B.M. Button

Postural drainage and gastro-oesophageal reflux in infants with cystic fibrosis

Gastro-oesophageal reflux is increased in cystic fibrosis and it is possible that postural drainage techniques may exacerbate reflux, potentially resulting in aspiration and further impairment of pulmonary function. AIM To evaluate the effects of physiotherapy with head down tilt (standard physiotherapy, SPT) on gastro-oesophageal reflux and to compare this with physiotherapy without head down tilt (modified physiotherapy, MPT). METHOD Twenty (mean age 2.1 months) infants with cystic fibrosis underwent 30 hour oesophageal pH monitoring during which SPT and MPT were carried out for two sessions each on consecutive days. RESULTS The number of reflux episodes per hour, but not their duration, was significantly increased during SPT compared with MPT (SPT 2.5 (0.4) v MPT 1.6 (0.3), p = 0.007) and to background (1.1 (0.)1, p = 0.0005). Fractional reflux time was also increased during SPT (11.7 (2.6)%) compared with background (6.9 (1.3)%, p = 0.03) but not compared with MPT (10.7 (2.7)%). There was no significant difference between MPT and background for number of reflux episodes, their duration, or fractional reflux time. CONCLUSION SPT, but not MPT, was associated with a significant increase in gastro-oesophageal reflux in infants with cystic fibrosis.

Gastro-oesophageal reflux in infants under 6 months with cystic fibrosis

AIM To establish the incidence of pathological gastro-oesophageal reflux (GOR) in newly diagnosed infants with cystic fibrosis and to identify clinical predictors of increased reflux. METHODS 26 infants with cystic fibrosis less than 6 months of age (14 male, 12 female; mean (SEM) age 2.1 (0.21) months, range 0.8 to 5.6 months) underwent prolonged oesophageal pH monitoring (mean duration 27.1 (0.49) hours; range 21.3 to 30.2 hours). Reflux symptoms, anthropometric variables, pancreatic status, meconium ileus, genotype, and chest x ray findings were correlated with pH monitoring data. RESULTS Five infants (19.2%) had an abnormal fractional reflux time of greater than 10%, seven (26.9%) of 5–10%, and 14 (53.8%) of below 5%. Infants who presented with frequent vomiting had a significantly higher fractional reflux time than infants who had infrequent or no vomiting. There was no significant association between abnormal chest x rays and pathological GOR. Sex, genotype, nutritional status, meconium ileus, and pancreatic enzyme supplementation were not significantly associated with pathological GOR. CONCLUSIONS About one in five newly diagnosed infants with cystic fibrosis had pathological GOR. Pathologically increased reflux was present before radiological lung disease was established. Apart from frequent vomiting, no useful clinical predictors of pathological reflux were found.

Pulmonary Rehabilitation Following Lung Transplantation

Pulmonary rehabilitation (PR) following lung transplantation (LT) is regarded as part of best practice management; however, the optimal duration and composition of PR programs for LT patients is unknown. This study aimed to describe changes in functional outcomes of LT patients who participated in our standard outpatient post-LT PR program. A prospective, repeated measures design was used. Functional exercise capacity (6-minute walk distance [6MWD]), lung function (forced expiratory volume in 1 second [FEV(1)], forced vital capacity [FVC]) and quality of life (Short Form 34 [SF-36]) were assessed at 1, 2, and 3 months following LT. All subjects attended a 1-hour outpatient group exercise training class 3 days per week until 12 weeks post-LT and education sessions facilitated by the multidisciplinary team. Patients with postoperative complications (mechanical ventilation, major myopathy) were excluded. Data were analyzed using descriptive statistics and analysis of variance with repeated measures. Thirty-six subjects (50% men), 81% bilateral LT, mean age 46 +/- 14 years were included. Significant improvements were demonstrated in 6MWD (451 +/- 126 m to 543 +/- 107 m, P < .001), FEV(1) (71% +/- 18% to 81% +/- 4%, P < .0001), FVC (69% +/- 14% to 81% +/- 18%, P < .0001), and all SF36 domains (P < .05). Large improvements were seen in the first month of rehabilitation, with smaller but clinically significant improvements continuing in the second month. Further prospective, longitudinal studies are required to determine whether a longer period of pulmonary rehabilitation would result in additional improvements.

Chest physiotherapy, gastro-oesophageal reflux, and arousal in infants with cystic fibrosis

Background: Postural drainage chest physiotherapy in infants with cystic fibrosis (CF) exacerbates gastro-oesophageal reflux (GOR) and may contribute to a more rapid deterioration in lung function. Aims: To compare standard postural drainage chest physiotherapy (SPT) and a modified physiotherapy regimen (MPT) without head-down tilt, with regard to GOR, arousal state, and cardiorespiratory function. Methods: Twenty infants with CF underwent 30 hour oesophageal pH monitoring, during which four chest physiotherapy sessions were administered (day 1: MPT–SPT; day 2: SPT–MPT). Arousal state, heart rate, and oxygen saturation were documented for each of the physiotherapy positions (supine, prone, right lateral, and left lateral with (SPT) or without (MPT) 30° head-down tilt). Results: Significantly more reflux episodes occurred during SPT than during MPT, but there were no significant differences in median episode duration or fractional reflux time. During SPT, left lateral positioning was associated with fewer reflux episodes compared to other positions. During supine and prone positioning, more reflux episodes occurred during SPT than during MPT. Infants were significantly more likely to be awake or cry during SPT. There was a significant association between crying and reflux episodes for SPT. Non-nutritive sucking was associated with a significant reduction in reflux episodes during SPT. Oxygen saturation during SPT was significantly lower during crying and other waking, and non-nutritive sucking during SPT was associated with a significant increase in oxygen saturation. Conclusions: SPT is associated with GOR, distressed behaviour, and lower oxygen saturation.

Postural drainage in cystic fibrosis : Is there a link with gastro-oesophageal reflux?

To determine the clinical effects of a change from postural drainage (PD) to positive expiratory pressure chest physiotherapy (PEP) in children with cystic fibrosis (CF) and symptoms of gastro‐oesophageal reflux (GOR). To measure the effects of PD on GOR in children with CF.

Structure and Function of the Mucus Clearance System of the Lung

In cystic fibrosis (CF), a defect in ion transport results in thick and dehydrated airway mucus, which is difficult to clear, making such patients prone to chronic inflammation and bacterial infections. Physiotherapy using a variety of airway clearance techniques (ACTs) represents a key treatment regime by helping clear the airways of thickened, adhered, mucus and, thus, reducing the impact of lung infections and improving lung function. This article aims to bridge the gap between our understanding of the physiological effects of mechanical stresses elicited by ACTs on airway epithelia and the reported effectiveness of ACTs in CF patients. In the first part of this review, the effects of mechanical stress on airway epithelia are discussed in relation to changes in ion transport and stimulation in airway surface layer hydration. The second half is devoted to detailing the most commonly used ACTs to stimulate the removal of mucus from the airways of patients with CF.

Clinical determinants of the 6-Minute Walk Test in bronchiectasis

BACKGROUND The 6-Minute Walk Test (6MWT) is a widely used measurement of functional exercise capacity in chronic lung disease. While exercise intolerance has been identified in patients with bronchiectasis, the clinical determinants of the 6MWT in this population have not been examined. The aim of this study was to 1) establish the relationship between the 6-Minute Walk Distance (6MWD), disease severity and Health-Related Quality of Life (HRQOL) and 2) identify predictors of exercise tolerance in adults with bronchiectasis. METHODS The 6MWT was performed in 27 patients with bronchiectasis (mean [SD] FEV(1) 73.9% predicted [23.4]). Disease severity was assessed using spirometry and HRCT scoring while HRQOL was evaluated using the St Georges Respiratory Questionnaire (SGRQ) and the Short-Form 36 (SF-36). The relationships were evaluated using correlation and multiple regression. RESULTS The 6MWD correlated positively with FVC (r=0.52, p<0.01), generations of bronchopulmonary divisions (r(s)=0.38, p<0.05) and SF-36 physical summary (r=0.71, p<0.001) while a negative correlation was observed between all domains of the SGRQ (all correlations r>0.5, p<0.001). Multiple regression analysis indicated that the SGRQ activity, symptom scores and generations of bronchial divisions involved were identified as independent predictors of the 6MWD, explaining 76% of the variance. CONCLUSIONS Measures of HRQOL demonstrated a stronger association with the 6MWD compared to physiological measures of disease severity in patients with predominantly mild to moderate bronchiectasis.

Proximal and distal gastro‐oesophageal reflux in chronic obstructive pulmonary disease and bronchiectasis

The aims of this observational study were (i) to examine the prevalence of symptomatic and clinically silent proximal and distal gastro‐oesophageal reflux (GOR) in adults with chronic obstructive pulmonary disease (COPD) or bronchiectasis, (ii) the presence of gastric aspiration, and (iii) to explore the possible clinical significance of this comorbidity in these conditions.

Medium-term outcome of fundoplication after lung transplantation

Gastroesophageal reflux disease (GERD) in lung transplant recipients has gained increasing attention as a factor in allograft failure. There are few data on the impact of fundoplication on survival or lung function, and less on its effect on symptoms or quality of life. Patients undergoing fundoplication following lung transplantation from 1999 to 2005 were included in the study. Patient satisfaction, changes in GERD symptoms, and the presence of known side effects were assessed. The effect on lung function, body mass index, and rate of progression to the bronchiolitis obliterans syndrome (BOS) were recorded. Twenty-one patients (13 males), in whom reflux was confirmed on objective criteria, were included, with a mean age of 43 years (range 20-68). Time between transplantation and fundoplication was 768 days (range 145-1524). The indication for fundoplication was suspected microaspiration in 13 and symptoms of GERD in 8. There was one perioperative death, at day 17. There were three other late deaths. Fundoplication did not appear to affect progression to BOS stage 1, although it may have slowed progression to stage 2 and 3. Forced expiratory volume-1% predicted was 72.9 (20.9), 6 months prior to fundoplication and 70.4 (26.8), six months post-fundoplication, P= 0.33. Body mass index decreased significantly in the 6 months following fundoplication (23 kg/m(2) vs. 21 kg/m(2), P= 0.05). Patients were satisfied with the outcome of the fundoplication (mean satisfaction score 8.8 out of 10). Prevalence of GERD symptoms decreased significantly following surgery (11 of 14 vs. 4 of 17, P= 0.002). Fundoplication does not reverse any decline in lung function when performed at a late stage post-lung transplantation in patients with objectively confirmed GERD. It may, however, slow progression to the more advanced stages of BOS. Reflux symptoms are well controlled and patients are highly satisfied. Whether performing fundoplication early post-lung transplant in selected patients can prevent BOS and improve long-term outcomes requires formal evaluation.

Pain impacts on quality of life and interferes with treatment in adults with cystic fibrosis.

BACKGROUND AND PURPOSE Pain has been reported in cystic fibrosis (CF), but its clinical significance and the physical and psychosocial impact in adults who are clinically stable and acutely unwell have not been well described. The aim of this study was to describe the intensity and location of pain and its relationship with health-related quality of life (HRQOL) and pain catastrophizing in adults with CF. METHODS This study was an observational study of adults with CF. Participants completed three questionnaires, the Brief Pain Inventory, Pain Catastrophizing Scale and the CF-Quality of Life questionnaire, when clinically stable and during an acute exacerbation. RESULTS A total of 73 participants were included during a period of clinical stability, with 33 repeating the measurements during an acute illness, with a mean (SD) age of 29 (9) years and forced expiratory volume (FEV(1) ) of 60.5 (24.9)% predicted. Mild pain was reported by 89% of stable participants and 79% of those with exacerbations. Severity of lung disease did not affect prevalence or intensity of pain. Pain interfered with airway clearance therapy during exacerbations (p < 0.012) and exercise regimens when participants were clinically stable (p < 0.002) and was related to a poorer physical function, regardless of clinical status (p < 0.05). Although pain intensity was associated with reduced HRQOL (p < 0.001), only FEV(1) and the degree of pain catastrophizing were independent predictors of poorer HRQOL. CONCLUSIONS Pain is common in adults with CF, irrespective of clinical status, and may interfere with important physiotherapy treatments. Although pain intensity is generally mild, those with a negative emotional response to pain have significantly impaired HRQOL.