View clinical trials related to Cystic Fibrosis.
Filter by:Cystic fibrosis is a rare chronic genetic disease that mainly affects the respiratory tract and the digestive system. Their management includes multi-year intravenous antibiotic treatments and repeated venous sampling. The venous access is a source of difficulties that nurses who take care of these patients face on a daily basis. In addition, multiple attempts at punctures can induce anxiety and pain in patients. It is therefore important to limit failures. Vein visualization technologies exist: guidance echo, portable trans lumination or infrared visualization can guide venipuncture and limit failures. Compared with the guided echo or the portable trans lumination, the infrared visualization is easy to use and does not pose a risk for the patient. Nevertheless, studies evaluating this technique are few in chronic diseases and mainly conducted in young children. This study aims to show that the use of a vein illumination system (VIS) should improve the peripheral venous access at the first attempt (thus limiting venous lesions) in adolescent and adult patients with cystic fibrosis, and improve comfort of the patient (pain, apprehension of the gesture).
The intimate life of patients with cystic fibrosis has not been the subject of specific research, it is even left behind in favor of respiratory, digestive or endocrinological dysfunctions endangering these patients. After 16 years of practice, it is significant that this subject is problematic, painful but difficult to tackle; No doubt just as much on the side of caregivers: this question has never found its way into team exchanges, it seems to be evaded doubtless difficult or too intimate to welcome. These disorders arise from the effects of the disease on the health of the mucous epithelial tissues also located in the genital area. These difficulties are more closely approached by the difficulties of procreation; PMA allows them to get around them, nevertheless making it possible to be a parent, with the residual frustration of a satisfactory sex life: a deaf "addition", adding to many care constraints. It is therefore in terms of sexual health and quality of sexual life that it is desirable to shed light on this aspect of cystic fibrosis in women. Bibliographic research confirms this approach: this subject is not explored, the rare publications concern the vulnerability of the cervix or the difficulty on both sides of tackling this subject.
This study aims to assess the role of some factors which can influence the chest physiotherapy adherence in adult with cystic fibrosis. At first, we are going to try to consider the adherence of chest physiotherapy. Then, after dividing the patients into two groups - adherent or not adherent - we will attempt to identify the factors which can influence this adherence. It consists in a short questionnaire that the patient will fill during usual visits. The hypothesis is that the following factors can play a role in adherence of chest physiotherapy: - Age - Age of diagnostic - Sex - Fev1 - Number of antibiotic course - Anxiety/depression - Work time - Socio-professional category - Individual situation - Physiotherapy with liberal physiotherapist and transport time - Sport practice - Family support - Feeling of work time burden - Feeling of physiotherapy efficiency - Feeling of physiotherapy burden
The investigators will recruit 15 patients with cystic fibrosis 18 years of age and older who present with constipation. The investigators will assess baseline motility symptoms with a survey. Patients will then ingest a SmartPill (trademark) to obtain baseline motility within the GI lumen. All patients will undergo intervention with taking polyethylene glycol (PEG) or Miralax (brand name) 17 grams once daily. After two weeks of therapy, the patient will repeat the motility survey and again ingest a smart pill to assess the change in motility symptoms while on therapy.
This study will investigate the contribution of SIMEOX technology on the effectiveness of bronchial drainage. This is a crossover study to evaluate the contribution of SIMEOX on the effectiveness of bronchial drainage (verified by the amount of sputum secretions, the rheology of sputum secretions and the subjective sensation of ease of sputum) in patients with cystic fibrosis. Patients will perform, in randomized order (1) a 30-min session of autogenic drainage, (2) a 30-min session of autogenic drainage with the SIMEOX device. Sputum will be collected during and after the session. The two sessions will be performed with minimum washout time of 24 hours.
This study will evaluate the long-term safety, tolerability, efficacy, and pharmacodynamics of elexacaftor (ELX, VX-445) in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF).
Cystic Fibrosis (CF) is the most common autosomal recessive lethal disorders affecting 1:2.500 newborns among Caucasians. CF patients are peculiarly susceptible to infection and colonization of the respiratory tract with pathogens. In particular, Methicillin-resistant Staphylococcus aureus (MRSA) has become the third most prevalent bacterium in CF in the U.S. and has been increasing in other countries. Apart from the difficulty of treating the infection because of its antimicrobial resistances, MRSA is transmissible between individuals with and without CF. Chronic MRSA infection is associated with worse outcomes, and treatment/eradication is challenging. Antibiotic dosing and choices should be optimized to minimize further resistance and to maximize chances of successful therapy. Yet, MRSA has several mechanisms to escape clearance by the immune system and antibiotic killing. For these reasons, a better understanding of preventive measures and early therapy is of key importance. In consideration of all these assessments there is an emerging consensus that MRSA is an important pathogen in CF rather than simply a marker of severe disease. However, to date there are no guidelines or recommendations on the choice of antibiotics for MRSA in CF. Glycopeptides are an important class of antibiotics active against Gram-positive pathogens. These include teicoplanin and vancomycin, which are currently in widespread use and are active against MRSA. Teicoplanin is often preferred to vancomycin for intravenous treatment because of its better safety profile but its use in MRSA lung infection is limited by its limited lung penetration. Teicoplanin is mainly used for injection/infusion. Inhalation of anti-microbial drugs is a cornerstone in the treatment of patients with CF, since inhaled antibiotics decrease the rate of decline of lung function, improve the quality of life, and reduce the frequency of exacerbations and hospital admissions. It is expected that, using inhalation route, efficacy would be improved and risk of resistance reduced. At present, no antibiotic active against MRSA is available as an inhaled formulation. The objective of this phase I, first-in-man clinical study is to identify the dose providing, after single inhalation administration, a sputum Teicoplanin concentrations exceeding the drug concentration required to inhibit bacterial growth for at least 8 hours, while minimizing the development of resistance.
Recently, the investigators described a new mucoid phenotype of Staphylococcus aureus cultured from the airways of cystic fibrosis (CF) patients.In this observational study, the investigators plan to determine the prevalence of mucoid S. aureus in respiratory specimens of CF patients and a possible impact of mucoid S. aureus on lung disease severity.
The effect of comprehensive respiratory physiotherapy applications on respiratory function, functional capacity and peripheral muscle strength in children with cystic fibrosis and non-cystic fibrosis will be compared.
The aim of this study is to compare pulmonary function, respiratory muscle strength, exercise capacity and peripheral muscle strength of patients with CF, PCD and healthy childrens.