Attitudes toward and preparedness for lung transplantation among individuals with cystic fibrosis in the era of highly effective modulators.

Background: Outcomes for individuals with cystic fibrosis (CF) have improved due to highly effective modulator therapy (HEMT). However, lung transplant (LTx) remains an important treatment for people with advanced lung disease. This study assessed attitudes and knowledge about LTx in the HEMT era.

A national postgraduate nurse practitioner and physician assistant fellowship in cystic fibrosis: An innovative approach to the provider shortage in complex and rare disease.

Cystic fibrosis (CF) is a complex life-limiting genetic condition that affects the respiratory, digestive, reproductive system, and sweat glands. Advances in treatment have led to improved survival and quality of life. Today, most persons with CF live to adulthood but require highly specialized care at accredited CF Care Centers.

Seroprevalence of SARS-CoV-2 IgG in people with cystic fibrosis.

Background: When the first known US case of COVID-19 (Coronavirus Disease 2019) was reported in early 2020, little was known about the impact of this novel virus on the cystic fibrosis community. As the majority of individuals with CF have chronic lung disease, this population was initially considered to be at high risk for severe disease as infection with a multitude of viruses has proven to cause pulmonary exacerbation. SARS-CoV-2 virus has proven challenging to study given the multiple disease manifestations, range of severity, and wave-like phenomenon that varies geographically.

A longitudinal analysis of respiratory symptoms in people with cystic fibrosis with advanced lung disease on and off ETI.

People with CF (PwCF), particularly those with advanced lung disease (ALD), experience frequent respiratory symptoms. A major CF breakthrough was the approval of elexacaftor/tezacaftor/ivacaftor (ETI) in 2019, which has been shown to improve symptoms and lung function in the CF population, and decrease pulmonary exacerbations. The purpose of this study was to analyze longitudinal changes in respiratory symptoms over 24 months in ETI-treated and untreated PwCF with ALD Symptoms were measured among CF adults with ppFEV < 40% (N = 48, 24 ETI-treated, 24 untreated) using the CFRSD-CRISS and the CFQ-R [respiratory].

Predicting Early Versus Late In-Hospital Mortality in the Trauma Population.

This study aimed to evaluate non-survivors who were admitted to a level I trauma center but later died, in terms of predicting who would expire early vs late. This is a single-center study of Trauma Registry data, from July 3, 2016, to February 24, 2022. The inclusion criteria were based upon age (≥18 years) and in-hospital mortality.