Active clinical trials for "Cystic Fibrosis"

The purpose of this research study is to learn about the activity of oral Pancrecarb® (a pancreatic enzyme preparation which contains proteins that help to digest food), administered by mouth as a capsule filled with specially coated granules in patients taking exogenous pancreatic enzyme therapy. Specific enzymes activities will be determined from samples of stomach and intestinal fluids after a standard liquid meal.

The purpose of this research study is to test a new combination of medicines, Phenylbutyrate and Genistein, to determine if they could be used to treat cystic fibrosis (CF). The most common genetic mutation found in patients with CF is called Delta F508. Due to this mutation, there is a lack of salt (chloride) movement in your nose, sinuses, lungs, intestines, pancreas and sweat glands. This lack of movement causes the clinical manifestations of the disease. Although Phenylbutyrate has been extensively used to treat patients with rare metabolic diseases, Phenylbutyrate is an investigational drug for the purpose of this study. Genistein is a naturally occurring substance that is found in food products such as soy and tofu, but is also an investigational drug for this study. When used together, both drugs may be able to restore normal chloride and salt (water) movements in body organs and glands in people with CF. We will be studying salt and water movement in the nose by a technique called nasal transepithelial potential difference (NPD).

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.

This is a project that will determine whether the use of daily bright light therapy has an effect on depressive symptoms experienced by adult inpatients with CF and COPD. The purpose of this project is to apply a daily 30-minute BLT intervention to hospitalized adult CF and COPD patients in order to decrease symptoms of depression as measured by depression inventory scoring.

Meropenem is an intravenous antibiotic commonly used to treat acute exacerbation of respiratory infections in cystic fibrosis. The research study aims to determine if a different method of infusing the drug over 3 hours or longer, instead of the traditional half-hour will improve target attainment of drug concentrations and bactericidal activity. A secondary aim is to assess if the pharmacokinetics of meropenem is different during active infection compared to non-infective stage. Twelve patients admitted with acute respiratory infection and who requires meropenem will be enrolled into the study. Meropenem blood concentrations collected over 8 hours will be measured after half-hour and 3-hour infusions on different days. A pharmacokinetic modelling and Monte Carlo simulation program will use the data to assess and predict the optimal method of dosing. When patients return for a follow-up clinic visit, a single dose of meropenem will be administered and blood concentrations will be measured to determine the pharmacokinetics during non-infective stage.

This is an add-on, randomized, open label, clinical trial that evaluates the use of quadriceps electrostimulation as an additional retraining procedure in patients suffering from cystic fibrosis.

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.

Cystic Fibrosis is a hereditary, chronic respiratory illness. Cystic Fibrosis leads to a progressive decline in lung function. School-aged children with cystic fibrosis experience backpack carrying everyday. Backpack carrying induce a restrictive effect responsible for lower lung function. Respiratory muscle strength is also impaired. No studies assessed aerobic capacities during children's gait while carrying a backpack. The investigators hypothesized that backpack carrying will induce an acute decline in lung function in children with cystic fibrosis compared to healthy children. Investigators also hypothesized that aerobic capacities will be impaired.

Randomized, double blind, placebo controlled study. The study has two parts: Dose-finding part, followed by longer term follow-up (6 months)

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.