Mucus plugging constitutes a nutrient‐rich nidus for a bacterial infection that has long been recognized as a potent stimulus for neutrophilic airway inflammation driving progressive lung damage in people with cystic fibrosis (CF). However, mucus plugging and neutrophilic…
November 2019
The Remaining Barriers to Normalcy in Cystic Fibrosis V
Cystic fibrosis precision therapeutics: Emerging considerations
Small molecules that address fundamental defects underlying cystic fibrosis (CF), including modulators such as the approved drugs ivacaftor, lumacaftor, tezacaftor, and elexacaftor, have advanced dramatically over the past few years and are transforming care and prognosis among individuals…
Improving outcomes of infections in cystic fibrosis in the era of CFTR modulator therapy
Currently, available single and dual‐combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies have favorably altered the life course of individuals with cystic fibrosis (CF) by decreasing morbidities and increasing survival. However, even with CFTR modulator use, questions…
Off‐label use of intravenous antimicrobials for inhalation in patients with cystic fibrosis
Management of infections in patients with cystic fibrosis (CF) presents challenges for healthcare providers, including the eradication of initial acquisition, treatment of acute exacerbations, and chronic infection with suppressive therapy. Inhaled antimicrobial therapy for infections in patients with…
Anti‐inflammatories and mucociliary clearance therapies in the age of CFTR modulators
Cystic fibrosis (CF) is a genetic and life‐limiting disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. This multi‐system disease is characterized by progressive lung disease and pancreatic insufficiency amongst other manifestations. CFTR primarily functions…
Nutrition in cystic fibrosis: From the past to the present and into the future
Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition…
The demographics of adverse outcomes in cystic fibrosis
Understanding variability in cystic fibrosis (CF) health outcomes requires an understanding of factors that go far beyond Cystic Fibrosis Transmembrane Receptor (CFTR) function caused by different gene mutations. Social and environmental factors that influence health have a significant…
Mucus, mucins, and cystic fibrosis
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and is characterized by the accumulation of…