Therapy of cystic fibrosis
ExpiredCystic fibrosis is a genetic disorder that affects the respiratory, digestive, and reproductive systems. There is no cure for cystic fibrosis, but various treatments are available to manage its symptoms and complications. Here are some common therapies used to manage cystic fibrosis:
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• Airway clearance techniques: People with cystic fibrosis often have thick, sticky mucus that can clog their airways. Airway clearance techniques, such as chest physiotherapy, can help loosen and clear this mucus. There are also devices such as the resi OPEP that can help with airway clearance.
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• Medications: Several medications are used to treat cystic fibrosis. Antibiotics are often used to treat lung infections. Bronchodilators can help open up airways, making it easier to breathe. Mucolytics can help break down mucus in the lungs. Orkambi, Symdeko, and Trikafta are three drugs that help treat the underlying cause of cystic fibrosis.
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• Nutritional support: People with cystic fibrosis often have difficulty absorbing nutrients from their food, which can lead to malnutrition. Nutritional support, such as high-calorie diets and pancreatic enzyme supplements, can help ensure adequate nutrition.
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• Lung transplant: For people with severe lung damage, a lung transplant may be necessary. This is a major surgery and carries significant risks, but can be life-saving for some individuals with cystic fibrosis.
It's important to work with a healthcare team to develop a personalized treatment plan for cystic fibrosis, as the condition can vary greatly from person to person.
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Accumulation of Mucus Plugs in COPD Linked to Increased Risk of Premature Death, Study Finds
ExpiredA review supported by the National Heart, Lung, and Blood Institute (NHLBI) found that people with chronic obstructive pulmonary disease (COPD) who accumulate extra mucus in their airways, known as mucus plugs, have a higher risk of premature death. The study analyzed data from 4,363 adults with COPD, aged 45-80, who were smokers. It revealed that 59% had no detectable mucus plugs, 22% had plugs in at least one airway, and 19% had plugs in at least three airways. Over the course of nearly 10 years, 41% of participants died, with higher mortality rates observed among those with mucus plugs. After adjusting for other factors, researchers concluded that the presence of mucus plugs in the airways increases the likelihood of premature death. These findings could potentially guide the development of targeted treatments aimed at breaking up and clearing mucus in individuals with COPD.
Source: https://www.nhlbi.nih.gov/news/2023/extra-mucus-accumulation-copd-linked-increased-risk-death
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Inspiratory muscle training reduces diaphragm activation and dyspnea during exercise in COPD
ExpiredAbstract
Among patients with chronic obstructive pulmonary disease (COPD), those with the lowest maximal inspiratory pressures experience greater breathing discomfort (dyspnea) during exercise. In such individuals, inspiratory muscle training (IMT) may be associated with improvement of dyspnea, but the mechanisms for this are poorly understood. Therefore, we aimed to identify physiological mechanisms of improvement in dyspnea and exercise endurance following inspiratory muscle training (IMT) in patients with COPD and low maximal inspiratory pressure (Pimax). The effects of 8 wk of controlled IMT on respiratory muscle function, dyspnea, respiratory mechanics, and diaphragm electromyography (EMGdi) during constant work rate cycle exercise were evaluated in patients with activity-related dyspnea (baseline dyspnea index <9). Subjects were randomized to either IMT or a sham training control group ( n = 10 each). Twenty subjects (FEV1 = 47 ± 19% predicted; Pimax = -59 ± 14 cmH2O; cycle ergometer peak work rate = 47 ± 21% predicted) completed the study; groups had comparable baseline lung function, respiratory muscle strength, activity-related dyspnea, and exercise capacity. IMT, compared with control, was associated with greater increases in inspiratory muscle strength and endurance, with attendant improvements in exertional dyspnea and exercise endurance time (all P < 0.05). After IMT, EMGdi expressed relative to its maximum (EMGdi/EMGdimax) decreased ( P < 0.05) with no significant change in ventilation, tidal inspiratory pressures, breathing pattern, or operating lung volumes during exercise. In conclusion, IMT improved inspiratory muscle strength and endurance in mechanically compromised patients with COPD and low Pimax. The attendant reduction in EMGdi/EMGdimax helped explain the decrease in perceived respiratory discomfort despite sustained high ventilation and intrinsic mechanical loading over a longer exercise duration. NEW & NOTEWORTHY In patients with COPD and low maximal inspiratory pressures, inspiratory muscle training (IMT) may be associated with improvement of dyspnea, but the mechanisms for this are poorly understood. This study showed that 8 wk of home-based, partially supervised IMT improved respiratory muscle strength and endurance, dyspnea, and exercise endurance. Dyspnea relief occurred in conjunction with a reduced activation of the diaphragm relative to maximum in the absence of significant changes in ventilation, breathing pattern, and operating lung volumes.
Keywords: chronic obstructive pulmonary disease; diaphragm; dyspnea; electromyogram; exercise; inspiratory muscle strength; respiratory mechanics.
Source: https://pubmed.ncbi.nlm.nih.gov/29543134/
Abstract
Background Unilateral diaphragm dysfunction (UDD) is an underdiagnosed cause of dyspnoea. Inspiratory muscle training (IMT) is the only conservative treatment for UDD, but the mechanisms of improvement are unknown. We characterised the effects of IMT on dyspnoea, exercise tolerance and respiratory muscle function in people with UDD.
Methods 15 people with UDD (73% male, 61±8 years) were randomised to 6 months of IMT (50% maximal inspiratory mouth pressure (PI,max), n=10) or sham training (10% PI,max, n=5) (30 breaths twice per day). UDD was confirmed by phrenic nerve stimulation and persisted throughout the training period. Symptoms were assessed by the transitional dyspnoea index (TDI) and exercise tolerance by constant-load cycle tests performed pre- and post-training. Oesophageal (Pes) and gastric (Pga) pressures were measured with a dual-balloon catheter. Electromyography (EMG) and oxygenation (near-infrared spectroscopy) of respiratory muscles were assessed continuously during exercise.
Results The IMT group (from 45±6 to 62±23% PI,max) and sham group (no progression) completed 92 and 86% of prescribed sessions, respectively. PI,max, TDI scores and cycle endurance time improved significantly more after IMT versus sham (mean between-group differences: 28 (95% CI 13–28) cmH2O, 3.0 (95% CI 0.9–5.1) points and 6.0 (95% CI 0.4–11.5) min, respectively). During exercise at iso-time, Pes, Pga and EMG of the scalene muscles were reduced and the oxygen saturation indices of the scalene and abdominal muscles were higher post- versus pre-training only in the IMT group (all p<0.05).
Conclusion The effects of IMT on dyspnoea and exercise tolerance in UDD were not mediated by an improvement in isolated diaphragm function, but may reflect improvements in strength, coordination and/or oxygenation of the extra-diaphragmatic respiratory muscles.
source: https://openres.ersjournals.com/content/9/5/00300-2023
Abstract
Oscillatory positive expiratory pressure (OPEP) devices have been utilized as an adjunct therapy to conventional chest physiotherapy (CPT) to promote the clearance of respiratory secretions in individuals with impaired ability to cough, particularly in chronic diseases. However, few studies have focused on the effectiveness of OPEP in lower respiratory tract infection. In the present study, all patients with lower respiratory tract infections hospitalized in the Department of Pulmonary and Critical Care Medicine, Ruijin Hospital (Shanghai, China) between February 2016 and July 2017 were analyzed. Daily sputum quantity and purulence were recorded on the first 7 days of physiotherapy. Oxygenation index, partial pressure carbon dioxide, white blood cell count, neutrophil percentage, C reactive protein (CRP) and procalcitonin (PCT) levels before and after CPT were compared between patients who received OPEP and patients who received mechanical percussion (MP). Sputum was collected prior to and following CPT. A total of 17 patients received OPEP, while 10 received MP. The OPEP group exhibited improved postural drainage compared with the MP group after 7 days of physiotherapy. After 7 days of CPT, patients who received OPEP also exhibited a significantly improved oxygenation index, while the oxygenation index in the MP group did not improve. The improvement of partial pressure carbon dioxide was not significantly different between groups. The OPEP group also exhibited a greater decrease in white blood cell count, neutrophil percentage and CRP levels, compared with the MP group. However, the decrease in PCT level was similar in the OPEP and MP groups. Sputum culture results revealed that the rate of negative conversion was very low in both groups. There was no difference between the two groups in terms of hospitalization outcomes. In conclusion, OPEP exhibited a greater effectiveness in draining sputum, improving oxygenation and reducing inflammatory status in patients with lower respiratory tract infections compared with MP; however, it did not promote the elimination of microbes.
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