory Medicine Authors: Traci M. Kazmerski, Olivia M. Stransky, Catherine E. Wright, Maya Albanowski, Joseph M. Pilewski, Mehret Birru Talabi, Lisa S. Callegari, Judy C. Chang, Kaleab Z. Abebe, Elizabeth Miller, Ashley Deal, Raelynn O'Leary, Sonya Borrero Tags: Original Article Source Type: research
Changes in fecal lipidome after treatment with ivacaftor without changes in microbiome or bile acids
Alterations in gastrointestinal health are prominent manifestations of cystic fibrosis (CF) and can independently impact pulmonary function. Ivacaftor has been associated with robust improvements in pulmonary function and weight gain, but less is known about the impact of ivacaftor on the fecal microbiome, lipidome, and bile acids. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 7, 2023 Category: Respiratory Medicine Authors: Rosara Bass, Ceylan Tanes, Kyle Bittinger, Yun Li, Hongzhe Lee, Elliot S ． Friedman, Imhoi Koo, Andrew D． Patterson, Qing Liu, Gary D． Wu, Virginia A． Stallings Tags: Original Article Source Type: research
Reduction in abdominal symptoms (CFAbd-Score), faecal M2-pyruvate-kinase and Calprotectin over one year of treatment with Elexacaftor-Tezacaftor-Ivacaftor in people with CF aged ≥12 years – The RECOVER study
Already at birth, most infants with cystic fibrosis (CF) have exocrine pancreatic insufficiency (PI). Malabsorption, malnutrition and growth failure follow PI, particularly with advancing age and evolving CF lung disease. Intestinal epithelial dysfunction, particularly in the setting of PI, can be associated with inflammation, dysbiosis and dysmotility, partial or complete bowel obstruction either in infancy with meconium ileus (MI) or later in life with constipation or distal intestinal obstruction syndrome (DIOS) [1]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 7, 2023 Category: Respiratory Medicine Authors: Jochen G. Mainz, Karen Lester, Basil Elnazir, Michael Williamson, Ed McKone, Des Cox, Barry Linnane, Carlos Zagoya, Franziska Duckstein, Anton Barucha, Jane C. Davies, Paul McNally, RECOVER Study Group Tags: Original Article Source Type: research
Impact of highly effective modulator therapy on chronic rhinosinusitis and health status: 2-year follow-up
In people with cystic fibrosis (PwCF) who are heterozygous or homozygous for F508del, treatment with the highly effective modulator therapy (HEMT) elexacaftor/tezacaftor/ivacaftor (ETI) yields substantial improvements in pulmonary function and health status, and is anticipated to extend life [1 –3]. Most PwCF endure comorbid chronic rhinosinusitis (CRS), which impairs quality-of-life (QOL), necessitates acute-on-chronic treatment, and worsens lower airway status [4–7]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 5, 2023 Category: Respiratory Medicine Authors: Daniel M. Beswick, Aastha Khatiwada, Jessa E. Miller, Stephen M. Humphries, Alexandra Wilson, Eszter K. Vladar, David A. Lynch, Jennifer L. Taylor-Cousar Tags: Short Communication Source Type: research
Characteristics associated with cystic fibrosis-related pulmonary exacerbation treatment location
Cystic fibrosis (CF) is a multisystem disease characterized by pulmonary exacerbations (PEx) that are often treated with intravenous (IV) antibiotics [1]. Treatment can occur at home or in the hospital, with some evidence indicating patients have better outcomes [2 –4] if they receive inpatient treatment. We were interested in understanding factors that impact whether patients are treated at home or in the hospital. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 5, 2023 Category: Respiratory Medicine Authors: Laura S. Gold, Ryan N. Hansen, Sonya L. Heltshe, Patrick A. Flume, Christopher H. Goss, Natalie E. West, Don B. Sanders, Larry Kessler Source Type: research
Standards for the care of people with cystic fibrosis (CF)
The European CF Society (ECFS) is a learned society committed to improving survival and quality of life for people with CF. An important task for the ECFS has been the development of standards for the care of people with CF. Over two decades, a series of publications have impacted significantly on the quality of care delivered by established and emerging CF services [1 –6]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 3, 2023 Category: Respiratory Medicine Authors: Kevin W Southern, Pierre-Regis Burgel, Carlo Castellani, Kris De Boeck, Jane C Davies, Fiona Dunlevy, Isabelle Fajac, Andrea Gramegna, Elise Lammertyn, Peter G Middleton, Felix Ratjen, Silke van Koningsbruggen-Rietschel Tags: Editorial Source Type: research
Patient perspectives on elexacaftor/tezacaftor/ivacaftor after lung transplant
Cystic fibrosis (CF) is a genetic disorder resulting in dysfunctional CF transmembrane conductance regulator (CFTR) protein. CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor (ETI), have emerged as a treatment to improve pulmonary function, sinus symptoms, and other patient outcomes. [1 –4] ETI may be prescribed to lung transplant recipients with CF (CF LTRs) to help alleviate non-pulmonary manifestations of CF. [5–9] Understanding CF LTRs' perspectives on the use of ETI can inform clinician practice and guide conversations regarding ETI therapy after transplant. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 3, 2023 Category: Respiratory Medicine Authors: Dave Young, Lauren E. Bartlett, Jennifer Guimbellot, Tijana Milinic, Nora Burdis, Eliana R. Gill, Erika D. Lease, Christopher H. Goss, Siddhartha G. Kapnadak, Kathleen J. Ramos Source Type: research
Editorial Board
(Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 1, 2023 Category: Respiratory Medicine Source Type: research
Publication Information
(Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 1, 2023 Category: Respiratory Medicine Source Type: research
Supplement title
(Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 1, 2023 Category: Respiratory Medicine Source Type: research
Table of Contents
(Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 1, 2023 Category: Respiratory Medicine Source Type: research
