scholarly journals 321: Asynchronous educational modules improve learner knowledge and confidence of cystic fibrosis gastrointestinal disease and management

2021 ◽  
Vol 20 ◽  
pp. S154
Author(s):  
G. Hartman ◽  
K. Holland ◽  
M. Bozic ◽  
R. Hinrichs ◽  
J. Molleston
2013 ◽  
Vol 48 (12) ◽  
pp. 1181-1189 ◽  
Author(s):  
Neil Shah ◽  
Hui-leng Tan ◽  
Neil Sebire ◽  
Ranjan Suri ◽  
Ku Leuven

2021 ◽  
Vol 10 (4) ◽  
pp. e001427
Author(s):  
Rachel Dunk ◽  
Susan Madge

Cystic fibrosis (CF) is a genetic, life-limiting disease without a cure; treatment is complex and lifelong. Respiratory failure is the most common cause of death; however, gastrointestinal disease, diabetes and liver disease are common comorbidities. Coronavirus disease (COVID-19) rapidly changed healthcare services across the globe, including redeployment of healthcare professionals. This adult CF service was challenged to continue a patient facing service within severe staffing and structural limitations.Not only were many members of the CF multidisciplinary team (MDT) redeployed at the start of the first wave, but also both the CF and ambulatory care wards were closed. Fortunately, the CF clinical nurse specialists (CF-CNSs) remained in their role. Rapid change and adaptation of the CF service was required to ensure that patients did not feel abandoned and access to treatment remained available. The role of the CF-CNS was therefore pivotal in this change.The aim of this project was to use quality improvement methodology to plan an emergency service allowing a reintroduction of ambulatory care services. Success was measured by the number of patients clinically reviewed with or without intervention, and the reasons for patients contacting the CF-CNS via email and phone were recorded.In weeks 1 and 2 of the emergency service, the CF-CNSs triaged patients by phone, then reviewed face-to-face when necessary. This first step allowed the CF-CNSs to start two patients on home intravenous antibiotics. This service continued to be developed over the following 12 weeks, leading to a total of 36 patient attendances. In March 2020, n=1187 patients made contact (mostly COVID-19, unwell and medication related), in April n=904 and May n=870 (blood test results, unwell and medication related).The motivation of the CF-CNSs was pivotal to the success of this initiative with the CF MDT available to provide some support and advice. It concluded at week 12, which then saw the opening of the formal ambulatory care ward and returning redeployed ward staff.


2018 ◽  
Vol 52 (3) ◽  
pp. 1702529 ◽  
Author(s):  
Karin M. de Winter-de Groot ◽  
Hettie M. Janssens ◽  
Rick T. van Uum ◽  
Johanna F. Dekkers ◽  
Gitte Berkers ◽  
...  

Forskolin-induced swelling (FIS) of intestinal organoids from individuals with cystic fibrosis (CF) measures function of the cystic fibrosis transmembrane conductance regulator (CFTR), the protein mutated in CF.We investigated whether FIS corresponds with clinical outcome parameters and biomarkers of CFTR function in 34 infants diagnosed with CF. Relationships with FIS were studied for indicators of pulmonary and gastrointestinal disease.Children with low FIS had higher levels of immunoreactive trypsinogen (p=0.030) and pancreatitis-associated protein (p=0.039), more often had pancreatic insufficiency (p<0.001), had more abnormalities on chest computed tomography (p=0.049), and had lower z-scores for maximal expiratory flow at functional residual capacity (p=0.033) when compared to children with high FIS values. FIS significantly correlated with sweat chloride concentration (SCC) and intestinal current measurement (ICM) (r= −0.82 and r=0.70, respectively; both p<0.001). Individual assessment of SCC, ICM and FIS suggested that FIS can help to classify individual disease severity.Thus, stratification by FIS identified subgroups that differed in pulmonary and gastrointestinal outcome parameters. FIS of intestinal organoids correlated well with established CFTR-dependent biomarkers such as SCC and ICM, and performed adequately at group and individual level in this proof-of-concept study.


Author(s):  
Meghana Sathe ◽  
Preeti Sharma ◽  
Adrienne Savant

The multisystemic manifestations of cystic fibrosis (CF) involve all parts of the gastrointestinal (GI) system, including the pancreas, intestine and liver. As providers who care for people with CF (PwCF), knowledge of the manifestations, treatment and research related to nutrition and GI disease is important. This review is last installment of the CF Year in Review 2020 series, focusing on the multisystem effects of CF. Part one focused on the literature related to CFTR (cystic fibrosis transmembrane conductance regulator protein) modulators, while part two focused on pulmonary outcomes, radiographic and physiologic assessments, as well as infection and inflammation. Part three was split into Part 3A, focusing on the multisystem impact of CF, and this review, Part 3B, focusing on nutritional, gastrointestinal and hepatobiliary articles. Articles were chosen from Pediatric Pulmonology but also include articles published in 2020 from other journals that are of particular interest to clinicians.


2021 ◽  
Author(s):  
Meghana Sathe ◽  
Preeti B. Sharma ◽  
Adrienne P. Savant

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Michael J. Coffey ◽  
Shaun Nielsen ◽  
Bernd Wemheuer ◽  
Nadeem O. Kaakoush ◽  
Millie Garg ◽  
...  

AbstractIntestinal dysbiosis has been observed in children with cystic fibrosis (CF), yet the functional consequences are poorly understood. We investigated the functional capacity of intestinal microbiota and inflammation in children with CF. Stool samples were collected from 27 children with CF and 27 age and gender matched healthy controls (HC) (aged 0.8–18 years). Microbial communities were investigated by iTag sequencing of 16S rRNA genes and functional profiles predicted using Tax4Fun. Inflammation was measured by faecal calprotectin and M2-pyruvate kinase. Paediatric CF gastrointestinal microbiota demonstrated lower richness and diversity compared to HC. CF samples exhibited a marked taxonomic and inferred functional dysbiosis when compared to HC. In children with CF, we predicted an enrichment of genes involved in short-chain fatty acid (SCFA), antioxidant and nutrient metabolism (relevant for growth and nutrition) in CF. The notion of pro-inflammatory GI microbiota in children with CF is supported by positive correlations between intestinal inflammatory markers and both genera and functional pathways. We also observed an association between intestinal genera and both growth z-scores and FEV1%. These taxonomic and functional changes provide insights into gastrointestinal disease in children with CF and future gastrointestinal therapeutics for CF should explore the aforementioned pathways and microbial changes.


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