Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study.

Background: Factors associated with severe COVID-19 infection have been identified; however, the impact of infection on longer-term outcomes is unclear. The objective of this study was to examine the impact of COVID-19 infection on the trajectory of lung function and nutritional status in people with cystic fibrosis (pwCF).

Methods: This is a retrospective global cohort study of pwCF who had confirmed COVID-19 infection diagnosed between January 1, 2020 and December 31, 2021.

Mobile health platform for self-management of pediatric cystic fibrosis: Impact on patient-centered care outcomes.

Background: Previously, we adapted a mobile health platform (Genia) to the needs of patients and families in a pediatric CF center in the United States. In this feasibility study, we tested the impact of Genia on measures of patient-centered care.

Methods: In a one-group pre-post study with adolescents with CF and caregivers of children with CF, we tested Genia's effect over 6 months on patient satisfaction with chronic illness care (PACIC) and shared decision-making (CollaboRate).

Outcomes of children with cystic fibrosis screen positive, inconclusive diagnosis/CFTR related metabolic syndrome.

Background: Some infants undergoing newborn screening (NBS) tests have inconclusive sweat chloride test (SCT) results that lead to the designation of Cystic Fibrosis Screen Positive, Inconclusive Diagnosis/CFTR-related metabolic syndrome (CFSPID/CRMS). Some proportion of them transition to a CF diagnosis, but no predictive markers can stratify which are at risk for this transition. We report single-center outcomes of children with CRMS.

A Mobile Health Platform for Self-Management of Pediatric Cystic Fibrosis: Qualitative Study of Adaptation to Stakeholder Needs and Integration in Clinical Settings.

Background: Cystic fibrosis (CF) is an inherited chronic condition that requires extensive daily care and quarterly clinic visits with a multidisciplinary care team. The limited exchange of information outside of the quarterly clinic visits impedes optimal disease self-management, patient engagement, and shared decision making.

Objective: The aim of this study is to adapt a mobile health (mHealth) app originally developed in Sweden to the needs of patients, families, and health care providers in a CF center in the United States and to test it as a platform for sharing patient-generated health data with the CF health care team.

Tobacco smoke exposure and socioeconomic factors are independent predictors of pulmonary decline in pediatric cystic fibrosis.

Background: Pulmonary decline in CF is heterogeneous, with socio-environmental factors contributing to this variability. Few studies have attempted to disentangle the effects of tobacco smoke exposure and socioeconomic factors on lung function deterioration in pediatric CF. The current study evaluates their contributions longitudinally across the entire U.

Area Deprivation as a Risk Factor for Methicillin-resistant Staphylococcus aureus Infection in Pediatric Cystic Fibrosis.

Background: In US cystic fibrosis (CF) patients, methicillin-resistant Staphylococcus aureus (MRSA) rates have tripled in the past 2 decades. Known clinical risk factors include exposure to a healthcare setting, Pseudomonas aeruginosa and CF-related diabetes. Area-level socio-environmental exposures have not been evaluated.

Objective Versus Self-Reported Adherence to Airway Clearance Therapy in Cystic Fibrosis.

Background: Historically, studies of adherence to airway clearance therapy in cystic fibrosis (CF) have relied on self-reporting. We compared self-reported airway clearance therapy adherence to actual usage data from home high-frequency chest wall compressions (HFCWC) vests and identified factors associated with overestimation of adherence in self-reports.

Methods: Pediatric patients who perform airway clearance therapy with a HFCWC vest were eligible to participate.

Risk stratification model to detect early pulmonary disease in infants with cystic fibrosis diagnosed by newborn screening.

Objective: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes.

Methods: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012).

Adherence to airway clearance therapy in pediatric cystic fibrosis: Socioeconomic factors and respiratory outcomes.

Objectives: The evidence linking socioeconomic status (SES) and adherence in cystic fibrosis (CF) is inconclusive and focused on medication uptake. We examined associations between SES, adherence to airway clearance therapy (ACT), and CF respiratory outcomes.

Study Design: Socioeconomic, clinical, and adherence data of CF patients (N = 110) at a single CF Center were evaluated in this cross-sectional observational study.

Sustained improvement in nutritional outcomes at two paediatric cystic fibrosis centres after quality improvement collaboratives.

Objective: To describe the characteristics of sustained improved nutritional outcomes through the use of quality improvement (QI) methodology.

Design: Retrospective analysis of a QI intervention in two institutions, implemented as part of larger national collaboratives.

Setting: Paediatric cystic fibrosis (CF) programmes in academic centres in Alabama and Illinois.

Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort.

Summary Background: The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young children with cystic fibrosis (CF).

Objectives And Hypothesis: To describe the baseline characteristics of the cohort and evaluate associations between potential risk factors and nutritional and respiratory characteristics at enrollment. We hypothesized that distinct demographic and environmental risk factors could be identified for poorer nutritional status and lung function at enrollment.

Improving the quality of care for patients with cystic fibrosis.

Purpose Of Review: Improvement in cystic fibrosis (CF) outcomes over the past 50 years has been dramatic. This article describes the factors that have contributed to the recent acceleration in this improvement and the important role of the Cystic Fibrosis Foundation.

Recent Findings: Initiatives to improve CF care over the past decade include the refinement of a sophisticated patient registry that allows a comparison of center processes and outcomes; development of evidence-based and consensus-based guidelines regarding standards of care; cultural transformation, including the training of care center teams in a systems-oriented approach to quality improvement; data transparency; and encouragement of a patient-centered and family-centered orientation.

Cystic fibrosis care in Chile.

Purpose Of Review: Cystic fibrosis (CF) has been underdiagnosed and undertreated for many years in Latin American countries, including Chile. This article describes the evolution of CF care in view of recent reforms in healthcare delivery in Chile, and the opportunities that exist to improve outcomes.

Recent Findings: The characteristics of the population of CF patients are described using recently collected data.