Exploring the complexity of cystic fibrosis (CF) and psychosocial wellbeing in the 2020s: Current and future challenges.

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]. However alongside, or perhaps because of, this exciting progress, the past few years have also brought important novel challenges to the psychosocial wellbeing of people with CF. This article, written as a collaboration between CF psychologists, social workers, physicians and nurses aims to provide an accessible overview of the novel psychosocial challenges now faced by children, their families, and adults with CF, and to invite consideration of their changing psychosocial requirements to inform future holistic care. Themes include geopolitical stressors such as the pandemic and its wake, a growing divide between those able or unable to access CFTR modulators, potential rapid changes in life expectancy secondary to these drugs and the inevitable associated challenges this brings; evolving body image, mental health side effects of CFTR modulators, the challenges of adherence in apparently well children and young adults, as well as the diagnostic conundrum and associated anxiety of the cystic fibrosis screen positive inconclusive diagnosis (CFSPID) label. It also highlights some unmet research and service delivery needs in the area.

Striving for perfection, accepting the reality: A reflection on adherence to airway clearance and inhalation therapy for paediatric patients with chronic suppurative lung disease.

Non-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.

The trachea with an air-fluid level: a rare and bizarre radiological sign.

We 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. We propose that in these children impaired airway mucus clearance may be also be caused by voluntary cough suppression. However, the complex physiology of coughing means it is difficult to distinguish between true voluntary cough suppression and paucity of cough due to a subtle neurological deficit. In two patients, the cycle has led to permanent lung damage with bronchiectasis and reduced lung function. In the third, early diagnosis and multidisciplinary intervention has so far delayed progression to bronchiectasis. With greater awareness of this phenomenon in children, there is potential for effective early intervention with medical, physical, and psychological therapies.

"Passage to Paradise" ethics and end-of-life decisions in children.

We 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). Key is careful planning by the whole multidisciplinary team and unhurried and frequent communication with the parents by a few key people. It is important to keep re-checking levels of understanding of the family; at times of stress, little information may be retained. Care should extend to the wider family and friends, and it is important also to consider the needs of hospital staff, both the immediate core team and beyond. Respect for religious and cultural issues is essential, but should not be allowed to interfere with optimum treatment of the child. The validity of different patterns of grieving should be acknowledged. Regular review is essential. There need to be open discussions about capping care, so that only appropriate interventions are given. This may be more difficult if the dying child is awaiting transplantation. The place of death (home, hospice, hospital) will be a matter of individual choice. After the death of the child, we offer follow up care for both the family and the staff.