Improvements in Serum 25(OH)D Following Stoss Dosing in People With Cystic Fibrosis and Variable Adherence to Maintenance Regimens: A Retrospective Chart Review.

Background And Aims: People with cystic fibrosis (pwCF) often have vitamin D deficiency and require vitamin D supplementation. The primary objective of this study was to evaluate the impact of ultrahigh dose cholecalciferol ("stoss dosing") in pwCF with variable adherence to maintenance cholecalciferol.

Methods: Retrospective cohort study of pwCF who received initial stoss dose per protocol between January 2017 and November 2021 at University of Iowa Health Care adult and pediatric CF centers.

Fueling Health: Overcoming Food Barriers in Cystic Fibrosis Care.

Background: Food insecurity is increasing in prevalence throughout the general population and has been noted to substantially exist within the cystic fibrosis (CF) community as well. Nutritional grant programs are in place to provide additional support to people with cystic fibrosis (PwCF). This study was designed to determine the current usage rate of nutritional grant funding as well as clinical outcomes such as body mass index (BMI).

Impact of elexacaftor/tezacaftor/ivacaftor on bacterial cultures from people with cystic fibrosis.

Background: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have shown beneficial effects on both forced expiratory volume in 1 s (FEV ) and frequency of pulmonary exacerbations in people with cystic fibrosis (CF). These positive outcomes may be related to changes in bacterial colonization within the lungs. Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is the first triple therapy CFTR modulator approved for use in people with CF 6 years and older.

Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients.

Background: Cystic fibrosis (CF) is a multisystem disorder that results in the buildup of mucus in various organs. Ninety percent of CF patients are classified as pancreatic insufficient, leading to malabsorption of nutrients and fat-soluble vitamins without the assistance of exogenous pancreatic enzymes. This study was designed to determine if serum 25-hydroxyvitamin D concentrations were impacted by initiation of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA).

Off-label use of intravenous antimicrobials for inhalation in patients with cystic fibrosis.

Management of infections in patients with cystic fibrosis (CF) presents challenges for healthcare providers, including the eradication of initial acquisition, treatment of acute exacerbations, and chronic infection with suppressive therapy. Inhaled antimicrobial therapy for infections in patients with CF has been used in these capacities, often in an effort to achieve optimal concentrations in sputum for antimicrobial efficacy while mitigating potential toxicities associated with systemic therapy. Unfortunately, there are few commercially available products formulated for inhalation, resulting in the off-label use of other formulations, such as intravenous products, administered via nebulization.