Active clinical trials for "Cystic Fibrosis"

31 CF patients and 23 healthy sex and age-matched volunteers were enrolled. All subjects underwent pulmonary function tests, 6-minute walking tests. Body Mass Index and Fat Free Mass Index were measured via a body composition analyser. Diaphragm ultrasonography (to measure thickening fraction), quadriceps femoris muscle ultrasonography were performed. A dynamometer was used to measure each subjects handgrip strength.

This project is an attempt to understand how Orkambi treatment affects exercise capacity and the function of the arteries in CF patients who are homozygous F508del. Our goal is to perform the exercise and vascular measurements every 3 months after a patient starts taking Orkambi.

This study compares the lung clearance index (LCI) in cystic fibrosis and healthy adults obtained with two different multiple breath nitrogen washout (MBWN2) devices. Each participants will perform the test (LCI) in duplicate on each device.

The aim of this study is to develop a reliable and valid instrument to measure symptom management (experience and / or management strategies) during exacerbation episodes in CF patients. For the development a sequential exploratory two phase mixed-method study will be applied. In a first step, up to 25 CF patients will be interviewed: approximately 15 CF patients who experienced at least one episode of exacerbation in the past year and approximately CF 10 patients who are currently experiencing an exacerbation. The latter group will be interviewed up to 3 times during and after the antibiotic treatment. The instruments initial item list will be developed on basis of the interviews. Up to five health professionals will rate the items' relevance. In a further step, items' clarity and relevance will be assessed by cognitive debriefing interviews with no more than 10 patients. In the last step (part III), the instrument's preliminary construct and concurrent validity and reliability will be tested in a larger sample up to 150 patients experiencing exacerbation. Patients will be asked to complete the newly developed questionnaires and a set of other questionnaires at one time point. For part III, ethic approval will be asked at a later date (2015).

This study compares the lung clearance index (LCI) in cystic fibrosis and healthy children obtained with two different multiple breath nitrogen washout (MBWN2) devices. Each participants will perform the test (LCI) in duplicate on each device.

The Impact of cystic fibrosis (CF) on psychological and emotional functioning has been the focus of several studies over the past 20 years. The results from a current meta-analysis indicated that depressed patients were three times more likely to be noncompliant with treatment recommendations than nondepressed patients with chronic illness. To date, most studies of the prevalence of psychiatric symptoms have been limited by small sample size; reliance on samples of convenience that are potentially biased in term of rates of symptomatology, and measures that contain legitimate symptoms that are part of the respondent's chronic disease. Estimating the prevalence of depression and anxiety has become important as new evidence indicates that these symptoms may have a significant impact on health outcomes, including adherence to medical treatments, utilization of health care services, and rates of morbidity and mortality. More depressive the symptoms are associated with poorer lung function, and that in the absence of depression, poor lung function is minimally associated with lower patient ratings of quality of life. The purpose of the current study is to estimate the regional, and if possible the national prevalence of depressive and anxious symptoms in children and adults with CF and parents caregivers. Patients with CF ages 14 to 17, and parents of children ages 14 to 17, will complete and depression/anxiety screening measure at a routine clinic visit. These will then be linked with demographic and medical variables reported to the CF registry and analyzed cross sectionally. Adults with CF ages 18 and older will also complete the depression / anxiety screening measure at a routine clinic visit. Efforts will be made to recruit a representative sample of patients from each clinic.

Main objective : This project aims to have a better understanding of the influence of representations of patients with mucoviscidosis, their assistants and their medical persons in the choice of patients to accept or not lung transplantation. Specific objectives : Identify the knowledge, representations, or feelings of patients which could constitute bridles or factors of motivation to use for transplantation. Identify the knowledge, representations, or feelings of medical persons which could constitute bridles or factors of motivation to the "encouraging" proposal to patients of lung transplant. Identify the knowledge, representations, or feelings of assistants which could constitute bridles or factors of motivation to the "encouragement" of lung transplantation at its close. Identify the needs of patients, assistants and medical persons to successfully reduce these bridles to transplantation and increase the factors of motivation. Compare the representations of patients, assistants and medical persons Search representations associated with patients who refuse the transplantation and those who accept the transplantation

Pseudomonas aeruginosa (Pa) is the bacterium that causes one of the most consequential lung infections in people with CF. Many young children do not have Pa in their lungs but will become infected as they get older. The investigators want to learn more about how Pa is passed from person to person, especially to someone with Cystic Fibrosis (CF).

The purpose of this study is the psychometric validation of a self-administered dyspnea questionnaire, usable in clinical practice in order to assess dyspnea and its impact on patients with chronic respiratory diseases.

Our research is comparing the lungs of babies and young children with CF (cystic fibrosis) to those without CF. We are looking at the blood flow in the lungs to help doctors better understand how CF damages lungs and how to prevent this damage in the future. We will use MRI (Magnetic Resonance Imaging) and special blood tests as ways to understand early changes in the lungs of babies and young children with CF. We will look at the special blood tests to see if they can work as signals for the doctors to better understand when changes are happening in the lungs. For the babies with CF, we will compare MRI images of the lungs to their CT images (also sometimes called CAT scans or Computerized Tomography). We hope that this study will help us reduce the number of X-rays and CT scans children with CF might get in the future. Because MRI's do not use radiation, this could reduce the amount of radiation exposure that children with CF would get over their lifetime.