View clinical trials related to Cystic Fibrosis.
Filter by:The study will assess if administration of high-dose vitamin D and a commonly used prebiotic (inulin) is effective to reduce gastrointestinal dysbiosis and to improve critical intestinal functions in Cystic Fibrosis with the additive or synergistic effects of the combination of vitamin D + inulin.
The objective of the study is to assess the utility of "Acceptance and Commitment Therapy" (ACT) in which subjects learn new ways to manage uncomfortable experiences and feelings and to engage in positive behaviors, over "Supportive Psychotherapy" in which subjects talk about their experiences to date in a cohort of adult Cystic Fibrosis patients. The hypothesis is that six telehealth/webcam sessions of ACT will lead to an improvement in medication and visit compliance, as well as an overall improved sense of well-being and coping skills, particularly as compared with 6 telehealth/webcam sessions of supportive psychotherapy.
This study will evaluate the efficacy, safety, and pharmacodynamics of elexacaftor (ELX, VX-445) in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF) who are homozygous for F508del.
Regular bronchial clearance is essential in patients with cystic fibrosis for their bronchial health. SIMEOX® (Physio-Assist, Aix en Provence, France) is an innovative medical device for the drainage of the bronchial tree. By changing the rheological properties of mucus, SIMEOX® helps to mobilize secretions and assists their transport to the upper airways. This technology is based on fundamental research on bronchial mucus rheology. At the present time, SIMEOX® device is mainly used over a short period at the time or after an exacerbation in healthcare structures (hospitals, physiotherapy practices, post-care, and rehabilitation units, etc…). The clinical effects observed in the short term encourages long-term autonomous use by the patients themselves. The overall objective of this study is to evaluate the efficiency and acceptability of SIMEOX® used at home by the patient himself for bronchial clearance in patients with cystic fibrosis.
Regular bronchial clearance is essential in patients with cystic fibrosis for their bronchial health. SIMEOX® (Physio-Assist, Aix en Provence, France) is an innovative medical device for the drainage of the bronchial tree. By changing the rheological properties of mucus, SIMEOX® helps to mobilize secretions and assists their transport to the upper airways. This technology is based on fundamental research on bronchial mucus rheology. At the present time, SIMEOX® device is mainly used over a short period at the time or after an exacerbation in healthcare structures (hospitals, physiotherapy practices, postcare, and rehabilitation units, etc…). The clinical effects observed in the short term encourages long-term autonomous use by the patients themselves. The overall objective of this study is to evaluate the efficiency and acceptability of SIMEOX® used at home by the patient himself for bronchial clearance in patients with cystic fibrosis.
"The usefulness of respiratory physiotherapy and its execution modalities remains highly debated even though reviews of the literature show that respiratory physiotherapy is able to improve the drainage of bronchial secretions and pulmonary function tests during cystic fibrosis in periods of stability. Different physiotherapy techniques have been developed but the choice of one or the other facing a patient can not currently be recommended. The VirtualChest project, supported by a grant from the National Agency for Research (ANR), aims to develop and validate a physical model of respiratory physiotherapy (6 stages including model establishment pulmonary: bronchial tree and pulmonary mechanics and parietal [steps 1 and 3], a model of mucus [step 2] and modeling the effect of physiotherapy [step 5]). This project is integrated with stages 3 and 5 of this broad project and aims to get on a limited number of children with cystic fibrosis a proof of concept (prediction of drainage efficiency) and especially to feed the proposed physical models in order to subsequent optimization of the model (step 6). The choice cystic fibrosis of the child was justified by the effect demonstrated respiratory physiotherapy, particularly on respiratory functional criteria, and the fact that the parietal mechanics varies physiologically at this age."
Chest physiotherapy plays a crucial role in treatment of lung disease in cystic fibrosis (CF). New airway clearance techniques (ACTs) adapted to individual needs are still being sought to achieve the best effect of airway clearance. The primary aim of this study is to assess the efficacy of a new ACT (Simeox) on pulmonary function in children with CF. 40 CF patients with stable respiratory function will be randomized 1:1 to Simeox or conventional chest physiotherapy (CCPT) therapy (control group) and treated at home during 1 month. After a short washout period, patients will be treated at home onto the alternative treatment for 1 month (crossover design). Lung function, quality of life, pulmonary exacerbation and safety will be evaluated at 1 month for each therapy period.
The purpose of this protocol is to begin an exercise program combined with behavioral counseling for patients who are hospitalized with a cystic fibrosis exacerbation. The exercise program will begin during the hospital stay. Beginning an exercise program during this period of reduced mobility and isolation may be an ideal time to deliver a structured exercise prescription along with a behavioral program to promote long-term adherence to exercise (structured physical activity) . Hospitalized patients have an acute awareness that their lung function is declining and may be more motivated and open to changing their behavior and adding exercise to their treatment regimen.
The investigators have established the "Evaluating the Alimentary and Respiratory Tracts in Health and disease" (EARTH) research program. It provides a structured approach to analysing gastrointestinal and respiratory microbiomes, along with diet and symptomatology, in children with a gastrointestinal and/or respiratory condition with recognised long-term morbidity (e.g. cystic fibrosis, obstructive sleep apnoea, or Hirschsprung's disease). The EARTH program consists of a series of prospective, longitudinal, controlled, observational studies, with each individual study comparing children with a chronic gastrointestinal and/or respiratory condition to healthy controls (HC). It will be conducted in an Australian tertiary paediatric hospital (although the methodology is applicable to other settings). Children with a chronic gastrointestinal and/or respiratory condition will be compared to age and gender matched HC across a 12-month period. The following will be collected at baseline, 6 and 12 months: (i) a stool sample, (ii) an oropharyngeal swab or sputum sample, (iii) a semi-quantitative food frequency questionnaire, (iv) details of disease symptomatology, (v) health-related quality of life, and (vi) psychosocial factors. Data on the intestinal and respiratory microbiomes and diet will be compared between children with a condition and HC. Correlations between dietary intake (energy, macro- and micro-nutrients), intestinal and respiratory microbiomes within each group will be explored. Data on disease symptomatology, quality of life and psychosocial factors will also be compared between children with a condition and HC. The investigators hypothesise that: (i) Children with chronic gastrointestinal and/or respiratory conditions will have altered intestinal and respiratory microbiomes compared to healthy children, and (ii) Diet plays a key role in influencing the intestinal and respiratory microbiomes and this may impact on clinical outcomes, biomarkers of disease, and health-related quality of life.
The purpose of this study is to identify new methods of measuring and improving collaborative goal setting between patients and clinicians in adult cystic fibrosis care.