Sydney Children's Hospital Research Articles

Impacts of paediatric chronic pain on parents: A qualitative study.

Paediatric chronic pain adversely impacts the child's functioning, health-related quality of life and development. However, there is a need for a holistic assessment of parental impacts of caring for a child with chronic pain. This qualitative study aimed to investigate the possible psychosocial, functional and work impacts of caring for a child with chronic pain on parents, including any positive effects of the experience. Ten parents (eight mothers and two fathers), whose child attended the Sydney Children's Hospital Interdisciplinary Chronic and Complex Pain Clinic, participated in semi-structured interviews exploring the impact of caring for a child with chronic pain. Interviews were audio-recorded, transcribed, coded (with good inter-coder agreement) and analysed using thematic analysis. The parental experience of caring for a child with chronic pain was encapsulated by four overarching themes: (1) 'the constant and all-consuming nature of pain' - parents described the unpredictable, yet constant nature of chronic pain, contributing to wide-reaching impacts in various areas of their life; (2) 'dealing with uncertainty' - their experience was commonly characterized by a sense of uncertainty, stress, hopelessness and fear; (3) 'importance of support and self-care' - strong support networks and prioritizing self-care were crucial in alleviating the negative effects of paediatric chronic pain; and (4) 'a revitalized and optimistic view on life and relationships' - some parents identified unique and positive effects, such as stronger relationships, personal growth and a reformed view on life. This study provided rich data on the various impacts of caring for a child with chronic pain, highlighting the need for the development of holistic, family-centred interventions addressing both child and parental functioning.

Impact of integrated care coordination on pediatric asthma hospital presentations.

IntroductionFrequent asthma attacks in children result in unscheduled hospital presentations. Patient centered care coordination can reduce asthma hospital presentations. In 2016, The Sydney Children's Hospitals Network launched the Asthma Follow up Integrated Care Initiative with the aim to reduce pediatric asthma emergency department (ED) presentations by 50% through developing and testing an integrated model of care led by care coordinators (CCs).MethodsThe integrated model of care was developed by a multidisciplinary team at Sydney Children's Hospital Randwick (SCH,R) and implemented in two phases: Phase I and Phase II. Children aged 2–16 years who presented ≥4 times to the ED of the SCH,R in the preceding 12 months were enrolled in Phase I and those who had ≥4 ED presentations and ≥1 hospital admissions with asthma attack were enrolled in Phase II. Phase I included a suite of interventions delivered by CCs including encouraging parents/carers to schedule follow-up visits with GP post-discharge, ensuring parents/carers are provided with standard asthma resource pack, offering referrals to asthma education sessions, sending a letter to the child's GP advising of the child's recent hospital presentation and coordinating asthma education webinar for GPs. In addition, in Phase II CCs sent text messages to parents/carers reminding them to follow-up with the child's GP. We compared the change in ED visits and hospital admissions at baseline (6 months pre-enrolment) and at 6-and 12-months post-enrolment in the program.ResultsDuring December 2016-January 2021, 160 children (99 in Phase I and 61 in Phase II) were enrolled. Compared to baseline at 6- and 12-months post-enrolment, the proportion of children requiring ≥1 asthma ED presentations reduced by 43 and 61% in Phase I and 41 and 66% in Phase II. Similarly, the proportion of children requiring ≥1 asthma hospital admissions at 6- and 12-months post-enrolment reduced by 40 and 47% in Phase I and 62 and 69% in Phase II.ConclusionOur results support that care coordinator led integrated model of asthma care which enables integration of acute and primary care services and provides families with asthma resources and education can reduce asthma hospital presentations in children.

Multistate outbreak of melioidosis associated with imported aromatherapy spray

Journal of Paediatrics and Child HealthVolume 58, Issue 5 p. 933-934 Heads UpFree Access Multistate outbreak of melioidosis associated with imported aromatherapy spray First published: 26 March 2022 https://doi.org/10.1111/jpc.15956 edited by Craig Mellis (craig.mellis@sydney.edu.au) AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat In 2021, a cluster of four cases of acute melioidosis were reported in patients from Kansas, Texas, Minnesota and Georgia, with no history of travel to endemic areas.1 Two of the four patients, including a 5-year-old boy, died; the surviving patients have significant morbidity related to their illness. The isolates from each patient were noted to be clonal. After extensive investigation of potential exposure items, Burkholderia pseudomallei was isolated from Better Homes & Gardens Lavender & Chamomile Essential Oil Infused Aromatherapy Room Spray with Gemstones from the house of patient 4, an aromatherapy sold across 55 Walmart stores within the United States. Whole-genome sequencing indicated the isolate from the spray bottle was the same strain found in all four patients. This strain (named aromatherapy spray 2021, ATS2021) clustered with samples of Burkholderia pseudomallei from South Asia that are consistent with the origins of the spray (Fig. 1). These cases highlight the potential risk of melioidosis due to imported products from regions where B. pseudomallei is endemic and the importance of astute clinical suspicion when patients do not respond to first-line empiric antibiotic regimens. The CDC has urged consumers to return the aromatherapy bottles to prevent the potential for B. pseudomallei to become established in the United States.2 Fig. 1Open in figure viewerPowerPoint Phylogenomic Comparisons of B. pseudomallei Genomes with Isolates Obtained from the Four Patients and from a Contaminated Aromatherapy Spray Bottle. (a) Core genome phylogeny= the core single-nucleotide polymorphism phylogeny of 1696 genomes in which the new isolates were initially placed. Geographic origins were selected from the BioSample database of the National Center for Biotechnology Information, and the specific geographic regions listed were based on definitions from the 2021 World Factbook of the Central Intelligence Agency (https://www.cia.gov/the-world-factbook/. opens in new tab). Samples of unknown geographic origin are white. The red branch inside the dashed red square denotes the outbreak strains, surrounded by isolates predominantly from South Asia. (b) Maximum-likelihood phylogenetic tree= the high-resolution clade with the closest isolates to the outbreak strain from maximum-likelihood analysis in Parsnp (Harvest, version 1.3) rendered with the use of MEGA X software (https://www.megasoftware.net/. opens in new tab). ATS2021 = aromatherapy spray 2021; GA2021a = patient 4, Georgia; MN2021a = patient 3, Minnesota; GA2021_Spray_1A = aromatherapy spray from house of patient 4; TX2021a = patient 2, Texas; KS2021a = patient 1, Kansas. References 1Gee JE, Bower WA, Kunkel A et al. Multistate outbreak of Melioidosis associated with imported aromatherapy spray. NEJM 2022; 386: 861– 8. 2 US Centers for Disease Control and Prevention. 2021. CDC lab testing confirms cause of melioidosis outbreak. Available from: https://www.cdc.gov/media/releases/2021/p1026-melioidosis-outbreak.html [accessed 6 March 2022]. Reviewers: Robert C Duguid, robert.duguid@health.nsw.gov.au, Sydney Children's Hospital, Sydney, NSW, Australia; Phoebe CM Williams, phoebe.williams1@health.nsw.gov.au, Sydney Children's Hospital, Sydney, NSW, Australia Volume58, Issue5May 2022Pages 933-934 FiguresReferencesRelatedInformation

Paediatric dog bite injuries: a 10-year retrospective cohort analysis from Sydney Children's Hospital.

Dog bite injuries are largely preventable yet present the most common animal related cause of hospitalisation. This study examines the demographics and clinical cost of patients with dog bite related injuries who presented to Sydney Children's Hospital (SCH) from 2010 to 2020. The results from our study will be used to raise awareness regarding the impact of dog bite injuries in our community. Data was obtained from the SCH database using ICD-10-AM code W54.0, which captures all patients presenting to SCH with dog bite injuries from 2010 to 2020. A chart review was then performed to retrieve demographic data for analysis. Data analysis was performed using SAS® software version 9.4 and cost for each patient retrieved from the SCH clinical costing department. A total of 628 patients presented to SCH with dog bites during the study period. 273 (43.5%) patients received treatment in ED only with the remaining 355 (56.5%) patients admitted for treatment. The average age was 5.69 years old. There were 321 males (51.1%) and 307 females (48.9%). Facial and other head & neck injuries were most common (64.4%). Pitbull, Labrador and Rottweiler were the most commonly documented offending breeds (25%) with the family dog most likely to offend (49%). The mean clinical cost for per dog bite injury was $2968. As part of the largest single centre study exploring dog bite injuries, we expect that this study will stimulate potential public health campaigns targeted at educating parents and children on interacting with dogs to minimise these injuries.

Onasemnogene abeparvovec in spinal muscular atrophy: an Australian experience of safety and efficacy.

ObjectiveTo provide a greater understanding of the tolerability, safety and clinical outcomes of onasemnogene abeparvovec in real‐world practice, in a broad population of infants with spinal muscular atrophy (SMA).MethodsA prospective cohort study of children with SMA treated with onasemnogene abeparvovec at Sydney Children's Hospital Network, Australia was conducted from August 2019 to November 2021. Safety outcomes included clinical and laboratory evaluations. Efficacy assessments included World Health Organisation (WHO) motor milestones, oral and swallowing abilities, and requirements for respiratory support. The implementation of a model of care for onasemnogene abeparvovec administration in health practice is described.Results21 children were treated (age range, 0.65–24 months; body weight range, 2.5–12.5 kg) and 19/21 (90.4%) had previous nusinersen. Transient treatment‐related side effects occurred in all children; vomiting (100%), transaminitis (57%) and thrombocytopaenia (33%). Incidence of moderate/severe transaminitis was significantly greater in infants weighing ≥8 kg compared with <8 kg (p < 0.05). Duration of prednisolone following treatment was prolonged (mean 87.5 days, range 57–274 days). 16/21 (76%) children gained at least one WHO motor milestone. Stabilisation or improvement in bulbar or respiratory function was observed in 20/21 (95.2%) patients. Implementation challenges were mitigated by developing standard operating procedures and facilitating exchange of knowledge.InterpretationThis study provides real‐world evidence to inform treatment decisions and guide therapeutic expectations for onasemnogene abeparvovec and combination therapy for SMA in health practice, especially for children weighing ≥8 kg receiving higher vector loads. Proactive clinical and laboratory surveillance is essential to facilitate individualised management of risks.

Factors influencing reintroduction of peanut and tree nuts after negative oral food challenges in children

BackgroundCompliance with reintroduction of foods after a negative oral food challenge (OFC) is variable. Ongoing avoidance of tolerated foods is associated with recurrence of allergy and a reduced quality of life. ObjectiveTo determine the proportion of children who reintroduced peanut or tree nuts after a negative OFC and to describe factors that influenced decisions regarding reintroduction or avoidance of nonallergic (negative) nuts. MethodsFamilies of children that had undergone an OFC for peanut or tree nuts at Sydney Children's Hospital were invited to participate. Consenting families were sent an online questionnaire. ResultsThe response rate to the questionnaire was 64%. More than 85% of respondents had introduced all or some of the negative nuts after the OFC and most had maintained at least some regular exposure in the child's diet at the time of the study. The age at diagnosis of the nut allergy and an awareness of the benefit of introducing foods after a negative OFC were significantly (P < .05) associated with introducing negative nuts. There was improved quality of life in those that introduced negative nuts. ConclusionMost families introduced or attempted to introduce negative nuts after a negative OFC. Educating families on the benefits of introducing foods after a negative OFC result is an important factor contributing to successful reintroduction.

The National Paediatric Medicines Forum: Who we are and what are we doing.

An increasing understanding of the pathophysiology of complex diseases has contributed to the explosion in availability of newer medications in recent years. Newer medications frequently have more specific mechanisms of action and improved clinical outcomes for their approved indications compared to standard care. This translation of research into practice is exciting because it reflects precision medicine principles and offers renewed hope for patients. Unfortunately, clinical decision-making is not always straight forward. Data are extremely limited in some patient groups, including those with rare conditions, or others not routinely included in clinical trials; paediatrics commonly feature in both scenarios.1 Newer medications are usually markedly more expensive than comparators. These factors complicate clinical decision-making in an individual patient. The Drug and Therapeutics Committees (DTCs) in each hospital are responsible for achieving effective medicines governance, equity of medicines access and quality use of medicines to underpin patient's therapy. The assessment of medication treatments has become an increasingly onerous workload for DTCs, reflecting the increase in newer medications often used for novel indications. In the context of limited high-quality evidence, there is substantial duplication of effort across tertiary paediatric institutions, with multiple paediatric DTCs considering similar applications with variability in processes and outcomes between DTCs.2 Furthermore, resources and training may be insufficient given the technical complexity of some submissions.2 In 2016, the Sydney Children's Hospitals Network (SCHN) proposed at the Children's Healthcare Australasia (CHA) Children's Hospitals CEOs Forum that a national approach for paediatric medication assessment should be developed. The National Paediatric Medicines Forum (NPMF) was established to identify shared issues and to develop and disseminate recommendations, including guidance for evaluating the clinical- and cost-effectiveness of high-cost medications. Through sharing experiences and skills, NPMF aimed to optimise consistency and quality in decision-making and equity of access. Tertiary paediatric services nominated members, including medical specialists and hospital pharmacists with expertise in clinical pharmacology, health economics, quality use of medicines and/or clinical ethics. Medications for evaluation were chosen via horizon scanning issues identified by regional DTCs, including high cost and/or novel drugs with limited evidence in paediatric patients. Since June 2019, 11 meetings with 16 members have been held by videoconference. Amongst a range of topics discussed, 10 drugs/classes have been reviewed in detail including vedolizumab, ranibizumab, burosumab, trametinib, canakinumab, eltrombopag, romiplostim, palivizumab, Glucagon-like peptide 1 (GLP-1) analogues and melatonin. Here, ‘medication summaries’ were developed, following a survey of their usage in paediatrics across Australia (including indications and local restrictions), systematic review of the literature and economic analysis modelling for financial implications. Consensus recommendations for the indications, dosage and endpoints for monitoring the effect were proposed and discussed. Guests with subspecialist expertise contributed as required, including the fields of neonatology, ophthalmology, gastroenterology and oncology. Feedback was also sought from other subspecialists utilising professional networks. The interplay and challenges with Medicines Access Programs for innovative therapies in paediatrics were explored.3 The purpose of the medication summaries is to support both clinician and DTC decision-making in individual patients, contributing to the stated goals of the NPMF. Plain language summaries have been developed for some medications to engage patients and parents with the findings. Looking forward, the NPMF will update medication summaries as new information is available and develop summaries for other drugs. We will increase NPMF membership and clinician input. Medication summaries will be submitted for publication in peer-reviewed journals to invite further discussions, and other methods to broaden their dissemination will be explored. Research to measure the impact of the medication summaries on decision-making in practice is under development. A systematic approach to multisite data collection is anticipated to provide valuable clinical information on trends, usage and outcomes from the drugs, further contributing to the published literature; collaborations with clinical researchers will be invaluable. This collective clinical experience also offers an opportunity to be a voice of paediatric advocacy, including for submissions to the Pharmaceutical Benefits Advisory Committee (PBAC). We hope that these collective activities will make significant improvements in outcomes for paediatric patients.

Lived experiences of pain in children and young people with cerebral palsy.

To explore the lived experiences of pain in children and young people with cerebral palsy (CP). Participants were recruited from the Sydney Children's Hospitals Network and the New South Wales/Australian Capital Territory CP Registers. Inclusion criteria were as follows: CP; aged 9 to 17 years; current/past experience of pain; fluent in English; no greater than mild intellectual disability. Purposive sampling ensured representation across age, motor subtypes, and Gross Motor Function Classification System (GMFCS) levels. Semi-structured face-to-face interviews were conducted. Data were analysed following an interpretative phenomenological approach. Ten participants (three male) were included (mean age 14y 5mo, SD 2y), GMFCS levels I (n=4), II (n=3), III (n=2), and IV (n=1). Analysis led to three superordinate themes: (1) Everybody's experience of pain is different; (2) When the pain is winning; (3) 'I know how to deal with it'. Pain contributors and locations varied between children. Pain intruded on school, physical activity, and psychosocial functioning. Children described personalized strategies used to deal with pain. In this study, children self-reported highly individualized pain experiences which interfered with their daily life and psychosocial well-being. There is a need for improvement in pain assessment and a personalized approach to pain management.

The COVID-19 System Shock Framework: Capturing Health System Innovation During the COVID-19 Pandemic.

Coronavirus disease 2019 (COVID-19) has resulted in over 2 million deaths globally. The experience in Australia presents an opportunity to study contrasting responses to the COVID-19 health system shock. We adapted the Hanefeld et al framework for health systems shocks to create the COVID-19 System Shock Framework (CSSF). This framework enabled us to assess innovations and changes created through COVID-19 at the Sydney Children's Hospitals Network (SCHN), the largest provider of children's health services in the Southern hemisphere. We used ethnographic methods, guided by the CSSF, to map innovations and initiatives implemented across SCHN during the pandemic. An embedded field researcher shadowed members of the emergency operations centre (EOC) for nine months. We also reviewed clinic and policy documents pertinent to SCHN's response to COVID-19 and conducted interviews and focus groups with stakeholders, including clinical directors, project managers, frontline clinicians, and other personnel involved in implementing innovations across SCHN. The CSSF captured SCHN's complex response to the pandemic. Responses included a COVID-19 assessment clinic, inpatient and infectious disease management services, redeploying and managing a workforce working from home, cohesive communication initiatives, and remote delivery of care, all enabled by a dedicated COVID-19 fund. The health system values that shaped SCHN's response to the pandemic included principles of equity of healthcare delivery, holistic and integrated models of care, and supporting workforce wellbeing. SCHN's resilience was enabled by innovation fostered through a non-hierarchical governance structure and responsiveness to emerging challenges balanced with a singular vision. Using the CSSF, we found that SCHN's ability to innovate was key to ensuring its resilience during the pandemic.

CM-AVM syndrome - A prospective observational study of unrelated paediatric cases.

The main objective of this study is to describe the clinical spectrum of CM-AVM syndrome as well as radiological and genetic findings. This is a single-centre prospective observational study performed at Sydney Children's Hospital. Patients under the age of 18years that presented to our paediatric dermatology clinic or vascular birthmark clinic between January 2015 and September 2020 with one or more geometric shaped pink/ red/ brown macule with a peripheral pallor characteristic of a high-flow vascular stain were included. Children subsequently diagnosed with other diagnosis or family members with CM-AVM syndrome were excluded. Sixty children were included, with two subsequently excluded. A third of patients (n=22, 38%) presented with a single characteristic HFVS, whereas the remaining two thirds (n=36; 62%) had multiple HFVS. In children with multiple HFVS, one notably larger HFVS was detected in the majority of children (n=32, 88%). In 33 patients, a brain and spine MRI was performed, which detected a spine AVM in one symptomatic patient with sensorimotor deficits. No cerebral AVM or AVF was picked up in the cohort. A RASA 1 result was available for evaluation in 24, of which 16 (67%) were positive. An EPHB4 result was available in eight, two (25%) of which were positive. One large HFVS often accompanied by multiple small HFVS can be seen in most patients. Despite of the lack of genetic confirmation of diagnosis in single lesions, this phenotype might be of interest and warrants further investigation.

"We needed this": perspectives of parents and healthcare professionals involved in a pilot newborn screening program for spinal muscular atrophy.

BackgroundNewborn screening (NBS) for spinal muscular atrophy (SMA) is a recognised model through which health outcomes can be improved. However, perspectives of parents and healthcare professionals (HCPs) involved in such programs are largely unknown.MethodsA pilot program for SMA ran from August 2018-July 2020. Using a mixed-methods convergent methodology, we used a self-administered questionnaire to understand parents’ perceptions and psychological impact of the program from diagnosis to treatment. We thematically analysed successes/challenges encountered by HCPs and recommendations for service improvement from both participant groups.Findings202,388 infants were screened for SMA and the perceptions of 44 parents and HCPs affected by a positive result in eighteen newborns was ascertained. Parents (n=29, 100%) were satisfied with NBS for SMA. Although screen-positive result was distressing for all parents, quality of life improved over time [CarerQoL-7D baseline median score 4 (SD=1.4) vs six-month median score 8 (SD=1.3), p<0.001)]. Challenges for HCPs included managing the time-critical nature of the pathway whilst remaining cognisant of limitations associated with the predictive screening test.InterpretationInterpretation: NBS for SMA fulfils criteria for population-wide screening. Net benefits are acknowledged by stakeholders to optimise lifelong outcomes. Harms including psychological distress associated with a screen-positive result may be managed by targeted psychosocial support, information provision and a personalised model of care together strengthening healthcare systems.FundingThe NSW Pilot NBS study was funded by Luminesce Alliance. Dr Kariyawasam received funding from the RTP Scholarship, University of New South Wales and The Freedman Family Foundation Scholarship, Sydney Children's Hospital Foundation.

Motivation for change in the health care of children with developmental disabilities: Pilot continuing professional development-quality improvement project.

People with developmental disabilities (DDs) experience significant barriers accessing and receiving optimal health care resulting in poorer health-care outcomes. Continuing professional development (CPD) represents an effective means to alter health-care staff behaviour to improve the care of people with DDs. However, given the scepticism regarding the effectiveness of certain CPD models' ability to alter learner's workplace behaviour, the current pilot study developed and determined the feasibility of a novel CPD programme aimed at improving the health care provided to children with DDs. Motivation for Change (MFC) is a novel CPD programme based on empirically based behaviour and educational strategies including motivational interviewing, flipped classroom and process mapping. It utilises input of patients, practitioners, and family members during administration of the programme. MFC was administered with 14 staff members in a Sydney Children's Hospital Sleep service. After MFC engagement, staff reported significant improvements in their knowledge of behavioural characteristics of children with DDs, the difficulties they face, how best to support them within the learner's work setting and confidence in working with children with DDs. There was a non-significant decline in their reported need for further training and expressed high level of satisfaction with the MFC programme. MFC represents a feasible means of providing CPD to health-care staff but further research is needed to determine objective clinical behavioural change. Evaluation of the impact on patient health outcomes, parent/child satisfaction, staff sustainability and overall system functioning is also needed. It may represent an effective model of CPD for other targets of health-care improvement.

The COVID-19 pandemic: Distance-delivered care for childhood cancer survivors.

The COVID-19 pandemic: Distance-delivered care for childhood cancer survivors.

Use of Onlay Hydroxyapatite Cement for Secondary Cranioplasty.

Children who undergo bi-fronto-orbital advancement (BFOA) frequently develop a contour deformity on the temporal and supra-orbital region, with an incidence reported as high as 55% and 75%, respectively. Up to 20% of patients may require correction. Hydroxyapatite cement (HAC) is a good alternative to autogenous tissue. The available literature on its use focusses on the reconstruction of bone defects, but little has been published on its efficacy and safety as an onlay graft over intact cranium. To describe our institution's experience with HAC in the pediatric population. Retrospective chart review from 1998 to 2018 on all patients from the Craniofacial Unit at the Sydney Children's Hospital who had either coronal or metopic craniosynostosis and underwent BFOA and later in life required cranioplasty with HAC for contour repair. We have performed 166 BFOA and nineteen secondary cranioplasties for contour repair using onlay HAC. The mean age at the time of operation was 14 years. Bi-coronal craniosynostosis was most frequently associated with secondary cranioplasty and 37% had an associated syndrome. The mean volume of HAC used was 37 mL. There was only 1 patient who had a complication (5.3%) and required partial removal of allograft. The mean length of admission was 2 days. Mean follow up time of 22.4 months. HAC represents a safe option when used correctly, with low rates of complication and satisfactory cosmetic outcomes.

Does Combined Multichannel Intraluminal Impedance and pH (MII-pH) Testing Improve Clinical Outcomes in Children With Gastroesophageal Reflux Disease?

The aim of the study was to investigate the role of combined multichannel intraluminal impedance and pH (MII-pH) testing in clinical management of children with gastroesophageal reflux disease (GERD) by exploring the impact of treatment changes made based on MII-pH testing results on symptoms and quality of life outcomes. All patients (<18 years) referred to the Sydney Children's Hospital for MII-pH testing were recruited. Patients were classified by acid suppression therapy (AST) status (on AST and off AST) and changes in medical and surgical management were evaluated. Validated questionnaires (Pediatric Gastroesophageal Symptom and Quality of Life Questionnaire and Infant Gastroesophageal Reflux Questionnaire Revised) were administered at baseline at the time of MII-pH testing, and 4 weeks after treatment changes were made and questionnaire scores were compared. Of the 45 patients recruited, 24 patients (53.3%) were off AST and 21 patients (46.7%) were on AST. MII-pH testing led to medication changes in 30 patients (66.7%). This included 15 of 24 (62.5%) in those off AST and 15 of 21 (71.4%) in those on AST. More than 98% of patients who had treatment changes showed a significant improvement in both symptoms and quality of life scores. Our study is one of the first pediatric studies to evaluate the clinical validity of MII-pH testing in the pediatric population referred for suspected GERD, and its ability in guiding clinical management. Our study has shown that treatment decisions guided by and based on results of MII-pH testing led to a significant improvement in symptoms and quality of life in infants and children with GERD.

The utility of multichannel intraluminal impedance-pH testing in tailoring the management of paediatric gastro-oesophageal reflux disease.

To determine whether combined multichannel intraluminal impedance and pH (MII-pH) testing led to a change in management of children with gastro-oesophageal reflux disease (GORD). Retrospective chart review was done in all patients who underwent MII-pH testing for GORD symptoms at Sydney Children's Hospital between 2008 and 2016. Changes to anti-reflux medications and referral for anti-reflux surgery were evaluated. There were 365 patients, 260 (71.2%) of whom were on acid-suppressing therapy. The median age was 4.1±4.8years, 205 patients (56%) were males, 83 (22.7%) were infants (<1year of age) and 145 (39.7%%) had comorbid conditions. We found 72.1% had abnormal MII-pH results, of which 17.5% had abnormal acid reflux, 8.2% had abnormal number of retrograde bolus movements and 46.3% had hypersensitive oesophagus (positive symptom association only). Infants were significantly more likely to have abnormal MII-pH results compared to older children (P=.04). Results of MII-pH testing led to medication changes in 44.7% and referral for anti-reflux surgery in 6.8% of patients. Combined multichannel intraluminal impedance and pH testing is clinically useful in the management of children with symptoms of GORD and over half the patients had changes to their medical treatment or referral for anti-reflux surgery based on the results of MII-pH testing. It resulted in a treatment change in an additional 32% of patients over traditional pH-metry.

Digital psychosocial assessment: An efficient and effective screening tool.

A large proportion of young people with chronic health conditions are surviving into adulthood. They face the same challenges as their healthy peers and are at increased risk of mental health problems. Psychosocial assessment is a crucial aspect of clinical care. Interviews using the internationally used and accepted HEEADSSS (home environment, education/employment, eating, peer-related activities, drugs, sexuality, suicide/depression, and safety) framework require trained clinicians, rapid interpersonal engagement, time and manual documentation. HEEADSSS-derived digital self-report surveys can be initiated by non-trained staff. This study compares the utility and information recorded using both methods. A retrospective analysis comparing documentation from HEEADSSS guided face-to-face interview and a digital survey tool was conducted using 146 records collected by the Trapeze transition service across the two locations of the Sydney Children's Hospital Network (NSW, Australia) between 2013 and 2016. A panel of four experts used an iterative process to identify 29 data verification points, falling into seven categories. Wilcoxon signed-rank tests were used to compare category scores. The digital survey took an average of 15 min and showed a significantly higher rate of disclosure across all psychosocial categories, particularly in the sensitive areas of emotions, drug use, sex and safety, compared to electronic medical record documentation of interview. Digital survey provided a time-efficient psychosocial screening tool that was self-administered, able to be introduced by non-trained staff, had a consistent record of responses, and elicited a substantially higher disclosure rate for important areas of strength and risk that may otherwise be avoided or not recorded.

DOZ047.19: Outcomes in the management of long-gap esophageal atresia: is the Foker technique superior?

Abstract Background Long-gap esophageal atresia (LGEA) has traditionally been managed by delayed anastamosis. However, over the last 10 years the Foker technique of esophageal growth elongation has been used. There is limited data evaluating outcomes using both techniques. Aims The aim of this study was to compare the outcomes in LGEA repair using the traditional delayed repair technique versus the Foker technique. Methods A retrospective chart review was done of LGEA patients at Sydney Children's Hospital between 1997 and 2016, comparing the Foker technique with the delayed repair technique. Results There were 9 children repaired using the Foker technique, and 10 by delayed repair. There were 7 patients with Type A, 1 with Type B, and 1 with Type C in the Foker group and in the delayed repair cohort, 5 were Type A, 2 Type B, 2 Type C, and 1 Type D esophageal atresia/tracheoesophageal fistula. There were 4 males in the Foker group and 5 in the non-Foker group. Median time to join was 59 days (15–117 days) for Foker repair and 173 days (16–433 days) for delayed repair. There was no significant difference in the incidence of post-operative leak, incidence of strictures needing dilation, presence of reflux symptoms, presence of reflux esophagitis on endoscopy, proton pump inhibitor use, need for fundoplication, incidence of cyanotic spells, occurrence of recurrent fistula, and symptoms of dysphagia when comparing both groups. Data on time to oral feeds and incidence of malnutrition was incomplete. Details are shown in Table 1. Conclusions Although the time to anastamosis was shorter in the Foker group and the incidence of post-operative leak, strictures needing dilation, need for fundoplication, and dysphagia were lower in the Foker group, the difference was not significant probably secondary to the small sample size. These results require validation in larger cohorts.

DOZ047.04: Upper gastrointestinal microbiome in esophageal atresia

Abstract Background Esophageal atresia (EA) is a rare defect, resulting in the condition being understudied. Specifically, information on the upper gastrointestinal (GI) microbiome of EA patients is lacking. Aims This study aimed to examine the composition of the upper GI (oral, esophageal, and gastric fluid) microbiomes of EA patients as compared to controls. Methods A pediatric cohort was prospectively recruited at Sydney Children's Hospital in 2018, and comprised children with a history of EA and those without EA. The groups were subdivided into children with GERD, eosinophilic esophagitis (EoE), and those with no disease. Biopsies, saliva, blood, and both tracheal and gastric aspirates were collected. Histology was reviewed for changes related to reflux and peak eosinophil count in subjects with EoE. Ethics approval has been granted and informed consent obtained from all patients and/or their guardians. The bacterial component (16S rRNA gene) of the microbiome was amplified using the 515F–806R primers and sequenced with Illumina MiSeq 2 × 250 bp chemistry. Reads were analyzed using mothur and R. Results Within the cohort (n = 47), 29 (61.7%) had EA and 18 (39.3%) were non-EA patients. The mean age of the whole cohort was 8.36 years (SD 5.05) and 24 (44%) were male. Non-EA patients were having endoscopic assessment of GERD, EoE, suspected celiac or inflammatory bowel disease (IBD). Active GERD, defined as histological changes on biopsy, an abnormal acid reflux index, or retrograde bolus movement on pH/impedance testing, was present in 11 (23.4%; 5 with EA) children. The mean eosinophil count in patients with EoE was 11.1 (SD 14.74) in the EA cohort and 15.9 (SD 21) in the non-EA cohort. Proton pump inhibitors were used by 29 (61.7%; 16 with EA). EoE was present in 18 (44.4%; 8 with EA). Swallowed steroids were used in 13 (27.6%) patients with EoE. Microbiome changes were observed in patients with EA as compared to controls. Conclusions This study is the first to examine the upper GI microbiome of children with EA. Changes in the upper GI microbiomes of children with EA require validation in larger cohorts.

DOZ047.18: Diagnosing reflux aspiration in esophageal atresia: role of microbiome and pepsin

Abstract Background Children with esophageal atresia (EA) often suffer from gastroesophageal reflux disease (GERD) and it's complications, including reflux aspiration. Reflux aspiration can be difficult to diagnose in these patients who often have other causes for their respiratory symptoms. Tracheal pepsin has been studied as a marker of reflux aspiration in other conditions but not in EA. The role of the airway microbiome in the diagnosis of reflux aspiration has not been studied previously. Aims The aim of this study was to examine the composition of the airway (tracheal aspirate) microbiome of EA patients and correlate the results of the microbial analysis with the presence of gastric pepsin in the airways as a marker of reflux aspiration. Methods A cohort of children with EA was prospectively recruited at Sydney Children's Hospital in 2018. The EA group were subdivided into children with GERD, eosinophilic esophagitis (EoE), and those with no disease. Tracheal aspirates were collected. The bacterial component (16S rRNA gene) of the microbiome was amplified. Pepsin was assayed using a monoclonal antibody specific for pepsin A by the ELISA technique. Results There were 26 EA patients. The mean age was 6.96 years (0.7–18) and 15 (58%) were female. Eighteen (69%) children were on proton pump inhibitors for GERD and 8 (30%) were on swallowed steroids for EoE. GERD, defined as reflux esophagitis on biopsy and/or abnormal result on pH/impedance testing, was present in 7 (27%) and EoE defined as &gt;15 eosinophils/HPF was seen in 5 (19%) children. Tracheal pepsin was detected in a significant proportion of EA patients and correlation between the pepsin results and the airway microbial analysis was seen in some of the EA patients who were pepsin positive. Conclusions This study is the first to examine the role of the microbiome and pepsin in tracheal aspirates as markers of reflux aspiration in children with EA. These results require validation in larger cohorts.