View clinical trials related to Cystic Fibrosis.
Filter by:The purpose of this study is to test the efficacy of a peer support coaching intervention to improve activated chronic illness self-management versus an attention control group in 225 adolescents and young adults with childhood onset chronic conditions.
A double-blind, randomised study of OligoG DPI compared to placebo DPI, both on top of standard-of-care, to assess safety, efficacy and tolerability. Adult patients with Cystic Fibrosis will be included in the study.
This is an open-label, single center, prospective study of patients with a diagnosis of cystic fibrosis (CF) and a history of methicillin-resistant Staphylococcus aureus (MRSA), also known as oxacillin-resistant Staphylococcus aureus (ORSA), being treated with intravenous ceftaroline. Patients will have intravenous ceftaroline dosed according to current recommendations based upon its approved usage in pediatric patients for community acquired pneumonia. The investigators will analyze pharmacokinetic (PK) and pharmacodynamic (PD) data using serum concentrations of ceftaroline measured at various time-points following infusion of ceftaroline to develop PK/PD guided dosing strategies of ceftaroline in pediatric CF patients that would be tailored to account for bacterial susceptibility and disease progression.
It is estimated that one in every 3,600 children in Canada has cystic fibrosis (CF). CF is a genetic disease that affects the glands that produce mucus and sweat. In CF, mucus production increases and the mucus becomes thick and sticky. This can block the airways, making it difficult to breathe. Mucus production also causes bacteria to grow, which can lead to infections in the lungs. Individuals with CF suffer from shortness of breath, wheezing, cough, and poor exercise capacity. There are limited treatment options to reduce shortness of breath in these individuals. Some medications known as bronchodilators are commonly prescribed to reduce breathlessness in patients with CF. These drugs help open the airways making it easier to breathe. Unfortunately, there is limited scientific proof that these drugs can reduce shortness of breath and improve exercise capacity in patients with CF. As a result, some experts have recommended that these drugs should not be prescribed for patients with CF. The purpose of this study is to examine the effects of a bronchodilator on shortness of breath, exercise performance, and breathing responses compared to a placebo drug in adults with CF.
The aim of the study is to assess the diagnostic sensitivity of MRI to detect changes in Helbich-Bhalla scoring over time in patients with cystic fibrosis
There have been substantial advances in care for individuals with CF, including improved nutrition and respiratory care. Women with CF are now likely to survive into adulthood with a median predicted survival of 49.7 years in 2012, which have resulted in increasingly normal lifestyles, including successful pregnancies in women with CF. The number of pregnancies in CF women has increased as reported by the 2013 Annual Data Report from the French CF Registry (6 pregnancies in 1992 to 53 in 2012). Pregnancy adds many physiological stresses to the body, particularly on pulmonary function (decrease in residual volume, expiratory reserve capacity) and nutritional status (increase in nutritional needs). In women with CF, these added stresses could theoretically affect survival, with difficulties to maintain adequate nutrition and an unpredictable effect on lung function. A number of studies published over the past 15 years have attempted to clarify the risk of pregnancy in women with CF to guide clinicians and women. Many authors compared the outcomes of pregnant CF women to non-pregnant CF women regardless of nutritional status, lung function, diabetes mellitus, bacteriological colonization. In literature, pregnancy was possible and well tolerated in CF women with mild disease if associated with more intensive monitoring and aggressive treatment during pregnancy, with particular emphasis on nutrition and weight gain. CF women have an increased likelihood of receiving treatment for diabetes both during and after pregnancy. Furthermore diabetes mellitus has been associated with more severe pulmonary disease, more frequent pulmonary exacerbations, poorer nutritional status and a reduced life expectancy. Pre-pregnancy diabetes mellitus is associated with a poorer prognosis for pregnancy in general population. Severe forms of CF, including pre-pregnancy diabetes mellitus might as well do badly with accelerated decline after pregnancy. To determine the effect of pre-pregnancy diabetes on maternal decline after pregnancy, we will compare FEV1 and BMI rates of decline, during a two-years follow-up period after pregnancies, reported in the French CF Registry, according to their diabetic status.
Prospective, randomized, cross-over, multicenter, trial comparing the efficacy and tolerability of nasal irrigation with Respimer® mineral salts solution versus saline solution both administered with Respimer® Netiflow® medical device among patients aged ≥ 11 years and older with cystic fibrosis and suffering from chronic rhinosinusitis.
Cystic fibrosis (CF) is the most common fatal inherited condition in Caucasians, causing recurrent chest infections (pulmonary exacerbations). People with CF experiencing pulmonary exacerbation often require a 14day course of intravenous antibiotics and this treatment can either be delivered in hospital or in the community. Patients admitted to hospital are seen regularly by members of the CF multidisciplinary team (MDT), including doctors, nurses, dieticians and physiotherapists. This allows patients' progress to be closely monitored and also gives patients the opportunity to discuss any concerns or questions. In contrast, patients receiving IV antibiotics in the community are only seen by the MDT at the beginning and end of their 14 day course of IV antibiotics. Although CF nurses often visit patients on 12 occasions during the course of treatment, patients are not routinely reassessed by the rest of the MDT. There is therefore less opportunity for the MDT to adjust patients' treatment and for patients to communicate with the MDT. We therefore aim to study whether patients receiving IV antibiotics in the community benefit from monitoring their own oxygen saturations and lung function, as well as taking part in twice-weekly videoconferences with the MDT ('virtual care'). We expect that this will enable the MDT to to assess patients' progress more closely and allow patients to communicate with the MDT more easily. We will assess whether this improves patients' experience of their care and whether this translates into improvements in health and economic outcomes. One hundred subjects will be recruited over a 12month period, with 50 subjects allocated to 'virtual care' and 50 subjects allocated to 'routine care'. Subjects receiving 'routine care' will receive usual clinical care. The research team are well placed to perform the study because we are part of a large CF centre with an excellent record in clinical research.
Liprotamase powder is a non-porcine, soluble and stable mixture of biotechnology-derived lipase, protease, and amylase digestive enzymes. The purpose of the present study is to to evaluate the non-inferiority of liprotamase compared with porcine-derived, enterically-coated pancreatic enzyme replacement therapy (PERT). The primary efficacy endpoint of the study will be comparative efficacy measured as the change from baseline in the coefficient of fat absorption (CFA) in Cystic Fibrosis patients with exocrine pancreatic insufficiency (EPI).
The general aims of this project are (i) to identify predictive epigenetic biomarkers of lung disease severity in Cystic Fibrosis, (ii) to characterize a non-invasive cellular model, spontaneous sputum, for the analysis of these epigenetic biomarkers, (iii) to analyze the variations in DNA methylation for a same patient over time.