Changes in UK paediatric long-term ventilation practice over 10 years

Abstract

ObjectivesTo provide up-to-date information on the use of long-term ventilation (LTV) in the UK paediatric population and to compare the results with data collected 10 and 20 years previously.DesignA single timepoint census completed by LTV centres in the UK, carried out via an online survey.Setting and patientsAll patients attending paediatric LTV services in the UK.ResultsData were collected from 25 LTV centres in the UK. The total study population was 2383 children and young people, representing a 2.5-fold increase in the last 10 years. The median age was 9 years (range 0–20 years). Notable changes since 2008 were an increase in the proportion of children with central hypoventilation syndrome using mask ventilation, an increase in overall numbers of children with spinal muscular atrophy (SMA) type 1, chronic lung disease of prematurity and cerebral palsy being ventilated, and a 4.2-fold increase in children using LTV for airway obstruction. The use of 24-hour ventilation, negative pressure ventilation and tracheostomy as an interface had declined. 115 children had received a disease-modifying drug. The use of ataluren and Myozyme did not influence the decision to treat with LTV, but in 35% of the children with SMA type 1 treated with nusinersin, the clinician stated that the use of this drug had or may have influenced their decision to initiate LTV.ConclusionThe results support the need for national database for children and young people using LTV at home to inform future recommendations and assist in resource allocation planning.

Shared decision-making experiences in child long-term ventilation: a systematic review

“…In the past decade, there has been a 2.5-fold increase in the number of children receiving LTV in the UK [ 12 ]. As both the prevalence and survival of children with life-limiting conditions increase [ 12 ], decisions about whether to initiate LTV will more frequently need to be made by patients, their caregivers and healthcare professionals. While the principles of SDM should be applied in decisions related to treatment for children with life-limiting conditions more broadly.…”

“…Despite the publication of such guidelines, there is minimal empirical evidence or understanding of the application of SDM in the practice of LTV initiation. This is significant because the number of children and young people (0–24 years) on LTV is increasing rapidly, with an estimated 2382 in 2019 in the UK [ 12 ]. Consequently, there is a growing need to substantiate existing opinion-based guidance with empirical evidence and understanding.…”

‘It gives me more freedom’: Family perspectives on travelling with children on nocturnal ventilation

“…Whilst individual neuromuscular and central hypoventilation diagnoses might be rare, the total population of children affected by these conditions is increasing (Rose et al, 2019; Woodcock et al, 2016). Moreover, these children are living longer lives (Barker et al, 2023; Castro‐Codesal et al, 2018; Flotats‐Bastardas & Hahn, 2020; National Confidential Enquiry into Patient Outcome and Death [NCEPOD], 2020; Turner, 2023; Wallis et al, 2011), with associated and changing expectations about the scope of their anticipated life experiences and opportunities (Bayes et al, 2012; Edwards et al, 2021). Understanding the value of expanding opportunities for children requiring nocturnal ventilation to include air travel requires consideration of the clinical implications for the individual child, knowledge of the practical issues and appreciation of the meaning of these ambitions for the child and family.…”

Transitions in tracheostomy care: from childhood to adulthood

“…Indications for short-term tracheostomy placement in children and young people (CYP) due to chronic airway obstruction secondary to infections, are infrequent due to mass immunisation programmes [5 ▪▪ ]. Current indications for tracheostomy in children and young people reflect the advances in managing respiratory conditions, particularly associated with prematurity resulting in long-term ventilation, and identification of congenital upper airway obstruction in utero or early infancy [6,7 ▪ ]. Additionally, advancing pharmacological and clinical expertise have enabled survival from significant acquired conditions such as brain injury and trauma [8].…”