I am a Nutrition Scientist in Newcastle Upon Tyne.
Cystic fibrosis is a multi-system disease, gastro-oesophageal reflux is one of the complications
Cystic fibrosis; reflux into your throat, and into your lungs?
In 10 seconds: Cystic fibrosis is a disease whereby the CFTR gene is faulty. The CFTR protein is expressed in the pancreas, airways and reproductive system. Due to problems with ion transport, a thick and sticky mucus causes blockages in these areas. People with cystic fibrosis experience problems in the gastro-intestinal tract . Including abnormal pH levels in the intestines, gastro-oesophageal reflux, and extra oesophageal reflux.
Reflux is the retrograde movement of the stomachs contents back into the oesophagus. This causes symptoms of heartburn and regurgitation. Extra-oesophageal reflux causes cough, runny nose, wheezing and a tickle in the throat. This refluxate can also travel into the lungs by being aspirated into the airways.
Don't believe it? Pepsin is a digestive enzyme found in the stomach, yet it is found in samples from the airways. Human studies show that refluxate in the airways may lead to decreased lung function. Our research group found that bile acids travelled from the duodenum to the airways. Also, we found that patients treated with a CFTR correcting drug had significantly less reflux symptoms. Increasing evidence has emerged showing gastro-intestinal tract is implicated in the CF disease.
But antacid medication is available to treat reflux! These are proton pump inhibitors and H2 histamine receptor antagonist. They reduce the acidity of the refluxate in the stomach. Yet, the reflux episode still occurs and the potent agents remain. Despite the use of antacid medication, many patients still experience symptoms of reflux
The problem persists. Many experience the negative impact of gastro/extra oesophageal reflux despite medication. This board focuses on the prevalence of reflux in the cystic fibrosis population. Research is ongoing in this area across the world.
Abstract: Extra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study.Data from 12 patients with CF and the G551D mutation prescribed ivacaftor (150mg bd) was collected at baseline, 6, 26 and 52weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)).Six patients presented EOR at baseline (RSI >13; median 13; range 2-29) and 5 presented airway reflux (HARQ >13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P<0∙04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire.Ivacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration.
Pub.: 01 Aug '16, Pinned: 24 Apr '17
Abstract: We conducted a nested case-control study to examine the prevalence and risk factors for current gastroesophageal reflux disease (GERD) symptoms in young adults with a history of childhood GERD.We identified a cohort of individuals diagnosed with GERD in childhood during 1995-1996, and controls without childhood GERD. Patients with neurodevelopmental disorders, tracheoesophageal anomalies, or cystic fibrosis were excluded. A computer-assisted telephone interview was conducted during 2004-2005. We calculated the prevalence of GERD symptoms, and examined the potential determinants of symptoms in unadjusted and adjusted logistic regression analyses.A total of 113 cases completed the questionnaires (participation rate, 70.6%). The mean age of participants was 18 years, and their mean age at the time of childhood GERD diagnosis was 10 years. At least weekly heartburn or regurgitation was reported in 52 (46%) participants, 94% of whom were taking proton pump inhibitors, H2RA, or antacids. On the other hand, 33 controls were identified (44% participation rate) in whom weekly heartburn or regurgitation was reported in 30%. GERD was significantly more frequent in females using oral contraceptive pills (76.5%) as compared with females not on oral contraceptive pills (47.9%), or males (33.3%) (P = .008). Weight, height, nonsteroidal anti-inflammatory drug use, race, family history of GERD, education level, employment status, tobacco smoking, alcohol, or coffee drinking were not associated significantly with adulthood GERD.Frequent GERD symptoms requiring antisecretory therapy are present in approximately half of young adults with a history of childhood GERD. The use of oral contraceptives is a risk factor for GERD symptoms in these individuals.
Pub.: 05 Jun '07, Pinned: 24 Apr '17
Abstract: Gastroesophageal reflux (GER) in adults with cystic fibrosis (CF) is poorly characterized. This study examines the frequency and predictors of GER symptoms and their relationship to lung function in adults with CF.Cross-sectional study of adults at the University of Minnesota CF Clinic using two validated self report surveys: The Mayo GER questionnaire and the GERD Symptom Assessment Scale (GSAS).Of 274 invited patients, 201 (73%) completed the surveys and 173 performed spirometry at the same visit. Frequent symptoms (at least weekly) were reported by 24% of the patients and an additional 39% experienced occasional symptoms. Heartburn, acid regurgitation and dysphagia were the most common symptoms and 18% reported that GER symptoms worsened their respiratory condition. Females and patients reporting weight loss had more symptoms (mean GSAS symptom score 4.9 vs. 4.0, p=0.025 and 5.3 vs. 4.2, p=0.04) and more severe symptoms (mean GSAS distress score 5.6 vs. 3.8, p=0.005 and 6.8 vs. 4.0, p=0.01) compared to males and those who did not report weight loss. Patients on acid suppression (n=122, 61%) continued to report heartburn (n=80, 66%) and acid regurgitation (n=47, 23%). GER symptoms and severity of symptoms were not predictive of FEV(1) or FVC.GER symptoms were present in a majority of patients. Females and patients with weight loss require special attention to their GER symptoms. Many patients on acid suppression continued to be report symptoms.
Pub.: 03 Aug '10, Pinned: 24 Apr '17
Abstract: Malabsorption of fat and protein contributes to poor nutritional status in people with cystic fibrosis. Impaired pancreatic function may also result in increased gastric acidity, leading in turn to heartburn, peptic ulcers and the impairment of oral pancreatic enzyme replacement therapy. The administration of gastric acid-reducing agents has been used as an adjunct to pancreatic enzyme therapy to improve absorption of fat and gastro-intestinal symptoms in people with cystic fibrosis. It is important to establish the evidence regarding potential benefits of drugs that reduce gastric acidity in people with cystic fibrosis. This is an update of a previously published review.To assess the effect of drug therapies for reducing gastric acidity for: nutritional status; symptoms associated with increased gastric acidity; fat absorption; lung function; quality of life and survival; and to determine if any adverse effects are associated with their use.We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals, abstract books and conference proceedings.Most recent search of the Group's Trials Register: 12 May 2016.All randomised and quasi-randomised trials involving agents that reduce gastric acidity compared to placebo or a comparator treatment.Both authors independently selected trials, assessed trial quality and extracted data.The searches identified 39 trials; 17 of these, with 273 participants, were suitable for inclusion, but the number of trials assessing each of the different agents was small. Seven trials were limited to children and four trials enrolled only adults. Meta-analysis was not performed, 14 trials were of a cross-over design and we did not have the appropriate information to conduct comprehensive meta-analyses. All the trials were run in single centres and duration ranged from five days to six months. The included trials were generally not reported adequately enough to allow judgements on risk of bias.However, one trial found that drug therapies that reduce gastric acidity improved gastro-intestinal symptoms such as abdominal pain; seven trials reported significant improvement in measures of fat malabsorption; and two trials reported no significant improvement in nutritional status. Only one trial reported measures of respiratory function and one trial reported an adverse effect with prostaglandin E2 analogue misoprostol. No trials have been identified assessing the effectiveness of these agents in improving quality of life, the complications of increased gastric acidity, or survival.Trials have shown limited evidence that agents that reduce gastric acidity are associated with improvement in gastro-intestinal symptoms and fat absorption. Currently, there is insufficient evidence to indicate whether there is an improvement in nutritional status, lung function, quality of life, or survival. Furthermore, due to the unclear risks of bias in the included trials, we are unable to make firm conclusions based on the evidence reported therein. We therefore recommend that large, multicentre, randomised controlled clinical trials are undertaken to evaluate these interventions.
Pub.: 23 Aug '16, Pinned: 24 Apr '17
Abstract: This study aimed to investigate pepsin as a marker of extra-oesophageal reflux disease by examining its presence in tracheal aspirates and correlating it with macroscopic changes on laryngobronchoscopy, along with the results of standard tests for gastro-oesophageal reflux disease and clinical features.A retrospective review was undertaken of a cohort of 188 paediatric patients who underwent laryngobronchoscopy at a tertiary children's hospital and for whom pepsin assay results of tracheal aspirates were available. An association analysis was performed.The mean patient age was 3.99 (3.40-4.58) years, with a male preponderance (55 per cent). Positive changes on laryngobronchoscopy were significantly associated with positive tracheal pepsin findings (p < 0.0001) but not with positive standard gastro-oesophageal reflux disease investigations. A positive pepsin assay was significantly associated with a history of recurrent croup (p = 0.0385) and a diagnosis of cystic fibrosis (p = 0.0232).Macroscopic changes on laryngobronchoscopy were significantly associated with positive tracheal pepsin findings in this paediatric population, suggesting that extra-oesophageal reflux disease may be a contributing aetiology.
Pub.: 26 May '15, Pinned: 23 Apr '17
Abstract: Gastro-oesophageal reflux is associated with a wide range of respiratory disorders, including asthma, isolated chronic cough, idiopathic pulmonary fibrosis, chronic obstructive pulmonary disease and cystic fibrosis. Reflux can be substantial and reach the proximal margins of the oesophagus in some individuals with specific pulmonary diseases, suggesting that this association is more than a coincidence. Proximal oesophageal reflux in particular has led to concern that microaspiration might have an important, possibly even causal, role in respiratory disease. Interestingly, reflux is not always accompanied by typical reflux symptoms, such as heartburn and/or regurgitation, leading many clinicians to empirically treat for possible gastro-oesophageal reflux. Indeed, costs associated with use of acid suppressants in pulmonary disease far outweigh those in typical GERD, despite little evidence of therapeutic benefit in clinical trials. This Review comprehensively examines the possible mechanisms that might link pulmonary disease and oesophageal reflux, highlighting the gaps in current knowledge and limitations of previous research, and helping to shed light on the frequent failure of antireflux treatments in pulmonary disease.
Pub.: 07 Jul '16, Pinned: 23 Apr '17
Abstract: Gastro-Oesophageal Reflux (GOR) is a key problem in Cystic Fibrosis (CF), but the relationship between lung and gastric microbiomes is not well understood. We hypothesised that CF gastric and lung microbiomes are related. Gastric and sputum cultures were obtained from fifteen CF patients receiving percutaneous endoscopic gastrostomy feeding. Non-CF gastric juice data was obtained through endoscopy from 14 patients without lung disease. Bacterial and fungal isolates were identified by culture. Molecular bacterial profiling used next generation sequencing (NGS) of the 16S rRNA gene. Cultures grew bacteria and/or fungi in all CF gastric juice and sputa and in 9/14 non-CF gastric juices. Pseudomonas aeruginosa(Pa) was present in CF sputum in 11 patients, 4 had identical Pa strains in the stomach. NGS data from non-CF gastric juice samples were significantly more diverse compared to CF samples. NGS showed CF gastric juice had markedly lower abundance of normal gut bacteria; Bacteroides and Faecalibacterium, but increased Pseudomonas compared with non-CF. Multivariate partial least squares discriminant analysis demonstrated similar bacterial profiles of CF sputum and gastric juice samples, which were distinct from non-CF gastric juice. We provide novel evidence suggesting the existence of an aerodigestive microbiome in CF, which may have clinical relevance.
Pub.: 02 Jun '16, Pinned: 23 Apr '17
Abstract: Gastro-oesophageal reflux (GOR) is common in patients with cystic fibrosis (CF). The aim of this study was to investigate the relationship between gastric emptying (GE) and GOR in children with CF.Multichannel intraluminal impedance-pH monitoring (MII-pH) to measure GOR and GE breath test (GEBT) to measure GE were performed in 28 children with symptoms suggestive for GOR disease (GORD) (group 1). GEBT was performed in another 28 children with/without GOR symptoms who agreed to undergo GEBT but not MII-pH (group 2).In group 1, we found increased acid GOR (AGOR) in 46.4% and delayed GE (DGE) in 21.4% but no relationship between increased AGOR and DGE. There was no DGE in group 2. We found DGE in 10.7% and rapid GE in 12.5% of the whole group.Almost half of the children with CF and symptoms suggestive for GORD have increased AGOR and almost a quarter has DGE. However, there was no relation between GOR and GE.
Pub.: 19 Jan '16, Pinned: 23 Apr '17
Abstract: Chronic respiratory infections are a leading global cause of morbidity and mortality. However, the molecular triggers that cause respiratory pathogens to adopt persistent and often untreatable lifestyles during infection remain largely uncharacterised. Recently, bile aspiration caused by gastro-oesophageal reflux (GOR) has emerged as a significant complication associated with respiratory disease, and cystic fibrosis (CF) in particular. Based on our previous finding that the physiological concentrations of bile influence respiratory pathogens towards a chronic lifestyle in vitro, we investigated the impact of bile aspiration on the lung microbiome of respiratory patients. Sputum samples (n = 25) obtained from a cohort of paediatric CF patients were profiled for the presence of bile acids using high-resolution liquid chromatography-mass spectrometry (LC-MS). Pyrosequencing was performed on a set of ten DNA samples that were isolated from bile aspirating (n = 5) and non-bile aspirating (n = 5) patients. Both denaturing gradient gel electrophoresis (DGGE) and pyrosequencing revealed significantly reduced biodiversity and richness in the sputum samples from bile aspirating patients when compared with non-aspirating patients. Families and genera associated with the pervasive CF microbiome dominated aspirating patients, while bacteria associated with the healthy lung were most abundant in non-aspirating patients. Bile aspiration linked to GOR is emerging as a major host trigger of chronic bacterial infections. The markedly reduced biodiversity and increased colonisation by dominant proteobacterial CF-associated pathogens observed in the sputum of bile aspirating patients suggest that bile may play a major role in disease progression in CF and other respiratory diseases.
Pub.: 13 May '14, Pinned: 23 Apr '17
Abstract: Gastro-oesophageal reflux (GER) has a special meaning for patients with cystic fibrosis (CF). Twelve voluntary patients with CF up to the age of 25 underwent an oesophageal manometry and a 24-hour impedance-pH monitoring. These patients were without symptoms of GER. The examination proved an acid GER in 42 %. In the total population the frequency is ≤ 10 %. In 11 of 12 patients a pathologically low pressure of the lower oesophageal sphincter (LES) was found. No significant correlations between the DeMeester score and the pressure of the LES, the reflux and respiratory symptomatology, the lung function as well as the quality of life could be proven. However, there was a significant correlation between the DeMeester score and the acid clearance time. 37 % of the registered cough pushes were related to a GER, of which 78 % were associated with an acid GER. Therefore, coughing in patients with CF must not necessarily be caused by the underlying disease. The timely detection of a pathological GER in patients with CF, but without symptoms of GER, and its prompt therapy could protect the lung function.
Pub.: 11 Apr '14, Pinned: 23 Apr '17
Abstract: Increased gastro-oesophageal reflux (GER) is common in patients with cystic fibrosis (CF). Previous studies showed delayed gastric emptying (GE) and a high prevalence of bile acids in saliva suggesting duodenogastro-oesophageal reflux (DGER).To assess different types of reflux (acid, weakly acidic and bile) and their relationship with rate of GE in adult CF patients.Gastric emptying was assessed in 33 CF patients using breath tests, reflux was monitored in 42 patients using impedance-pH-metry and 14 CF patients underwent combined impedance-pH-Bilitec monitoring.Delayed GE was found in 33%, increased GER (predominantly acid) in 67% and pathological DGER in 35% of the CF patients. There was a significant correlation between oesophageal bile and acid exposure (P < 0.0001, r = 0.85). Patients with increased DGER had a higher proximal extent of reflux compared to those without DGER [17 (9-35) vs. 5 (1-12), P = 0.04]. There was no correlation between GE and reflux parameters, however, in a subgroup of 10 patients studied by impedance-pH-Bilitec and GE, there was a strong correlation between GE rate and bile exposure (P = 0.005, r = 0.83).Delayed gastric emptying is present in 1/3 of patients with cystic fibrosis. There is a subgroup of these patients with both delayed gastric emptying and increased acidic duodenogastro-oesophageal reflux with high proximal extent and risk of aspiration. Controlled studies should be performed to evaluate the effect of prokinetics or antireflux surgery on the clinical cystic fibrosis evolution in these patients.
Pub.: 29 Jul '11, Pinned: 23 Apr '17
Abstract: There have been several recent articles which have attempted to refine the appropriate timing of listing for lung transplantation and the identification of factors which increase the risk with particular emphasis on pretransplant microbiology in patients with cystic fibrosis. Moreover, there are several series describing long-term outcomes and demonstrating improved long-term survival following lung transplantation for patients with cystic fibrosis. This review attempts to summarize the main points.Although forced expiratory volume in 1 s still plays an important role in timing for listing for lung transplantation, other factors such as arterial PaCO2, number of exacerbations, sex, weight, development of pneumothorax and microbiology of colonizing bacteria with particular relevance to Burkholderia all have been shown to influence survival in patients with advanced cystic fibrosis. The median survival should now be around 10 years, and strategies to reduce renal damage and chronic allograft dysfunction including preventing gastro-oesophageal reflux and airway infection receive greater emphasis.This article attempts to provide an overview of the current important topics for the clinician involved in cystic fibrosis lung transplantation.
Pub.: 09 Dec '08, Pinned: 23 Apr '17
Abstract: Gastro-oesophageal reflux (GOR) is increased in cystic fibrosis (CF), but its prevalence, characteristics, association with gastric aspiration and respiratory impact are not well characterised. We investigated acid and weakly acidic reflux, aspiration and respiratory symptoms/function in adult CF patients.Thirty-three CF patients [19 men; 29 (18-55) years, [10 post-lung transplant (LTx)] underwent impedance-pH monitoring for detection of acid (pH<4) and weakly acid GOR (pH 4-7). In 16 patients cough was objectively recorded with oesophageal manometry, and the symptom association probability (SAP) was calculated. Saliva and bronchoalveolar lavage fluid (BALF) were tested for bile acids.Twenty-eight patients had increased GOR (21 acid, 5 weakly acidic and 2 acid+weakly acidic) and 10 had a positive SAP for reflux cough. GOR parameters were similar in non-LTx and post-LTx CF patients. The sequence reflux cough was significantly more common than cough reflux. Sixteen of 38 patients had bile acids in saliva and 6/10 in BALF and this was almost exclusively observed in patients with genotype DF508/DF508. Only 12/28 with increased GOR and 9/22 with bile acids in saliva/BALF had typical reflux symptoms. There was a positive correlation (r = 0.53, p = 0.03) between oesophageal acid exposure and cough. SAP-positive patients with for reflux cough had a lower lung function than SAP-negative patients.Increased GOR is prevalent in CF and not secondary to cough. Acid GOR is common, but weakly acidic GOR may also occur. CF patients have a high risk of aspiration and reflux seems to be associated with more cough and poorer lung function. Outcome studies with intense anti-reflux therapy are needed to confirm the deleterious role of reflux in CF progression.
Pub.: 29 Mar '08, Pinned: 23 Apr '17
Abstract: Cystic Fibrosis (CF) is an autosomal recessive disease that affects the function of a number of organs, principally the lungs, but also the gastrointestinal tract. The manifestations of cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction in the gastrointestinal tract, as well as frequent antibiotic exposure, undoubtedly disrupts the gut microbiota. To analyse the effects of CF and its management on the microbiome, we compared the gut microbiota of 43 individuals with CF during a period of stability, to that of 69 non-CF controls using 454-pyrosequencing of the 16S rRNA gene. The impact of clinical parameters, including antibiotic therapy, on the results was also assessed.The CF-associated microbiome had reduced microbial diversity, an increase in Firmicutes and a reduction in Bacteroidetes compared to the non-CF controls. While the greatest number of differences in taxonomic abundances of the intestinal microbiota was observed between individuals with CF and the healthy controls, gut microbiota differences were also reported between people with CF when grouped by clinical parameters including % predicted FEV1 (measure of lung dysfunction) and the number of intravenous (IV) antibiotic courses in the previous 12 months. Notably, CF individuals presenting with severe lung dysfunction (% predicted FEV1 ≤ 40%) had significantly (p < 0.05) reduced gut microbiota diversity relative to those presenting with mild or moderate dysfunction. A significant negative correlation (-0.383, Simpson's Diversity Index) was also observed between the number of IV antibiotic courses and gut microbiota diversity.This is one of the largest single-centre studies on gut microbiota in stable adults with CF and demonstrates the significantly altered gut microbiota, including reduced microbial diversity seen in CF patients compared to healthy controls. The data show the impact that CF and it's management have on gut microbiota, presenting the opportunity to develop CF specific probiotics to minimise microbiota alterations.
Pub.: 11 Mar '17, Pinned: 23 Apr '17
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