Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is an inherited life-limiting disorder. Over time persistent infection and inflammation within the lungs contribute to severe airway damage and loss of respiratory function. Chest physiotherapy, or airway clearance techniques (ACTs), are integral in removing airway secretions and initiated shortly after CF diagnosis. Conventional chest physiotherapy (CCPT) generally requires assistance, while alternative ACTs can be self-administered, facilitating independence and flexibility. This is an updated review. OBJECTIVES: To evaluate the effectiveness (in terms of respiratory function, respiratory exacerbations, exercise capacity) and acceptability (in terms of individual preference, adherence, quality of life) of CCPT for people with CF compared to alternative ACTs. SEARCH METHODS: We used standard, extensive Cochrane search methods. The latest search was 26 June 2022. SELECTION CRITERIA: We included randomised or quasi-randomised controlled trials (including cross-over design) lasting at least seven days and comparing CCPT with alternative ACTs in people with CF. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcomes were 1. pulmonary function tests and 2. number of respiratory exacerbations per year. Our secondary outcomes were 3. quality of life, 4. adherence to therapy, 5. cost-benefit analysis, 6. objective change in exercise capacity, 7. additional lung function tests, 8. ventilation scanning, 9. blood oxygen levels, 10. nutritional status, 11. mortality, 12. mucus transport rate and 13. mucus wet or dry weight. We reported outcomes as short-term (seven to 20 days), medium-term (more than 20 days to up to one year) and long-term (over one year). MAIN RESULTS: We included 21 studies (778 participants) comprising seven short-term, eight medium-term and six long-term studies. Studies were conducted in the USA (10), Canada (five), Australia (two), the UK (two), Denmark (one) and Italy (one) with a median of 23 participants per study (range 13 to 166). Participant ages ranged from newborns to 45 years; most studies only recruited children and young people. Sixteen studies reported the sex of participants (375 males; 296 females). Most studies compared modifications of CCPT with a single comparator, but two studies compared three interventions and another compared four interventions. The interventions varied in the duration of treatments, times per day and periods of comparison making meta-analysis challenging. All evidence was very low certainty. Nineteen studies reported the primary outcomes forced expiratory volume in one second (FEV1)and forced vital capacity (FVC), and found no difference in change from baseline in FEV1 % predicted or rate of decline between groups for either measure. Most studies suggested equivalence between CCPT and alternative ACTs, including positive expiratory pressure (PEP), extrapulmonary mechanical percussion, active cycle of breathing technique (ACBT), oscillating PEP devices (O-PEP), autogenic drainage (AD) and exercise. Where single studies suggested superiority of one ACT, these findings were not corroborated in similar studies; pooled data generally concluded that effects of CCPT were comparable to those of alternative ACTs. CCPT versus PEP We are uncertain whether CCPT improves lung function or has an impact on the number of respiratory exacerbations per year compared with PEP (both very low-certainty evidence). There were no analysable data for our secondary outcomes, but many studies provided favourable narrative reports on the independence achieved with PEP mask therapy. CCPT versus extrapulmonary mechanical percussion We are uncertain whether CCPT improves lung function compared with extrapulmonary mechanical percussions (very low-certainty evidence). The annual rate of decline in average forced expiratory flow between 25% and 75% of FVC (FEF25-75) was greater with high-frequency chest compression compared to CCPT in medium- to long-term studies, but there was no difference in any other outcome. CCPT versus ACBT We are uncertain whether CCPT improves lung function compared to ACBT (very low-certainty evidence). Annual decline in FEF25-75 was worse in participants using the FET component of ACBT only (mean difference (MD) 6.00, 95% confidence interval (CI) 0.55 to 11.45; 1 study, 63 participants; very low-certainty evidence). One short-term study reported that directed coughing was as effective as CCPT for all lung function outcomes, but with no analysable data. One study found no difference in hospital admissions and days in hospital for exacerbations. CCPT versus O-PEP We are uncertain whether CCPT improves lung function compared to O-PEP devices (Flutter device and intrapulmonary percussive ventilation); however, only one study provided analysable data (very low-certainty evidence). No study reported data for number of exacerbations. There was no difference in results for number of days in hospital for an exacerbation, number of hospital admissions and number of days of intravenous antibiotics; this was also true for other secondary outcomes. CCPT versus AD We are uncertain whether CCPT improves lung function compared to AD (very low-certainty evidence). No studies reported the number of exacerbations per year; however, one study reported more hospital admissions for exacerbations in the CCPT group (MD 0.24, 95% CI 0.06 to 0.42; 33 participants). One study provided a narrative report of a preference for AD. CCPT versus exercise We are uncertain whether CCPT improves lung function compared to exercise (very low-certainty evidence). Analysis of original data from one study demonstrated a higher FEV1 % predicted (MD 7.05, 95% CI 3.15 to 10.95; P = 0.0004), FVC (MD 7.83, 95% CI 2.48 to 13.18; P = 0.004) and FEF25-75 (MD 7.05, 95% CI 3.15 to 10.95; P = 0.0004) in the CCPT group; however, the study reported no difference between groups (likely because the original analysis accounted for baseline differences). AUTHORS' CONCLUSIONS: We are uncertain whether CCPT has a more positive impact on respiratory function, respiratory exacerbations, individual preference, adherence, quality of life, exercise capacity and other outcomes when compared to alternative ACTs as the certainty of the evidence is very low. There was no advantage in respiratory function of CCPT over alternative ACTs, but this may reflect insufficient evidence rather than real equivalence. Narrative reports indicated that participants prefer self-administered ACTs. This review is limited by a paucity of well-designed, adequately powered, long-term studies. This review cannot yet recommend any single ACT above others; physiotherapists and people with CF may wish to try different ACTs until they find an ACT that suits them best.

The 2 min step test: A reliable and valid measure of functional capacity in older adults post coronary revascularisation.

PURPOSE: Exercise training is the central component in Cardiac Rehabilitation (CR). A baseline assessment of aerobic capacity is paramount for exercise prescription and safety. The Two-Minute Step Test (2MST) has been used to measure aerobic capacity in healthy older adults. However, the reliability and validity of the 2MST in older adults post-coronary revascularisation (CRV) is unknown. METHODS: A prospective observational study was conducted in a single CR centre. Two 2MSTs and one six-minute walk test (6MWT) were completed in a single session. The 2MST measurements were recorded by two raters for each individual. The six-minute walk distance (6MWD) and 2MST steps recorded by both raters were analysed to determine the relationship and agreement between measurements. RESULTS: Thirty one participants with a median (IQR) age of 66 (62,73) years old were included in the study post CRV. Strong positive correlations were found between steps achieved during the 2MSTs and the 6MWD (r = 0.87, 95% CI 0.82-0.91, p < 0.0001). Excellent inter-rater reliability was demonstrated between raters during the 2MSTs (ICC = 0.999-1.000, p = 0.000). Excellent relative test-retest reliability was demonstrated in both 2MSTs recorded by both raters (ICC = 0.927-0.934, p = 0.000). However, absolute test-retest reliability may have been limited by a learning effect between repeated 2MSTs. CONCLUSIONS: The results of this study indicate that the 2MST may be used as an alternative to the 6MWT as an outcome measure for aerobic capacity in older adults post-CRV. However, a practice trial is recommended at baseline to account for a learning effect.

The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus.

BACKGROUND: The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such guidelines. METHOD: On 30th June and 1st July 2021, 39 stakeholders from 11 countries, including researchers, healthcare professionals and patients participated in a virtual conference to agree an evidence-based and informed expert consensus about PA and exercise for people with CF. This consensus presents the agreement across six themes: (i) patient and system centred outcomes, (ii) health benefits, iii) measurement, (iv) prescription, (v) clinical considerations, and (vi) future directions. The consensus was achieved by a stepwise process, involving: (i) written evidence-based synopses; (ii) peer critique of synopses; (iii) oral presentation to consensus group and peer challenge of revised synopses; and (iv) anonymous voting on final proposed synopses for adoption to the consensus statement. RESULTS: The final consensus document includes 24 statements which surpassed the consensus threshold (>80% agreement) out of 30 proposed statements. CONCLUSION: This consensus can be used to support health promotion by relevant stakeholders for people with CF.

Feasibility, safety and acceptability of select outcome measures in a physiotherapy study protocol for boys with haemophilia.

BACKGROUND: There is a lack of functional performance measures for children and young people with haemophilia (CYPwH) with associated control data from typically developing boys (TDB). The literature advocates development of a core set of outcome measures for different chronic conditions. As medical treatment improves, CYPwH are experiencing better outcomes; therefore, more challenging measures are required to monitor physical performance. Such testing is not performed routinely, due to practical and safety concerns. AIM: Evaluate the feasibility, safety and acceptability of select outcome measures as part of a study protocol testing CYPwH; including myometry, 10 metre incremental shuttle walk test (10-m ISWT), iSTEP (an incremental step test, with data from TDB), and 1 week of accelerometry-wear at home. METHODS: Sixty-six boys aged 6-15 years with mild, moderate or severe haemophilia A or B (including inhibitors) attending routine clinics at Great Ormond Street Hospital were approached to participate. Descriptive statistics and content analysis were used to assess outcomes of feasibility, safety and acceptability, which included recruitment/retention rates, protocol completion within routine appointment timeframes, performance testing without serious adverse events/reactions (SAE/SARs), and acceptability to CYPwH of high-level performance measures. RESULTS: Outcomes were met: 43 boys completed testing at clinic review (Jan-Nov 2018) within a 10-month timeframe, retention was 95% at completion of protocol and no SAE/SARs were reported throughout testing. CONCLUSION: Feasibility, safety and acceptability of the study protocol have been established in this population. Both high-level performance tests, iSTEP and 10-m ISWT, were an acceptable addition to boys' routine clinic appointments and could be safe, acceptable choices of outcome measure as part of a core set of tests for CYPwH. Further investigation of the psychometric properties for the iSTEP is now justified, in order for it to be used as a standardised, validated, reliable outcome measure in clinical or research settings. TRIAL REGISTRATION: Retrospectively registered on September 3, 2019, on ClinicalTrials.gov (ID: NCT04076306 ).

The impact of physical activity and exercise interventions for physical health in people with cystic fibrosis: protocol for a systematic review.

BACKGROUND: Cystic fibrosis (CF) is a genetically inherited, life-limiting condition, affecting ~90,000 people globally. Physical activity (PA) and exercise form an integral component of CF management, and have been highlighted by the CF community as an area of interest for future research. Previous reviews have solely focused on PA or structured exercise regimens independent of one another, and thus a comprehensive assessment of the physical health benefits of all PA, including exercise, interventions, is subsequently warranted. Therefore, the purpose of this review is to evaluate the effects of both PA and exercise upon outcomes of physical health and healthcare utilisation in people with CF. METHODS: A systematic review has been registered and reported in line with Preferred Reporting Items for Systematic Reviews and Meta-Analysis-P guidelines. This will include randomised control trials on the effects of PA and exercise, relative to usual treatment, upon people with CF. Primary outcomes will include variables associated with fitness, PA, lung health, inflammation, body composition, glycaemic control and patient-reported outcomes. Secondary outcomes will include adverse events and healthcare utilisation. Searches will be undertaken in Ovid MEDLINE, OVID EMBASE, PsychINFO, ERIC, SPORTDiscus, ASSIA, CCTR, CINHAL and Web of Science databases, and will be searched from date of inception onwards. Two reviewers will independently screen citations and abstracts, and full-texts, for inclusion and data extraction, respectively. Methodological quality will be assessed using the Cochrane Risk of Bias-2 tool. If feasible, random-effects meta-analyses will be conducted where appropriate. Additional analyses will explore potential sources of heterogeneity, such as age, sex, and disease severity. DISCUSSION: This systematic review will build on previous research, by comprehensively assessing the impact of both PA and exercise upon physical health and healthcare utilisation in people with CF. Results of this review will be utilised to inform discussions that will ultimately result in a consensus document on the impact of physical activity and exercise for people with CF. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42020184411.

Hypermobility, developmental coordination disorder and physical activity in an Irish paediatric population.

OBJECTIVE: The primary aim of the study was to explore the prevalence of generalized joint hypermobility (GJH) and generalized hypermobility spectrum disorder (gHSD) using the new classification system in a community paediatric physiotherapy service in Ireland. The second aim was to explore the relationship between GJH, gHSD and physical activity level, while considering the association of probable developmental coordination disorder (pDCD). METHODS: A case-controlled cross-sectional study of children aged 6-12 years, recruited from the community paediatric physiotherapy department (n = 32) and a local school (n = 41), was carried out. A Beighton score of ≥6/9 distinguished GJH. The new framework for hypermobility spectrum disorder (HSD) was used. Self-reported physical activity level was measured using the Physical Activity Questionnaire-Older Children. A parent-reported validated questionnaire screened for pDCD. RESULTS: The prevalence of GJH was 21.9% of children attending physiotherapy. One child in the physiotherapy group was identified as having gHSD, with a prevalence of 3.1%. There was no significant difference in physical activity level between children with and without GJH attending physiotherapy (independent samples t-test, p = 0.28). Probable developmental coordination disorder (pDCD) was observed in 71.9% of children attending physiotherapy. There was no significant difference in the number of children with pDCD in those with and without GJH (Fisher's exact test, p = 0.370). CONCLUSIONS: This study was the first to explore the prevalence of GJH and gHSD in the paediatric physiotherapy population in Ireland. The presence of GJH did not affect self-reported physical activity level or motor coordination in children attending physiotherapy.

Quality of hypertension care: An improvement initiative in two outpatient health care centers.

RATIONALE, AIMS, AND OBJECTIVES: Hypertension control is an important public health goal; however, significant barriers remain in primary care practice. Our objective was to identify areas for improvement in hypertension care and implement changes in management to improve outcomes. We also aimed to evaluate whether quality improvement influences physician attitudes towards and adherence to current hypertension guidelines. METHOD: We conducted a non-experimental pre- vs post- design quality improvement study for ambulatory patients with a history of hypertension. Specific measures of hypertension care were assessed at baseline and 3 months post-implementation of the quality improvement initiative. De-identified data were collected from 100 charts, randomly selected from the practice's electronic medical records, and compared with a national sample of peer data. The Intervention was based on the American Academy of Family Physicians METRIC Performance Improvement module. This consisted of creating a computerized registry, system improvements to the electronic medical records, and peer education workshops on best practices. A 7-item survey was completed by primary care physicians pre- and post-intervention. RESULTS: Improvement was demonstrated in several primary outcome measures: increased number of patients counselled on sodium intake (P = 0.005), physical activity (P = 0.001), alcohol consumption (P = 0.03), and weight reduction (P < 0.0001). Practice self-assessment findings did not show a statistically significant change following the intervention. CONCLUSIONS: This quality improvement increased provider compliance with hypertension guidelines. However, more effort is required to modify physician practices for full compliance with the 2017 updated hypertension guidelines.

Airway clearance strategies in cystic fibrosis and non-cystic fibrosis bronchiectasis.

Many patients with cystic fibrosis (CF) and non-CF bronchiectasis present with common symptoms in clinical domains that appear to benefit from airway clearance strategies. These symptoms include chronic productive cough, retention of excessive, purulent mucus in dilated airways, impairment of normal mucociliary clearance (MCC), atelectasis, breathlessness, fatigue, respiratory inflammation, fever, infection, and airflow obstruction. Airway clearance strategies may involve singular and focused interventions for the purpose of removing secretions and improving lung recruitment and gas exchange in patients with atelectasis. Strategies may also involve indirect or adjunctive interventions that facilitate or enhance effective airway clearance at different ages or stages of the disease process, for example, inhalation therapy, exercise, oxygen therapy, or noninvasive ventilation. The aim is to optimize care by selecting any one or combination of these in responding intelligently and sensitively to individual and changing patient requirements during their lifetime. Currently, a solid evidence base does not exist for airway clearance strategies in CF and non-CF bronchiectasis, and much of airway clearance clinical practice remains in the domain of clinical expertise. The paucity of evidence is partly explained by the relatively immature research machinery in allied health care internationally but is also partly to do with inadequate or inappropriate research designs. This article aims to provide an overview of the nature of, and physiological basis for, the direct and indirect airway clearance strategies in CF and non-CF bronchiectasis with reference to the best available evidence.

Exercise as part of a cystic fibrosis therapeutic routine.

The role of exercise in cystic fibrosis (CF) is well established, and over the last three decades it has become an important component in the management of all individuals with CF. The role of exercise as a prognostic indicator or therapeutic tool is an important area of research interest in CF care internationally. This article summarizes the currently available evidence regarding exercise capacity in CF, the potential effects of exercise on health outcomes in CF and the challenges faced when trying to incorporate exercise into a CF therapeutic routine, and highlights some methods to facilitate the incorporation of exercise into CF therapeutic routines.