Use Of A Pulmonary Exacerbation Score (PES) To Improve Treatment Of Adult Cystic Fibrosis Pulmonary Exacerbations

Author(s):  
Matthew D. Fletcher ◽  
B S. Prato ◽  
Jonathan B. Zuckerman ◽  
Karen Jarosz ◽  
Scott Morin
2020 ◽  
pp. 1357633X2097200
Author(s):  
Beste Ozsezen ◽  
Nagehan Emiralioglu ◽  
Dilber A Tural ◽  
Birce Sunman ◽  
Halime N Buyuksahin ◽  
...  

Introduction The global burden of the 2019 novel coronavirus disease pandemic on the healthcare system, as well as the high transmission risk of coronavirus disease has led to the use of alternative strategies for evaluation of children with chronic conditions. In this context, telemedicine has become the effective and affordable choice. In this study we aimed to evaluate the efficiency of telephone visits to determine pulmonary exacerbations and hospitalization rates of children with cystic fibrosis and interstitial lung disease. Methods A total of 119 children with cystic fibrosis or interstitial lung disease were enrolled and provided cases in which telephone visits were applied during the peak time of the coronavirus disease pandemic in our country. The recordings of respiratory, gastrointestinal and other symptoms, nutrition status, rate of acute pulmonary exacerbation, treatments initiated by telephone visits, referral to hospital and hospitalization were established from the electronic health reports of the patients. Results Thirteen patients (10.9%) were symptomatic, 12 of them (10%) were diagnosed with acute pulmonary exacerbation. One patient was diagnosed with peripheral facial paralysis. Nine patients were recalled to the hospital and seven patients (5.8%) were hospitalised. Discussion Using telemedicine the health status of patients can be defined, and patients can be guided on proper healthcare that they need, especially during the times of pandemics which we are facing. Communication with patients while minimising the risk of exposure to coronavirus disease is an important advantage of telemedicine. Telemedicine will have to be implemented on our daily medical practice in the near future.


2020 ◽  
Vol 68 (4) ◽  
pp. 856-863
Author(s):  
Kristina L Bailey ◽  
Peter J Murphy ◽  
Olena K Lineberry ◽  
Matthew R Haack ◽  
John D Dickinson ◽  
...  

Patients with cystic fibrosis (CF) experience multiple pulmonary exacerbations throughout their lifetime, resulting in repeated antibiotic exposure and hospital admissions. Reliable diagnostic markers to guide antibiotic treatment in patients with CF, however, are lacking. Given that the CF airway is characterized by persistent and frequent bacterial infection, our goal was to determine if procalcitonin (PCT) could be used as a severity and prognostic marker of CF exacerbation. We enrolled 40 participants at the time of diagnosis of CF pulmonary exacerbation. Inclusion criteria: age ≥19 years with exacerbation requiring antibiotics as determined by the treating physician. Exclusion criteria: antibiotics initiated more than 48 hours prior to enrollment, and pregnancy. Blood samples were collected on enrollment day and after 7–10 days of treatment. Of the 40 patients enrolled, 23 (57.5%) had detectable levels of PCT (≥0.05 ng/mL). PCT levels were significantly associated with pulmonary exacerbation scores (p=0.01) and per cent decrease in forced expiratory volume in 1 second (FEV1) (p=0.01) compared with the best in the last 12 months. Those who had worsening PCT during treatment had less improvement in FEV1 (p=0.001) and were more likely to be readmitted to the hospital sooner (p<0.0001). Likewise, those who had a detectable PCT at the time of admission were more likely to be readmitted sooner (p=0.03). PCT elevation during antibiotic treatment is associated with less improvement in FEV1 and earlier readmission. A detectable PCT level occurs only in more severe CF exacerbations. Multicenter trials are needed to confirm whether PCT may play a role in the clinical care of patients with CF.


2019 ◽  
Vol 40 (06) ◽  
pp. 792-803 ◽  
Author(s):  
Christopher H. Goss

AbstractWith the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator therapy, the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is an acute pulmonary exacerbation. Clinical and microbial epidemiology studies of CF pulmonary exacerbations continue to provide important insight into the disease course, prognosis, and complications. This work has now led to a number of large scale clinical trials with the goal of improving the treatment paradigm for CF pulmonary exacerbation. The primary goal of this review is to provide a summary of the pathophysiology, the clinical epidemiology, microbial epidemiology, outcome and the treatment of CF pulmonary exacerbation.


2018 ◽  
Vol 51 (6) ◽  
pp. 1702509 ◽  
Author(s):  
Marieke van Horck ◽  
Kim van de Kant ◽  
Bjorn Winkens ◽  
Geertjan Wesseling ◽  
Vincent Gulmans ◽  
...  

To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009–2014).Lung disease progression was expressed as a decline in lung function (forced expiratory volume in 1 s (FEV1) % pred) and pulmonary exacerbation rate. Potential risk factors at baseline included sex, age, best FEV1 % pred, best forced vital capacity % pred, genotype, body mass index z-score, pancreatic insufficiency, medication use (proton pump inhibitors (PPIs), prophylactic antibiotics and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis and colonisation with Pseudomonas aeruginosa.The data of 545 children were analysed. PPI use was associated with both annual decline of FEV1 % pred (p=0.017) and future pulmonary exacerbation rate (p=0.006). Moreover, lower FEV1 % pred at baseline (p=0.007), prophylactic inhaled antibiotic use (p=0.006) and pulmonary exacerbations in the baseline year (p=0.002) were related to pulmonary exacerbations in subsequent years.In a cohort of Dutch children with CF followed for 5 years, we were able to identify several risk factors for future exacerbations. In particular, the association between PPI use and lung disease progression definitely requires further investigation.


F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 575 ◽  
Author(s):  
Kate Skolnik ◽  
Bradley S. Quon

Pulmonary exacerbations are common events in cystic fibrosis and have a profound impact on quality of life, morbidity, and mortality. Pulmonary exacerbation outcomes remain poor and a significant proportion of patients fail to recover their baseline lung function despite receiving aggressive treatment with intravenous antibiotics. This focused review provides an update on some of the recent advances that have taken place in our understanding of the epidemiology, pathophysiology, diagnosis, and management of pulmonary exacerbations in cystic fibrosis as well as direction for future study.


2015 ◽  
Vol 47 (3) ◽  
pp. 829-836 ◽  
Author(s):  
Felix Ratjen ◽  
Valerie Waters ◽  
Michelle Klingel ◽  
Nancy McDonald ◽  
Sharon Dell ◽  
...  

Lung disease in patients with both primary ciliary dyskinesia (PCD) or cystic fibrosis (CF) is associated with impaired mucociliary clearance; however, clinical outcomes are typically worse in CF patients. We assessed whether CF and PCD patients differ in inflammatory response in the airways during pulmonary exacerbation.We first studied clinically stable PCD patients with a spectrum of bacterial pathogens to assess inflammatory response to different pathogens. Subsequently, PCD and CF patients with similar bacterial pathogens were studied at the time of a pulmonary exacerbation and after 21 days of antibiotics treatment. Qualitative and quantitative microbiology, cell counts, interleukin-8 concentrations, and neutrophil elastase activity were assessed in sputum samples obtained before and after treatment.In stable PCD patients, no significant differences were found in sputum inflammatory markers between individuals colonised with different bacterial pathogens. Pulmonary exacerbation severity assessed by a pulmonary exacerbation score and lung function decline from their previous baseline did not differ between CF and PCD patients. Bacterial density for Staphylococcus aureus and Haemophilus influenzae was higher in CF versus PCD (p<0.05), but absolute neutrophil counts were higher in PCD patients (p=0.02). While sputum elastase activity was similar in PCD and CF at the time of exacerbation, it decreased with antibiotic therapy in PCD (p<0.05) but not CF patients.PCD patients differ from those with CF in their responses to treatment of pulmonary exacerbations, with higher neutrophil elastase activity persisting in the CF airways at the end of treatment.


2019 ◽  
Vol 109 (3) ◽  
pp. 544-553 ◽  
Author(s):  
Vin Tangpricha ◽  
Joshua Lukemire ◽  
Yuqing Chen ◽  
José Nilo G Binongo ◽  
Suzanne E Judd ◽  
...  

ABSTRACT Background Patients with cystic fibrosis (CF) have increased risk of vitamin D deficiency owing to fat malabsorption and other factors. Vitamin D deficiency has been associated with increased risk of pulmonary exacerbations of CF. Objectives The primary objective of this study was to examine the impact of a single high-dose bolus of vitamin D3 followed by maintenance treatment given to adults with CF during an acute pulmonary exacerbation on future recurrence of pulmonary exacerbations. Methods This was a multicenter, double-blind, placebo-controlled, intent-to-treat clinical trial. Subjects with CF were randomly assigned to oral vitamin D3 given as a single dose of 250,000 International Units (IU) or to placebo within 72 h of hospital admission for an acute pulmonary exacerbation, followed by 50,000 IU of vitamin D3 or an identically matched placebo pill taken orally every other week starting at 3 mo after random assignment. The primary outcome was the composite endpoint of the time to next pulmonary exacerbation or death within 1 y. The secondary outcomes included circulating concentrations of the antimicrobial peptide cathelicidin and recovery of lung function as assessed by the percentage of predicted forced expiratory volume in 1 s (FEV1%). Results A total of 91 subjects were enrolled in the study. There were no differences between the vitamin D3 and placebo groups in time to next pulmonary exacerbation or death at 1 y. In addition, there were no differences in serial recovery of lung function after pulmonary exacerbation by FEV1% or in serial concentrations of plasma cathelicidin. Conclusions Vitamin D3 initially given at the time of pulmonary exacerbation of CF did not alter the time to the next pulmonary exacerbation, 12-mo mortality, serial lung function, or serial plasma cathelicidin concentrations. This trial was registered at clinicaltrials.gov as NCT01426256.


2021 ◽  
Author(s):  
Lily Amanda Cook ◽  
Bethany Collins ◽  
Aaron Trimble

Abstract PurposeBecause pulmonary exacerbations in cystic fibrosis cause a step-wise decline in FEV1 function and contribute significantly to disease progression, it is important to identify potential environmental triggers. Studies have been done on general air quality and its relationship to cystic fibrosis disease activity, but none have examined air pollution caused by wildfire smoke. Our study intends to better understand this relationship. MethodsA retrospective cohort study was conducted using data collected from people with cystic fibrosis (CF) between 2012 and 2019. Data on pulmonary exacerbations was extracted from the patient registry hosted and maintained by the Cystic Fibrosis Foundation. Exposures were determined using measurements of fine particulate matter (PM2.5) from the Environmental Protection Agency. A logistic regression model was created in order to identify both univariate and adjusted odds ratios and their associated confidence intervals.Results82.7% (n = 415) of individuals with CF experienced an exposure to wildfire smoke during the study period. The adjusted odds ratio for a pulmonary exacerbation within one month following an exposure to wildfire smoke was 1.50 (95% CI = 1.13 – 1.99, p = 0.006) for adults and 0.92 (95% CI = 0.69 – 1.23, p = 0.578) for children. ConclusionWildfire smoke exposure is associated with severe pulmonary exacerbation in adults but not in children. This suggests that wildfire smoke may be an environmental risk factor for exacerbation in adults with CF. Further study is needed to understand why and how wildfire smoke exposure affects adult with CF differently than the pediatric population.


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