Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy [1-9].This decade has seen transformative change in management for many, but not all, people with CF. The most notable change comes from Cystic Fibrosis Transmembrane Receptor (CFTR) modulators, which bring significant benefits for people who are eligible for, and able to access, them [10].
View Article and Find Full Text PDFNon-adherence to prescribed treatment is considered the foremost cause of treatment failure in chronic medical conditions. Airway clearance techniques (ACT) play a key role in the management of chronic suppurative lung disease yet, along with inhaled therapies such as nebulised antibiotics, adherence to these is often lower than to other treatments. In this review we discuss methods of monitoring adherence to these therapies and potential barriers and outline suggestions for improving adherence in the paediatric population.
View Article and Find Full Text PDFWe report three children with an unusual radiological sign: "trachea with an air fluid level." We suggest this is related to paucity of cough leading to recurrent chest infections. Voluntary cough suppression as a cause of chronic lower respiratory tract infection is recorded in adults (The Lady Windermere Syndrome) but has not previously been reported in children.
View Article and Find Full Text PDFWe describe our personal practice in the care of the dying child. There is no on right model of care, and the multidisciplinary team must be sensitive to individual needs. It is important to get the balance of palliation and active treatment right (important at all stages of the illness).
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