Active clinical trials for "Cystic Fibrosis"

The purpose of this research study was to determine if an experimental drug called Aztreonam for Inhalation Solution (AZLI) was safe and effective to treat Burkholderia lung infections in patients with cystic fibrosis (CF). Spirometry was used to assess pulmonary function, and the revised Cystic Fibrosis Questionnaire (CFQ-R) was used to assess quality of life. The CFQ-R is a validated, patient-reported outcome tool used to measure health-related quality of life for children and adults with CF. The study consisted of a 24-week randomized phase, and a 24-week open-label phase. Primary and secondary efficacy analyses were conducted for the 24-week randomized phase only. Safety data were collected for both the randomized and open-label phases.

A study to evaluate long term safety and effect on ability to thrive of EUR-1008 (APT-1008) 3,000 lipase units (Zenpep® [pancrelipase] delayed release capsules) in infants with exocrine pancreatic insufficiency (EPI) due to cystic fibrosis (CF).

The purpose of this study is to examine the role of a well-known and well-tolerated antibiotic, doxycycline, in the treatment of cystic fibrosis patients who are hospitalized. This antibiotic does not effectively treat the bacteria in airways of cystic fibrosis patients, but may reduce the activity of inflammatory molecules in the disease.

The aim of this study is to test the effect of a 6-month program of self-regulated, home-based exercise with telephone reinforcement on the cardiorespiratory fitness, pulmonary function and health-related quality of life of children with cystic fibrosis (CF), compared to controls. Exploring the exercise experiences of the children and parents is a secondary aim.

The purpose of this study is to determine whether sildenafil can decrease inflammation in CF lung disease.

Nonadherence to treatment regimes among patients with cystic fibrosis (CF) is well documented in the literature. Unfortunately, few interventions have been successful at improving adherence rates in this patient population. Within the pediatric population, specifically adolescents, attitude toward treatment directly effects adherence to treatment. Changing attitude includes making conscious decisions regarding adherence or non-adherence and should include an understanding of what outcomes will likely result from nonadherence. This study will determine if attitudes can be enhanced and adherence to treatment regimen improved in Cystic Fibrosis (CF) adolescent patients by using a simulation game "My Life with CF" . The target population is CF patients ages 12-21 years. Attitudes will be assessed utilizing four attitude scales in CF adolescents pre and post experiencing the simulation game. In the simulation game, patients make life decisions, including CF therapy decisions, using a fictional character diagnosed with CF. The game has been designed to help players understand the long-term effects of health care choices. Data from this pilot study will assess changes in attitudes and reported changes in adherence.

This is a dose-finding study for the investigational product Lancovutide (Moli1901) in the exploratory phase IIb to establish minimum effective dose, optimal dose, and maximum safe dose. Additionally, the tolerability of Moli1901 shall be investigated.

Patients with cystic fibrosis (CF) suffer from chronic infections of the lower respiratory tract that can be caused by one or multiple bacteria, including Pseudomonas aeruginosa, which has been particularly problematic to eradicate and been implicated as the major cause of morbidity and mortality in CF patients. Aerosol delivery of antibiotics directly to the lung increases the local concentrations of antibiotic at the site of infection resulting in improved antimicrobial effects compared to systemic administration. Bacterial resistance to current aerosol antibiotic treatments indicate a need for improved therapies to treat CF patients with pulmonary infections caused by multi-drug resistant Pseudomonas aeruginosa and other bacteria. High concentrations of MP-376 delivered directly to the lung are projected to have antimicrobial effects on even the most resistant organisms.

Cystic Fibrosis (CF) is a complex disease with a wide range of clinical problems. Despite enzyme replacement therapy, children with cystic fibrosis (CF) may still have problems absorbing some nutrients. Detailed studies of the nutrient status of children with CF and have found low amounts of choline, an essential dietary nutrient, and altered levels of some amino acids in almost all patients. Choline is an essential dietary nutrient that is important in many important body functions, which include proving a source of methyl groups, the structure of cell membranes and in acetylcholine. Most choline is present in our diets in a fat known as phosphatidylcholine. Research studies have shown that children with cystic fibrosis do not absorb fat, including phosphatidylcholine very well. In previous studies, we showed that choline provided as a dietary supplement for 2 weeks improved choline status in children with cystic fibrosis. The purpose of this research is to find out if choline supplements over a longer duration of 6 months will improve and maintain normal choline status in children with CF.

This study is a preliminary study designed to determine the safety and effectiveness of HFCWO using the InCourage vest system as an airway clearance technique for the treatment of CF. The study will compare HFCWO to the most commonly used airway clearance technique in Canada, namely the Positive Expiratory Pressure technique in patients with CF over a period of one year. As this is a preliminary study, it will not be able to determine if the vest is equivelant to PEP, but will provide the information needed to plan a larger study to answer that question.