Impact of Highly Effective Modulator Therapy on the Cystic Fibrosis Microbiome
Cystic FibrosisThe goal of this observational study is to learn about the effects of a specific cystic fibrosis therapy (Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy) on chronic sinonasal disease. The main questions it aims to answer are: How does this therapy impact bacterial communities in the paranasal sinuses? How does this therapy impact inflammation in the paranasal sinuses and olfactory cleft? How does this therapy impact sense of smell and sinonasal disease burden in individuals with cystic fibrosis? How does this therapy impact disease-specific and general quality of life of individuals with cystic fibrosis? Participants will be asked to provide samples from their nose, complete testing of their sense of smell, and complete surveys about their quality of life and sense of smell in this study. Researchers will compare study results between patients who are currently undergoing Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy and patients who are not currently undergoing therapy.
A Pilot Study of a Low Glycemic Load Diet in Adults With Cystic Fibrosis
Cystic FibrosisCystic Fibrosis-related Diabetes1 moreThis pilot study will evaluate the safety and tolerability of a low glycemic load dietary intervention in adult patients with cystic fibrosis (CF) in a rigorous feeding study. Specific emphasis will be placed on changes in weight, body composition, and glycemic measures obtained via continuous glucose monitor (CGM) usage.
Evaluation of Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) in Cystic Fibrosis...
Cystic FibrosisThis study will evaluate the efficacy, pharmacodynamics (PD) and safety of ELX/TEZ/IVA in participants 6 years of age and older with a non-F508del ELX/TEZ/IVA-responsive cystic fibrosis transmembrane conductance regulator gene (CFTR) mutation.
A Study to Assess the Effect of ELX/TEZ/IVA on Glucose Tolerance in Participants With Cystic Fibrosis...
Cystic FibrosisThis study was evaluate the effect of elexacaftor (ELX)/tezacaftor (TEZ)/ivacaftor (IVA) on glucose tolerance in CF participants, 12 years of age and older who are heterozygous for the F508del mutation and a minimal function mutation (F/MF genotypes), with abnormal glucose metabolism.
Ph 1/2 Study Evaluating Safety and Tolerability of Inhaled AP-PA02 in Subjects With Chronic Pseudomonas...
Cystic FibrosisPseudomonas Aeruginosa3 morePhase 1b/2a, double-blind, randomized, placebo-controlled, single and multiple ascending dose study to evaluate the safety, tolerability and phage recovery profile of AP-PA02 multi-bacteriophage therapeutic candidate administered by inhalation in subjects with cystic fibrosis and chronic pulmonary Pseudomonas aeruginosa (PA) infection.
A Study Evaluating Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Subjects 6 Through...
Cystic FibrosisThis study will evaluate the efficacy and safety of elexacaftor (ELX) / tezacaftor (TEZ) / ivacaftor (IVA) triple combination (TC) in subjects 6 through 11 years of age with cystic fibrosis (CF) who are heterozygous for F508del and a minimal function (MF) mutation (F/MF genotypes).
Aztreonam for Inhalation Solution (AZLI) for the Treatment of Exacerbations of Cystic Fibrosis
Cystic FibrosisInfection1 moreThis study evaluates the role of AZLI in the treatment of acute pulmonary exacerbations of CF. For consecutive exacerbations patients will receive AZLI + IV Colistin, or two IV anti-pseudomonals.
A Study Evaluating the Safety and Efficacy of VX-440 Combination Therapy in Subjects With Cystic...
Cystic FibrosisThis is a Phase 2, randomized, double-blind, placebo- and active-controlled, parallel group, multicenter study to evaluate the safety, tolerability, and efficacy of VX-440 in dual and triple combination with tezacaftor (TEZ; VX-661) and ivacaftor (IVA; VX-770) in subjects with cystic fibrosis (CF) who are homozygous for the F508del mutation of the CF transmembrane conductance regulator (CFTR) gene (F508del/F508del), or who are heterozygous for the F508del mutation and a minimal function (MF) CFTR mutation not likely to respond to TEZ and/or IVA therapy (F508del/MF).
Effects of an Early Rehabilitation Program During Hospitalization in Patients With Cystic Fibrosis...
Cystic FibrosisStudies demonstrate that exercise increases the maximal oxygen uptake, peak oxygen consumption, reduce effort-induced lactic acid production, and increase skeletal muscle oxidative capacity, as well as psychological aspects such as increased self-esteem and improvement of the quality of life. In the literature there is only one study involving rehabilitation in hospitalized pediatric patients with cystic fibrosis. Thus, more information on in-hospital rehabilitation is required in adult cystic fibrosis patients. The present study aims to evaluate the effects of an early rehabilitation program, based on aerobic training and muscle strength training, in adolescent and adult patients with cystic fibrosis hospitalized at Hospital de Clinicas de Porto Alegre for exacerbation of lung disease.
A Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of VX-661/Ivacaftor in Pediatric...
Cystic FibrosisThis is a Phase 3, 2-part (Part A and Part B), open label, multicenter study evaluating the pharmacokinetic (PK), safety, and tolerability of multiple doses of tezacaftor (TEZ) in combination with ivacaftor (IVA) in subjects 6 through 11 years of age with CF who are homozygous or heterozygous for the F508del- CF transmembrane conductance regulator protein (CFTR) mutation.