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Being at or above the 50th percentile body mass index (BMI) for age and gender in children with cystic fibrosis (CF) is associated with better lung functioning as measured by FEV1, yet diet is one of the least adhered to components of the CF treatment regimen. Investigators at Cincinnati Children's Hospital Medical Center (CCHMC) have developed an efficacious behavioral plus nutrition education program (Be In Charge) that improves adherence to dietary recommendations, and promotes weight gain in children with CF. To make Be In Charge (BIC) widely available to families of children with CF ages 3 to 10 years, the investigators translated the face-to-face intervention into a 10-week, web-based intervention (BeInCharge.org). The investigators tested it in a pilot study and the results were promising. In the first phase of the current study, the investigators worked with a team of clinicians, parents and technology developers to extend the usability and functionality of the web intervention, enable parent-clinician collaboration, and support concurrent use across multiple clinical sites. The long term goal of this research is to make BeInCharge.org available through CF Centers across the country to patients that would benefit in order to improve dietary adherence. The current phase of this protocol is a prospective, multicenter, nonrandomized study enrolling up to 150 parents of children with CF. Participants will complete the Be In Charge program outside of CF clinic on their own time. CF Center clinicians will be able to follow the participant's progress via the Be In Charge clinician dashboard. CF center clinicians will be asked to support participating families in completing the program as clinically appropriate. The primary study objectives are to: 1. Demonstrate that it is feasible and acceptable to use the Be In Charge program in clinical care and with fidelity to intervention parameters. 2. Demonstrate preliminary effectiveness on calorie intake and weight outcomes when the Be In Charge program is integrated into clinical care with implementation support for care teams. The secondary objective is to develop a well-defined, tested set of implementation strategies consolidated into a change package and an optimized technology platform that will support a dissemination trial for spreading the Be In Charge program across CF Centers.
This study will evaluate the pharmacokinetics (PK), safety, tolerability, efficacy, and pharmacodynamic effect of VX-659, tezacaftor (TEZ), and ivacaftor (IVA) when dosed in triple combination (TC) in Cystic Fibrosis (CF) subjects with F/F or F/MF genotypes.
This pilot interventional cohort study will examine the effects of intravenous iron in adults with cystic fibrosis and iron deficiency.
The impact of high-frequency chest wall oscillation therapy on spirometry values (Forced Expiratory Volume, Forced Vital Capacity, Peak Expiratory Flow, Forced Expiratory Flow and Tidal Volume is investigated during use of several products and comparing to baseline values
This study will explore the impact of lumacaftor/ivacaftor (LUM/IVA) on disease progression in subjects aged 2 through 5 years with cystic fibrosis (CF), homozygous for F508del (F/F)
Based on previous clinical findings, we hypothesize that ivacaftor will have synergistic effects with drugs that facilitate truncated but partially active W1282X CFTR protein processing (tezacaftor) in patients with W1282X CFTR. In the current study, we propose to directly test the efficacy of tezacaftor/ivacaftor (TEZ/IVA) for W1282X CFTR therapy in the clinic in comparison to ivacaftor alone.
Description of the presence of cell-free fetal DNA in maternal plasma allowed the possibility of non-invasive prenatal diagnosis. Whereas detection of paternally-inherited alleles is straightforward and being quickly implemented in routine, detection of maternally-inherited alleles remains challenging. To date, the main approach that is being developped, called Relative Haplotype Dosage Analysis, relies on the identification of an allelic imbalance between the mother's wild-type and mutant alleles, relative to the fetal's contribution. This approach therefore requires the study of a propositus to identify the morbid haplotype, which is not always possible in the context of an ongoing pregnancy. In this study, we aim to evaluate the contribution of new technologies, such as linked-read Sequencing, to allow direct identification of parental haplotype in the context of non-invasive prenatal diagnosis.
This study is testing a non invasive way to measure airway pH in individuals with Asthma and Cystic Fibrosis using a new inhaled drug. The airway pH will help health care providers in creating tailored treatment plans for individuals suffering from these specific conditions.
The aim of this observational trial is to evaluate the pulmonary function in cystic fibrosis patients that have been treated with inhaled aztreonam lysine comparing the previous 12 months before the treatment and the forward 12 months after initiating the treatment.
This study will evaluate the safety and pharmacokinetics (PK) of lumacaftor (LUM) and ivacaftor (IVA) in subjects 1 to less than 2 years of age with cystic fibrosis (CF), homozygous for F508del (F/F).