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Case 19
Published in Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta, Clinical Cases, 2021
Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta
Prior to starting antibiotics, patients should have stopped smoking, have completed pulmonary rehabilitation, have been vaccinated appropriately and have optimised inhaled management. They should also have sputum cultures sent and a CT thorax to rule out other lung pathologies. The patient should also be trained in airway clearance techniques.
Respiratory Diseases
Published in Amy J. Litterini, Christopher M. Wilson, Physical Activity and Rehabilitation in Life-threatening Illness, 2021
Amy J. Litterini, Christopher M. Wilson
There is no definitive cure for cystic fibrosis, and therefore, treatment primarily centers around symptom management. Pharmacological interventions as well as airway clearance techniques are the mainstays of care; however, lifestyle and behavioral choices including a healthy diet, physical activity, and avoiding tobacco use are recommended to improve health outcomes. Supplemental oxygen is recommended when indicated, and lung transplant is a consideration for some with advanced disease.11
Physiotherapy and airway clearance
Published in Claudio F. Donner, Nicolino Ambrosino, Roger S. Goldstein, Pulmonary Rehabilitation, 2020
Miguel R. Gonçalves, Amanda J. Piper
While these devices are widely used for patients with obstructive lung disease and secretion encumbrance (28,29), there is little evidence that PEP with or without oscillation is any more or less effective than other techniques aimed at peripheral airway clearance (30–32). Likewise, there is no clear evidence PEP improves short- or long-term outcomes when used during an exacerbation of COPD (33). A number of factors will influence clinician prescription and patient use of an airway clearance technique including cost, convenience, comfort and perceived effectiveness of therapy. Patient preference is important, as this will likely impact acceptance and adherence to routine airway clearance therapy. Patients with NMD and respiratory muscle weakness are unlikely to generate sufficient expiratory flow for PEP devices to be effective and a recent state-of-the-art review recommended against using PEP in this population (34).
Outpatient management of pulmonary exacerbations in children with cystic fibrosis
Published in Expert Review of Respiratory Medicine, 2023
Lucy Perrem, Isaac Martin, Felix Ratjen
To address mucus clearance, mainstays of treatment involve mucolytics (e.g. rhDNase) and therapies that rehydrate the airway mucus (e.g. hypertonic saline) which create an osmotic gradient and draw water into the dehydrated airway mucus. When these therapies are combined with effective airway clearance techniques, this helps clear pathogens and byproducts of the inflammatory cascade. These strategies can be especially important in the context of pulmonary exacerbations. If not already established as a twice daily treatment, in the context of a pulmonary exacerbation it may also be of clinical utility add 7% hypertonic saline to the treatment regimen as this has been shown to reduce symptom burden during an exacerbation in adults [74]. The introduction of rhDNase has not been shown to improve outcomes when used in acute pulmonary exacerbations when combined with IV antibiotics and airway clearance maneuvers [75].
Care of children with home mechanical ventilation in the healthcare continuum
Published in Hospital Practice, 2021
Benjamin Kalm, Khanh Lai, Natalie Darro
In patients with poor respiratory strength, it is important to optimize airway clearance. Airway clearance techniques (ACTs) include postural drainage, percussion, chest wall vibration, and coughing. In children on HMV, recommended home equipment includes portable suctioning equipment, a heated humidifier, and a nebulizer/compressor [17]. Of note, many of our patients do not use nebulized medications and therefore do not have a nebulizer/compressor. A mechanical insufflation-exsufflation device (commonly called cough assist) may benefit children with HMV with ineffective cough, such as those with neuromuscular disease with respiratory muscle weakness. In children large enough for proper fitment, high-frequency chest wall oscillation is indicated for impaired clearance of secretions with impaired mobility. There is a lack of randomized controlled trials regarding choice of different ACT modalities in the pediatric HMV population.
Bronchiectasis
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2021
Airway clearance is typically recommended as a mainstay of management in bronchiectasis and an analysis of 905 patients in the US Bronchiectasis and NTM Registry found 59% used an airway clearance technique.26 47% of these patients had a history of chronic Pseudomonas airway infection as well as an exacerbation or hospitalization in the preceding 2 years. However, at a 1 year follow up point, 58% of patients were no longer practicing airway clearance. Techniques for airway clearance are varied and experience with the high frequency chest wall oscillation vest was reported on in a review of a registry of 198 patients with bronchiectasis identified by ICD Coding.27 This review did not use validated questionnaires and instead relied on patient self-reporting via telephone surveys. It reported a reduction in hospitalizations from 49.1% to 24% in the 12 months’ following treatment initiation. This may be encouraging but further studies are necessary. Finally, a systematic review and meta-analysis to evaluate the efficacy of hypertonic saline compared with 0.9% saline as an adjunct to airway clearance in bronchiectasis found there to be no superiority for hypertonic saline on forced expiratory volume in 1 second, forced vital capacity, sputum expectoration or cough severity using the LCQ from four randomized controlled trials.28