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Background: A mechanical insufflation-exsufflation (MI-E) device is a commonly used tool for airway clearance in children with an ineffective cough. Whereas the device has been shown to have multiple benefits, limited evidence exists regarding parents' experiences with its home use in the Canadian context. This study's objective was to explore the perspectives and experiences of parents who receive service through the IWK Health Centre and use an MI-E device at home with their child., Methods: The study used an interpretive description design. Semi-structured interviews, conducted with 9 participants, were audio recorded and transcribed verbatim. Transcripts were analyzed using a reflective thematic process., Results: Seven mothers and 2 fathers were interviewed. Following analysis, 3 themes were identified: (1) Learning about the MI-E device described participants' journey from becoming aware of the device to acquiring knowledge and skills about its use; (2) using the device detailed the integral role the MI-E device played in their lives, including decisions around use, and parental role; and (3) changing lives outlined the physical, emotional, and social benefits the device provided to the child and their family., Conclusions: Participants provided detailed descriptions of their journey from learning to integrating the MI-E device into their child's daily routine and family life. Its multiple associated benefits improved the child's and their family's quality of life. However, better education on its use was highlighted as a need for both parents and the health care professionals who work with them., (Copyright © 2024 by Daedalus Enterprises.)

Existing barriers to care were exacerbated by the development and implementation of necessary public health restrictions during the COVID-19 pandemic. Children with complex care needs and their families represent a small portion of the paediatric population, and yet they require disproportionately high access to services. Little is known about the impact of COVID-19 public health measures on this population. This study will generate evidence to uncover the wider impact of COVID-19 measures on the lives of children with complex care needs and their families in relation to policy and service changes. This multi-site sequential mixed methods study will take place across the Canadian Maritime provinces and use an integrated knowledge translation approach. There are two phases to this study: 1) map COVID-19 public health restrictions and service changes impacting children with complex care needs by conducting an environmental scan of public health restrictions and service changes between March 2020 and March 2022 and interviewing key informants involved in the development or implementation of restrictions and service changes, and 2) explore how children with complex care needs and their families experienced public health restrictions and service changes to understand how their health and well-being were impacted., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Curran et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Spinal muscular atrophy (SMA), caused primarily by deletions in SMN1, leads to progressive loss of lower motor neurons. Newborn screening for SMA is under consideration for the Maritime Newborn Screening Program. The incidence of this disease has not been explored in Maritime Canada which includes the provinces of Nova Scotia (NS), New Brunswick (NB), and Prince Edward Island (PEI). In this retrospective chart review, patients were identified from the IWK Clinical Genomics Lab and Maritime Medical Genetics Service databases for SMN1 genetic testing between 2000 and 2020. The incidence of SMA in Maritime Canada was 1:11,900. Among patients born between 2000 and 2020, NB and PEI had lower proportions of type 1 SMA (12% and 0%, respectively) when compared to NS (50%). The majority of type 1 patients had 2 copies of SMN2, the majority of type 2 patients had 3 copies, and the majority of type 3 patients had 4 copies. There was a delay to molecular diagnosis for all subtypes, longest in type 3. This study provides the best available SMA epidemiology in Maritime Canada and expands our understanding of the pattern of disease severity relative to SMN2 copy number in this region., (© 2023 Wiley Periodicals LLC.)

Background: Power wheelchairs can empower children with physical limitations to gain independence in their everyday lives; however, traditional methods of power wheelchair training are often limited by poor accessibility and safety concerns. Immersive virtual reality technology (IVRT) uses advanced display technology to place users in a fully immersive web-based environment that can support real-time skills training, often requiring less resources and fewer safety concerns than real-world methods. IVRT interventions have shown to be a feasible training option among adult power wheelchair users; however, there is still a need to understand the technical and clinical feasibility of developing an IVRT power wheelchair training tool for the pediatric population., Objective: This proposed study aims to use expert feedback and an iterative design process to develop an IVRT training intervention for pediatric power wheelchair skill development., Methods: This 3-phase feasibility study will be conducted within the assistive technology unit of a public pediatric hospital. Separate participant groups will be recruited for each phase, consisting of approximately 10 to 15 clinicians (phase 1), 10 pediatric power wheelchair users (phase 2), and 15 to 20 additional pediatric power wheelchair users (phase 3). Phase 1 will be conducted to gather feedback on the baseline IVRT training intervention. Clinicians will test the intervention and assess its usability and acceptability using qualitative and quantitative methods. Phase 1 participants will also be invited back for a subsequent session to reassess a revised version of the training intervention that has been updated based on their previous feedback. Phase 2 and phase 3 will also use mixed methods to gather feedback on the usability, acceptability, and user experience of the IVRT training intervention from current pediatric power wheelchair users. In addition, phase 3 participants will perform a skills transfer assessment to compare power mobility skill performance between the virtual reality and real-life environments. Data gathered in phase 2 will be used to further refine the IVRT intervention, whereas phase 3 data will be used to statistically evaluate the final version., Results: This study was approved by the Izaak Walton Killam Health Centre research ethics board in August 2021. Phase 1 testing began in February 2022. The entire study is expected to be completed by 2023., Conclusions: The results of this study will be used to create an IVRT training intervention for pediatric power wheelchair skill development through an iterative and collaborative design process. Results may also assist in directing future studies in this area., International Registered Report Identifier (irrid): DERR1-10.2196/39140., (©Sara Drisdelle, Liam Power, Scott Thieu, Jordan Sheriko. Originally published in JMIR Research Protocols (https://www.researchprotocols.org), 06.10.2022.)

Introduction: Children with medical complexity and their families are an important population of interest within the Canadian healthcare system. Despite representing less than 1% of the paediatric population, children with medical complexity require extensive care and account for one third of paediatric healthcare expenditures. Opportunities to conduct research to assess disparities in care and appropriate allocation of health resources relies on the ability to accurately identify this heterogeneous group of children. This study aims to better understand the population of children with medical complexity in the Canadian Maritimes, including Nova Scotia (NS), New Brunswick (NB) and Prince Edward Island (PEI). This will be achieved through three objectives: (1) Evaluate the performance of three algorithms to identify children with medical complexity in the Canadian Maritimes in administrative data; then using the 'best fit' algorithm (2) Estimate the prevalence of children with medical complexity in the Canadian Maritimes from 2003 to 2017 and (3) Describe patterns of healthcare utilisation for this cohort of children across the Canadian Maritimes., Methods and Analysis: The research will be conducted in three phases. In Phase 1, an expert panel will codevelop a gold-standard definition of paediatric medical complexity relevant to the Canadian Maritime population. A two-gate validation process will then be conducted using NS data and the gold-standard definition to determine the 'best fit' algorithm. During phase 2 the 'best fit' algorithm will be applied to estimate the prevalence of children with medical complexity in NS, NB and PEI. Finally, in phase 3 will describe patterns of healthcare utilisation across the Canadian Maritimes., Ethics and Dissemination: Ethics approval for this protocol was granted by the institutional research ethics board at the IWK Health Centre (REB # 1026245). A waiver of consent was approved. This study will use an integrated knowledge translation approach, where end users are involved in each stage of the project, which could increase uptake of the research into policy and practice. The findings of this research study will be submitted for publication and dissemination through conference presentations and with our end users., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Children with intellectual disabilities (IDs) can have complex health conditions that require intense and ongoing care management by multiple healthcare professionals (HCPs). Families often experience frustrations and challenges sharing necessary information about their children's unique emotional and communicative needs with HCPs. In turn, these needs are often poorly documented and shared with other HCPs. This contributes to compromised care and frustrations for families and HCPs. We conducted a qualitative study using focus groups to examine how 10 parents and 3 HCPs experienced provision of care for children with ID, as well as their suggestions for developing a one-page personal health profile (PHP) to improve communication. Parents suggested including behavioural descriptors rather than diagnoses. All participants believed a one-page PHP that was child and parent led would be very helpful and would improve communication between HCPs, parents and children leading to effective and supportive care.

Pain is a significant health concern for children living with cerebral palsy (CP). There are no population-level or large-scale multi-national datasets using common measures characterizing pain experience and interference (ie, pain burden) and management practices for children with CP. The aim of the CPPain survey is to generate a comprehensive understanding of pain burden and current management of pain to change clinical practice in CP. The CPPain survey is a comprehensive cross-sectional study. Researchers plan to recruit approximately 1400 children with CP (primary participants) across several countries over 6-12 months using multimodal recruitment strategies. Data will be collected from parents or guardians of children with CP (0-17 years) and from children with CP (8-17 years) who are able to self-report. Siblings (12-17 years) will be invited to participate as controls. The CPPain survey consists of previously validated and study-specific questionnaires addressing demographic and diagnostic information, pain experience, pain management, pain interference, pain coping, activity and participation in everyday life, nutritional status, mental health, health-related quality of life, and the effect of the COVID-19 pandemic on pain and access to pain care. The survey will be distributed primarily online. Data will be analyzed using appropriate statistical methods for comparing groups. Stratification will be used to investigate subgroups, and analyses will be adjusted for appropriate sociodemographic variables. The Norwegian Regional Committee for Medical and Health Research Ethics and the Research Ethics Board at the University of Minnesota in USA have approved the study. Ethics approval in Canada, Sweden, and Finland is pending. In addition to dissemination through peer-reviewed journals and conferences, findings will be communicated through the CPPain Web site (www.sthf.no/cppain), Web sites directed toward users or clinicians, social media, special interest groups, stakeholder engagement activities, articles in user organization journals, and presentations in public media., (© 2021 The Authors. Paediatric and Neonatal Pain published by John Wiley & Sons Ltd.)

Background: Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population., Methods: The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials., Results: The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner., Conclusion: Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.