Qualitative understanding of experiences of people with cystic fibrosis in a treatment discontinuation trial: The QUEST study.

Background: As people with cystic fibrosis (PWCF) live longer due to the breakthrough drug elexacaftor-tezacaftor-ivacaftor (ETI), they have questioned whether other CF therapies could be safely discontinued. SIMPLIFY was the first prospective, randomized trial to evaluate non-inferiority of discontinuing versus continuing two therapies. The QUEST (Qualitative Understanding of Experiences in the SIMPLIFY Trial) study was conducted to understand experiences of PWCF enrolled in SIMPLIFY, including why they joined, perceptions of randomization, decision-making around study withdrawal, and considerations for future discontinuation studies.

Predicting lung function decline in cystic fibrosis: the impact of initiating ivacaftor therapy.

Background: Modulator therapies that seek to correct the underlying defect in cystic fibrosis (CF) have revolutionized the clinical landscape. Given the heterogeneous nature of lung disease progression in the post-modulator era, there is a need to develop prediction models that are robust to modulator uptake.

Methods: We conducted a retrospective longitudinal cohort study of the CF Foundation Patient Registry (N = 867 patients carrying the G551D mutation who were treated with ivacaftor from 2003 to 2018).

Nasal airway inflammatory responses and pathogen detection in infants with cystic fibrosis.

Background: Detecting airway inflammation non-invasively in infants with cystic fibrosis (CF) is difficult. We hypothesized that markers of inflammation in CF [IL-1β, IL-6, IL-8, IL-10, IL-17A, neutrophil elastase (NE) and tumor necrosis factor (TNF-α)] could be measured in infants with CF from nasal fluid and would be elevated during viral infections or clinician-defined pulmonary exacerbations (PEx).

Methods: We collected nasal fluid, nasal swabs, and hair samples from 34 infants with CF during monthly clinic visits, sick visits, and hospitalizations.

Longitudinal mental health trends in cystic fibrosis.

Background: Mental health screening in accordance with consensus guidelines became routine clinical practice in our cystic fibrosis (CF) Center in 2015. We hypothesized improvement in anxiety and depression symptoms over time and associations between elevated screening scores and disease severity. We aimed to observe the impact of the COVID-19 pandemic and modulator use on mental health symptoms.

Built environment factors predictive of early rapid lung function decline in cystic fibrosis.

Background: The extent to which environmental exposures and community characteristics of the built environment collectively predict rapid lung function decline, during adolescence and early adulthood in cystic fibrosis (CF), has not been examined.

Objective: To identify built environment characteristics predictive of rapid CF lung function decline.

Methods: We performed a retrospective, single-center, longitudinal cohort study (n = 173 individuals with CF aged 6-20 years, 2012-2017).

Patient and family perceptions of telehealth as part of the cystic fibrosis care model during COVID-19.

Background: Cystic Fibrosis (CF) is a chronic multi-system disease best cared for at Care centers with routine monitoring by interdisciplinary teams. Previously, remote home monitoring technology has been explored to augment in-person care. During the COVID-19 pandemic, traditional in-person care was limited and CF centers rapidly adapted to a telehealth delivery model.

Assessment of treatment burden and complexity in cystic fibrosis: A quality improvement project.

Background: Treatment regimens for cystic fibrosis (CF) continue to evolve and grow in complexity. Treatment regimen burden, and associated sequelae, are incompletely understood.

Objective: Quality improvement (QI) methods were used to investigate treatment burden of CF care, family and care team partnerships, and potential interventions to reduce burden.

A Pilot Study of Mindfulness-Based Cognitive Therapy to Improve Well-Being for Health Professionals Providing Chronic Disease Care.

Objective: To assess the efficacy of mindfulness-based cognitive therapy delivered onsite during work hours in reducing stress and improving well-being in an interdisciplinary chronic care health care team.

Study Design: A longitudinal, mixed methods, observational pilot study using a survey created from validated assessment tools to measure effectiveness of training. Surveys were completed before training, and 1 and 15 months after training.

Assessing and responding to stress related to pulmonary function testing in cystic fibrosis through quality improvement.

Background: Pulmonary function tests (PFTs) are performed routinely to evaluate lung function in patients with cystic fibrosis (CF). Staff at the Cincinnati Children's Hospital Medical Center CF Center observed stress in patients before PFTs. An interdisciplinary quality improvement (QI) team was assembled to address this clinical issue.

Executive functioning in pediatric cystic fibrosis: A preliminary study and conceptual model.

Background: Research has shown that broad cognitive functioning in individuals with CF is intact. Specific executive functioning (EF) deficits have been identified, however, and adults with CF report more symptoms of ADHD than the general population. EF skills are critical to the management of a complex disease like CF although studies have not adequately examined EF mechanisms in CF.

Acute Respiratory Distress Syndrome immediately following the removal of an aspirated foreign body.

Acute Respiratory Distress Syndrome (ARDS) is a form of diffuse lung injury with many potential etiologies, pneumonia being the most common cause developing outside of the hospital. Foreign body (FB) aspiration is a risk factor for pneumonia, and therefore, ARDS. Although these associations exist, the development of ARDS immediately following the removal of an aspirated FB appears quite rare.

Reduction of Recurrence Risk of Pancreatitis in Cystic Fibrosis With Ivacaftor: Case Series.

The effect of ivacaftor in patients with cystic fibrosis (CF) with recurrent pancreatitis is unknown. We conducted a multicenter retrospective study of patients with CF taking ivacaftor who had a history of recurrent pancreatitis. During the first 3 months of therapy, only 1 of the 6 patients had an episode of pancreatitis, which was managed on an outpatient basis.

Non-typeable Haemophilus influenzae purulent pericarditis in a child with cystic fibrosis.

Early airway colonization and infection with Haemophilus influenzae in children with cystic fibrosis (CF) is common. Although the pathogenicity of non-typeable H. influenzae (NTHi) in patients with CF is controversial, this organism can cause both upper and lower respiratory tract infections.

Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor.

Background: Previous studies of CF treatments have shown suboptimal adherence, though little has been reported regarding adherence patterns to ivacaftor. Electronic monitoring (EM) of adherence is considered a gold standard of measurement.

Methods: Adherence rates by EM were prospectively obtained and patterns over time were analyzed.

Adherence Determinants in Cystic Fibrosis: Cluster Analysis of Parental Psychosocial, Religious, and/or Spiritual Factors.

Rationale: Cystic fibrosis is a progressive disease requiring a complex, time-consuming treatment regimen. Nonadherence may contribute to an acceleration of the disease process. Spirituality influences some parental healthcare behaviors and medical decision-making.

The impact of transforming healthcare delivery on cystic fibrosis outcomes: a decade of quality improvement at Cincinnati Children's Hospital.

Background: In 2001, Cincinnati Children's Hospital embarked on a journey to improve healthcare delivery to patients with cystic fibrosis (CF). Data from the Cystic Fibrosis Foundation National Patient Registry revealed our below-average clinical outcomes, prompting us to initiate improvement interventions.

Objective: To improve clinical outcomes for patients with CF through a comprehensive quality-improvement approach directed at increasing patient centredness and improving healthcare delivery.