Effect of Sulforaphane in Broccoli Sprouts on Nrf2 Activation
Cystic FibrosisThe purpose of this study to investigate the effect of sulforaphane from macerated broccoli sprouts in humans and to evaluate less invasive methods of assessing potential anti-inflammatory drugs in CF.
Efficacy of Amiloride and Hypertonic Saline in Cystic Fibrosis
Cystic FibrosisThe purpose of this research study is to determine whether the combination of inhaled amiloride and a concentrated salt solution is better than the salt solution itself for cystic fibrosis (CF) patients. In CF, airway secretions are thick and dehydrated. Many patients use inhaled salt solutions to help draw water into their secretions so that they are easier to get rid of with chest physiotherapy ("chest PT") and cough. Unfortunately, these salt solutions are reabsorbed very quickly by the airways, so the beneficial effects may not last very long. In the hopes of prolonging their effects, the drug amiloride could be used in combination to slow salt and water reabsorption from airways. Amiloride is a medication that has been given by mouth for high blood pressure for many years. It is possible that the combination of salt solutions and inhaled amiloride may significantly improve the clearance of secretions in CF, which would be expected to improve lung function in CF.
Building Adherence to Live With And Navigate my Cystic Fibrosis (CF) Experience
Cystic FibrosisThe investigators research group and others have found that patient-reported adherence to all aspects of the regimen is suboptimal and objective measures suggest even poorer adherence. There is little data, however that identifies and examines the reason for nonadherence in an adult Cystic Fibrosis (CF) population or identifies effective strategies for improving adherence. The investigators propose to evaluate the relative efficacy of a Motivational Interviewing-focused intervention (MI) in improving adherence and reducing CF-related morbidity compared to a CF education intervention (CFE; attention control group). Specifically, The investigators hypothesize that MI will result in improved regimen adherence and reduced CF morbidity compared to the CFE control group.
Nutritional, Metabolic and Respiratory Status in Cystic Fibrosis
Cystic FibrosisDiabetesDiabetes is a important complication of cystic fibrosis (CF). The improved life expectancy of patients with cystic fibrosis, as a result of advances in medical therapy, has resulted in an increasing prevalence of cystic fibrosis-related diabetes (CFRD). CFRD is associated with accelerated pulmonary decline and increased mortality. Pulmonary effects are seen some years before the diagnosis of CFRD implying that impaired glucose tolerance may be very early detrimental. Insulin treatment is clearly indicated in patients with CFRD to control symptoms and reduce complications. However, at the state of impaired glucose tolerance or fasting hyperglycaemia, current screening methods are not suitable for the early management of hyperglycaemia.The recent introduction of the continuous glucose monitoring system (CGMS), which provides a continuous glucose profile, has revealed to be clinically relevant in the investigation of glucose excursions over a long period. This device, widely use in diabetic non cystic fibrosis patients, has been validated in non diabetic cystic fibrosis subjects. Previous studies of continuous glucose monitoring have been realized in CF patients with normal glucose tolerance and diabetes and compared with non CF controlThe aim of our study is to evaluate the glucose profile with continuous glucose monitoring the nutritional and respiratory status in cystic fibrosis subjects, according to their glucose tolerance.
Tele-Coaching Intervention to Improve Treatment Adherence in Cystic Fibrosis
Cystic FibrosisThis is a prospective, multicenter pilot study to investigate the feasibility and preliminary effectiveness of a tailored tele-coaching intervention to enhance medical adherence in patients with CF.
Investigating the Use of a Positive Parenting Programme to Improve Treatment Adherence in Cystic...
Cystic FibrosisCystic fibrosis (CF) is an illness that makes the lungs clog up with sticky mucus. There is no cure and so treatments are used to help make the illness easier to live with. The treatment can take lots of time and can feel not very nice sometimes. When children with CF become teenagers they need to learn to take charge of their treatment. This can be difficult. Teenagers with CF want to fit in with friends and can become more upset about their illness. Their parents have to learn to let their child take charge of their illness which can be hard for parents. These issues can put strain on parent-child relationships and this can make it harder for teenagers to stick to their treatment plans. A parenting program (called Teen Triple P) has been shown to help teenagers with other illnesses (such as diabetes) to be able to stick to their treatment plans. Parents are given a booklet to work through at home which helps them to build on the skills they already have. It aims to help families to support positive parent-child relationships, to manage difficult teenage behaviours, and to teach new skills and behaviours. So far no one has done any research to see if this program helps families of teenagers with CF. This research would like to see if the Triple P program can help teenagers with CF stick to their treatment plan. Helping teenagers stick to their treatment plan will help them to live happier and healthier lives.
Steady-state Pharmacokinetics of Ceftazidime/Avibactam in Cystic Fibrosis
Cystic FibrosisThe purpose of this research study is to characterize the pharmacokinetics of intravenous ceftazidime/avibactam in patients with Cystic Fibrosis.
Integrating Supportive Care in Cystic Fibrosis
Cystic FibrosisIndividuals living with cystic fibrosis (CF) commonly report high symptom burden, poor quality of life, and additional psychosocial stressors; these burdens are particularly heightened in advanced stages of the disease. Although supportive care (aka palliative care) has been shown to improve many of these outcomes among patients with illnesses such as cancer, no clinical trials to date have tested the impact of supportive care for patients with CF. The purpose of this pilot randomized clinical trial study is to evaluate the feasibility, acceptability, and perceived effectiveness of an embedded supportive care intervention, whereby a supportive care specialist will be integrated within the usual care experience of patients with advanced CF. The investigators will enroll 50 patients with advanced CF, who will be equally randomized to receive this embedded supportive care intervention or usual care. Secondary measures include: patient quality of life, mood, coping style, satisfaction with care, and symptom burden. This study will provide preliminary data to support the development of a larger, definitive, Phase III randomized clinical trial.
To Assess the Safety, Tolerability and Pharmacokinetics of AZD5634 Following Inhaled and Intravenous...
Cystic FibrosisThis is a Phase 1, first-in-human (FIH) single ascending dose study being conducted to better understand the safety, tolerability and pharmacokinetics of AZD5634 in healthy subjects
Pharmacokinetics of FDL169 in Healthy Female Subjects
Cystic FibrosisTo determine the pharmacokinetics of single and multiple doses of FDL169 in healthy female subjects.