Active clinical trials for "Cystic Fibrosis"

This is a pilot study. The purpose of the study is to facilitate the development of a new biomarker of cystic fibrosis transmembrane conductance regulator (CFTR) function using rectal tissue.

This study will be performed at the University of Washington. Forty subjects with Cystic Fibrosis will be enrolled, with 20 randomized into the usual care arm and 20 randomized into the intervention arm. All subjects will be enrolled for 6 months and have 3 study visits. One at baseline, midpoint, and final. The intervention arm subjects may have more study visits depending on their respiratory symptoms. The intervention arm subjects will perform home spirometry twice a week with a PiKo-6 hand held spirometer. They will also have a home-based telemonitoring system connected to their phone line. They will be prompted twice a week to answer questions regarding their health via the telemonitor. Subjects who are not able to utilize the home-based telemonitoring system will answer the questions regarding their health via the internet from their home computer. If the subject's spirometry falls by greater than 10% from baseline or the CF respiratory symptoms change in 3 or more of the 8 respiratory symptoms captured by the telemonitor questionnaire, the subject will be called by the research staff and clinically evaluated by the study PIs within three days. The usual care subjects will continue with their routine care at the University of Washington CF Clinic.

Inhaled medications are often used to treat lung diseases such as cystic fibrosis. We are performing this study to determine whether inhaled medications dissolved in surfactant-based solutions will distribute more evenly throughout the lungs when compared to standard saline-based solutions. We think that inhaling medication that is in a surfactant-based liquid will result in more medication reaching partially blocked parts of the lung. This study will use a special nuclear medicine test called an aerosol deposition scan to compare how a drug spreads in the lung using a surfactant-based aerosol compared to a saline-based aerosol.

The purpose of this study is to investigate how mucus (phlegm or spit) is broken down once it forms in the airways (bronchial tubes) of people with lung disease. This research study will also examine whether blood groups have an effect on lung function or the type of mucus found in the lung. This study is not designed to be a treatment for asthma, emphysema, cystic fibrosis, or other lung disease. It is designed to help the investigators learn more about the causes of airway disease.

The objective of this study is to assess the effects of using HRQL measures in the clinical care of pre- and post-lung transplant patients. The hypotheses are that the inclusion of HRQL measures, the Health Utilities Index System Mark 2(HUI2) and Mark 3 (HUI3), in routine clinical care of pre- and post-lung transplant patients, will: 1) improve patient-clinician communication;2) affect patient management; 3) improve patients' HRQL.

Lung transplantation is a life saving procedure for patients with a terminal lung disease such as cystic fibrosis. Approximately, one in 3,500 children in the United States are born with cystic fibrosis each year with the predicted survival reaching 36.9 years in 2006. Cystic fibrosis was the third lead indication for lung transplantation in 2006. Cystic fibrosis is a genetic disease that can affect the way the body can remove salt from various organs. It results in mucus blocking the ducts of the lungs and pancreas leading to inability to handle oxygen and malabsorption of nutrients. Malabsorption is a common complication of cystic fibrosis that can affect the way the anti-rejection medications are absorbed. One medication that is utilized after transplant to prevent rejection is mycophenolate mofetil. This medication may not be absorbed adequately in this population due to their disease thus placing these patients at increased risk of rejection. At the investigators' institution, all transplant patients are initiated at the same mycophenolate dose regardless of their underlying disease. The limited available literature regarding cystic fibrosis transplant patients and mycophenolate suggests that these patients require higher doses due to their erratic absorption. The purpose of this study is to evaluate the effects of mycophenolate mofetil on the body in lung transplant patients who have cystic fibrosis in efforts to improve survival outcomes.

Chronic bronchial inflammation is an important clinical feature in cystic fibrosis. Approximately 10% of patients with cystic fibrosis suffer from Allergic Bronchopulmonary Aspergillosis. In addition airway inflammation in patients with cystic fibrosis (CF) plays a major role in progression of CF lung disease. In patients with mild disease (Vital capacity >75%) airway inflammation is often under diagnosed. Severity of allergy against Aspergillus fumigatus will be examined using radioallergosorbent test and skin Prick-test. Subsequently, in patients with established sensitization (RAST ≥ 0.35 IU/mL) a specific bronchial provocation with Aspergillus will be performed. In addition, exhaled nitric oxide,carbon monoxide, exhaled air temperature and inflammatory cells in sputum is measured. 24 hours after bronchial allergen provocation, exhaled NO, CO, air temperature, and bronchial responsiveness is determined and a second sputum obtained. This study is designed to characterize patients with CF and sensitization against Aspergillus fumigatus in an early stage to prevent pulmonary complications of ABPA. In addition sputum cytokine profiles in CF patients with mild and moderate disease may be different in patients without and with involvement of small airway disease (SAD).

This study will examine 1) the role of hereditary factors in cystic fibrosis; i.e., the relationship of the disease to specific gene variations, and 2) the role of bacterial products involved in lung infections substances produced by bacteria may worsen the disease. Patients with cystic fibrosis who are being followed by the Medical College of Wisconsin or the University of Wisconsin-Madison are eligible for this study. Participants will have blood tests, pulmonary function tests, a sputum culture, and buccal swabbing (cotton swabbing of the inside of the cheek to collect cells for DNA study). In addition, their medical records will be reviewed for a history of lung infections and the results of various tests, including pulmonary function studies, chest X-rays and bacterial cultures. Blood samples collected previously at the Medical College of Wisconsin or the University of Wisconsin-Madison will also be analyzed for antibodies to bacteria. Although this is a one-time study, participants may be asked to return for repeated tests. ...

Some bacteria that cause disease can produce toxic substances that may worsen the disease. Pseudomonas aeruginosa is a bacteria that can produce a variety of toxins and is of special interest for patients with cystic fibrosis and repeated long term lung infections. The goal of this study is to determine whether specific toxins produced by Pseudomonas aeruginosa may be important in the disease process of chronic lung infections of patients with cystic fibrosis. This study will attempt to measure bacterial production of toxins in blood and sputum and immune system response to toxins in the blood....

With better medical care, patients with cystic fibrosis (CF), a life-threatening disease, are enjoying longer lives. As the CF life expectancy increases, conditions such as CF-related diabetes (CFRD) become more prevalent. Nutrition plays a major role in maintaining optimal health in cystic fibrosis (CF). This project is designed to investigate nutrition-related factors, such as diet and body composition, on outcomes in patients with CF. The data generated from this study will be used to inform future nutrition intervention studies in adults with CF and CFRD.