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Background Children with chronic respiratory failure and/or sleep disordered breathing due to a broad range of diseases may require long-term ventilation to be managed at home. Advances in the use of long-term non-invasive ventilation has progressively leaded to a reduction of the need for invasive mechanical ventilation through tracheostomy. In this study, we sought to characterize a cohort of children using long-term NIV and IMV and to perform an analysis of those children who showed significant changes in ventilatory support management. Methods We performed a retrospective cohort study of pediatric (within 18 years old) patients using long-term, NIV and IMV, hospitalized in our center between January 1, 2000 and December 31, 2017. A total of 432 children were included in the study. Long Term Ventilation (LTV) was defined as IMV or NIV, performed on a daily basis, at least 6 h/day, for a period of at least 3 months. Results 315 (72.9%) received non-invasive ventilation (NIV); 117 (27.1%) received invasive mechanical ventilation (IMV). Children suffered mainly from neuromuscular (30.6%), upper airway (24.8%) and central nervous system diseases (22.7%). Children on IMV were significantly younger when they start LTV [NIV: 6.4 (1.2–12.8) years vs IMV 2.1 (0.8–7.8) years] ( p  < 0.001)]. IMV was likely associated with younger age at starting ventilatory support (aOR 0.9428; p  = 0.0220), and being a child with home health care (aOR 11.4; p  < 0.0001). Overtime 39 children improved (9%), 11 children on NIV (3.5%) received tracheostomy; 62 children died (14.3%); and 74 children (17.1%) were lost to follow-up (17.8% on NIV, 15.4% on IMV). Conclusions Children on LTV suffered mainly from neuromuscular, upper airways, and central nervous system diseases. Children invasively ventilated usually started support younger and were more severely ills.

Background Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition characterized by an alveolar hypoventilation due to a deficient autonomic central control of ventilation and a global autonomic dysfunction. Paired-like homeobox 2B (PHOX2B) mutations are found in most of the patients with CCHS. In recent years, the condition has evolved from a life-threatening neonatal onset disorder to include broader and milder clinical presentations, affecting children, adults and families. Genes other than PHOX2B have been found responsible for CCHS in rare cases and there are as yet other unknown genes that may account for the disease. At present, management relies on lifelong ventilatory support and close follow up of dysautonomic progression. Body This paper provides a state-of-the-art comprehensive description of CCHS and of the components of diagnostic evaluation and multi-disciplinary management, as well as considerations for future research. Conclusion Awareness and knowledge of the diagnosis and management of this rare disease should be brought to a large health community including adult physicians and health carers.

The number of children with tracheostomies with and without home mechanical ventilation has grown continuously in recent years. For some of these children, the need for tracheostomy resolves and the child can be weaned from the tracheal cannula. Choosing the optimal time point for decannulation after elaborated prior diagnostic work-up needs careful consideration. The decannulation process requires an interdisciplinary team; however, these specialized structures for the experienced care of these children with tracheostomy are not available in all areas. The Working Group on Chronic Respiratory Insufficiency in the German Speaking Pediatric Pneumology Society (GPP) developed these recommendations to guide through a decannulation process. Initial evaluation of decannulation feasibility starts in the outpatient clinic with a detailed history, examination, and a speaking valve trial and is followed by an inpatient workup including sleep study, airway endoscopy and possibly modifications of the tracheal cannula. Downsizing the tracheal cannula allows a stepwise controlled weaning prior to removal of the tracheal cannula. After shrinking of the tracheostomy, the final surgical closure is performed.   Conclusion : An algorithm with diagnostic and therapeutic procedures for a safe and successful decannulation process is proposed. What is Known: • In children tracheostomy decannulation is a complex process that requires careful preparation and surveillance. What is New: • This statement of the German speaking society of pediatric pulmonology provides an expert practice guidance on the decannulation procedure and the value of one-way speaking valves.

Background It has been shown that home mechanical ventilation improves quality of life, but it has not been widely studied which particular patient groups benefit the most from starting this type of therapy. The purpose of this prospective observational study was to evaluate quality of life change patterns 6 months after initiation of home mechanical ventilation in patients suffering from chronic respiratory failure using patient reported outcomes. Methods We enrolled 74 chronic respiratory failure patients starting invasive or noninvasive home mechanical ventilation through the Semmelweis University Home Mechanical Ventilation Program. Quality of life was evaluated at baseline and at 6 months after initiation of home mechanical ventilation using the Severe Respiratory Insufficiency Questionnaire. Results Overall quality of life showed 10.5% improvement 6 months after initiation of home mechanical ventilation ( p  < 0.001). The greatest improvement was observed in Respiratory complaint (20.4%, p  = 0.015), Sleep and attendant symptoms (19.3%, p  < 0.001), and Anxiety related subscales (14.4%, p  < 0.001). Interface (invasive versus noninvasive ventilation) was not associated with improvement in quality of life ( p  = 0.660). Severely impaired patients showed the greatest improvement (CC = -0.328, p  < 0.001). Initial diagnosis contributed to the observed change ( p  = 0.025), with chronic obstructive pulmonary disease and obesity hypoventilation syndrome patients showing the greatest improvement, while amyotrophic lateral sclerosis patients showed no improvement in quality of life. We found that patients who were started on long term ventilation in an acute setting, required oxygen supplementation and had low baseline quality of life, showed the most improvement during the six-month study period. Conclusions Our study highlights the profound effect of home mechanical ventilation on quality of life in chronic respiratory failure patients that is indifferent of ventilation interface but is dependent on initial diagnosis and some baseline characteristics, like acute initiation, oxygen supplementation need and baseline quality of life. Trial registration This study was approved by and registered at the ethics committee of Semmelweis University (SE TUKEB 251/2017; 20th of December, 2017).

Dislocation of an endotracheal tube (ETT) during invasive ventilation can lead to serious events such as unilateral ventilation or unintentional extubation. The correct position of the endotracheal tube is determined visually. X-ray imaging or invasive procedures such as bronchoscopy are established for repeated position verification. However, these measures are time-consuming and only provide a limited number of snapshots. A new monitoring method can recognize dislocations of the ETT. The proposed system operates automatically without the need for continuous staff awareness or interaction. A ring-shaped permanent magnet is attached to the ETT. A small device is placed extracorporeally on the patient to detect the magnetic field. This device uses 64 magnetic sensors arranged as a sensor array in an 8x8 matrix. The sensor signals are digitally converted, enabling the position of the ETT with the attached magnet to be determined by software. Two processing methods (image similarity and localization) are tested for monitoring. The prototype system detects displacements with millimeter scale positioning deviations in our tests. Our system triggers an alarm upon detecting an impermissible dislocation, complete extubation, or unintended bronchial intubation. The proposed methods were validated on a sensor array prototype and assessed through a dedicated experimental setup. The results are promising and could lead to further development towards clinical usability. Early warnings would be particularly advantageous, even for minor or beginning dislocations of the ETT. An automated continuous tube monitoring process could help reduce the workload of the staff and improve patient safety.

The last two decades have seen increasing use of long-term ventilation (LTV) as an intervention in childhood. Children who use LTV have many risk factors for feeding and swallowing difficulties, including their underlying respiratory and/or neurological etiology, long hospitalizations, medical interventions, and limited exposure to oral feeding experiences. This review aimed to answer two questions: 1) ‘What specific swallowing and feeding characteristics do these children experience?’; and 2) ‘What impacts do these swallowing and feeding characteristics have on health status and quality of life?’. Texts were identified across bibliographic databases, reference lists, and grey literature. Studies were analyzed according to ventilation, feeding and swallowing, assessment and intervention, and quality of life parameters. Overall, 1919 papers were screened, with 31 papers included in the final data extraction process. A range of feeding and swallowing characteristics were observed, including oral secretion management difficulties, oral aversion, swallowing difficulties, and clinical signs of aspiration. Non-oral feeding was found to be the primary feeding method used. Little information on health status and quality of life was reported in scoping review texts. Children with LTV needs present with a range of feeding and swallowing concerns, and non-oral feeding is common. Further research is needed to understand the feeding and swallowing journey of this population. This will assist in future service planning and delivery, and in turn contribute to improving patient outcomes and quality of life.

Kaum ein anderer Bereich der modernen Medizin hat eine derart rasante Entwicklung genommen wie die Intensivmedizin. So sehen wir gerade im prolongierten Weaning häufig Patienten, die sich nicht ohne Komplikationen von ihrer Grunderkrankung erholen und entsprechend schwer von der maschinellen Beatmung zu entwöhnen sind. Die Entwöhnung von der maschinellen Beatmung ist eine Herausforderung für das behandelnde Team. Um das bestmögliche Outcome für die Patienten zu erzielen, den Patienten eine qualifizierte, hochspezialisierte und erfahrene Betreuung zukommen zu lassen, bedarf es einer fachübergreifenden, interdisziplinären Zusammenarbeit.

Background The decision to initiate invasive long-term ventilation for a child with complex medical needs can be extremely challenging. TechChild is a research programme that aims to explore the liminal space between initial consideration of such technology dependence and the final decision. This paper presents a best practice example of the development of a unique use of the factorial survey method to identify the main influencing factors in this critical juncture in a child’s care. Methods We developed a within-subjects design factorial survey. In phase 1 (design) we defined the survey goal (dependent variable, mode and sample). We defined and constructed the factors and factor levels (independent variables) using previous qualitative research and existing scientific literature. We further refined these factors based on expert feedback from expert clinicians and a statistician. In phase two (pretesting), we subjected the survey tool to several iterations (cognitive interviewing, face validity testing, statistical review, usability testing). In phase three (piloting) testing focused on feasibility testing with members of the target population ( n  = 18). Ethical approval was obtained from the then host institution’s Health Sciences Ethics Committee. Results Initial refinement of factors was guided by literature and interviews with clinicians and grouped into four broad categories: Clinical, Child and Family, Organisational, and Professional characteristics. Extensive iterative consultations with clinical and statistical experts, including analysis of cognitive interviews, identified best practice in terms of appropriate: inclusion and order of clinical content; cognitive load and number of factors; as well as language used to suit an international audience. The pilot study confirmed feasibility of the survey. The final survey comprised a 43-item online tool including two age-based sets of clinical vignettes, eight of which were randomly presented to each participant from a total vignette population of 480. Conclusions This paper clearly explains the processes involved in the development of a factorial survey for the online environment that is internationally appropriate, relevant, and useful to research an increasingly important subject in modern healthcare. This paper provides a framework for researchers to apply a factorial survey approach in wider health research, making this underutilised approach more accessible to a wider audience.

Background Home mechanical ventilation (HMV) is a viable and effective strategy for patients with chronic respiratory failure (CRF). The Chilean Ministry of Health started a program for adults in 2008. Methods This study examined the following data from a prospective cohort of patients with CRF admitted to the national HMV program: characteristics, mode of admission, quality of life, time in the program and survival. Results A total of 1105 patients were included. The median age was 59 years (44–58). Women accounted for 58.1% of the sample. The average body mass index (BMI) was 34.9 (26–46) kg/m 2 . A total of 76.2% of patients started HMV in the stable chronic mode, while 23.8% initiated HMV in the acute mode. A total of 99 patients were transferred from the children's program. There were 1047 patients on non-invasive ventilation and 58 patients on invasive ventilation. The median baseline PaCO 2 level was 58.2 (52–65) mmHg. The device usage time was 7.3 h/d (5.8–8.8), and the time in HMV was 21.6 (12.2–49.5) months. The diagnoses were COPD (35%), obesity hypoventilation syndrome (OHS; 23.9%), neuromuscular disease (NMD; 16.3%), non-cystic fibrosis bronchiectasis or tuberculosis (non-CF BC or TBC; 8.3%), scoliosis (5.9%) and amyotrophic lateral sclerosis (ALS; 5.24%). The baseline score on the Severe Respiratory Insufficiency questionnaire (SRI) was 47 (± 17.9) points and significantly improved over time. The lowest 1- and 3-year survival rates were observed in the ALS group, and the lowest 9-year survival rate was observed in the non-CF BC or TB and COPD groups. The best survival rates at 9 years were OHS, scoliosis and NMD. In 2017, there were 701 patients in the children's program and 722 in the adult´s program, with a prevalence of 10.4 per 100,000 inhabitants. Conclusion The most common diagnoses were COPD and OHS. The best survival was observed in patients with OHS, scoliosis and NMD. The SRI score improved significantly in the follow-up of patients with HMV. The prevalence of HMV was 10.4 per 100,000 inhabitants. Trial registration This study was approved by and registered at the ethics committee of North Metropolitan Health Service of Santiago, Chile (N° 018/2021).

Introduction: Children dependent on Long-Term Ventilation (LTV) need the planning, provision and monitoring of complex services generally provided at home by professionals belonging to different settings. The collaboration among professionals improves the efficiency and the continuity of care especially when treating children with complex needs. In this paper patterns of collaboration have been analysed across the 30 EU/EEA countries of the MOCHA project using Unified Modelling Language (UML). Methods: The level of collaboration was defined analysing the answers provided by local Country Agents (CAs) in a questionnaire adapted from standards of care for children with complex care needs. Particular attention was given to: management of the personalized plan, organization of the transition from the hospital to the child’s home, provision of general and preventive services and access to specialized services. Responses of the CAs were summarized using UML to capture: the professionals involved in each activity and their configuration in a multidisciplinary team (MDT) (Use case diagram); the flow of activities performed and the messages exchanged by the different actors/teams taking into account the location and the timeline of each activity (Activity diagram). Results: Twenty-three CAs responded to the questionnaire. Within a high variety of patterns of collaboration, primary care professionals are involved in the majority of countries providing general services, while specialists continue to be a reference point also when the child is discharged at home. Moreover, the presence of a social worker within a MDT indicates a trend of including the social component for the children and their families. The adoption of a personalized plan is one of the optimum features in the treatment of complex care, in particular considering that it should include the procedures to access care in case of medical crisis. Discussion: This approach provides important indications on the efficiency in performing and organizing health-related activities. The association of identified activities with data, such as number and cost of professionals, health outcomes and waiting times, could provide an in-depth analysis indicating best practices in the provision of care. Conclusions: The adoption of a business process analysis allowed us to identify different patterns of collaboration that may be distributed along a continuum of integration. Moreover, the use UML made it possible to represent the different organizational features in place in the MOCHA countries to manage children with LTV in a comparable way. Lessons learned: The adoption of a business process analysis contributed to the analysis of the integration of care among the primary, secondary and social level. It captured the patterns of care delivery in the highly dynamic, complex and multi-disciplinary nature of healthcare processes. Limitations: The description of the care process is provided by a single CA that describes the actual situation in his/her country. Further analysis can help to capture regional variations, differences in terms of services provided in urban and rural areas or changes depending on the severity. Suggestions for future research: Compare the results obtained for LTV with other complex care needs analysed in MOCHA such as, traumatic brain injury, intractable epilepsy.