Active clinical trials for "Cystic Fibrosis"

The aim of this project is to evaluate the psychological reshuffle induced by ORKAMBI. The particular focus of this study is the consequence of its introduction on anxiety, depression, quality of life and adherence to all cystic fibrosis (CF) treatment. To answer this question investigators will monitor the psychological function of CF adolescents and young adults treated with ORKAMBI and compare them to CF adolescents and young adults not treated with ORKAMBI.

Educational programs are essential for cystic fibrosis patients who need to take several different drugs every day. Compliance is often klow in these patients, and clinicals and paramedics try to increase it, through educational programs. As for inhaled therapies, the CF center of Montpellier uses a video conference tool to educate patients. The program may involve either the physiotherapist or the doctor or both. Through the present study, the investigators would like to evaluate the current practice at the CF center of Montpellier and verify if patients feel that the physiotherapist could handle the program on his own, without the support of a physician.

Cystic Fibrosis related diabetes (CFRD), a major factor of morbid-mortality in CF, is characterized by a preclinical phase of glucose intolerance particularly long reaching up to 10 years. At the physiopathology level, insulin secretion is determinant in the glucose tolerance abnormalities in CF. Indeed insulin secretion is dependent of the CFTR activity at the beta cell surface and inhibition of CFTR leads to a decrease in insulin secretion. Recently, the combination of the lumacaftor, a CFTR corrector, with Ivacaftor, a CFTR potentiator, was studied in patient with CF homozygous for the Phe508 del CFTR mutation patients and showed an improvement of the respiratory state in comparison with the placebo group. These data suggests that lumacaftor in combination with ivacaftor in targeting CFTR action may have an early impact on the insulin-secretion and consequently on the glucose tolerance.

This study will evaluate the performance of wearable technology in cystic fibrosis (CF) participants taking commercial Elexacaftor (ELX)/Tezacaftor (TEZ)/Ivacaftor (IVA) utilizing a fully decentralized trial design.

There is an urgent need for a noninvasive method to diagnose bronchial infections and exacerbations in patients with Cystic Fibrosis (CF). The current method to diagnosed infections involves either collecting a sputum sample or obtaining a bronchoalveolar lavage (BAL). However, some patients cannot produce sputum. At the same time the decision of when a patient has an exacerbation continues to be very subjective. In this exploratory study, we propose a new, noninvasive method to diagnose bronchial infections and to evaluate possible markers of inflammation that can assist in a noninvasive way in the determination of exacerbations.

The goal of this research study is to better understand current treatment practices for pulmonary exacerbations (lung infections) and whether the Cystic Fibrosis National Patient Registry (CFFNPR)can be used for this type of study.

There are very few studies comparing the accuracy, sensitivity and specificity of HemoCue glucose 201 RT system with the laboratory gold standard. The incidence of cystic fibrosis related diabetes (CFRD) has risen significantly as patients' survival improves. Around 30% of all cystic fibrosis (CF) adult patients have CFRD. Early diagnosis of CFRD is important to slow the deterioration of lung function and nutritional status, both of which increase mortality. The oral glucose tolerance test (OGTT) is the accepted method for detecting CFRD and the Cystic Fibrosis Trust guidelines recommend that patients with CF over the age of twelve years should be screened annually. The World Health Organisation has pointed out that due to the absence of a more specific biological marker to define diabetes, plasma glucose estimation remains the basis of diagnostic criteria. WHO recommends that venous plasma glucose should be the standard method for measuring and reporting glucose concentration in blood. However, it has also recognised that there is a widespread use of capillary sampling. Although fasting values for venous and capillary plasma glucose are the same, in the nonfasting state capillary samples will give higher results than venous samples, and glucose values require conversion which can be problematic. HemoCue Glucose 201 RT (HGS 201 RT) analyzer with plasma conversion provides laboratory quality test results at the point of care for the diagnosis, screening and monitoring of diabetes mellitus. The analyzer is easy to use and the system can be operated by nonlaboratory personnel, also the results can be presented immediately, leading to significant time and resource saving. The patient also benefits from having the result immediately instead of waiting for several hours. With immediate result, the doctor and health care professionals can provide education, support and reassurance, and implement care plans, if appropriate, at the earliest opportunity. The aim of the study is to compare the clinical accuracy of HemoCue Glucose 201 RT with the laboratory standard method in the analysis of blood glucose concentration during oral glucose tolerance test.

The goal of this research study is to collect blood and urine samples from people who have either the R117H type of CF or the non-G551D gating type of CF to be kept for future research.We will also use some of the collected blood to measure the number of neutrophils.

The goal of this study is to identify the immunological factors that influence a patient's response to the presence of the fungus Aspergillus fumigatus (A. fumigatus) in the lungs. In patients with cystic fibrosis (CF), this fungus is not known to cause damage to the lungs, but some patients respond with an allergic reaction that may cause wheeze, cough, or difficulty breathing. Approximately 230 patients will be enrolled with an additional 60 people who do not have CF and who do not have a history of asthma to serve as a comparison group.

The purpose of this study is to identify all the bacterial species present in the respiratory tracts of patients with Cystic Fibrosis (CF), and to connect them with clinical information. Traditional culture methods of throat swab and sputum samples can only identify the most prevalent bacteria in samples, those of which there are over about 5 million bacteria per teaspoon. A recently developed method has been found to be more sensitive and can detect up to several hundred bacterial species in throat swab or sputum sample of individual CF patients.