Article

An exploratory study investigating factors associated with adherence to chest physiotherapy and exercise in adults with cystic fibrosis

September 2009
Journal of Cystic Fibrosis 8(6):425-7

September 2009
8(6):425-7

DOI:10.1016/j.jcf.2009.08.005

Source
PubMed

Authors:

Lynn b Myers

Brunel University London

This study explored the relationship between psychological and demographic variables relating to chest physiotherapy (CP) and exercise in adults with cystic fibrosis. The main results were that adherence to both treatments was low and analysis of variance indicated that severity and gender were associated with exercise adherence, importance and burden. These results suggest potential areas for interventions to improve exercise adherence.

Factors influencing physical activity in adults with cystic fibrosis

Article

Full-text available

Apr 2021
BMC Pulm Med

Background Physical activity (PA) is a well-documented and accepted adjunct therapy for the maintenance and improvement of long-term health in cystic fibrosis (CF). Although the benefits of PA for CF populations are well-established, adherence to PA programmes within this population remains low. This study aimed to investigate the factors that influence engagement in physical activity, and to explore exercise preferences, among adults with cystic fibrosis (CF). Methods Semi-structured telephone interviews were conducted. Participants were twenty-one adults (mean age 35 years, SD ± 8) with an established diagnosis of CF, living in Ireland. Interview scripts were digitally recorded and transcribed verbatim. Thematic analysis was used to analyse the data. Results Four main themes emerged: barriers, motives, value of exercise-related outcomes, and exercise preferences . The main barriers included: low energy levels, time, the weather, and exercise-related confidence. Enjoyment and perceived competence underpinned autonomous motivation. Participants who self-identified as being regularly active valued personally identified exercise-related outcomes such as, accomplishment and affect regulation. Participants indicated a preference for home-based physical activity programs compared to gym- or facility-based programs. Conclusion Interventions aimed at promoting physical activity among adults with CF should involve programs that foster autonomous motivation, enjoyable activities, personally identified outcomes, competence and that can be conducted from the home environment. Clinical implications To increase physical activity participation among adults with CF, interventions that can be conducted from the home environment, that pay attention to the patients’ personally-valued exercise outcomes may be required.

Development and Validation of the AdT-Physio Scale: A Tool to Assess Adherence and Perception of Physical Therapist Intervention in Patients With Cystic Fibrosis

Article

Aug 2020
PHYS THER

Objective: The purpose of this study was to develop a scale to measure patient adherence to physical therapist intervention and to report psychometric properties in patients with cystic fibrosis (CF). Methods: This observational, multicenter, qualitative, and cross-sectional study was divided into 2 phases: development of scale items (content validity) and assessment of psychometric properties (construct validity, reliability, and convergent/discriminant validity). Recruited were 121 patients with CF (aged ≥16 y). Sociodemographic characteristics, lung function testing, Coping with Stress Self-Efficacy (EAEAE), brief Coping Orientation to Problems Experienced inventory (COPE-28), and the AdT-Physio scale were evaluated. Results: The final version of the AdT-Physio scale consists of 15 items distributed across 2 subscales: compliance and beliefs about treatment/therapist. The scale showed high internal consistency (Cronbach α = 0.897; 95% CI = 0.868-0.922) and subscales above 0.800. The 2-factor confirmatory factor analysis model fitted the data reasonably well: χ2 (76) = 111.96, comparative fit index = 0.982, Tucker-Lewis index = 0.978, root mean square error of approximation = 0.063, 95% CI = 0.036 to 0.086, weighted root mean square residual = 0.720. No floor or ceiling effects were identified. There was a positive, significant, and moderate-low magnitude correlation with the total COPE-28 (r = .360) and EAEAE subscale efficacy expectations scores (r = .304). For discriminant validity, there was a positive, significant, and moderate correlation between the total and the age of the patients (r = .354). Conclusion: The AdT-Physio scale is psychometrically valid and reliable for use in the clinic for the assessment of adherence to physiotherapy in patients with CF. Impact: Ultimately, the authors propose this documentary instrument to assess the evaluation of the therapeutic alliance in a valid and objective manner. The AdT-Physio scale provides us with a greater degree of understanding of the problems behind noncompliance with treatment to advance person-centered decisions in physical therapy and thereby enhance the effectiveness of care.

Videogames in the Treatment of Obstructive Respiratory Diseases: A Systematic Review

Article

Aug 2019

Introduction: Millions of people suffer from obstructive respiratory conditions globally. Including videogames in rehabilitation programs can be an interesting alternative to traditional programs or a complementary activity. Objective: To explore the use of videogames in the treatment of patients with obstructive respiratory diseases. Methods: A systematic literature search was performed to identify randomized clinical trials evaluating the effects of videogames on health outcomes in patients with respiratory obstructive diseases. The following databases were searched: PubMed, Web of Science, ScienceDirect, and Scopus. The methodological quality of the studies included was assessed with the Downs and Black quality assessment method. Results: Nine articles were included, of which three used videogames as physical training methods and managed to improve exercise capacity, strength, quality of life, and symptoms. The remaining six articles used videogames to educate patients about the disease and showed slight improvements in knowledge of the disease and use of medication. Conclusion: The results of this review show that videogames are a very useful complementary therapy. They can contribute to enhance rehabilitation programs, as they improve exercise capacity, muscle strength, quality of life, severity, control, and knowledge of the disease. Videogames should be supervised by a professional so that programs can be tailored to patients, proposing different activities in the game and controlling the correct performance, and generating an increase in adherence to treatment. Registry: prospective register of systematic reviews (PROSPERO) CRD42018094094.

Physiotherapy adherence and perception in patients with cystic fibrosis: A descriptive study

Article

Sep 2015

Predictors of adherence to treatment in bronchiectasis

Article

Apr 2015

CF-CATS: An uncontrolled feasibility study of using tai chi for adults with cystic fibrosis

Article

Dec 2013

Introduction Cystic fibrosis (CF) can adversely affect quality of life. This study on tai chi (TC) for cystic fibrosis (CF) was an uncontrolled pilot trial assessing the feasibility of a future controlled trial. Methods Adults with CF, recruited from the Royal Brompton Hospital, UK, were taught weekly TC (based on the “eternal spring” set) for 5 weeks in participants’ homes. They continued with DVD and home practice for 5 further weeks. Cystic fibrosis questionnaire revised (CFQ-R), hospital anxiety and depression scale and brief pain inventory were completed at baseline, 5 and 10 weeks. Healthcare use, TC practice, symptoms, and wellbeing were recorded weekly, medical data at baseline and 10 weeks. Data compared before and after TC. Two online focus groups explored experiences and feedback on research methods. Results Eleven patients were recruited, mean age 38, five working/studying, baseline predicted FEV1 between 26% and 74%. Treatment constraints and respiratory symptoms (CFQ-R) significantly improved (mean decrease 27.78 (p = 0.023) and 11.90 (p = 0.05) respectively). Numerous health fluctuations were recorded. TC was perceived as a “powerful tool”, relaxing and calming. Challenges to TC practice included hospitalisation, poor health, other treatments and forgetting movements. The DVD was helpful. No problems participating were reported. Conclusion TC may reduce CF treatment impact, improve respiratory symptoms, self-efficacy and sleep. Participants’ poor and fluctuating health and the small sample potentially affected results. Such research appears appropriate and feasible. Suggestions for a future trial include measurements of quality of life, dypsnoea, self-efficacy, coping, sleep, exercise levels, longer follow up and frequent assessment.

Gaming console exercise and cycle or treadmill exercise provide similar cardiovascular demand in adults with cystic fibrosis: A randomised cross-over trial

Article

Full-text available

Mar 2011

Does exercise using a gaming console result in similar cardiovascular demand and energy expenditure as formally prescribed exercise in adults with cystic fibrosis? How do these patients perceive gaming console exercise? Randomised cross-over trial with concealed allocation and intention-to-treat analysis. 19 adults with cystic fibrosis admitted to hospital for treatment of a pulmonary exacerbation. Participants underwent two 15-minute exercise interventions on separate days; one involving a gaming console and one a treadmill or cycle ergometer. Cardiovascular demand was measured using heart rate and rating of perceived exertion (RPE). Energy expenditure was estimated using a portable activity monitor. Perception (enjoyment, fatigue, workload, effectiveness, feasibility) was rated using a horizontal 10-cm visual analogue scale. There was no significant difference in average heart rate (mean difference 3 beats/min, 95% CI -3 to 9) or energy expenditure (0.1 MET, 95% CI -0.3 to 0.5) between the two interventions. Both interventions provided a 'hard' workout (RPE ∼15). Gaming console exercise was rated as more enjoyable (mean difference 2.6 cm, 95% CI 1.6 to 3.6) than formal exercise but they didn't differ significantly in fatigue (-1.0 cm, 95% CI -2.4 to 0.3), perceived effectiveness (-0.4 cm, 95% CI -1.2 to 0.3), or perceived feasibility for inclusion in routine management (0.2 cm, 95% CI -0.7 to 1.1). Gaming console exercise provides a similar cardiovascular demand as traditional exercise modalities. It is feasible that adults with cystic fibrosis could include gaming console exercise in their exercise program. ACTRN12610000861055.

Video Game‐Based Exercise in Children and Adolescents with Non‐Cystic Fibrosis Bronchiectasis: A Randomized Comparative Study of Aerobic and Breathing Exercises

Article

Jun 2022

Background: Video game-based systems have been proposed to improve effectiveness and compliance with exercise training in children and adolescents with non-cystic fibrosis bronchiectasis (NCFB). This study aimed to investigate the effects of aerobic and breathing video game-based exercises (VGE) on pulmonary function, respiratory and peripheral muscle strength, functional capacity, and balance in children and adolescents with NCFB. Method: Thirty-nine children and adolescents aged between 8-18 years with NCFB were randomly allocated into three groups as "home-based chest physiotherapy group" (CP), "aerobic VGE given in addition to home-based chest physiotherapy group" (CP+aerobic VGE), and "breathing VGE given in addition to home-based chest physiotherapy group" (CP+breathing VGE). All 3 groups performed chest physiotherapy program twice a day for 7 days per week for 8 weeks. Pulmonary function, respiratory and peripheral muscle strength, functional capacity, and balance were assessed at baseline and after 8 weeks of training. Results: The improvement in maximum expiratory pressure and balance scores were significantly higher in both CP+aerobic and CP+breathing VGE groups. The significant improvement in maximum inspiratory pressure was greater in the CP+breathing VGE group. The changes in peripheral muscle strength and functional capacity were significantly higher in the CP+aerobic VGE group. Conclusions: The present study showed that aerobic VGE provides additional benefits in improving peripheral muscle strength and functional capacity, while breathing VGE provides further increase in improving respiratory muscle strength. In addition, both aerobic and breathing VGE were effective in improving balance, but they were not superior to each other. This article is protected by copyright. All rights reserved.

Gaming Console Home-Based Exercise for Adults with Cystic Fibrosis: Study Protocol

Article

Full-text available

Apr 2020

Background: Despite evidence of exercise benefits to lung function, adherence to routine exercise in adults with cystic fibrosis (CF) is low. The incorporation of interactive virtual reality video exergame activities in home-based programs as an incentive may help improve motivation and adherence to exercise. This proposed study will attempt to improve the physical fitness and respiratory function of sedentary adults with CF by engaging them in a Nintendo Wii Fit Plus™ home-based exercise program. Methods: A single group pretest-posttest design will be used to examine the immediate (12-weeks) and long-term effect (24-weeks) of a home-based exergame program on improving pulmonary-related function (physical fitness and respiratory function) in sedentary adults with CF. Participants will receive a one-time orientation to the Wii Fit Plus, and will be requested to use it to exercise according to the recommended guidelines 3 times a week for 30 min in the following 24 weeks. Monthly phone monitoring will be conducted during the first 12 weeks. Besides evaluating the efficacy of a home-based exergame program on improving aerobic capacity, physical activity, and respiratory-related symptoms, we will examine the impact of the exergame on airway ion transport as measured by nasal potential difference, which will be collected at baseline and at the end of 12-weeks only. Discussion: This is the first study to evaluate the feasibility, acceptability and potential effectiveness of a low-cost exercise avenue (i.e., exergames) for adults with CF to improve their pulmonary-related function, which is important for CF disease management and prevention of complications. In addition, the proposed study will be the first to investigate the therapeutic efficacy of home-based exergames on airway ion transport among adults with CF. Through an increase in physical activity, it is expected that participants will improve their physical fitness and respiratory function at the end of the study. Trial registration: ClinicalTrials.gov ID: NCT02277860.

Tolérance à l’effort global et fatigabilité musculaire dans les maladies respiratoires chroniques : Méthodes d’évaluation et identification des mécanismes neuromusculaires sous-jacents. Whole-body exercise tolerance and muscle fatigability in chronic respiratory disorders: assessment methods and underlying neuromuscular mechanisms.

Research

Full-text available

Jun 2020

Mathieu Gruet

This post-doctoral dissertation (habilitation) aims to provide a detailed synthesis of my research activities performed after my PhD, across the 2011-2019 period. I have gathered and presented some works with the intention to better describe and understand the limitations to physical exercise in chronic respiratory disorders (CRD). My approach suggests a complementary between whole-body exercise tests and isolated muscle assessments. The usefulness of these tests is exemplified through the presentation of several studies conducted in people with cystic fibrosis, chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea. Collectively, some of our results suggest that each maximal and submaximal whole-body exercise test has its own interests and limitations. These tests should be viewed as complementary rather than interchangeable. The choice and frequency for the use of these tests should be considered with an individualized approach, taking into consideration the nature of the disease, its severity, some events modulating disease progression and hospital resources. Whole-body exercise tests, albeit useful to detect and quantify exercise intolerance, are often less adapted to identify some of the underlying physiological mechanisms. In particular, the various neuromuscular abnormalities observed in people with CRD legitimize the evaluation of physical abilities using tests that isolate peripheral muscle function. Despite arguments supporting peripheral muscle abnormalities in cystic fibrosis, our studies suggest a preserved muscle metabolic function and contractility during local fatiguing exercises in individuals with mild to moderate lung disease. Some of our recent works now investigate the potential influence of CFTR genotype severity on peripheral muscle function in people with cystic fibrosis. Beyond peripheral factors, there are strong evidences supporting corticospinal abnormalities in CRD, especially in severe phenotypes, possibly linked with peripheral muscle dysfunction in these individuals. We found that cerebrovascular and corticospinal abnormalities observed at rest in people with severe obstructive sleep apnea persisted during physical exercise, with a potential contribution to reduced muscle strength, increased muscle fatigability or reduced maximal aerobic capacities. The last part of this dissertation is dedicated to the presentation of future research perspectives. I propose a rationale that legitimate the examination of limitations to physical exercise in aging individuals with CRD in demanding cognitive-motor contexts. We are currently investigating the hypothesis of a particular increase in muscle fatigability in people with COPD during cognitive-motor dual-tasks, related with abnormalities in central command. The traditional neuromuscular indicators are, however, somewhat limited for the understanding of such a complex phenomenon, which is influenced by several interconnected components (i.e. cortical, spinal, muscular). We are currently developing a methodology for facilitating the use of various indicators of neuromuscular complexity derived from nonlinear dynamics, in the specific context of muscle fatigability. The use of such indicators adapted to the study of the complexity of the neuromuscular system will allow shedding light on the psychophysiological limitations experienced by the individuals with CRD in demanding cognitive-motor contexts. Such investigations will favor the development of new treatment modalities in these specific populations.

Perceptions towards physical activity in adult lung transplant recipients with cystic fibrosis

Article

Full-text available

Feb 2020
PLOS ONE

Background Barriers and motives towards physical activity (PA) in lung transplant (LTx) recipients with cystic fibrosis (CF) are largely unknown. We aimed to explore perceptions towards PA in LTx recipients with CF to better understand individuals’ needs and preferences. Methods Participants completed an online survey at two Swiss LTx and one follow-up shared care centre between June and December 2018. Results One hundred and eleven individuals completed the survey (87.4% response rate). Overall, survey participants perceive PA as important for their daily life and health. Perceived motives of PA were improving muscle strength, endurance and quality of life (QoL), to feel better, fun, to achieve personal goals and having more energy for everyday life. Fatigue was the most common perceived barrier to PA and associated with poorer QoL (r = -0.43, p<0.001) and health status (r = -0.31, p = 0.001). Participants with lung allograft dysfunction (LAD, n = 20) reported lower habitual PA (p = 0.009) and health status (p = 0.011), and rated shortness of breath, bad weather and concerns regarding lung rejection higher than those without LAD (all p<0.05). When we asked how an optimal training programme should look like, the majority would prefer individual, non-supervised (60%), outdoor (77%), endurance training (90%), once or twice a week (47%) for 40–60 minutes (48%). Only a minority of patients (14%) would be willing to use exercise applications for their home-based training. Conclusions LTx recipients with CF value PA as important for their health. People with CF should be encouraged individually by their multidisciplinary transplant team to implement PA in their daily life, potential barriers should be identified and addressed. Overall, knowledge on perceived barriers and motives for PA should be considered in the development of future patient-centred PA programmes.

Physical Activity Level and Perception of Exercise in Cystic Fibrosis

Article

Jan 2020

Background: More patients with cystic fibrosis (CF) are living longer, and lifestyle-related behavior is becoming increasingly important for improving morbidity and mortality. Declining levels of exercise leads to low cardiorespiratory fitness, which is a strong, independent predictor of mortality in patients with CF. As a result, exercise training has become a commonly accepted form of treatment for patients with CF. The purpose of this study was to determine physical activity levels and perception of exercise in adult patients with CF. Methods: Adult patients from an in-patient CF unit were recruited to participate. A structured interview and self-report questionnaires were used to collect information on levels of physical activity and exercise perception including preferences, readiness, and barriers. Results: Forty-six adult patients with CF consented to participate in the interview and completed self-report questionnaires. Subjects self-reported that the majority (84%) of their time was spent performing physical activity at a moderate level, with mean ± SD of 11.8 ± 12.2 h per week of moderate physical activity. Vigorous physical activity was described as hard and very hard physical activity, with a mean ± SD of 1.8 ± 4.6 h (13%) and 0.4 ± 1.6 h (3%), respectively. Most of the adult subjects with CF preferred walking, and 65% of them felt that exercise was very important. Lack of energy, lack of good health, lack of self-discipline, and lack of time were noted as the most frequent barriers to exercise. Conclusion: In this study, adult subjects with CF self-reported performing an adequate amount of moderate physical activity, although only a small proportion of time was spent at a vigorous level of physical activity. Clinicians providing rehabilitation have an opportunity to improve adherence to prescribed exercise by understanding the impact that physiological and psychological factors have on patients with CF. Further, motivating patients with CF to engage in more vigorous physical activity may provide a stimulus that improves clinical outcomes and potentially survival.

Exercise Physiology Across the Lifespan in Cystic Fibrosis

Article

Full-text available

Nov 2019

Cystic fibrosis (CF), a severe life-limiting disease, is associated with multi-organ pathologies that contribute to a reduced exercise capacity. At present, the impact of, and interaction between, disease progression and other age-related physiological changes in CF on exercise capacity from child- to adult-hood is poorly understood. Indeed, the influences of disease progression and aging are inherently linked, leading to increasingly complex interactions. Thus, when interpreting age-related differences in exercise tolerance and devising exercise-based therapies for those with CF, it is critical to consider age-specific factors. Specifically, changes in lung function, chronic airway colonization by increasingly pathogenic and drug-resistant bacteria, the frequency and severity of pulmonary exacerbations, endocrine comorbidities, nutrition-related factors, and CFTR (cystic fibrosis transmembrane conductance regulator protein) modulator therapy, duration, and age of onset are important to consider. Accounting for how these factors ultimately influence the ability to exercise is central to understanding exercise impairments in individuals with CF, especially as the expected lifespan with CF continues to increase with advancements in therapies. Further studies are required that account for these factors and the changing landscape of CF in order to better understand how the evolution of CF disease impacts exercise (in)tolerance across the lifespan and thereby identify appropriate intervention targets and strategies.

Overall and differentiated sensory responses to cardiopulmonary exercise test in patients with cystic fibrosis: kinetics and ability to predict peak oxygen uptake

Article

Full-text available

Sep 2018
EUR J APPL PHYSIOL

Purpose: We evaluated the validity of predicting peak oxygen uptake ([Formula: see text]O2peak) from submaximal ratings of perceived exertion (RPE) during incremental cardiopulmonary exercise test (CPET) in patients with cystic fibrosis (CF) and compared the predictive accuracy between overall and differentiated RPE scores. Methods: Thirty-five adults with CF (FEV1 = 58 ± 23%) performed a CPET on cycle ergometer with gas exchange measurements. Leg, chest and overall RPE were collected every minute throughout the test. Linear regressions between [Formula: see text]O2 and RPE ≤ 15 were extrapolated to maximal theoretical RPE (i.e. RPE18 and RPE19) to predict [Formula: see text]O2peak. Agreements between measured and all predicted [Formula: see text]O2peak were tested using Bland-Altman Plots, for the whole group and for subjects presenting significant exercise intolerance (n = 24). Results: Leg, chest and overall RPE increased similarly with exercise intensity. No differences were found between predicted [Formula: see text]O2peak and measured [Formula: see text]O2peak with RPE18 as maximal RPE, for both overall and differentiated RPE (P range 0.94-0.98). Ranges for Pearson correlations and limits of agreements were 0.88-0.91 and 380-461 mL min-1 for the whole group and 0.92-0.94 and 269-365 mL min-1 for subjects with significant exercise intolerance. The greatest association and narrowest limits of agreements were obtained from chest RPE scores. Conclusions: Submaximal RPE scores obtained during CPET can provide acceptable estimate of [Formula: see text]O2peak in adults with CF, particularly in those having significant exercise intolerance. Future studies should assess whether the prediction can be improved, particularly by encouraging the regular use of RPE scales during physical activities/exercise rehabilitations sessions.

Effectiveness of a Home-Based Active Video Game Programme in Young Cystic Fibrosis Patients

Article

Oct 2017
RESPIRATION

Background: Exercise-based rehabilitation is already a part of cystic fibrosis (CF) treatment; however, patient adherence is low. Objectives: To assess the effectiveness of a home exercise programme using active video games (AVGs) as a training modality for children and adolescents with CF. Methods: Thirty-nine children with CF were randomised to a control group (CG, n = 20, age 11 ± 6 years; FEV1 86.2 ± 20.5% of predicted) or a training group (AVGG, n = 19, age 13 ± 3 years; FEV1 82.7 ± 21.7% of predicted). The home training protocol consisted of 30- to 60-min sessions, 5 days/week, for 6 weeks using a Nintendo Wii™ platform. Exercise capacity was measured by the 6-min walk test (6MWT) and modified shuttle walk test (MSWT); muscular strength was estimated using the horizontal jump test (HJT), medicine ball throw (MBT), and hand grip strength (right [RHG]; left [LHG]); and quality of life was rated using the Cystic Fibrosis Questionnaire-Revised (CFQ-R). All the children were measured at baseline, after rehabilitation, and at 12 months. Results: For the group × time interaction ANOVAs, the AVGG showed significant between-group differences in exercise capacity: 6MWT farthest walking distance, 38.4 m (p < 0.01); MSWT farthest walking distance, 78.4 m (p < 0.05); and muscular strength: HJT 9.8 cm, MBT 30.8 cm, RHG 7 kg, and LHG 6.5 kg (p < 0.01), before versus after intervention. The CFQ-R reported significantly higher scores on respiratory symptoms after the intervention and favoured the AVGG, and there was an improvement in other domains after 12 months. Adherence to the home exercise programme was 95% during the 6- week intervention period. Conclusion: A home-based programme using AVGs can effectively improve exercise capacity, muscular strength and quality of life in the short-term in children and adolescents with CF. The effects of training on muscle performance and quality of life were sustained over 12 months.

Health Locus of Control and Health Seeking Behaviour among Medical Outpatients at Benue State University Teaching Hospital

Article

Aug 2017

Effects Of Exercise Intensity Compared To Albuterol In Individuals With Cystic Fibrosis

Article

Dec 2014
RESP MED

Although exercise is a vital component of the therapy prescribed to individuals with cystic fibrosis (CF), it is not a priority due to a finite amount of treatment time and the view that exercise is not as beneficial as pharmacological treatments by many individuals with CF. We sought to compare the therapeutic benefits of exercise and their prescribed bronchodilator albuterol. CF (n = 14) and healthy (n = 16) subjects completed three visits, a baseline screening with VO2 max test and two treatment visits. On the two treatment visits, subjects completed spirometry and diffusing capacity of the lungs for nitric oxide (DLNO) maneuvers either at baseline, 60, and 110 min post-albuterol administration, or at baseline and the midway point of three separate 15 min exercise bouts at low, moderate and vigorous intensity (25, 50 and 65% of the maximum workload, respectively). With moderate exercise the increase in DLNO was double (39 ± 8 vs 15 ± 6% change) and the level of bronchodilation similar (23% change) when compared to 110 min post-albuterol in individuals with CF. During exercise FVC became reduced (-309 ± 66 mL with moderate exercise) and the increase in FEV1 was attenuated (103 ± 39 vs 236 ± 58 mL, exercise vs. albuterol) when compared with the response to albuterol in individuals with CF. Epinephrine (EPI) release increased 39, 72 and 144% change with low, moderate and vigorous intensity exercise respectively for individuals with CF, but this increase was blunted when compared to healthy subjects. Our results suggest that moderate intensity exercise is the optimal intensity for individuals with CF, as low intensity exercise increases EPI less than 50% and vigorous intensity exercise is over taxing, such that airflow can be restricted. Although the duration of the beneficial effect is uncertain, exercise can promote greater improvements in gas diffusion and comparable bronchodilation when compared to albuterol. Copyright © 2014 Elsevier Ltd. All rights reserved.

Rehabilitation in Patients with Chronic Respiratory Disease Other than Chronic Obstructive Pulmonary Disease: Exercise and Physical Activity Interventions in Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Article

Feb 2015

A relevant proportion of children and adults with cystic fibrosis (CF) have a marked decrease in exercise tolerance, which can be partly related to impaired muscle function and decreased physical activity levels in daily life, in addition to lung disease. Preliminary findings suggest that patients with non-CF bronchiectasis face the same problems. These patients might be excellent candidates for exercise and physical activity interventions. This review elaborates on the rationale for exercise training and activity behaviour changes and summarizes the existing evidence for these rehabilitation strategies in patients with bronchiectasis, both CF and non-CF bronchiectasis. Furthermore, practical considerations and safety aspects are discussed. © 2015 S. Karger AG, Basel.

Inflammation, Oxidative Stress, and Cardiovascular Disease Risk Factors in Adults with Cystic Fibrosis

Article

Full-text available

Nov 2014
FREE RADICAL BIO MED

Cystic Fibrosis (CF) represents one of a number of localized lung and non-lung diseases with an intense chronic inflammatory component associated with evidence of systemic oxidative stress. Many of these chronic inflammatory diseases are accompanied by an array of atherosclerotic processes and cardiovascular disease (CVD), another condition strongly related to inflammation and oxidative stress. As a consequence of a dramatic increase in long lived patients with CF in recent decades, the specter of CVD must be considered in these patients who are now reaching middle age and beyond. Buttressed by recent data documenting that CF patients exhibit evidence of endothelial dysfunction, a recognized precursor of atherosclerosis and CVD, the spectrum of risk factors for CVD in CF are reviewed here. Epidemiological data further characterizing the presence and extent of atherogenic processes in CF patients would seem important to interrogate. Such studies should further inform and offer mechanistic insights into how other chronic inflammatory diseases potentiate the processes leading to CVDs.

Cystic fibrosis and the relevance of the whole-body vibration exercises in oscillating platforms: a short review

Article

Full-text available

Jan 2011

Objective: The aim is to present a review about the whole-body vibration (WBV) exercises in the management of cystic fibrosis (CF). Methods: Computerized literature searches were per-formed using the PubMed databases. Results: A strong interest in studies, considering the number of publications, involving CF and lung was found. Although, the exercises and physi-cal activities seem to have a high relevance in the management of the patients with CF, the number of publications involving these proce-dures is limited. Moreover, a modality of exer-cise involving WBV seems to be poorly utilized by the patients with CF and only two papers were found with positive actions. Conclusion: Oscillating platform is a promising technique to manage patients with CF. Moreover, as 1) the use of the oscillating platforms is inexpensive, 2) the vibration exercise requires less technical abilities as compared to the performance of conventional resistance training and 3) positive clinical findings have been noticed with the use of WBV in CF, it is suggested to implement the studies involving the application of the exer-cises with WBV in oscillating platforms to manage the patients with CF.

Meditative Movement for Respiratory Function: A Systematic Review

Article

Full-text available

Jul 2013

BACKGROUND: Meditative movement (MM; tai chi/yoga/qi gong) may be beneficial for people with cystic fibrosis (CF) as a form of gentle exercise incorporating meditation, breathing and relaxation. Respiratory function is the most common issue in CF. This systematic review synthesised the evidence for MM for respiratory function to provide a clinical summary and inform research into MM for CF. METHODS: Chinese and English language databases were searched using keywords for tai chi/yoga/qi gong, and respiratory function/cough/dyspnoea. Articles were screened and selected by two researchers. Controlled studies published in English/Chinese after 1980 were included. Data were extracted using a specially designed spreadsheet. Two researchers independently evaluated study quality and reporting using three standardised checklists. Meta-analysis was not possible due to heterogeneous methodology. RESULTS: 1649 papers were identified, 43 included (30 English language, 13 Chinese), 23 RCTs, 20 nonrandomised trials. None studied CF; 11 studied patients with respiratory disorders, 27 healthy people. Very few were high quality. The main bias with RCTs was randomisation and nonrandom/poorly reported sampling, for non-randomised studies poor reporting of samples and non-equivalent groups. Although no clinically significant changes were shown, MM may improve FEV1 in healthy people compared to no treatment/exercise (intervention groups showed changes from 0.07 to 0.83) but MM did not appear to affect FEV1/FVC ratio in COPD. Key study limitations were: poor reporting of sampling or methods; small, potentially underpowered samples; non-randomised design; lacking description of randomisation; randomisation by centre; no blinding; lack of reporting of important aspects of MM; short-term follow up. CONCLUSION: There is no evidence for MM in CF, and very limited evidence for respiratory function in healthy populations. Due to heterogeneity of populations and lack of sampling information, clinicallyrelevant conclusions cannot be drawn and more research is needed in this area, in particular powered, randomised studies.

Xbox Kinect (TM) represents high intensity exercise for adults with cystic fibrosis

Article

Jun 2013

Background: Exercise is important for patients with cystic fibrosis (CF). Interactive gaming consoles are a new trend in exercise. This study sought to determine the exercise intensity of training using the Xbox Kinect(™). Methods: Participants with CF completed two sessions separated by ≤10 days. The first session involved a cardiopulmonary exercise test (CPET) to measure peak exercise capacity. The second session involved 20 min of exercise using the Xbox Kinect™. Results: Ten participants (median [interquartile range] FEV1 58 [46]%, 29 [6] years, 6 males) completed the study. The average heart rate over the final 10 min of exercise using the Xbox Kinect™, expressed as a percentage of the peak heart rate achieved on the CPET, was 86% (95% confidence interval, 81 to 92%). Conclusions: Training using the Xbox Kinect™ represents high intensity exercise for adults with CF and may be a suitable alternative to conventional exercise modalities.

Exercise with incorporated expiratory manoeuvres was as effective as breathing techniques for airway clearance in children with cystic fibrosis: A randomised crossover trial

Article

Full-text available

Dec 2012

: Can a session of exercise with incorporated expiratory manoeuvres substitute for a session of breathing techniques for airway clearance in children with cystic fibrosis? Are children with cystic fibrosis as co-operative and satisfied with the exercise regimen as with the breathing techniques?. Randomised, cross-over trial with concealed allocation and intention-to-treat analysis. 34 children with cystic fibrosis in a stable clinical state. Participants underwent two 20-min airway clearance interventions on two scheduled clinic days: one involving three bouts of various whole-body exercise modalities each followed by independent expiratory manoeuvres, and the other involving breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique. Wet weight of expectorated sputum, change in lung function, co-operation with treatment, perceived treatment quality, and satisfaction with treatment were all assessed after each intervention. The wet weight of sputum after exercise was 0.6g higher after the exercise intervention, which was not statistically or clinically significant (95% CI -0.2 to 1.4). However, lung function and participant satisfaction with the treatment were both significantly better after the exercise intervention. Co-operation with treatment and perceived treatment quality were equally high for each intervention. A session of various whole-body exercises interspersed with independent expiratory manoeuvres could be an acceptable substitute for a session of breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique in children with mild cystic fibrosis lung disease.

Exercise as part of a cystic fibrosis therapeutic routine

Article

Jun 2012
Expet Rev Respir Med

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.

Exercise and quality of life in patients with cystic fibrosis: A 12-week intervention study

Article

Full-text available

Jul 2011
Physiother Theor Pract

It was hypothesised that increased exercise capacity is related to improved quality of life (QoL) in patients with cystic fibrosis (CF). A 12-week individually tailored unsupervised aerobic exercise programme was offered to 42 patients with CF. At the start and at the end of the exercise programme, data on QoL, current exercise habits and preferences, anthropometric data, exercise test, and lung function test were collected. Adherence was observed by a heart rate (HR) monitor. A total of 24 patients accepted to be enrolled in the exercise programme and 14 completed the programme. Another 14 patients declined to be enrolled in the exercise programme but completed the Cystic Fibrosis Questionnaire for Adolescents and Adults (CFQ-R 14+). Four patients did not want to participate at all. The 14 patients completing the exercise programme had a significantly increased VO(2max), but they showed no significant change in total QoL score. However, the scores in the domain of treatment burden and emotional functioning increased significantly. There was no significant difference in QoL and lung function between patients participating in the exercise programme (n = 24) and non-participants (n = 14). A 12-week individually tailored unsupervised aerobic exercise programme where HR monitors were used significantly affected VO(2max). Improvement in QoL could not be demonstrated in this study.

Self-care and cystic fibrosis: A review of research with adults

Article

Nov 2010
HEALTH SOC CARE COMM

The issue of self-care is becoming increasingly central to both policy and practice in health and social care in the community. It is imperative therefore that research in this important area is drawn together and presented coherently so as to ensure that change can be informed by evidence and implemented sensitively. As cystic fibrosis (CF) has until recently been regarded as a paediatric condition, there is relatively little research that focuses on the self-care of adults. Although not entirely uncritical of traditional biomedicine, these studies focus on individual patient deficits and are directed primarily at facilitating their 'compliance'. After discussing some important methodological, evidential and theoretical limitations of this research, other recent CF literature will be considered that suggests the possibility of developing a 'social model' for self-care research. The proposed model is more pluralistic and less prescriptive than its predecessors and the resulting 'types' of self-care indicate that both old and new, mainstream and marginal discourses should co-exist. Indeed, recognising the legitimacy of distinct varieties of self-care not only guards against unwarranted moralising and pathologising but may also enable self-care support to be negotiated and tailored more appropriately.

Incorporating exercise into the routine care of individuals with cystic fibrosis: Is the time right?

Article

Full-text available

Apr 2010

Trajectories of Adherence to Airway Clearance Therapy for Patients with Cystic Fibrosis

Article

Full-text available

Mar 2010
J PEDIATR PSYCHOL

Although cross-sectional studies have demonstrated poor adherence to airway clearance therapy (ACT) for patients with cystic fibrosis (CF), no studies have identified longitudinal patterns of adherence. The objective was to characterize and identify predictors of ACT adherence trajectories for individuals with CF. Secondary data analyses were conducted for a randomized clinical trial examining differences in three ACTs. Participants (n = 153; M = 14.3 years, 55% male, 86% Caucasian, baseline FEV(1)% predicted: M = 86.7)/primary caregivers completed Daily Phone Diaries, an empirically supported adherence measure, every 4 months. Group-based trajectory modeling revealed the best-fitting solution was a three-group model: low-adherence (14%), medium-adherence (49%), and high-adherence (37%) groups. ACT type was the only significant predictor of adherence trajectories. Three trajectories of adherence to ACT for patients with CF were found. With the identification of trajectories, adherence interventions can be targeted for the subgroup at highest risk in order to prevent poor health outcomes.

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

Article

May 2023

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 safety and sustainability of bottle-pep therapy in pediatric patients with cystic fibrosis

Article

Dec 2021

Introduction Airway clearance techniques, which include positive expiratory pressure (PEP) devices, are essential in the pulmonary rehabilitation of cystic fibrosis (CF). Bottle-PEP is a low-cost but an effective alternative. Objective The aim of this study is to document the sustainability and safety of Bottle-PEP therapy as a home rehabilitation aid. Methods The study has been designed as a prospective case series. Patients with CF at the age of 6–18 years in acute exacerbation period were included in the study. Bottle-PEP training was given by a competent physiotherapist to those patients who did not use any method, and those who currently use another device were followed up with their existing devices. Thus, patients divided into two groups were followed up for 1 year. The patients were evaluated by phone every 2 weeks for exacerbation, regular and proper use of the device, and satisfaction during their follow-up. The patients were evaluated every 3 months with pulmonary function tests, 6-minute walking test (6MWT) and quality of life. Results Thirty-four patients were included in the study. The acute exacerbation score of the patients was 4.5 in the Bottle-PEP group and 6 in the other group, showing no significant difference (p = .1). Treatment compliance scores were compared, the median value of the Bottle-PEP group was 24 the other group was 27 and there was no significant difference (p = .6). During follow-up of, there were no significant differences in FEV1, 6MWT and quality of life data (p > .05). Conclusion Bottle-PEP treatment is not different from other devices in terms of long-term usability and safety in patients diagnosed with cystic fibrosis.

Elicitation interview study to identify salient beliefs about exercise participation in adults with cystic fibrosis

Article

Oct 2019
PHYSIOTHERAPY

Objectives: Conduct an elicitation study, using the Theory of Planned Behaviour framework, to identify salient beliefs about exercise participation in adults with cystic fibrosis (CF). Specifically, identify attitudes on advantages and disadvantages of exercise (behavioural beliefs); individuals and groups who apply social pressure to exercise (normative beliefs); and perceived control over facilitators and barriers to exercise (control beliefs) for adults with CF. Design: Qualitative interviews using open-ended, structured questions. Setting: Adult CF clinic in a large Australian hospital. Participants: Sixteen adults with CF, three relatives/friends of adults with CF and six CF clinic staff. Results: The most common positive attitudes about exercise were to keep fit and healthy (68%) and feel better and happier (60%), and negative attitude was to feel breathless (36%). Social pressure to exercise mainly came from parents/family (72%) and friends (52%), and 60% of participants reported that no-one discourages exercise. Having someone to exercise with (44%) and be encouraged (36%) were the most common facilitators of perceived control to exercise, whereas being unwell (96%) and not having sufficient time (56%) were the most common barriers. Conclusions: Attitudes, social pressure and perceived control to exercise for adults with CF were similar to beliefs previously reported by the general population and some patient groups. A number of CF-specific exercise beliefs, mainly related to pulmonary function, were also reported. These findings can help develop questionnaires for larger groups of adults with CF, interpret relationships between exercise beliefs and participation, and inform clinicians to target interventions to increase exercise participation.

Current perspectives of physical activity in cystic fibrosis

Article

Nov 2018

Introduction: Regular exercise and physical activity participation are recommended in guideline-based care for individuals with cystic fibrosis (CF) across the spectrum of age and disease severity. However, the best training methods to improve physical function and encourage ongoing exercise and activity participation are not clear, which is an ongoing challenge for clinicians. Areas covered: This perspective provides an overview of current evidence for exercise and physical activity relative to clinical outcomes and healthcare utilisation in people with CF, and highlights areas of future research need. Expert commentary: What kind, how much, how often and how best to support people with CF to be physically active is uncertain. Whether new methods of training, the use of technology, or pharmaceutical developments, could best deliver increased activity and physiological benefit without increased therapeutic burden is unclear. At present, if people with CF are going to be physically active, seemingly they should aim to perform this activity in the way most likely to confer some health benefit i.e. concerted exercise bouts of at least ten minutes in addition to any incidental (habitual) activity performed during the course of daily life.

Singing Lessons for Respiratory Health: A Literature Review

Article

Apr 2017

Rachel B. Goldenberg

Objective: Several studies have explored the role of music and singing as a treatment for respiratory symptoms. The objective of this paper was to review the current body of literature in regard to the use of singing as both a physiological and a psychological therapy for respiratory disease and assess the role the singing teacher might play in this treatment. Study design: This is a literature review, discussion of results and directions for further research. Method: Multiple databases were searched using keywords such as "respiratory," "physiotherapy," and "pulmonary" in conjunction with "singing." Studies that met selection criteria were summarized and analyzed. Results: Seventeen studies pertaining to multiple conditions including chronic obstructive pulmonary disease, asthma, cystic fibrosis, cancer, Parkinson disease, quadriplegia, and multiple sclerosis were analyzed. All studies reported trends of positive physical and/or quality of life outcomes after a series of singing lessons, regardless of statistical significance. Several noted improvements in maximum expiratory pressure and overall breathing technique. Many studies included open-ended interviews revealing participants' perception of singing as an effective therapy that was fun, improved mood, taught breathing and breath control, was a good exercise for the lungs, and had improved physical functioning. Conclusions: Singing can be used as an adjunctive treatment for respiratory disease, with the best results occurring after long-term study. Group lessons and a strong teacher relationship feed the need for social interaction and support, which can facilitate treatment compliance. Further research is warranted.

REGULAR PHYSICAL ACTIVITY POSITIVELY INFLUENCES THE CLINICAL STATUS OF CF PATIENTS

Article

Jan 2011

Helge Hebestreit

Airway Clearance Strategies in Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Article

Apr 2015
SEMIN RESP CRIT CARE

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. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Mucoviscidose : les techniques instrumentales de désencombrement des voies aériennes

Article

Feb 2012
REV MAL RESPIR

Introduction Chest physiotherapy is an essential part of the life-long therapeutic routine in patients with cystic fibrosis. State of the art Various manoeuvres are available but there is no consensus supporting one specific technique over others. These techniques can be classified as “manual” or “instrumental”. Instrumental airway clearance techniques are based on physiological principles and consist in the application of vibrations or positive expiratory pressure during expiratory phase to enhance sputum clearance. Positive expiratory pressure can be delivered continuously or in an oscillating pattern. The effects of these devices have been investigated in many studies. Perspectives Results suggest a potential place for these techniques in the management of cystic fibrosis, but they remain poorly used. Conclusion A better knowledge of these approaches could enable them to integrate more widely into the physiotherapy management of patients with cystic fibrosis.

[Cystic fibrosis: instrumental airway clearance techniques].

Article

Feb 2012

Chest physiotherapy is an essential part of the life-long therapeutic routine in patients with cystic fibrosis. Various manoeuvres are available but there is no consensus supporting one specific technique over others. These techniques can be classified as "manual" or "instrumental". Instrumental airway clearance techniques are based on physiological principles and consist in the application of vibrations or positive expiratory pressure during expiratory phase to enhance sputum clearance. Positive expiratory pressure can be delivered continuously or in an oscillating pattern. The effects of these devices have been investigated in many studies. Results suggest a potential place for these techniques in the management of cystic fibrosis, but they remain poorly used. A better knowledge of these approaches could enable them to integrate more widely into the physiotherapy management of patients with cystic fibrosis.

The role of exercise in maintaining health in cystic fibrosis

Article

Aug 2011
CURR OPIN PULM MED

Consensus statements about the care of people with cystic fibrosis (CF) recommend exercise as part of a wider management strategy. Many of these recommendations are based on high-quality evidence that regular exercise improves some important clinical outcomes, such as lung function and quality of life. However, the evidence about the effect of exercise on other clinical outcomes is less extensive or lower in quality. This article will review the physiological effects of exercise on a range of outcomes in people with CF, the mechanisms by which exercise may improve these outcomes and the quality and findings of clinical research into the effects of exercise in the management of CF. Substantial evidence confirms that exercise significantly reduces the rate of decline in lung function in people with CF, at least in part by increasing mucus clearance. Regular exercise training over 6 months improves aerobic exercise capacity. Bone health is often poor in people with CF, but only indirect evidence supports that increasing the amount of exercise will have a beneficial effect on bone density. CF-related diabetes is also a common sequela of the disease, but again only evidence from type-2 diabetes exists to support exercise as a way of managing it. Although its effects on some outcomes are unclear, the overall effect of exercise on quality of life is substantially beneficial and the evidence available for other specific outcomes is directly or indirectly supportive, so it appears appropriate to recommend it in clinical practice.

Effects of Gender and Age at Diagnosis on Disease Progression in Long-term Survivors of Cystic Fibrosis

Article

Full-text available

May 2010
AM J RESP CRIT CARE

Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. Little is known concerning disease progression and outcomes in these cohorts. Examine effects of age at diagnosis and gender on disease progression, setting of care, response to treatment, and mortality in long-term survivors of CF. Retrospective analysis of the Colorado CF Database (1992-2008), CF Foundation Registry (1992-2007), and Multiple Cause of Death Index (1992-2005). Patients with CF diagnosed in childhood and who survive to age 40 years have more severe CFTR genotypes and phenotypes compared with adult-diagnosed patients. However, past the age of 40 years the rate of FEV(1) decline and death from respiratory complications were not different between these cohorts. Compared with males, childhood-diagnosed females were less likely to reach age 40 years, experienced faster FEV(1) declines, and no survival advantage. Females comprised the majority of adult-diagnosed patients, and demonstrated equal FEV(1) decline and longer survival than males, despite a later age at diagnosis. Most adult-diagnosed patients were not followed at CF centers, and with increasing age a smaller percentage of CF deaths appeared in the Cystic Fibrosis Foundation Registry. However, newly diagnosed adults demonstrated sustained FEV(1) improvement in response to CF center care. For patients with CF older than 40 years, the adult diagnosis correlates with delayed but equally severe pulmonary disease. A gender-associated disadvantage remains for females diagnosed in childhood, but is not present for adult-diagnosed females.

The relationship between control beliefs and self-reported adherence with cystic fibrosis

Article

Full-text available

Nov 1999

This study investigated the relationship between locus of control and self-reported adherence in 31 adults with cystic fibrosis. Participants completed the Multidimensional Health Locus of Control Scale, Form C (MHLC-C; Wallston et al ., 1994) and an adherence questionnaire. The two 'powerful others' sub-scales of the MHLC-C, 'doctors' and 'others', were significantly correlated with overall self-reported adherence. Multiple regression indicated that the 'doctors' sub-scale explained 35% of the variance in overall adherence. It is concluded that an external locus of control for powerful others, especially doctors, may promote greater adherence.

Determinants of non-compliance with short-term antibiotic regimens

Article

Full-text available

Nov 1987

The contribution of doctor, patient, and consultation interaction patterns to compliance with antibiotic treatment was examined in 233 adult patients seen in general practice. Twelve variables were shown to discriminate between compliers and non-compliers. Discriminating variables relating to patients included health state, employment state, knowledge of tablet, and perception of anxiety level, difficulty in complying, and their observed anger, distance, and assertiveness in the consultation. Discriminating variables relating to doctors included provision of advice on duration of treatment, complexity of dosage schedule, age of doctor, and number of years in practice. For the most part these results confirmed previous research. It is concluded that the doctor should consider both the dosage schedule and the patient's daily routine when prescribing antibiotic tablets. Advice on how to take the tablets should be given in specific rather than in general terms. The significant effect of the age of the doctor and the years spent in practice has not been found in previous work. This finding may reflect differences in behavior between younger doctors and their patients. This difference was not detected in the observation of consultation events.

Treatment compliance in adults with cystic fibrosis

Article

Full-text available

Mar 1994

The study comprised three interrelated aims: (1) to ascertain (a) patient compliance with physiotherapy, exercise, enzyme and vitamin regimens, (b) how compliance was perceived by patients, and (c) the reasons for poor compliance (2) to identify demographic and clinical variables associated with compliance; and (3) to determine how accurately patient compliance can be predicted by carers. Demographic and medical history data were obtained from medical records and a patient questionnaire. The data obtained included age, sex, employment status, inpatient or outpatient status, frequency of contact with the clinic, age at diagnosis, and the number of years practising physiotherapy. Measures of clinical status, including FEV1 and FVC percentage predicted, Shwachman score, and 24 hour sputum weight were recorded before completion of the questionnaire. The questionnaire, administered by a psychologist, assessed the reported degree of patient compliance, their perception of compliance, and their reasons for poor compliance. Sixty patients participated in the study and 51/60 and 41/55 patients were considered compliant with enzyme and exercise therapies, respectively. Compliance was lower with physiotherapy (32/60) and vitamin treatment (21/45). Patients reporting immediate benefits following exercise and physiotherapy were more compliant than those reporting no improvement. The perception by patients that compliance was sufficient ("about right") was physiotherapy 67%, exercise 37%, enzymes 78%, and vitamins 9%. Compliance was not influenced by demographic details nor by severity of disease, although patients producing large amounts of sputum and receiving help with physiotherapy were more compliant with physiotherapy. The physiotherapist and physician judged correctly the degree of compliance with physiotherapy in 83% and 75% of cases, respectively, and with exercise in 68% and 67% of cases, respectively. The reported degree of compliance and reasons for poor compliance were treatment specific. Demographic and disease severity variables were not associated with compliance. Those involved in the care of patients with cystic fibrosis were able to predict patient compliance.

Adherence to Chest Physiotherapy in Adults with Cystic Fibrosis

Article

Full-text available

Dec 2006

Chest physiotherapy (CP) is seen as a cornerstone of Cystic Fibrosis (CF) treatment. However, previous studies have suggested that adherence to CP is low. This study of adults with CF (N = 563) investigated CP adherence and associated factors. Only 29.5 per cent reported undertaking daily CP. Predictors of adherence included problems with fitting CP into lifestyle, a perception that CP does not help, physical consequences of CP, doing exercises instead and doing CP as and when necessary. These variables accounted for 45 per cent of the variance in adherence. Content analysis revealed a number of themes related to adherence to CP. Future studies should explore the benefits of daily CP and attempt to devise simple interventions to maximize adherence to CP.

Cystic fibrosis mortality and survival in the UK: 1947-2003

Article

Full-text available

Mar 2007

Data up to 1995 on the survival of 3-yr cohorts of patients with cystic fibrosis (CF) born in the UK in the period 1968-1992 have previously been published. The present study reports survival data up to the end of 2003 together with a 2003 population estimate. All subjects with CF born in the UK in the period 1968-1992 were identified up to 1997 by active enquiry through recognised CF clinics and other hospital consultants. Information from the death certification authorities up to the end of 2003 was added. Death certificates that could not be matched with UK Cystic Fibrosis Survey records were investigated and the data reconciled. The observed survival up to 2003 of CF patients born in 1978 was 55% for males and 49% for females. For 1988 and 1992 the data were 91 and 88%, and 97 and 96%, respectively. The estimated 2003 mid-year CF population was 8,284. The continuing improvement in survival of cystic fibrosis patients in successive cohorts means that the previous prediction of median survival of >50 yrs of age for individuals born in 2000 continues to look realistic, even in the absence of proven effective therapy aimed at correcting the basic cystic fibrosis defect.

Methodological and Conceptual Issues in Adherence

Chapter

Mar 1998

Treatments for cystic fibrosis: The role of adherence, importance and burden

Chapter

Jan 2010

L.B. Myers

Introduction: Cystic fibrosis (CF) is the most frequent lethal genetic disease of childhood. Nowadays due to improvements in treatment most people with CF can lead fairly normal lives. To achieve this people with CF need daily treatment consisting of various medications, chest physical therapy and pancreatic enzymes as well as paying attention to dietary needs. There has been a gradual but significant improvement in the life expectancy of CF patients due to advances in the efficacy of treatment. So what was considered a disease of childhood has now also become a chronic condition of adulthood. The current study investigated factors associated with treatments in adults with CF. Method: Participants were 563 adults with CF recruited from the UK's Cystic Fibrosis Trust mailing list. Patients rated adherence to each treatment and reported any other treatments e.g. complementary therapies. Other measures included importance of each treatment, burden of each treatment and demographic information. Results: The mean number of current treatments was high (10.49). Adherence varied with different behaviors, with the highest adherence for enzymes, insulin and antibiotics and lowest for physical therapy, exercise, overnight feeding and dietary supplements. Statistical analyses were correlations and Analysis of Variance. Adherence was correlated with importance and burden for the majority of treatments, meaning high adherence was related to high importance and low burden. Treatments varied in importance, with insulin and antibiotics rated the highest and dietary treatments the lowest. For burden, treatments which are administered by injection or are time consuming were rated the highest e.g. insulin, nebulized medications, with easily administered treatments rated the lowest e.g. oral steroids, enzymes. For most treatments importance and burden were positively correlated, meaning that the more important the treatment the least troublesome it was rated. Nearly a quarter of participants reported using some form of complimentary therapy, with more females reporting such use. However, over 50% said that CF never or only occasionally interfered with their enjoyment of life. There were few gender differences, but some age differences. Conclusion: Results from this study provide an insight into treatments for adults with CF, indicating a complex picture of adherence and indicate that adherence, importance and burden are usually related.

Sawicki GS, Sellers DE, Robinson WM. High treatment burden in adults with cystic fibrosis: challenges to disease self-management. J Cyst Fibros.8(2):91-96

Article

Oct 2008

More aggressive management of cystic fibrosis (CF), along with the use of new therapies, has led to increasing survival. Thus, the recommended daily treatment regimens for most CF adults are complex and time consuming. In the Project on Adult Care in CF (PAC-CF), an ongoing longitudinal study of CF adults, we assessed self-reported daily treatment activities and perceived treatment burden as measured by the CF Questionnaire-Revised (CFQ-R), a disease-specific quality of life measure. Among the 204 respondents, the median number of daily therapies reported was 7 (IQR 5-9) and the mean reported time spent on treatment activities was 108 minutes per day (SD 58 min). Respondents reported a median of 3 inhaled and 3 oral therapies on the day prior to the survey. Only 49% reported performing airway clearance (ACT) on that day. There were no differences in the number of medications or the time to complete therapies based on gender, age or FEV1. The mean CFQ-R treatment burden domain score was 52.3 (SD 22.1), with no significant differences in the treatment burden based on age or FEV1. In a multivariable model controlling for age, gender, and FEV1, using 2 or more nebulized medications and performing ACT for >or=30 min were significantly associated with increased treatment burden. The level of daily treatment activity is high for CF adults regardless of age or disease severity. Increasing number of nebulized therapies and increased ACT time, but not gender, age, or pulmonary function, are associated with higher perceived treatment burden. Efforts to assess the effects of high treatment burden on outcomes such as quality of life are warranted.

Cystic fibrosis: Adult clinical aspects

Article

Mar 1996
Br J Hosp Med

Steven P Conway

The number of adults with cystic fibrosis is increasing by 120 per year. It is essential that doctors and paramedical staff are trained to appreciate the complex nature of this multisystem disease in adults, and that adequate resources are allocated to provide for proper standards of patient care.

Adherence of adult cystic fibrosis patients with airway clearance and exercise regimens

Article

Jun 2007
J CYST FIBROS

Regular airway clearance and exercise form an important part of the physiotherapy management of patients with cystic fibrosis (CF). Previous research has found that adherence of these patients with physiotherapy regimens is variable and influenced by factors such as sex and disease severity. To date, the adherence of Australian patients with CF has not been investigated. The aim of this study was to measure the adherence of a sample of Australian adult patients with CF and to ascertain factors that improved or decreased their adherence with physiotherapy. Patients attending an Australian CF Unit were surveyed by an independent physiotherapist using a questionnaire based on the Manchester Cystic Fibrosis Compliance Questionnaire. Fifty seven of the 84 patients registered with the Unit (67.9% response rate) completed the survey. Over the previous six months, 96.5% of patients reported doing some form of airway clearance, with 70.2% doing this daily or only occasionally missing one or two days. Regular exercise was performed by 91.2% of patients when well, with 77.8% also exercising regularly when unwell. The most common reasons for non-adherence with airway clearance regimens were being too busy and not being bothered. Being too busy and too tired were the most frequent reasons for decreased adherence with exercise. Frequency of performing airway clearance regimens significantly improved when patients felt unwell. Adherence with exercise regimens was significantly higher in those who worked or studied full time. Overall, the levels of adherence with physiotherapy regimens found in this study were considered to be satisfactory and higher than those previously reported in the literature, with time related factors being the most commonly reported reasons for decreasing adherence.

Treatments for Cystic Fibrosis: The role of adherence, importance and burden. In Cystic Fibrosis: Etiology, Diagnosis and Treatments

Lb Myers

Myers, LB. Treatments for Cystic Fibrosis: The role of adherence, importance and burden. In Cystic Fibrosis: Etiology, Diagnosis and Treatments. Int J Psychol Res in press.

An exploratory study investigating factors associated with adherence to chest physiotherapy and exercise in adults with cystic fibrosis

Abstract

No full-text available

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