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Aims The aim was to evaluate whether standardised exercise performance during the incremental shuttle walk test (ISWT) can be used to assess disease severity in children and young people (CYP) with chronic conditions, through (1) identifying the most appropriate paediatric normative reference equation for the ISWT, (2) assessing how well CYP with haemophilia and cystic fibrosis (CF) perform against the values predicted by the best fit reference equation and (3) evaluating the association between standardised ISWT performance and disease severity.Methods A cross-sectional analysis was carried out using existing data from two independent studies (2018–2019) at paediatric hospitals in London,UK. CYP with haemophilia (n=35) and CF (n=134) aged 5–18 years were included. Published reference equations for standardising ISWT were evaluated through a comparison of populations, and Bland-Altman analysis was used to assess the level of agreement between distances predicted by each equation. Associations between ISWT and disease severity were assessed with linear regression.Results Three relevant reference equations were identified for the ISWT that standardised performance based on age, sex and body mass index (Vardhan, Lanza, Pinho). A systematic proportional bias of standardised ISWT was observed in all equations, most pronounced with Vardhan and Lanza; the male Pinho equation was identified as most appropriate. On average, CYP with CF and haemophilia performed worse than predicted by the Pihno equation, although the range was wide. Standardised ISWT, and not ISWT distance alone, was significantly associated with forced expiratory volume in 1 s in CYP with CF. Standardised ISWT in CYP with haemophilia was slightly associated with haemophilia joint health score, but this was not significant.Conclusions ISWT performance may be useful in a clinic to identify those with worsening disease, but only when performance is standardised against a healthy reference population. The development of validated global reference equations is necessary for more robust assessment.

Abstract Background Clinical outcomes are normally captured less frequently than data from remote technologies, leaving a disparity in volumes of data from these different sources. To align these data, flexible polynomial regression was investigated to estimate personalised trends for a continuous outcome over time. Methods Using electronic health records, flexible polynomial regression models inclusive of a 1st up to a 4th order were calculated to predict forced expiratory volume in 1 s (FEV1) over time in children with cystic fibrosis. The model with the lowest AIC for each individual was selected as the best fit. The optimal parameters for using flexible polynomials were investigated by comparing the measured FEV1 values to the values given by the individualised polynomial. Results There were 8,549 FEV1 measurements from 267 individuals. For individuals with > 15 measurements (n = 178), the polynomial predictions worked well; however, with

Abstract Background Cystic fibrosis (CF) is a genetically inherited, life-limiting condition, affecting ~90,000 people globally. Physical activity (PA) and exercise form an integral component of CF management, and have been highlighted by the CF community as an area of interest for future research. Previous reviews have solely focused on PA or structured exercise regimens independent of one another, and thus a comprehensive assessment of the physical health benefits of all PA, including exercise, interventions, is subsequently warranted. Therefore, the purpose of this review is to evaluate the effects of both PA and exercise upon outcomes of physical health and healthcare utilisation in people with CF. Methods A systematic review has been registered and reported in line with Preferred Reporting Items for Systematic Reviews and Meta-Analysis-P guidelines. This will include randomised control trials on the effects of PA and exercise, relative to usual treatment, upon people with CF. Primary outcomes will include variables associated with fitness, PA, lung health, inflammation, body composition, glycaemic control and patient-reported outcomes. Secondary outcomes will include adverse events and healthcare utilisation. Searches will be undertaken in Ovid MEDLINE, OVID EMBASE, PsychINFO, ERIC, SPORTDiscus, ASSIA, CCTR, CINHAL and Web of Science databases, and will be searched from date of inception onwards. Two reviewers will independently screen citations and abstracts, and full-texts, for inclusion and data extraction, respectively. Methodological quality will be assessed using the Cochrane Risk of Bias-2 tool. If feasible, random-effects meta-analyses will be conducted where appropriate. Additional analyses will explore potential sources of heterogeneity, such as age, sex, and disease severity. Discussion This systematic review will build on previous research, by comprehensively assessing the impact of both PA and exercise upon physical health and healthcare utilisation in people with CF. Results of this review will be utilised to inform discussions that will ultimately result in a consensus document on the impact of physical activity and exercise for people with CF. Systematic review registration PROSPERO CRD42020184411

Introduction Daily physiotherapy is believed to mitigate the progression of cystic fibrosis (CF) lung disease. However, physiotherapy airway clearance techniques (ACTs) are burdensome and the evidence guiding practice remains weak. This paper describes the protocol for Project Fizzyo, which uses innovative technology and analysis methods to remotely capture longitudinal daily data from physiotherapy treatments to measure adherence and prospectively evaluate associations with clinical outcomes.Methods and analysis A cohort of 145 children and young people with CF aged 6–16 years were recruited. Each participant will record their usual physiotherapy sessions daily for 16 months, using remote monitoring sensors: (1) a bespoke ACT sensor, inserted into their usual ACT device and (2) a Fitbit Alta HR activity tracker. Real-time breath pressure during ACTs, and heart rate and daily step counts (Fitbit) are synced using specific software applications. An interrupted time-series design will facilitate evaluation of ACT interventions (feedback and ACT-driven gaming). Baseline, mid and endpoint assessments of spirometry, exercise capacity and quality of life and longitudinal clinical record data will also be collected.This large dataset will be analysed in R using big data analytics approaches. Distinct ACT and physical activity adherence profiles will be identified, using cluster analysis to define groups of individuals based on measured characteristics and any relationships to clinical profiles assessed. Changes in adherence to physiotherapy over time or in relation to ACT interventions will be quantified and evaluated in relation to clinical outcomes.Ethics and dissemination Ethical approval for this study (IRAS: 228625) was granted by the London-Brighton and Sussex NREC (18/LO/1038). Findings will be disseminated via peer-reviewed publications, at conferences and via CF clinical networks. The statistical code will be published in the Fizzyo GitHub repository and the dataset stored in the Great Ormond Street Hospital Digital Research Environment.Trial registration number ISRCTN51624752; Pre-results.

Abstract Background Communication skills (CS) are vital for doctors. Indeed, as the most important element of consultations, are highly valued by patients. CS are core, teachable skills, however, have not been widely taught in South Asian medical schools, unlike their western counterparts. Patan Academy of Health Sciences, is one of the first in Nepal to have CS central to its’ aims and curriculum. CS are taught from the first weeks of medical school and re-enforced during preclinical study (first 2 years). Our study seeks to explore students’ perceptions and experiences of CS teaching in this South Asian, Nepal context. Methods This study is a qualitative evaluation of a CS course in Nepal, exploring the experiences and perceptions of participants. The study aims to also identify aspects that were helpful or not for student learning and areas for potential development. A purposive sample of twenty: second, fourth and Intern year students was selected for interview. Data were collected through audio recorded semi-structured interviews following a piloted schedule. Interview transcripts were manually coded and thematically analysed. Codes were arranged into themes and subthemes. Results The two main themes: 1. Positivity 2. Experiential learning. Results demonstrate participants’ positive perceptions of CS teaching: believing it is important, effective, relevant and valuable for personal development. Participants identified experiential learning features as valuable for CS acquisition. Intern students recognised CS relevance and requested expanding teaching to clinical years,incorporating challenging communication scenarios. Discussion This study shows that PAHS’ CS course is well perceived and valuable to learners. Experiential learning is powerful for CS development. Expansion of formal, structured CS teaching through all years in a spiral curriculum, should be considered. Violence towards doctors in South Asia is increasing. Students recognised CS teaching’s significance in addressing this. Conclusion CS teaching,still in its’ infancy in South Asia, is a pressing issue for medical educators here. Our study provides evidence it is well perceived with positive impacts in this context, particularly when employing experiential learning. Medical schools in south Asia should be encouraged to incorporate and strengthen their CS teaching curriculum. .

As the student population in the United States (US) is becoming more diverse, educator preparation programs are seeking ways to make the teacher workforce more diverse, which involves diversification of training experiences. Informal spaces, which can include integrated science, mathematics, engineering and technology (STEM) opportunities, are not traditionally part of teacher preparation, although teachers are being asked to occupy these STEM spaces with more frequency. Including informal experiences in a community STEM makerspace, which highlights digital fabrication, creativity, and problem solving, diversifies teacher preparation by extending beyond both the university setting and the elementary classroom. This exploratory case study with elementary (primary) pre-service teachers in a science and math methods (vis a vis STEM) class examines experiences they have in a community makerspace in a qualitative study, which uses Data Engagement (DE) as a methodological framework. DE draws upon intersectional feminist, critical, and materialist theories. Assembled and made data from observations in the makerspace, pre- and post-maker reflections, and responses to a modified in-person interview protocol reveal that, despite more open-ended and complex challenges than those experienced in class, participants reported positive future-thinking, less content dread, and more agency in perceived efficacy. These results invite further study and conversation about including informal STEM maker experiences in US teacher preparation as a step to increasing diversity in the teacher workforce.

Abstract Background Doctors’ empathy: the understanding of patients’ experiences, concerns and perspectives, is highly valued by patients yet often lacking in patient care. Medical Humanities has been introduced within undergraduate curriculum to address this lack in empathy. There is a paucity of research on the impact of a course on medical humanities on the empathy of medical students, particularly in South Asia. Here we report on the impact of such an intervention in first-year medical students and aim to help outcome-based medical education and the evaluation and promotion of humanities within medical courses. Methods This study is a quantitative evaluation of student empathy before and after a Medical Humanities Module. The study employs the Jefferson Scale of Empathy-Student version (JSE-S). Participants were first-year medical students at Patan Academy of Health Sciences, Nepal. All cohort students were invited to participate and written consent was obtained. Data were collected both prior-to and on-completion-of, a six-week Medical Humanities Module. Pre- and post-module data were analyzed and the resulting empathy scores compared using the paired t-test or Wilcoxon signed-rank test. Subgroup analysis was undertaken to determine the association of the score with gender and preferred future speciality. Results Sixty-two student responses were analyzed, 32 (52%) of whom were male. In the pre-module scores females had a slightly higher mean score than males:108 and 103 respectively. Participants who preferred people-oriented specialities also scored higher than those preferring procedure and technology-oriented specialities: 107 and 103 respectively. There was a significant increase in mean score for the entire class from pre-module to post-module: 105 to 116, p-value of

Abstract Background Communication skills (CS) are important and teachable, however, many Asian medical schools’ curricula do not incorporate them. Patan Academy of Health Sciences in Nepal identifies CS within its’ aims and curriculum. CS are taught from commencement of medical school and re-emphasised throughout preclinical learning (first 2 years). There is no explicit CS teaching in clinical years but placements allow students to learn through observation. These ‘role-modelling’ interactions form part of CS learning and development. Methods This study is a qualitative evaluation of CS learning in PAHS, through participants’ experiences. Through purposive sampling, twenty medical students from 2nd, 4th and Intern years were selected for inclusion. Data were collected via audio recorded, semi-structured interviews, employing a piloted schedule. Transcripts were manually coded and analysed thematically. Codes were organised into themes and subthemes. This paper discusses themes related to role-modelling. Results The majority of participants described role-modelling in CS learning, recounting both positive and negative incidents, reflected in the themes of; Positive and Negative experiences. Subthemes of Personal Qualities and Inspiring, emerged from positive experiences, describing students’ desire to imitate or aspire to be like their role models. Learners reported predominantly negative experiences and interns exclusively so. From these emerged subthemes of; Good doctors but.., Contradictory messages, How not to behave, Unprofessional behaviour and Affect-Emotional Distress. Learners received conflicting messages from observing behaviour contradictory to explicit CS teaching. Many identified learning “how not to behave” from such incidents, however, several described feeling distressed. Discussion Role-modelling is a powerful and important CS learning tool, seen as positively reinforcing or negatively contradicting explicit CS teaching. Negative modelling created internal conflict, confusion and distress amongst learners, despite its’ potential for positive learning. The worldwide problem of negative role-modelling is also prevalent in Nepal. Medical educators need to ensure the explicit curriculum aligns with implicit learning. Clinical tutors must be alerted to their powerful role-model position and supported in developing intentional modelling skills. Learners’ reflections upon their experiences should be facilitated, enabling them to critically evaluate observations and hence consciously adopt or reject role-modelled behaviour and attitudes.

Background: Perinatal palliative care can offer compassionate support to families following diagnosis of a life-limiting illness, to enable them to make valued choices and the most of the time that they have with their newborn. However, home birth is usually only offered in low-risk pregnancies., Case: A couple who received an antenatal diagnosis of hypoplastic left heart syndrome and who had made a plan to provide palliative care to their baby after birth requested the option of a home birth., Possible Courses of Action: Recommend birth at hospital or explore the possibility of a home birth with perinatal palliative care support., Formulation of a Plan: Multidisciplinary discussion and collaboration enabled a plan for home birth to be made which anticipated potential complications., Outcome: The baby was born at home and died on day 5 of life receiving outreach nursing, paediatric and palliative care support and buccal and oral opioids for symptom management. We include reflections from the family on the importance of this experience., Lessons: We provide a list of potential criteria for considering home birth in the setting of perinatal palliative care., View: Facilitating a home birth in the setting of perinatal palliative care is an option that can be hugely valued by families, but this service may be practically difficult to deliver in many contexts. Further research is needed to understand the preferences of women and families receiving perinatal palliative care., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Breakthrough pain is common in life-limiting conditions and at end-of-life. Despite over 30 years of study, there is little consensus regarding the definition and characteristics of breakthrough pain., Objective: This study aims to update and expand a 2010 systematic review by Haugen and colleagues to identify (1) all definitions of breakthrough pain and (2) all descriptions and classifications of breakthrough pain reported by patients, caregivers, clinicians, and experts., Design: This rapid systematic review followed the Cochrane Rapid Review Methods Group guidelines. A protocol is published on PROSPERO (CRD42019155583)., Data Sources: CINAHL, MEDLINE, PsycINFO, and the Web of Science were searched for breakthrough pain terms from the inception dates of each database to 26th August 2022., Results: We identified 65 studies that included data on breakthrough pain definitions, descriptions, or classifications from patients ( n = 30), clinicians ( n = 6), and experts ( n = 29), but none with data from caregivers. Most experts proposed that breakthrough pain was a sudden, severe, brief pain occurring in patients with adequately controlled mild-moderate background pain. However, definitions varied and there was no consensus. Pain characteristics were broadly similar across studies though temporal factors varied widely. Experts classified breakthrough pain into nociceptive, neuropathic, visceral, somatic, or mixed types. Patients with breakthrough pain commonly experienced depression, anxiety, and interference with daily life., Conclusions: Despite ongoing efforts, there is still no consensus on the definition of breakthrough pain. A compromise is needed on breakthrough pain nomenclature to collect reliable incidence and prevalence data and to inform further refinement of the construct., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2023.)

Infants, children and young people with life-limiting or life-threatening conditions often experience acute, transient pain episodes known as breakthrough pain. There is currently no established way to assess breakthrough pain in paediatric palliative care. Anecdotal evidence suggests that it is frequently underdiagnosed and undertreated, resulting in reduced quality of life. The development of a standardised paediatric breakthrough pain assessment, based on healthcare professionals' insights, could improve patient outcomes. This study aimed to explore how healthcare professionals define and assess breakthrough pain in paediatric palliative care and their attitudes towards a validated paediatric breakthrough pain assessment. This was a descriptive qualitative interview study. Semi-structured interviews were conducted with 29 healthcare professionals working in paediatric palliative care across the UK. An inductive thematic analysis was conducted on the data. Five themes were generated: 'the elusive nature of breakthrough pain', 'breakthrough pain assessment', 'positive attitudes towards', 'reservations towards' and 'features to include in' a paediatric breakthrough pain assessment. The definition and assessment of breakthrough pain is inconsistent in paediatric palliative care. There is a clear need for a validated assessment questionnaire to improve assessment, diagnosis and management of breakthrough pain followed by increased healthcare professional education on the concept.

Background: Assessing pain in infants, children and young people with life-limiting conditions remains a challenge due to diverse patient conditions, types of pain and often a reduced ability or inability of patients to communicate verbally., Aim: To systematically identify pain assessment tools that are currently used in paediatric palliative care and examine their psychometric properties and feasibility and make recommendations for clinical practice., Design: A systematic literature review and evaluation of psychometric properties of pain assessment tools of original peer-reviewed research published from inception of data sources to April 2021., Data Sources: PsycINFO via ProQuest, Web of Science Core, Medline via Ovid, EMBASE, BIOSIS and CINAHL were searched from inception to April 2021. Hand searches of reference lists of included studies and relevant reviews were performed., Results: From 1168 articles identified, 201 papers were selected for full-text assessment. Thirty-four articles met the eligibility criteria and we examined the psychometric properties of 22 pain assessment tools. Overall, the Faces Pain Scale-Revised (FPS-R) had high cross-cultural validity, construct validity (hypothesis testing) and responsiveness; while the Faces, Legs, Activity, Cry and Consolability (FLACC) scale and Paediatric Pain Profile (PPP) had high internal consistency, criterion validity, reliability and responsiveness. The number of studies per psychometric property of each pain assessment tool was limited and the methodological quality of included studies was low., Conclusion: Balancing aspects of feasibility and psychometric properties, the FPS-R is recommended for self-assessment, and the FLACC scale/FLACC Revised and PPP are the recommended observational tools in their respective age groups.

Contexts: Inadequate pain management in community pediatric palliative care is common. Evidence to inform improved pain management in this population is limited., Objectives: To explore the barriers and facilitators to pediatric community-based pain management for infants, children and young people at end-of-life as perceived by healthcare professionals., Methods: A qualitative interview study was conducted. Semi-structured interviews were undertaken with 29 healthcare professionals; 12 nurses, five GPs, five consultants and registrar doctors, two pharmacists and five support therapists working in primary, secondary or tertiary care in the United Kingdom and involved in community end-of life care of 0 to 18-year-olds., Results: The data corpus was analyzed using an inductive thematic analysis and seven themes emerged: parents' abilities, beliefs and wellbeing; working relationships between families and healthcare professionals, and between healthcare teams; healthcare professionals' knowledge, education and experience; health services delivery; nature of pain treatment; and pediatric-specific factors. Across themes, the concepts of partnership working between families and healthcare professionals, and within healthcare teams, and sharing expertise were prevalent., Conclusion: Partnership working and trust between healthcare professionals and parents, and within healthcare teams, is needed for effective at-home pediatric palliative pain management. Community healthcare professionals require more education from experienced multidisciplinary teams to effectively manage pediatric pain at end-of-life and prevent emergency hospice or hospital admissions, particularly during the COVID-19 pandemic., (Copyright © 2021 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.)

Context: Breakthrough pain (BTP) is common in cancer and other conditions yet there is a lack of validated BTP measurement tools., Objectives: We aimed to identify all tools assessing or characterising BTP in patients of any age with any condition, and to critically appraise their psychometric properties., Methods: The Cochrane Library, PROSPERO, Embase, CINAHL, Medline, PsycINFO, Web of Science, Google Scholar, ProQuest, Evidence Search and OpenGrey were searched to identify all available tools used to assess BTP. A second search identified studies that had evaluated psychometric properties of tools identified in Search 1. Databases were searched from inception to November 2020. Studies were assessed using COSMIN criteria and GRADE guidelines., Results: Search 1 found 51 tools used to assess BTP. Search 2 found six tools that had a development study and/or a study evaluating a tool psychometric property. No tool had more than one study evaluating psychometric properties so a meta-analysis could not be conducted. Studies were of inadequate to very good quality. Only the Breakthrough Pain Assessment Tool (BAT) had sufficient content validity and at least low-quality evidence for sufficient internal consistency., Conclusion: The BAT is recommended to characterise BTP in adults with cancer; its applicability to other conditions is unknown. The remaining tools need further evaluation. Only the Breakthrough Pain Questionnaire for Children was designed for children with cancer, but no psychometric properties were evaluated. There is a need for a tool to assess and characterise BTP in children with non-cancer diagnoses and those who cannot self-report., (Copyright © 2021 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.)

Background: Oral morphine is frequently used for breakthrough pain but the oral route is not always available and absorption is slow. Transmucosal diamorphine is administered by buccal, sublingual or intranasal routes, and rapidly absorbed., Aim: To explore the perspectives of healthcare professionals in the UK caring for children with life-limiting conditions concerning the assessment and management of breakthrough pain; prescribing and administration of transmucosal diamorphine compared with oral morphine; and the feasibility of a comparative clinical trial., Design/ Participants: Three focus groups, analysed using a Framework approach. Doctors, nurses and pharmacists ( n  = 28), caring for children with life-limiting illnesses receiving palliative care, participated., Results: Oral morphine is frequently used for breakthrough pain across all settings; with transmucosal diamorphine largely limited to use in hospices or given by community nurses, predominantly buccally. Perceived advantages of oral morphine included confidence in its use with no requirement for specific training; disadvantages included tolerability issues, slow onset, unpredictable response and unsuitability for patients with gastrointestinal failure. Perceived advantages of transmucosal diamorphine were quick onset and easy administration; barriers included lack of licensed preparations and prescribing guidance with fears over accountability of prescribers, and potential issues with availability, preparation and palatability. Factors potentially affecting recruitment to a trial were patient suitability and onerousness for families, trial design and logistics, staff time and clinician engagement., Conclusions: There were perceived advantages to transmucosal diamorphine, but there is a need for access to a safe preparation. A clinical trial would be feasible provided barriers were overcome.