View clinical trials related to Cystic Fibrosis.
Filter by:In contrary to what is seen in FRT cells, rectal organoids of patients with a R334W mutation do respond to CFTR modulators ivacaftor and lumacaftor. The present study will investigate the response to modulators in organoids of 30 patients with CF and a R334W mutation, to allow further stratificaton for a future clinical trial assessing the clinical effect of ivacaftor/tezacaftor in patients with CF and a R334W mutation.
The investigators propose to assess the contributions of the thermic effect of food intake (TEF), which is the amount of energy expended to digest food, to overall energy expenditure in Cystic Fibrosis (CF).
The study is a prospective, double blinded, placebo controlled, randomized study to evaluate the effects of daily oral estrogen supplements on bone health, sexual and reproductive health, quality of life and markers of inflammation and lung function when given to hypogonadal women with Cystic Fibrosis related Bone Disease (CFBD).
Randomized, double blind, placebo controlled study. The study has two parts: Dose-finding part, followed by longer term follow-up (6 months)
This is a crossover study to determine the possible factors that might be related to the development of diabetes in CF and examines the relationship between pancreatic fatigue if any, and the development of diabetes. In addition hormones affecting the release of insulin and stomach motility will be studied to determine if there is a variation in response to solid and liquid meals and if this variation increases as fatigue progresses.
The Effectiveness of the Jamboxx Respiratory Therapy Device in Treatment of Patients with Decreased Respiratory Function is a proposal for investigation of the application of gaming to improving respiratory health. The Jamboxx device combines gaming with traditional incentive spirometry to provide users with a fun experience to keep them engaged in their respiratory therapy routine. The device allows users to play a series of mini-games that walk them through their routines. The Jamboxx also records airflow and lung parameters with an external mouthpiece attachment to provide users with real time feedback, and helps to assess increases or decreases in relative lung function over time. The Jamboxx has the potential to significantly impact the field of respiratory therapy by being one of the first gaming devices for patient therapy, and the first respiratory therapy gaming device that is accessible to users with limited mobility. Jamboxx provides a fun and engaging, low cost alternative to the traditional therapy techniques used and aims to improve patient compliance. This study addresses the ongoing challenge of clearing the burden of bronchial secretions resulting from cystic fibrosis. These patients are dependent upon mechanical devices to help clear secretions. A device autonomous means for clearing secretions is well defined in the literature (autogenic drainage) but is difficult to learn. This study proposes to teach cystic fibrosis patients to master autogenic drainage, and seeks to determine at what age it can be taught. As it would be expected that gaming could easily teach adults this procedure, the study will include children for whom learning autogenic drainage based upon conceptualization would be expected to be very difficult.
Intrapulmonary Percussive Ventilation (IPV) will show greater improvements in Forced Expiatory Volume in 1 second (FEV1) shorter duration of stay, and greater time to next pulmonary exacerbation in pediatric Cystic Fibrosis patients admitted for pulmonary exacerbation. FEV1 is a measurement of obstruction in the airway which is seen to be elevated in CF patients with worsening lung disease. FEV1 is used as a primary endpoint in pulmonary trials because it is an acute indicator of worsening or improving lung function.
A Safety Run-In will be followed by a Double-Blinded Randomized Phase. All subjects shall meet the inclusion/exclusion criteria, and will be evaluated prior to the scheduled infusion to establish baseline. There will be 3 subjects in the safety run-in phase and 15 subjects in the double-blinded phase.
A qualitative study assessing the impact of early narrative medicine practice on Medical Honors Program (MHP) students' attitudes regarding patient-centered interactions, through interviewing patients with chronic or life-limiting illnesses to obtain their illness stories. MHP students will develop a patient narrative for the patients interviewed. These narratives will be edited by the patient, and, with the permission of the patients, may be published as a collection of stories.
The purpose of this research study is to find out if the combined therapy lumacaftor-ivacaftor effects how people with cystic fibrosis respond to an oral glucose tolerance test, a test for diabetes.