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Parkins M.D.,University of Calgary | Parkins V.M.,University of Calgary | Rendall J.C.,Northern Ireland Regional Adult Cystic Fibrosis Center | Elborn S.,Queens University of Belfast
Therapeutic Advances in Respiratory Disease | Year: 2011

Improvements in the quality and implementation of medical care for individuals with cystic fibrosis (CF) have resulted in a dramatic improvement in survival. Many of these strategies have focused on the effective management of pulmonary disease which has delayed its manifestations into later years. With an increasing number of patients surviving to later years the impact of chronic inflammation and nutritional compromise on other organ systems over a lifetime are increasingly manifest. This review highlights the changing epidemiology of the ageing CF population and the complications that may ensue. © The Author(s), 2010. Source

Quittner A.L.,University of Miami | Abbott J.,University of Central Lancashire | Georgiopoulos A.M.,Massachusetts General Hospital | Georgiopoulos A.M.,Harvard University | And 19 more authors.
Thorax | Year: 2016

Studies measuring psychological distress in individuals with cystic fibrosis (CF) have found high rates of both depression and anxiety. Psychological symptoms in both individuals with CF and parent caregivers have been associated with decreased lung function, lower body mass index, worse adherence, worse health-related quality of life, more frequent hospitalisations and increased healthcare costs. To identify and treat depression and anxiety in CF, the CF Foundation and the European CF Society invited a panel of experts, including physicians, psychologists, psychiatrists, nurses, social workers, a pharmacist, parents and an individual with CF, to develop consensus recommendations for clinical care. Over 18 months, this 22-member committee was divided into four workgroups: Screening; Psychological Interventions; Pharmacological Treatments and Implementation and Future Research, and used the Population, Intervention, Comparison, Outcome methodology to develop questions for literature search and review. Searches were conducted in PubMed, PsychINFO, ScienceDirect, Google Scholar, Psychiatry online and ABDATA by a methodologist at Dartmouth. The committee reviewed 344 articles, drafted statements and set an 80% acceptance for each recommendation statement as a consensus threshold prior to an anonymous voting process. Fifteen guideline recommendation statements for screening and treatment of depression and anxiety in individuals with CF and parent caregivers were finalised by vote. As these recommendations are implemented in CF centres internationally, the process of dissemination, implementation and resource provision should be closely monitored to assess barriers and concerns, validity and use. © 2016, BMJ Publishing Group. All rights reserved. Source

Parkins M.D.,University of Calgary | Parkins M.D.,Northern Ireland Regional Adult Cystic Fibrosis Center | Rendall J.C.,University of Calgary | Elborn J.S.,Northern Ireland Regional Adult Cystic Fibrosis Center | Elborn J.S.,Queens University of Belfast
Chest | Year: 2012

Background: Pulmonary exacerbations (PEx) are responsible for much of the morbidity and mortality associated with cystic fibrosis (CF). However, there is a paucity of data on outcomes in CF PEx and factors influencing outcomes. Methods: We reviewed all PEx in patients infected with Pseudomonas aeruginosa treated with parenteral antibiotics over 4 years at our center. Treatment failures were categorized a priori as those PEx requiring antibiotic regimen change, prolongation of therapy. >20 days because of failure to respond, an early recurrent event within, <45 days, or failure to recover lung function to. >90% of baseline FEV 1. Results: A total of 101 patients were followed for 452 PEx. Treatment failures were observed in 125 (28%) of PEx;antibiotic regimen change was observed in 27 (6%), prolongation of therapy in 29 (6%), early recurrent events in 63 (14%), and failure to recover lung function to. >90% of baseline FEV 1 in 66 (15%). Demographic factors associated with one or more treatment failures per year included advanced airways disease, use of enteric feeds, CF-related diabetes, and CF liver disease but did not include female sex or F508del homozygosity. Increased treatment failure risk was associated with lower admission FEV 1 and increased markers of inflammation. At therapeutic completion, increased inflammatory markers correlated with treatment failure. Failure rates decreased with increasing number of active antimicrobial agents used based on in vitro susceptibility (zero, 28/65 [43%]; one, 38/140 [27%]; two, 59/245 [24%]; three, 0/2 [0%];P =.02). Conclusions: One-fourth of PEx fail to respond adequately to initial management. Patient demographic and episode-specific clinical information can be used to identify individuals at increased risk of initial management failure. ©2012 American College of Chest Physicians. Source

Shteinberg M.,Pulmonology Institute | Shteinberg M.,Technion - Israel Institute of Technology | Shteinberg M.,Queens University of Belfast | Kis-Papo T.,Haifa University | And 9 more authors.
Journal of Water and Health | Year: 2015

Clinical cystic fibrosis (CF) Pseudomonas aeruginosa (n = 6) and Burkholderia cenocepacia (n = 4) were inoculated in marine brines from the Dead Sea and the Atlantic Ocean and their survival was monitored over a 1 month duration. In Dead Sea samples, all P. aeruginosa and B. cenocepacia isolates were non-detectable by culture following 24 h incubation, including the non-selective enrichment samples. In the Atlantic Ocean brine, over a 1 month period, mean P. aeruginosa counts decreased by only 0.25 log10 units and mean B. cenocepacia counts decreased by approximately 4 log10 units (10,000 cfu/ml). This study demonstrated that Dead Sea brine exerted a lethal effect within 24 h on planktonic P. aeruginosa and B. cenocepacia. Thus, the Dead Sea effectively purges these organisms from its environment on a daily basis. © IWA Publishing 2015. Source

Moore J.E.,Northern Ireland Public Health Laboratory | Moore J.E.,Northern Ireland Regional Adult Cystic Fibrosis Center | Moore J.E.,Queens University of Belfast | Moore J.E.,University of Ulster | And 4 more authors.
British Journal of Biomedical Science | Year: 2015

D-mannitol has been approved in dry powder formulation as an effective antimucolytic agent in patients with cystic fibrosis. What is not known is the effect of adding a metabolisable sugar on the biology of chronic bacterial pathogens in the CF lung. Therefore, a series of simple in vitro experiments were performed to examine the effect of adding D-mannitol on the phenotype of the CF respiratory pathogens Pseudomonas aeruginosa and Burkholderia cenocepacia. Clinical isolates (n=86) consisting of P. aeruginosa (n=51), B. cenocepacia (n=26), P. putida (n=4), Stenotrophomonas maltophila (n=3) and Pseudomonas spp. (n=2) were examined by supplementing basal nutrient agar with varying concentrations of D-mannitol (0-20% [w/v]) and subsequently examining for any change in microbial phenotype. The effect of supplementation with mannitol was four-fold, namely i) To increase the proliferation and increase in cell density of all CF organisms examined, with an optimal concentration of 2-4% (w/v) D-mannitol. No such increase in cell proliferation was observed when mannitol was substituted with sodium chloride. ii) Enhanced pigment production was observed in 2/51 (3.9%) of the P. aeruginosa isolates examined, in one of the P. putida isolates, and in 3/26 (11.5%) of the B. cenocepacia isolates examined. iii). When examined at 4.0% (w/v) supplementation with mannitol, 11/51 (21.6%) P. aeruginosa isolates and 3/26 (11.5%) B. cenocepacia isolates were seen to exhibit the altered adhesion phenotype. iv). With respect to the altered mucoid phenotype, 5/51 (9.8%) P. aeruginosa produced this phenotype when grown at 4% mannitol. Mucoid production was greatest at 4%, was poor at 10% and absent at 20% (w/v) mannitol. The altered mucoid phenotype was not observed in the B. cenocepacia isolates or any of the other clinical taxa examined. Due consideration therefore needs to be given, where there is altered physiology within the small airways, leading to a potentially altered biological state of the colonising microorganisms in novel inhaled pharmaceutical interventions in CF, particularly those, which are not designated as antimicrobial agents. Source

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