View clinical trials related to Cystic Fibrosis.
Filter by:The purpose of this study is to evaluate the effect of ivacaftor (VX-770) on lung clearance index (LCI) in subjects aged 6 years and older with cystic fibrosis (CF) who have the G551D-CFTR mutation on at least 1 allele.
The purpose of this study is to determine whether the dipeptidyl peptidase IV (DPPIV) inhibitor sitagliptin is effective in the treatment of cystic fibrosis-related diabetes (CFRD). We hypothesize that sitagliptin will improve meal-stimulated insulin secretion.
UCSD researchers are conducting a study aimed to develop and evaluate a chronic disease self management web and text message based program on health-related self-efficacy and frequency of adolescent-conducted healthcare interactions. We hypothesize that users of the program will demonstrate greater gains between baseline and 8 month measures of health related self-efficacy and adolescent-conducted healthcare interactions as compared to the usual care comparison group.
The purpose of this research study is to perfect the technique of EVLP and learn about the safety of transplanting lungs that have been ventilated (attached to a breathing machine or ventilator to deliver oxygen) and perfused with a lung perfusion solution (Steen solution™, made by Vitrolife). This ventilation and perfusion will be done outside the body (ex-vivo) in a modified cardiopulmonary bypass circuit (the kind of device used routinely during most heart surgeries). The purpose of performing ex-vivo lung perfusion and ventilation (EVLP) is to learn how well the lungs work, and whether they are likely safe to transplant.
Pulmonary exacerbations of CF are an important cause for the experienced disability of patients, respiratory symptoms, and decreases in lungfunction, which require antibiotic therapy at home or in the hospital. Therefore, prevention of exacerbations in CF is important. The aim of this study was to assess the predictive properties of inflammatory markers in exhaled breath for pulmonary exacerbations in children with CF. In addition the reliability of home monitor assessments of symptoms and lungfunction was investigated.
Respiratory viruses, such as those that cause colds and influenza, are very common in the general population. The average adult will experience between 2 and 4 viral respiratory illnesses each year. It is known that respiratory viruses can cause flare-ups of chronic lung diseases such as asthma and chronic obstructive pulmonary disease. In children with CF, respiratory viral infections have been identified in nearly half of all exacerbations and are associated with a decline in lung function and increased frequency of hospital admission and acquisition of new bacterial pathogens. Currently, little is known about the impact of viral infections on the course of CF lung disease in adults and no large-scale prospective studies in this area have been performed to date. It is unknown how often respiratory viruses can be found in patients with CF when they are well and what consequences they have on the course of exacerbations of CF lung disease. This study will identify the frequency of common viral infections in adults with CF and determine the effects they have on lung function, the rate and diversity of bacterial infection and patients' treatment burden. The information gained from this study will lead to improved prevention and treatment of respiratory infections in CF.
The iCARE study, a clustered-randomized controlled trial, is evaluating an adherence promotion intervention for adolescents that Cystic Fibrosis (CF) care teams will implement. Half the centers will receive the Comprehensive Adherence Program (CAP) for 2 years. CAP consists of training in the CF My Way program (a validated problem-solving adherence promotion intervention). The other half of the centers will receive CAP in year 2 of the study. Participants are patients age 11-20 years old who are diagnosed with CF and have been prescribed at least one of the following medications for at least 6 months prior to signing the informed consent: azithromycin, hypertonic saline, Pulmozyme®, TOBI®, or inhaled compounded tobramycin. Rate of refilling prescriptions is the primary outcome with lung function decline rates, exacerbation rates, and patient reported measures including health related quality of life and CF knowledge and skills, as secondary outcomes. A central goal of this study is to test the effectiveness of the comprehensive adherence program (CAP), described above, versus standard care (SC) for adolescents and young adults seen in outpatient CF clinics.
The primary objective of this study is to measure efficacy of our new protocol by monitoring the results of our routine respiratory cultures at the end of the new standard treatment, and during routine visits for 1 year from initiation of therapy for Staphylococcus aureus. The secondary objective will include determining the clinical course (pulmonary exacerbations, antibiotic use, hospitalizations, pulmonary function tests) of patients who underwent the protocol.
The purpose of this study is to evaluate of the safety, efficacy, pharmacokinetics (PK) and pharmacodynamic (PD) effects of lumacaftor (VX-809) alone and when coadministered with ivacaftor (VX-770) in participants with cystic fibrosis, homozygous or heterozygous for the F508del-CFTR mutation.
Blockage of the breathing tubes of the lungs by thick, sticky mucus is a major cause of lung problems for people with cystic fibrosis (CF). Many researchers now believe that people with CF absorb too much water from the insides of their lungs, and that the mucus in their lungs becomes so thick and sticky because there is not enough water in it. The investigators are trying to develop ways to measure how fast water is absorbed from the breathing tubes in the lung so that the investigators can more quickly test new medications that are being developed to fix this problem for CF patients. The investigators have already done studies showing that people with CF absorb a particular radioactive drug (Indium-111 diethylenetriaminepentaacetic acid or In-DTPA) from their lungs more quickly than people without CF. Now the investigators are trying to prove that the absorption of this drug is related to the absorption of water. The investigators measure the absorption of In-DTPA by delivering it in an aerosol (inhaled mist) along with another radioactive drug (Technetium 99m sulfur colloid or Tc-SC). This other drug helps us measure how much material is cleared from the lungs in other ways (like coughing) without being absorbed. In this study, the investigators will measure how the absorption of In-DTPA is affected by inhaling isotonic saline and hypertonic saline (salt water), both of which the investigators know affect the absorption of water in the airways.