BackgroundCystic 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.MethodA 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.ResultsTrends 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.ConclusionMTIs 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 idNCT04696198.
The European Respiratory Society (ERS) recognizes that there are an increasing number of national and international guidelines for the management of asthma, chronic obstructive pulmonary disease (COPD) and other chest diseases. Some of these guidelines recommend nebulizer use in specific circumstances, using either a jet nebulizer or an ultrasonic nebulizer to administer a drug to the airways or lungs in the form of an aerosolized mist of fine droplets. Although many patients with severe chest disease are given nebulized treatment both in hospitals and in their own homes, it is recognized that much of this practice may not be evidence-based. Some present practice may be ineffective or even harmful. The manufacturers of hand-held inhalers are obliged to meet exacting standards such as dose-to-dose reproducibility. However, nebulizer devices are sold separately from nebulized drugs and the dose delivered to the lung can be increased 10-fold or more by changing from an inefficient nebulizer system to a highly efficient one. For these reasons, the ERS commissioned a Task Force to review the scientific and clinical principles of nebulized therapy and to produce a set of guidelines (evidence-based whenever possible) for users of nebulized treatment in Europe.
It is hoped that the guidelines will improve clinical practice in the use of nebulized therapy throughout Europe. The most important considerations should be efficacy and patient safety. The guidelines will also serve as an educational and scientific resource for clinicians and scientists with an interest in inhaled therapy. These guidelines are aimed at a wide group of healthcare professionals practising in very different healthcare systems throughout Europe. The immediate target audience for the guidelines will be pulmonary physicians, but it is hoped that the messages will be communicated to all healthcare workers who are involved in treating patients with nebulized medication (doctors, nurses, pharmacists, paramedics, …
Abstract 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).
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.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.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.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.
Cystic Fibrosis (CF) is a severe hereditary disorder leading to progressive deterioration of lung function and death. This thesis describes the demography of CF in Sweden, its treatment, with special reference to physiotherapy, and its effects on lung function and peak working capacity (PWC). The study is based on Swedish CF patients and evaluates our current CF treatment package, which differs from those in use in other countries in that it includes preventive physical exercise from the day of diagnosis as an essential part of physiotherapy. Airway clearance therapy based upon physical exercise has been used since the early 1980s, when it replaced the previous airway clearance technique, which was postural drainage with percussion and vibrations.
Study I used inhaled radioactive aerosols to compare the effect on mucus mobilisation of three different airway clearance techniques, of which one was based upon physical exercise. There were no significant differences between the techniques. We found increased mucus mobilisation in the dependent lung during postural drainage in the lateral position. Ventilation has a central role in mucus mobilisation and we felt that it was justifiable to continue with physical exercise as the basis for airway clearance therapy.
Study II evaluated the demography of the Swedish CF population, its mortality rate and social situation. Survival rate for CF patients in Sweden is good, the median age of the population is high, the age at death is high, and the mortality rate is low. 75% of all patients who have finished school are in full or part time work, and 22% of all adults
have at least one child.
Studies III and IV investigate the physical condition of patients ≥7 years of age. They include summaries of lung function and PWC and their rates of change in different sub-groups over a 3 year period. Lung function and PWC were good in all age groups with no gender difference and with teenagers at least equally good as other age groups. The rate of decline in lung function and PWC was low. The proportion chronically colonised with Pseudomonas aeruginosa was low, but associated with worse lung function
and PWC, and a more rapid rate of decline.
These studies show that the Swedish treatment package, which includes preventive physical exercise, is good in an international perspective. It was not however possible to evaluate whether there is a mechanism that make PWC influence Pseudomonas colonisation. Further long term studies are desired.
Infections in cystic fibrosis (CF), often involving Pseudomonas aeruginosa, result from a dysregulated airway immunity where one hallmark is the accumulation of necrotic and apoptotic immune cells, in particular neutrophils. In addition, neutrophils actively release DNA, forming neutrophil extracellular traps (NETs) that contain antimicrobial proteins. Altogether, free DNA in complex with actin accumulates in the airway lumen, resulting in highly viscous sputum that provides an anionic matrix, binding cationic antimicrobial proteins. In this study, granulocyte chemotactic protein 2 (GCP-2)/CXCL6, a neutrophil-activating chemokine with bactericidal properties, was detected in the airway epithelium of CF patients and was also present in azurophilic and specific granules of neutrophils. Elastase of neutrophils, but not of P. aeruginosa, completely degraded CXCL6 (chemokine (C-X-C motif) ligand 6). In addition, CXCL6 colocalized with extracellular DNA in both CF sputa and in in vitro-formed NETs. In vitro, CXCL6 bound DNA with a KD of 2,500 nM. Interestingly, both the bactericidal and the receptor-activating properties of CXCL6 (against neutrophils) remained largely unaffected in the presence of DNA. However, the chemotactic properties of CXCL6 were reduced by the presence of DNA. Taken together, CXCL6 is expressed in CF, retaining its functional properties even after binding to the anionic scaffold that extracellular DNA provides in CF.
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