Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis.

BACKGROUND: Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis (CF). Positive expiratory pressure (PEP) devices provide back pressure to the airways during expiration. This may improve clearance by building up gas behind mucus via collateral ventilation and by temporarily increasing functional residual capacity. The developers of the PEP technique recommend using PEP with a mask in order to avoid air leaks via the upper airways and mouth. In addition, increasing forced residual capacity (FRC) has not been demonstrated using mouthpiece PEP. Given the widespread use of PEP devices, there is a need to determine the evidence for their effect. This is an update of a previously published review. OBJECTIVES: To determine the effectiveness and acceptability of PEP devices compared to other forms of physiotherapy as a means of improving mucus clearance and other outcomes in people with CF. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. The electronic database CINAHL was also searched from 1982 to 2017. Most recent search of the Group's CF Trials Register: 20 February 2019. SELECTION CRITERIA: Randomised controlled studies in which PEP was compared with any other form of physiotherapy in people with CF. This included, postural drainage and percussion (PDPV), active cycle of breathing techniques (ACBT), oscillating PEP devices, thoracic oscillating devices, bilevel positive airway pressure (BiPaP) and exercise. DATA COLLECTION AND ANALYSIS: Three authors independently applied the inclusion and exclusion criteria to publications, assessed the risk of bias of the included studies and assessed the quality of the evidence using the GRADE recommendations. MAIN RESULTS: A total of 28 studies (involving 788 children and adults) were included in the review; 18 studies involving 296 participants were cross-over in design. Data were not published in sufficient detail in most of these studies to perform any meta-analysis. In 22 of the 28 studies the PEP technique was performed using a mask, in three of the studies a mouthpiece was used with nose clips and in three studies it was unclear whether a mask or mouthpiece was used. These studies compared PEP to ACBT, autogenic drainage (AD), oral oscillating PEP devices, high-frequency chest wall oscillation (HFCWO) and BiPaP and exercise. Forced expiratory volume in one second was the review's primary outcome and the most frequently reported outcome in the studies (24 studies, 716 participants). Single interventions or series of treatments that continued for up to three months demonstrated little or no difference in effect between PEP and other methods of airway clearance on this outcome (low- to moderate-quality evidence). However, long-term studies had equivocal or conflicting results regarding the effect on this outcome (low- to moderate-quality evidence). A second primary outcome was the number of respiratory exacerbations. There was a lower exacerbation rate in participants using PEP compared to other techniques when used with a mask for at least one year (five studies, 232 participants; moderate- to high-quality evidence). In one of the included studies which used PEP with a mouthpiece, it was reported (personal communication) that there was no difference in the number of respiratory exacerbations (66 participants, low-quality evidence). Participant preference was reported in 10 studies; and in all studies with an intervention period of at least one month, this was in favour of PEP. The results for the remaining outcome measures (including our third primary outcome of mucus clearance) were not examined or reported in sufficient detail to provide any high-quality evidence; only very low- to moderate-quality evidence was available for other outcomes. There was limited evidence reported on adverse events; these were measured in five studies, two of which found no events. In a study where infants performing either PEP or PDPV experienced some gastro-oesophageal reflux , this was more severe in the PDPV group (26 infants, low-quality evidence). In PEP versus oscillating PEP, adverse events were only reported in the flutter group (five participants complained of dizziness, which improved after further instructions on device use was provided) (22 participants, low-quality evidence). In PEP versus HFCWO, from one long-term high-quality study (107 participants) there was little or no difference in terms of number of adverse events; however, those in the PEP group had fewer adverse events related to the lower airways when compared to HFCWO (high-certainty evidence). Many studies had a risk of bias as they did not report how the randomisation sequence was either generated or concealed. Most studies reported the number of dropouts and also reported on all planned outcome measures. AUTHORS' CONCLUSIONS: The evidence provided by this review is of variable quality, but suggests that all techniques and devices described may have a place in the clinical treatment of people with CF. Following meta-analyses of the effects of PEP versus other airway clearance techniques on lung function and patient preference, this Cochrane Review demonstrated that there was high-quality evidence that showed a significant reduction in pulmonary exacerbations when PEP using a mask was compared with HFCWO. It is important to note that airway clearance techniques should be individualised throughout life according to developmental stages, patient preferences, pulmonary symptoms and lung function. This also applies as conditions vary between baseline function and pulmonary exacerbations.

Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis.

BACKGROUND: Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis. Positive expiratory pressure (PEP) devices provide back pressure to the airways during expiration. This may improve clearance by building up gas behind mucus via collateral ventilation and by temporarily increasing functional residual capacity. Given the widespread use of PEP devices, there is a need to determine the evidence for their effect. This is an update of a previously published review. OBJECTIVES: To determine the effectiveness and acceptability of PEP devices compared to other forms of physiotherapy as a means of improving mucus clearance and other outcomes in people with cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. The electronic database CINAHL was also searched from 1982 to 2013.Most recent search of the Group's Cystic Fibrosis Trial Register: 02 December 2014. SELECTION CRITERIA: Randomised controlled studies in which PEP was compared with any other form of physiotherapy in people with cystic fibrosis. This included, postural drainage and percussion, active cycle of breathing techniques, oscillating PEP devices, thoracic oscillating devices, bilevel positive airway pressure (BiPaP) and exercise. Studies also had to include one or more of the following outcomes: change in forced expiratory volume in one second; number of respiratory exacerbations; a direct measure of mucus clearance; weight of expectorated secretions; other pulmonary function parameters; a measure of exercise tolerance; ventilation scans; cost of intervention; and adherence to treatment. DATA COLLECTION AND ANALYSIS: Three authors independently applied the inclusion and exclusion criteria to publications and assessed the risk of bias of the included studies. MAIN RESULTS: A total of 26 studies (involving 733 participants) were included in the review. Eighteen studies involving 296 participants were cross-over in design. Data were not published in sufficient detail in most of these studies to perform any meta-analysis. These studies compared PEP to active cycle of breathing techniques (ACBT), autogenic drainage (AD), oral oscillating PEP devices, high frequency chest wall oscillation (HFCWO) and Bi level PEP devices (BiPaP) and exercise.Forced expiratory volume in one second was the review's primary outcome and the most frequently reported outcome in the studies. Single interventions or series of treatments that continued for up to three months demonstrated no significant difference in effect between PEP and other methods of airway clearance on this outcome. However, long-term studies had equivocal or conflicting results regarding the effect on this outcome. A second primary outcome was the number of respiratory exacerbations. There was a lower exacerbation rate in participants using PEP compared to other techniques when used with a mask for at least one year. Participant preference was reported in 10 studies; and in all studies with an intervention period of at least one month, this was in favour of PEP. The results for the remaining outcome measures were not examined or reported in sufficient detail to provide any high-level evidence. The only reported adverse event was in a study where infants performing either PEP or postural drainage with percussion experienced some gastro-oesophageal reflux. This was more severe in the postural drainage with percussion group. Many studies had a risk of bias as they did not report how the randomisation sequence was either generated or concealed. Most studies reported the number of dropouts and also reported on all planned outcome measures. AUTHORS' CONCLUSIONS: Following meta-analyses of the effects of PEP versus other airway clearance techniques on lung function and patient preference, this Cochrane review demonstrated that there was a significant reduction in pulmonary exacerbations in people using PEP compared to those using HFCWO in the study where exacerbation rate was a primary outcome measure. It is important to note, however, that there may be individual preferences with respect to airway clearance techniques and that each patient needs to be considered individually for the selection of their optimal treatment regimen in the short and long term, throughout life, as circumstances including developmental stages, pulmonary symptoms and lung function change over time. This also applies as conditions vary between baseline function and pulmonary exacerbations.However, meta-analysis in this Cochrane review has shown a significant reduction in pulmonary exacerbations in people using PEP in the few studies where exacerbation rate was a primary outcome measure.

Physiotherapy and cystic fibrosis: what is the evidence base?

PURPOSE OF REVIEW: To provide a comprehensive overview and evidence to support the role of physiotherapy in the management of individuals with cystic fibrosis (CF) including airway clearance, exercise, and musculoskeletal concerns which can affect activities of daily living and respiratory health. RECENT FINDINGS: Several long-term studies have looked at the efficacy of airway clearance techniques, including active cycle of breathing techniques, autogenic drainage, high frequency chest wall oscillation, postural drainage, positive expiratory pressure (PEP), and oscillating PEP. Each of these studies reported some efficacy of airway clearance in maintaining health with no one technique being superior to another. However, one study suggested that high frequency chest wall oscillation was not as effective as PEP in maintaining health in CF patients. Individual preference needs to be considered when selecting a technique. Recent studies have found exercise to increase mucociliary clearance peripherally. Musculoskeletal issues, including posture, bone density, urinary incontinence, and pain should be assessed and managed in individuals to improve the mechanics of breathing and overall well-being. SUMMARY: The role of physiotherapy in CF is complex and includes airway clearance, exercise, and management of the long-term sequelae of musculoskeletal issues. More rigorous physiotherapy studies are required to assist with evidence based practice.

Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis.

BACKGROUND: Positive expiratory pressure (PEP) is the most commonly used method of airway clearance (AC) in Canada for patients with cystic fibrosis (CF) whereas, in some countries, high frequency chest wall oscillation (HFCWO) is the preferred form of AC. There have been no long-term studies comparing the efficacy of HFCWO and PEP in the CF population. OBJECTIVES: To determine the long-term efficacy of HFCWO compared with PEP mask therapy in the treatment of CF as measured by the number of pulmonary exacerbations (PEs). METHODS: A randomised controlled study was performed in 12 CF centres in Canada. After a 2-month washout period, subjects were randomised to perform either HFCWO or PEP mask therapy for 1 year. RESULTS: 107 subjects were enrolled in the study; 51 were randomised to PEP and 56 to HFCWO. There were 19 dropouts within the study period, of which 16 occurred prior to or at the time of randomisation. There were significant differences between the groups in the mean number of PEs (1.14 for PEP vs 2.0 for HFCWO) and time to first PE (220 days for PEP vs 115 days for HFCWO, p=0.02). There was no significant difference in lung function, health-related quality of life scores or patient satisfaction scores between the two groups. PEP mask therapy required a shorter treatment time. CONCLUSIONS: The results of this study favour PEP and do not support the use of HFCWO as the primary form of AC in patients with CF. CLINICAL TRIAL REGISTRATION NUMBER: NCT00817180.

Personalising airway clearance in chronic lung disease.

This review describes a framework for providing a personalised approach to selecting the most appropriate airway clearance technique (ACT) for each patient. It is based on a synthesis of the physiological evidence that supports the modulation of ventilation and expiratory airflow as a means of assisting airway clearance. Possession of a strong understanding of the physiological basis for ACTs will enable clinicians to decide which ACT best aligns with the individual patient's pathology in diseases with anatomical bronchiectasis and mucus hypersecretion.The physiological underpinning of postural drainage is that by placing a patient in various positions, gravity enhances mobilisation of secretions. Newer ACTs are based on two other physiological premises: the ability to ventilate behind obstructed regions of the lung and the capacity to achieve the minimum expiratory airflow bias necessary to mobilise secretions. After reviewing each ACT to determine if it utilises both ventilation and expiratory flow, these physiological concepts are assessed against the clinical evidence to provide a mechanism for the effectiveness of each ACT. This article provides the clinical rationale necessary to determine the most appropriate ACT for each patient, thereby improving care.

Long-term comparative trial of two different physiotherapy techniques; postural drainage with percussion and autogenic drainage, in the treatment of cystic fibrosis.

UNLABELLED: In N. America, over the past decade, various airway clearance techniques (ACT) have been introduced for the treatment of cystic fibrosis (CF). We hypothesized that autogenic drainage (AD), an ACT developed in Belgium would be as effective as postural drainage with percussion (PD) in treating patients with CF. METHODS: Thirty-six CF patients, aged 12-18 years, with Shwachman score 65-98, were enrolled in a 2-year cross-over trial. Patients were matched as pairs and members of each pair were randomly assigned to two groups. For the first study year, Group A performed PD while Group B performed AD. At the end of 1 year Groups A and B crossed over physiotherapy techniques. Clinical status and pulmonary function (FVC, FEV(1), FEF(25-75)) were measured at 3 monthly intervals. Only results from the first year of the study are reported, as 10 out of 17 patients who had completed performing AD for the first year refused to change back to PD for the second year. RESULTS: During the first year of the study, both the AD and PD groups demonstrated improved pulmonary function with no significant difference between the two groups. Change in FVC and FEV(1) percent predicted for Groups A and B was 0.47 ± 1.65(se) versus 2.35 ± 1.51(se) and 2.09 ± 2.2(se) versus 0.92 ± 2.25(se). However, CF patients exhibited a marked preference for the AD technique. Results suggest that both AD and PD are effective methods of performing physiotherapy for CF patients and that the benefits of either technique are enhanced by measures which encourage adherence.

Chest physical therapy, breathing techniques and exercise in children with CF.

Chest physiotherapy in the form of airway clearance techniques and exercise has played an important role in the treatment of cystic fibrosis. Until the 1990s the primary airway clearance technique used was postural drainage combined with percussion and vibration (PD&P). It was introduced into the treatment of CF with little evidence to support its efficacy and once established, it has been difficult ethically to perform a study comparing PD&P to no treatment. A common question, yet unanswered is when should it be commenced, especially for the newly diagnosed asymptomatic CF patient? Recently, the technique of PD&P has been modified to include only non-dependant head-down positioning due to the detrimental effects of placing a person in a Trendelenburg position. In the 1990s other airway clearance techniques gained popularity, in that they could be performed independently, in a sitting position and avoided many of the detrimental effects of PD&P. These techniques include the Active cycle of breathing technique, formally called the Forced expiration technique and Autogenic drainage. Both these breathing techniques aim at using expiratory airflow to mobilize secretions up the airways and incorporate breathing strategies to assist in the homogeneity of ventilation. Studies suggest that both these techniques are as effective if not more effective than as PD&P and offer many advantages over PD&P. It has been suggested that exercise can be used as an airway clearance technique; however the literature does not support this. Rather, when exercise is used in addition to an airway clearance technique there is enhanced secretion removal and an overall benefit to the patient. Further research needs to be directed at assessing the effects of an airway clearance technique on the individual patient using appropriate outcome measures.