Repercussões Clínicas da Gameterapia no Manejo do Indivíduo com Fibrose Cística / Clinical repercussions of game therapy in the management of individuals with cystic fibrosis

Introdução: A gameterapia (GT) tem sido utilizada como método para estimular a prática de atividade física em indivíduos com fibrose cística (FC), podendo proporcionar melhora no sistema cardiorrespiratório e na adesão ao tratamento. Objetivo: analisar as repercussões clínicas da gameterapia no manejo de pacientes com FC. Métodos:Conduziu-se uma revisão sistemática de literatura nas seguintes bases de dados: PubMed, Embase, Scopus e MEDLINE via Ovid, utilizando-se os descritores:"Cystic fibrosis","video games" e seus cognatos, por meio da qual se incluíram estudos que descrevessem as repercussões do uso da GT em indivíduos com FC. Resultados: Foram identificados 293 estudos, sendo 7 selecionados ao final. Em 6 estudos, a frequência cardíaca alcançada durante a GT apresentou-se dentro dos padrões recomendados para treinamento. A avaliação do consumo máximo de oxigênio (VO2máx) foi realizada em 3 publicações, e este parâmetro apresentou níveis mais elevados em comparação ao identificado no teste de caminhada de seis minutos. Os consoles mais utilizados foram Nintendo Wii TM e Xbox 360 ™ e a intensidade na realização dos exercícios com a GT esteve entre moderada e intensa. A adesão dos indivíduos quanto a esse recurso também foi verificada, e a GT apresentou-se mais aceitável que outros e foi considerada lúdica. Conclusão: a GT se mostra uma intervenção capaz de gerar respostas fisiológicas que correspondem às necessidades de treinamento, além de maior adesão e satisfação dos indivíduos com FC em sua realização (AU),

Game therapy (GT) has been used as a method to encourage the practice of physical activity in individuals with cystic fibrosis (CF), which may provide improvement in the cardiorespiratory system and treatment adherence. Objective: analyze the clinical repercussions of game therapy in the management of patients with CF. Methods: A systematic review of the literature was carried out in the following databases: PubMed, Embase, Scopus and MEDLINE via Ovid, using the descriptors: "Cystic fibrosis", "video games" and their cognates, through which they included Studies describing the repercussions of the use of GT in individuals with CF were sought. Results: 293 studies were identified, seven of which were selected at the end. In six studies, the heart rate reached during the TG was within the recommended standards for training. The assessment of maximum oxygen consumption (VO2max) was performed in three publications, and this parameter showed higher levels compared to that identified in the six-minute walk test. The most used consoles were Nintendo WiiTM and Xbox 360™and the intensity in carrying out the exercises with the TG was moderate and intense. The individuals' adherence to this resource was also verified, and the GT was more acceptable than others and was considered playful. Conclusion: TG proves to be an intervention capable of generating physiological responses that correspond to training needs and greater adherence and satisfaction of individuals with CF in its performance (AU).

Indicaciones de rehabilitación respiratoria en niños y adolescentes con enfermedades respiratorias crónicas / Indications for respiratory rehabilitation in children and adolescents with chronic respiratory diseases

Resumen El aumento de la expectativa de vida de niños y adolescentes con enfermedades respiratorias crónicas ha llevado a implementar estrategias como la rehabilitación respiratoria (RR). El presente artículo profundiza en las distintas indicaciones de la RR en distintos niveles de atención médica, distinto grado de dependencias tecnológicas y diversas patologías como: Fibrosis Quística, Bronquiolitis Obliterante, Enfermedades Neuromusculares, anomalías de caja torácica y escoliosis.

The increase in the life expectancy of children and adolescents with chronic respiratory diseases has led to implement strategies such as respiratory rehabilitation (RR). This article delves into the different indications of RR at different levels of medical care, different level of technological dependencies and different pathologies such as: Cystic Fibrosis, Bronchiolitis Obliterans, Neuromuscular Diseases, Rib cage abnormalities and Scoliosis.

Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF).

Rationale: The long-term effects of vigorous physical activity (PA) on lung function in cystic fibrosis are unclear. Objectives: To evaluate effects of a 12-month partially supervised PA intervention using motivational feedback. Methods: In a parallel-arm multicenter randomized controlled trial (ACTIVATE-CF), relatively inactive patients aged at least 12 years were randomly assigned (1:1 ratio) to an intervention group or control group. The intervention group consented to add 3 hours of vigorous PA per week, whereas the control group was asked not to change their PA behavior. Primary endpoint was change in percent predicted FEV1 (ΔFEV1) at 6 months. Secondary endpoints included PA, exercise capacity, exercise motives, time to first exacerbation and exacerbation rates, quality of life, anxiety, depression, stress, and blood glucose control. Data were analyzed using mixed linear models. Measurements and Main Results: A total of 117 patients (40% of target sample size) were randomized to an intervention (n = 60) or control group (n = 57). After 6 months, ΔFEV1 was significantly higher in the control group compared with the intervention group (2.70% predicted [95% confidence interval, 0.13-5.26]; P = 0.04). The intervention group reported increased vigorous PA compared with the control group at each study visit, had higher exercise capacity at 6 and 12 months, and higher PA at 12 months. No effects were seen in other secondary outcomes. Conclusions: ACTIVATE-CF increased vigorous PA and exercise capacity, with effects carried over for the subsequent 6 months, but resulted in better FEV1 in the control group.

Development and validation of the Cystic Fibrosis Decisional Balance for Physical Activity scale (CF-DB-PA).

BACKGROUND: People with cystic fibrosis (pwCF) derive several physiological and psychological benefits from regular physical activity (PA), but the practice is lower than recommended. Knowledge about the facilitators of and barriers to PA at the individual level is important to act positively on PA behaviors. This study validated the Cystic Fibrosis Decisional Balance for Physical Activity scale (CF-DB-PA) for adults with CF. METHODS: French adults with CF were recruited in several specialist centres in France. The CF-DB-PA scale was validated following a quantitative study protocol comprising four stages: (1) tests of the clarity and relevance of a preliminary 44-item version and reduction analysis, (2) confirmatory factor analysis and tests of dimensionality through equation modelling analysis, (3) tests of reliability with Cronbach alphas for the internal consistency and a test-retest with a 2-to-3 week interval for temporal stability, and 4) tests of construct validity with Spearman correlations to measure the associations between each subscale and the theoretically related constructs (i.e., quality of life, PA and exercise tolerance). RESULTS: A total of 201 French adults with CF participated in the validation study. The CF-DB-PA comprises 23 items divided into two factors: facilitators of and barriers to PA. Each factor is divided into three subscales: physical, psychological and environmental. The factors (facilitators and barriers) can be used independently or combined as a whole. A general score of decisional balance for PA can also be calculated. The bi-factor model presented satisfactory adjustment indexes: χ2 (194) = 362.33; p < .001; TLI = .87; CFI = .90; RMSEA = .067. The scale showed satisfactory internal consistency (Cronbach's α = .77). The test-retest reliability was not significant for either subscale, indicating stability over time. The facilitators subscale correlated significantly with the self-reported score of PA (r = .33, p < .01) and quality of life (r = .24, p < .05). The barriers subscale correlated significantly with the self-reported scores of PA (r = - .42, p > .01), quality of life (r = - .44, p < .01), exercise tolerance (r = - .34, p < .01) and spirometry tests (r = - .30, p < .05). CONCLUSIONS: The CF-DB-PA is a reliable and valid questionnaire assessing the decisional balance for PA, the facilitators of and the barriers to PA for adults with CF in French-speaking samples.

Interval versus constant-load exercise training in adults with Cystic Fibrosis.

BACKGROUND: The efficacy of interval exercise (IE) compared to constant-load exercise (CLE) training remains unsettled in adults with Cystic Fibrosis (CF). METHODS: Twenty-four adults with CF were randomised to 30-min IE (100 % peak work capacity (WRpeak) for 30-s alternated with 40 % WRpeak for 30-s; n = 12) or 30-min CLE (70 % WRpeak; n = 12) training, 3 times weekly, for 12 weeks. Isometric quadriceps muscle strength was assessed using a strain gauge Myometer. RESULTS: The magnitude of improvement in quadriceps muscle strength was greater (p = 0.037) in the IE (by 32 ±â€¯13 Nm) compared to the CLE (by 23 ±â€¯12 Nm) groups. Maximum inspiratory and expiratory mouth pressures were significantly improved only in the IE group (by 30 ±â€¯10 cmH2O; p = 0.009 and 13 ±â€¯4 cmH2O; p = 0.007, respectively). Arterial oxygen saturation during training was higher (p = 0.002) for IE (94 ±â€¯1%) compared to CLE (91 ±â€¯1%), whereas dyspnoea scores were lower (p = 0.001) for IE (3.8 ±â€¯0.7) compared to CLE (5.9 ±â€¯0.8) CONCLUSIONS: IE is superior to CLE in improving peripheral and respiratory muscle strength and preferable to CLE because it is associated with lower exercise-induced arterial oxygen desaturation and breathlessness.

Effects of rehabilitation methods on lower-limb muscle function and functional performance in patients with cystic fibrosis: A systematic review.

OBJECTIVE: To investigate the effects of rehabilitation methods on leg muscle function and functional performance in cystic fibrosis. DATA SOURCES: A literature search was conducted in PubMed (MEDLINE), Scopus and the Cochrane Library from inception to October 12, 2020. A secondary hand search through reference lists from identified articles was conducted. REVIEW METHODS: Three authors independently checked the full-text copies for eligibility of relevant articles. Randomized controlled trials were included. Methodological quality was assessed using the Physiotherapy Evidence Database scale. The PRISMA guidelines were followed. Results suggestive of leg muscle function (e.g. strength, power, endurance, and fatigue) and functional performance were reported. RESULTS: The search identified 8 studies (233 patients). The overall quality of these articles was good. Rehabilitation modalities investigated were physical exercises (aerobic, anaerobic and resistance training) (n = 7) and neuromuscular electrical stimulation (n = 1). Most studies (n = 4) reported on the effects of combined aerobic and resistance training and showed improvement on leg strength when exercises were supervised. Aerobic training alone or the use of neuromuscular electrical stimulation also enhanced leg strength, whereas anaerobic training did not. Two studies evaluated leg fatigue and found no improvement compared to standard care. One study assessed the effect of exercises on functional performance and found no improvement when compared to standard care. No studies emphasized the impact of rehabilitation methods on leg muscle endurance or power. CONCLUSION: Combined aerobic and resistance training enhances leg muscle strength in cystic fibrosis. There is insufficient data on other leg muscle outcomes, nor on alternative rehabilitation strategies.

Effects of a long-term exercise program on motor performance in children and adolescents with CF.

OBJECTIVES: The aim of this study was to examine motor performance and trainability in youths with cystic fibrosis (CF). METHODS: Twenty-two children and adolescents (11 f/11 m), age range 6-17 years (11.3 ± 3.3 years), mean FEV1 91.0 ± 21.7% pred.finished the partially monitored 12-months exercise program. Patients performed the Deutsche Motorik Test (DMT) to assess flexibility, balance, strength, power and totalmotor performance. An incremental ergometer cycle test was used to assess maximal exercise capacity (Wpeak). All tests were performed before (T1), after 6 months of monitored exercise training (T3) and another 6 months without monitoring (T4). RESULTS: Motor Competence in total and test-items of the DMT (except foreward bend) improved to T3 (p < .05). No further improvement could be observed after the end of the monitoring (T3). However, the values remained stable at the improved level (T4). Girls scored lower in test items depending on strength/power but scored higher in balancing compared to boys (p > .05). Wpeak and FEV1 were not influenced by the training program. From T3 to T4 a slight decrease was observed (p ≤ .05). CONCLUSIONS: The findings demonstrate benefits of an individualizedmonitored long-term exercise intervention on motor performance in CF with improvements of test-tasks to predicted normal. Monitoringseems to be a facilitator in maintaining motivation toward physical activity as no further increase in motor performance was observed after stopping supervision. The results suggest that an individually tailored monitoredregular exercise program should include all aspects of physical fitness with a variety of movement experiences.

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

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 years). Sociodemographic characteristics, lung function testing, Coping with Stress Self-Efficacy, brief Coping Orientation to Problems Experienced inventory, and the scale of Adherence to Treatment of Physiotherapy (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 = .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, and 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 Coping Orientation to Problems Experienced inventory (r = .360) and Coping with Stress Self-Efficacy 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). CONCLUSIONS: The AdT-Physio scale is psychometrically valid and reliable for use in the clinic for the assessment of adherence to physical therapy 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.

Nutritional rehabilitation for children with cystic fibrosis: Single center study.

BACKGROUND: Cystic fibrosis (CF) is an inherited disorder that causes severe damage to multiple organs in the body. Nutritional management has a dramatic effect on growth and survival in CF patients. This study aimed to assess the nutritional status of Egyptian pediatric patients with CF before and after proper nutritional counseling and supplementation. METHODS: This is an interventional observational study conducted on fifty children with CF recruited from CF clinic, Children's Hospital, Cairo University. The patients were subjected to history taking, clinical examination, full nutritional assessment, including signs of vitamins deficiencies, Subjective global assessment (SGA), anthropometric measurements, laboratory assessment (complete blood count, kidney, liver function tests and Prealbumin as a nutritional marker). After the initial assessment all the patients were subjected to a proper nutritional plan plus vitamin and mineral supplementations then reassessed them after 3 months. RESULTS: SGA showed that 60% of the patients had severe malnutrition, 62%, 78%, and 48% of the patients were below -2 Z score for height, weight, and BMI respectively which decreased to 45%, 40%, and 32% after nutritional supplementation. 43% of the patients were anemic, 29% were hypoalbuminemic, and 80% had low serum Prealbumin levels. After 3 months of follow up, only 17% had anemia, 11% had hypoalbuminemia, and 41% had low serum Prealbumin. Compliance was detected in 76% of caregivers during the follow up. CONCLUSION: CF patients had considerable impairment in their nutritional status. Proper caloric intake and vitamin supplementation had a valuable impact on their growth and prognosis.

The feasibility of online video calling to engage patients with cystic fibrosis in exercise training.

INTRODUCTION: Physical activity, including structured exercise, is an essential component in the management of cystic fibrosis. The use of telehealth such as video-calling may be a useful method for the delivery of exercise and physical activity interventions, though the feasibility of this remains unknown. METHODS: Nine patients with cystic fibrosis (three female, six male, 30.9 ± 8.7 years) volunteered to participate. Participants completed an eight-week exercise training intervention conducted via Skype, using personalised exercises, with all sessions supervised by an exercise therapist. Feasibility was assessed by demand, implementation, practicality and acceptability. Changes in anthropometric, pulmonary, physical activity and quality of life variables were also assessed. RESULTS: Two male participants withdrew from the study, citing lack of available time. The remaining participants found use of Skype useful, with a mean satisfaction rating of 9/10, and three participants requesting to continue the sessions beyond the duration of the study. Mean compliance with sessions was 68%, with mean duration of sessions being 20 min. A total of 25% of calls suffered from technical issues such as video or audio lags. Anthropometric, pulmonary, physical activity and quality of life variables remained unchanged over the course of the study period. DISCUSSION: The use of Skype to deliver an exercise intervention to patients withcystic fibrosis was found to be technologically feasible, and acceptable among participants. Findings have implications for clinical practice and could allow care teams to engage patients remotely in exercise. Further research is required to assess the efficacy of this modality on increasing physical activity and associated health outcomes.

Consenso chileno para la atención integral de niños y adultos con fibrosis quística / Chilean consensus for the integral care of children and adults with cystic fibrosis

Cystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.

La Fibrosis Quística (FQ) es la enfermedad hereditaria de pronóstico reservado más frecuente en raza blanca. Desde el año 2003, Chile inicia un Programa Nacional de Fibrosis Quística, de carácter integral, dirigido por la Unidad de Salud Respiratoria del Ministerio de Salud. Hasta la fecha, los principales resultados del Programa registran una significativa mayor sobrevida (promedio 27 años) y una significativa reducción en la edad de diagnóstico de los pacientes ingresados desde 2006 en adelante. El acceso a la canasta GES (Garantías Explícitas en Salud), la implementación del tamizaje neonatal en algunas regiones del país, la organización y la constitución de equipos entrenados en FQ de diversas especialidades, ha contribuido a mejorar los resultados. Si bien las principales manifestaciones son del aparato respiratorio y digestivo, el carácter multisistémico de la FQ obliga a conocer los distintos aspectos involucrados en su manejo, a fin de optimizar los resultados del tratamiento y los recursos invertidos, tanto en el sector público como privado. Este documento es una revisión y actualización sobre los principales aspectos del diagnóstico, seguimiento y tratamiento de las manifestaciones respiratorias y no respiratorias de la FQ.

Effects of aerobic interval training on glucose tolerance in children and adolescents with cystic fibrosis: a randomized trial protocol.

BACKGROUND: Individuals with cystic fibrosis (CF) may develop CF-related diabetes (CFDR). This comorbidity is related to a poorer quality of life, microvascular complications, a decline in lung function, and an increase in exacerbations, as well as delayed growth and puberty. Evidence exists that physical exercise contributes to glycemic control in individuals with non-CF-related diabetes. This exercise is usually continuous with moderate intensity and long duration, which can cause muscle dyspnea and fatigue in CF individuals. Aerobic interval training (AIT) emerges as a safe and effective alternative for treating these individuals. The objective of this study is to evaluate the effects of AIT on glucose tolerance in children and adolescents with CF. METHODS: This study will be a two-arm, prospectively registered, randomized controlled trial with blind assessors and twenty 6- to 18-year-old individuals with cystic fibrosis (CF) from two different Brazilian states. People with CF will be randomly allocated to either the experimental or control group using block randomization, stratified by puberty stage,. Participants from both groups will receive an educational intervention and will be asked to continue their usual daily treatment for the full duration of the study. Those in the experimental group will perform AIT on a cycle ergometer at home three times a week, for 8 consecutive weeks. The sample characterization will include an assessment of puberty stage, socioeconomic status, dyspnea, and anthropometry. The primary outcome will be the change in glucose tolerance, while the secondary outcomes will include lung function, exercise tolerance, respiratory muscle strength, quality of life, and CF exacerbations. All outcomes will be assessed at baseline, week 9, and week 17. DISCUSSION: This is the first study to evaluate the effects of AIT on glucose tolerance in children and adolescents with CF. This study will serve as a basis for guiding clinical practice and decision-making in treating glucose intolerance and CF-related diabetes (CFRD) in children and adolescents with CF. TRIAL REGISTRATION: ClinicalTrials.gov Protocol Registration System: NCT03653949. Registered on August 31, 2018.

Nutrition in cystic fibrosis: From the past to the present and into the future.

Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition care.

Using photo-elicitation to explore perceptions of physical activity among young people with cystic fibrosis.

BACKGROUND: Physical activity is recommended in the management of cystic fibrosis (CF). The aim of this study was to explore motives, barriers and enablers to physical activity among this population. METHODS: Twelve participants (12-18 years) were recruited via convenience sampling. Photo-elicitation alongside semi-structured interviews were used to explore participants' views and experiences of physical activity. RESULTS: Our findings revealed motives for physical activity including health, enjoyment and autonomy. Those with families who valued physical activity tended to have positive attitudes towards physical activity, and valued and integrated it into their lives. Moreover, they were likely to be intrinsically motivated to be active. Several factors enable and act as barriers to physical activity. Whilst CF influenced physical activity, the majority of enablers and barriers raised where congruent with the general populations. CONCLUSION: This study provides support that healthcare providers should encourage both young people with CF and their families to be active, and subsequently informs the development of clinical interventions to support physical activity among young people with CF and their families.

Employment after lung transplantation in Poland - a single center study.

OBJECTIVES: Lung transplantation not only saves a patient's life but also creates the opportunity for becoming more self-reliant and getting back to work. The aim of this single center study was to assess the prospects of employment, as well as its influence on the quality of life and physical activity, of the lung transplant recipients of the Silesian Center for Heart Diseases in Zabrze, Poland. MATERIAL AND METHODS: A retrospective study covered 67 lung transplant recipients of the Silesian Center of Heart Diseases. Only patients with ≥ 6-month follow-up were included. All of the patients gave their written consent to be included in the study before filling out the questionnaire containing questions about employment, income, education and how work affected their quality of life before and after lung transplantation. A physical capability assessment was performed by climbing flights of stairs and by means of a 6-min walk test, and spirometry parameters were also measured. RESULTS: Twenty of the patients included in the study (31.7%) were employed after lung transplantation, 63.2% of whom worked full-time. Profession was changed by 2 patients (14.3%). The patients diagnosed with cystic fibrosis were found to have the highest chance of finding employment after lung transplantation. The statistical analysis revealed that the employed patients were able to cover longer distances during the 6-min walk test (556 m, on average) than the unemployed ones (494 m, on average). CONCLUSIONS: One in 3 patients finds employment after lung transplantation. Work improves the quality of life of the majority of lung transplant recipients. The patients who are employed are also in a better physical condition, and they are more self-reliant in comparison to those who remain unemployed. Lung transplant recipients with cystic fibrosis are most likely to find employment, and so are patients with higher education. Int J Occup Med Environ Health. 2019;32(3):379-86.

Effects of inspiratory muscle training on postural stability, pulmonary function and functional capacity in children with cystic fibrosis: A randomised controlled trial.

BACKGROUND: Previous research has found conflictive results regarding the benefits of inspiratory muscle training (IMT) for cystic fibrosis (CF) patients. Also, involvement of postural stability is a rising concern in chronic lung diseases but its role in CF patients is poorly understood. Our aim was to investigate the effects of IMT in CF patients as well as analysing the factors which may be related to postural stability. METHODS: Thirty-six children aged between 8 and 18 years with CF were randomly allocated to either "comprehensive chest PT" group (PT) or "IMT alongside comprehensive chest PT" group (PT+IMT). Both groups trained for 8 weeks. Dynamic and static postural stability tests on Biodex Balance system®, spirometry, respiratory muscle strength and 6-min walk distance (6MWD) was assessed at baseline and after 8 weeks of training. Determinants of postural stability was also analysed on baseline values. RESULTS: Maximum expiratory pressure (MEP) was found to be an independent predictor for overall limits of stability (LOS) score explaining %26 of variance (R = 0.514, p = 0.003). Overall LOS score, FVC, FEV1, peak expiratory flow, MEP and 6MWD significantly improved in both groups, with no significant differences between groups. Maximum inspiratory pressure (MIP) also improved in both groups but the magnitude of improvement in MIP was greater in PT+IMT group (38 cmH2O vs 13 cmH2O; p < 0.001). CONCLUSIONS: Combining IMT with chest PT failed to provide further improvements, except for MIP, suggesting that a comprehensive chest PT program may be individually effective in improving overall LOS score, spirometry, respiratory muscle strength and 6MWD. TRIAL REGISTRATION: www.ClinicalTrials.gov; registration number: NCT03375684.

Cardiopulmonary responses to maximal aerobic exercise in patients with cystic fibrosis.

Cystic fibrosis (CF) is a debilitating chronic condition, which requires complex and expensive disease management. Exercise has now been recognised as a critical factor in improving health and quality of life in patients with CF. Hence, cardiopulmonary exercise testing (CPET) is used to determine aerobic fitness of young patients as part of the clinical management of CF. However, at present there is a lack of conclusive evidence for one limiting system of aerobic fitness for CF patients at individual patient level. Here, we perform detailed data analysis that allows us to identify important systems-level factors that affect aerobic fitness. We use patients' data and principal component analysis to confirm the dependence of CPET performance on variables associated with ventilation and metabolic rates of oxygen consumption. We find that the time at which participants cross the gas exchange threshold (GET) is well correlated with their overall performance. Furthermore, we propose a predictive modelling framework that captures the relationship between ventilatory dynamics, lung capacity and function and performance in CPET within a group of children and adolescents with CF. Specifically, we show that using Gaussian processes (GP) we can predict GET at the individual patient level with reasonable accuracy given the small sample size of the available group of patients. We conclude by presenting an example and future perspectives for improving and extending the proposed framework. The modelling and analysis have the potential to pave the way to designing personalised exercise programmes that are tailored to specific individual needs relative to patient's treatment therapies.

CyFiT telehealth: protocol for a randomised controlled trial of an online outpatient physiotherapy service for children with cystic fibrosis.

BACKGROUND: Telehealth and telemonitoring is an emerging area of study in people with cystic fibrosis (CF), with the potential of increasing access to care, and minimising infection control risks to patients without compromising their health outcomes. To date, limited evidence is available to support the use of telehealth in paediatric population with CF in a clinical setting. This study aims to investigate the utility of a multimodal telehealth-based outpatient physiotherapy service and assess its effect on quality of life, functional exercise capacity, hospital admission and intravenous antibiotic requirements, lung function, processes of care, participation in activities of daily living, and health economics associated with operating an innovative service. METHOD: This single centre, prospective, parallel, randomised, controlled, non-inferiority trial aims to recruit 110 children with CF between the ages 8 to 18 years of age. Participants will be randomised to the Usual Outpatient Physiotherapy Service group (Usual OPS) or the telehealth intervention group (CyFiT OPS). Quality of life, participation in activity of daily living, functional exercise capacity and patient perception of care will be examined every six months using the Cystic Fibrosis Questionnaire-Revised (CFQ-R), Children's Assessment of Participation and Enjoyment (CAPE), Preferences for Activities of Children (PAC) questionnaire, Modified Shuttle Test-25 (MST25), and Measure of Process of Care (MPOC-20) questionnaire. Physiological measurements collected during routine clinical visits such as spirometry, body weight and height, information will be retrospectively retrieved via a chart review at the end of the study. DISCUSSION: We anticipate that this multi-modal telehealth service will deliver a comparable service to traditional face-to-face models. An alternative to existing outpatient physiotherapy services may potentially increase patient options for access to care and patient-orientated outcomes such as quality of life. If deemed appropriate, the new model of care can be integrated into clinical practice immediately. TRIAL REGISTRATION: This trial is registered with the Australian and New Zealand Clinical Trial Registry ( ACTRN12617001035314 ) last updated 17th July 2018.