Effectiveness of manual therapy interventions in cystic fibrosis care: a pilot study.

BACKGROUND: Cystic fibrosis (CF) is a severe genetic condition that affects multiple organ systems and imposes a substantial treatment burden. Regarding the lungs and airways, the progressive pathophysiological changes place a significant strain on the musculoskeletal components of the respiratory system for people with CF. This pilot study investigated the effectiveness of manual therapy interventions (MTIs) on thoracic mobility, respiratory muscle strength, lung function, and musculoskeletal pain. METHOD: A study with a pretest-posttest design was conducted with 15 eligible people with CF at the Sahlgrenska University Hospital CF Centre. After an initial set of diagnostic tests at baseline, the participants underwent eight weekly 30-min MTIs. The MTIs included passive joint mobilisation and soft tissue manipulation of primary and secondary anatomical areas of the musculoskeletal respiratory system. On the day of the final intervention, the baseline measurements were repeated. RESULTS: Trends of increased thoracic mobility were observed following the intervention, with a statistically significant increase in respiratory muscle strength. No change in lung function was observed. Musculoskeletal pain before and after the intervention showed a significant decrease in tender points, and all participants reported positive experiences with MTIs. CONCLUSION: MTIs may improve thoracic mobility, alleviate pain, and enhance respiratory muscle strength in people with CF. Further research is needed to confirm their potential role as a CF physiotherapy supplement. CLINICAL TRIAL ID: NCT04696198.

Musculoskeletal aspects of respiratory function in cystic fibrosis: a cross-sectional comparative study.

Background: Respiration is an intricate interaction between visceral and musculoskeletal structures. In cystic fibrosis (CF), the airways and lungs are subject to progressive obstruction and destruction. However, knowledge about the musculoskeletal aspects of respiratory function and symptoms is still limited in this patient group. Methods: In a cross-sectional comparative study, 21 adults with CF enrolled at the Gothenburg CF Centre were matched with 42 healthy controls. The two groups were examined and compared in terms of thoracic mobility, respiratory muscle strength, lung function, and musculoskeletal pain in accordance with a predefined protocol. Results: Significant differences were observed between the groups in the number of tender points, thoracic excursion, forced vital capacity (FVC), and forced expiratory volume (FEV). The CF group also demonstrated a tendency toward reduced function in other measurements, although these were not statistically significant. Conclusion: This cross-sectional study revealed that people with CF have reduced thoracic mobility and an increased prevalence of muscular tender points, alongside decreased lung function, compared to healthy controls. These findings stress the need for greater emphasis on the often-overlooked musculoskeletal aspects of CF care, especially as people with CF are living longer and may require more musculoskeletal health support.

Variations in respiratory and functional symptoms at four months after hospitalisation due to COVID-19: a cross-sectional study.

BACKGROUND: Much remains unknown about complex respiratory symptoms after COVID-19. Here we aimed to describe and analyse patients' various respiratory symptoms 4 months after discharge from hospitalisation for COVID-19, focusing on sex, previous pulmonary disease, and prolonged mechanical ventilation. METHODS: This cross-sectional study involved five hospitals and included 52 patients with self-assessed respiratory dysfunction at 4 months after discharge from hospitalisation for severe COVID-19. Their average age was 63 years, 38% were women, 15 had a previous diagnosed pulmonary disease, and 29 were current or previous smokers. Additionally, 31 had required intensive care-among whom 21 were intubated and 11 needed mechanical ventilation for ≥20 days. Respiratory function was tested concerning lung volumes, expiratory flow, muscle strength, physical capacity (including concurrent oxygen saturation), thoracic expansion, and respiratory movements. RESULTS: Among 52 patients, 47 (90%) had one or several objectively measured respiratory function abnormalities. Decreased thoracic expansion was observed in 32 patients (62%), abnormal respiratory movements in 30 (58%), decreased vital capacity in 21 (40%), low physical function in 13 (26%), and desaturation during the test in 9 (17%). Respiratory inspiratory muscle strength was more commonly diminished than expiratory strength (27% vs. 8%). We did not observe differences between men and women, or between patients with versus without diagnosed pulmonary disease, except that those with pulmonary disease had significantly lower physical capacity assessed with 6MWD (70% vs. 88% predicted, p = 0.013). Compared to those who did not, patients who required ≥20 days of mechanical ventilation performed similarly on most tests, except that all thoracic breathing movements were significantly smaller (p < 0.05). The numbers and combinations of abnormal findings varied widely, without clear patterns. CONCLUSION: Patients with remaining respiratory symptoms 4 months after discharge from hospitalization due to COVID-19 may suffer from various abnormal breathing functions, and dysfunctional breathing that is not detected using traditional measurements. These patients may benefit from multidimensional measuring of breathing movement, thoracic expansion, and respiratory muscle strength, along with traditional measurements, to assess their symptoms and enable prescription of optimal treatment interventions and rehabilitation. TRIAL REGISTRATION: FoU i Sverige (Research & Development in Sweden, Registration number: 274476, registered 2020-05-28).

Thoracic configuration in patients ageing with cystic fibrosis: A retrospective single-blinded cohort study.

BACKGROUND: In patients with cystic fibrosis (CF), thoracic morphology and its role in respiratory function is conditioned by anthropometric factors, as well as by pathological changes. While the lungs are continuously monitored, examinations of potential thoracic cage adaptations to the disease are rare. Hence, the aim of this study was to investigate thoracic configuration, and its correlation to spirometry measures over time. METHODS: In total, 344 high-resolution computed tomography (HRCT) examinations from 90 patients were assessed and analysed. Those results were subsequently related to spirometry measurements performed within the same period. RESULTS: The cohort displayed no homogenous change in thoracic configuration over time, and correlation between thoracic area and spirometry variables could not be supported statistically. CONCLUSIONS: Although the current study included a larger cohort of patients with CF compared to previous studies on thoracic morphology, no patient group-specific changes in thoracic configuration were revealed. Furthermore, no correlations between structural findings and functional respiratory measurements were found.

Technical Aspects of Devices and Equipment for Positive Expiratory Pressure With and Without Oscillation.

BACKGROUND: Breathing exercises with positive expiratory pressure (PEP) and oscillating PEP are common treatments for patients with respiratory impairments. There are several trials evaluating the clinical effects of a variety of commercially available and self-made devices. There is a lack of evaluation concerning technical aspects and construction of the devices. The aims of this review were to describe and compare technical aspects of devices and equipment used for PEP and oscillating PEP as a basis for clinical decisions regarding prescriptions. METHODS: In this systematic review, we included trials evaluating different technical aspects of devices and equipment for PEP and oscillating PEP until June 2019. The literature search was performed in PubMed, CINAHL, Cochrane Library, Embase and PEDro. RESULTS: The literature search resulted in 812 studies, which, after being read by 2 independent reviewers, were reduced to 21 trials that matched the inclusion criteria. The achieved PEP is dependent on the given resistance or achieved expiratory flow through the devices and their separate parts. Oscillation frequency in oscillating PEP devices affects the pressure and oscillation amplitude and flow. For some devices, the device's position also has an impact on the outcome. There are similarities and differences among all of the devices, and the equipment components are not interchangeable without changing the achieved PEP levels. CONCLUSIONS: Many devices are available to provide PEP and oscillating PEP treatment. These devices differ substantially in design as well as in performance. When using PEP devices, it is important to understand how all parts of the devices affect outcomes. An increased understanding of how PEP is produced for the spontaneously breathing patient is important to achieve desired treatment effects.

Positive expiratory pressure - Common clinical applications and physiological effects.

Breathing out against resistance, in order to achieve positive expiratory pressure (PEP), is applied by many patient groups. Pursed lips breathing and a variety of devices can be used to create the resistance giving the increased expiratory pressure. Effects on pulmonary outcomes have been discussed in several publications, but the expected underlying physiology of the effect is seldom discussed. The aim of this article is to describe the purpose, performance, clinical application and underlying physiology of PEP when it is used to increase lung volumes, decrease hyperinflation or improve airway clearance. In clinical practice, the instruction how to use an expiratory resistance is of major importance since it varies. Different breathing patterns during PEP increase or reduce expiratory flow, result in movement of EPP centrally or peripherally and can increase or decrease lung volume. It is therefore necessary to give the right instructions to obtain the desired effects. As the different PEP techniques are being used by diverse patient groups it is not possible to give standard instructions. Based on the information given in this article the instructions have to be adjusted to give the optimal effect. There is no consensus regarding optimal treatment frequency and number of cycles included in each treatment session and must also be individualized. In future research, more precise descriptions are needed about physiological aims and specific instructions of how the treatments have been performed to assure as good treatment quality as possible and to be able to evaluate and compare treatment effects.

European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre.

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries.

Lung function in the aging Swedish cystic fibrosis population.

Swedish Cystic Fibrosis (CF) care follows international guidelines in general. The only difference in our CF care package since 25 years is the physiotherapy regimen. Airway clearance therapy has since the early 1980s, from the day of diagnosis, been based upon daily physical exercise in conjunction with techniques for transporting and evacuating mucus. Postural Drainage+/-percussion and vibration has not been used in any age. The aim of this study was to evaluate our CF care package. Lung function data from the start of the study and with a 3-year interval were collected in the entire Swedish CF population > or =7 years old. Data were analysed for the age groups 7-17 and > or =18 years of age. Change of lung function over the study period was calculated. The impact of chronic Pseudomonas aeruginosa (Pa) colonisation and basal FEV(1)%p was also evaluated in a linear mixed model. Data from 99% of the country-wide CF population were available at inclusion. Mean FEV(1)%p was 90+/-21 vs 73+/-26 in the different age groups and mean VC%p was 94+/-18 vs 91+/-20. Forty percent of the adult group was > or =30 years old. Overall, 41% were chronically Pa colonised. Mean annual FEV(1)%p rate of decline was 0.77 and 0.64 in the different age groups. Lung function among Swedish CF patients is good and annual rate of decline low, even in an old cohort. The large proportion of adult patients emphasises future demands on CF care.