Nancy L. Young

Background: Children with transfusion‐dependent thalassemia (TDT), also known as thalassemia major, require monthly blood transfusions and regular iron chelation therapy for long‐term survival. While the burden of caring for these children is recognized, little has been done to address the parents’ experience. Procedure: A 38‐item parent self‐report was included in the previously designed disease‐specific tool, the transfusion‐dependent quality‐of‐life questionnaire (TranQol), to identify the issues they may be facing. We wanted to ensure that the self‐report was reliable and valid. Questionnaires were given to parents during their children's routine clinic visits as part of a multi‐center North American study of the TranQol. Construct validity was assessed using Pearson's correlation with the Pediatric Quality of Life (PedsQL) Family Impact Module (FIM) (hypothesis: correlation of 0.4–0.6). Results: Sixty‐seven parents completed both the TranQol and PedsQL Family Impact Module questionnaires at baseline. There was substantial correlation ( r = 0.76) between TranQol parent self‐report scores (median 63, interquartile range [IQR]: 53–76) and PedsQL Family Impact Module scores (median 77, IQR: 59–89). Conclusions: The median PedsQL FIM score was similar to previously reported scores for parents of children with sickle cell disease. The TranQol parental burden tool demonstrated construct validity was internally reliable (Cronbach's α = 0.94) and had acceptable test–retest reliability (intraclass correlation coefficient [ICC] = 0.81), for future research into TDT. In addition, this tool could also be used to help clinicians assess the impact of the disease on parents to potentially target appropriate support and care that is required.

Introduction: Culturally appropriate assessment is vital for learning health systems; however, few such tools for Indigenous children exist. In 2011, Indigenous health leaders and scientists collaborated to create the Aaniish Naa Gegii: the Children’s Health and Well-being Measure (ACHWM) for Indigenous children aged 8 to 18 years. It was adapted for Inuit in Ottawa and named Qanuippit. The Government of Nunavut (GN) expressed interest in integrating the Qanuippit territory-wide. This study aimed to adapt the Ottawa Inuit version for Nunavut, to ensure they had a valid wellness measure to guide community-based health services planning for Inuit children in Iqaluit. Methods: We used a mixed-methods approach to consider the full spectrum of health, from wellness to illness. All data gathered was governed by Inuit Qaujimajatuqangit (IQ) principles. We consulted eight Iqaluit-based experts in Inuit education, culture, and children’s health to ensure cultural relevance and identify any missing content. In February 2024, interviews were conducted by the local mental health team, with 15 Inuit children (7.7 -17.9 years). Each child completed the draft of the Qanuippit that resulted from the Expert review, and local mental health workers examined the children’s understanding of each item. The analysis focused on items with critical input from two or more participants. Once finalized, a pilot implementation study explored how mental health workers integrated the Qanuippit in practice. Results: Experts reported eight items of concern; one had a clear solution, and the remaining seven were identified for monitoring. Children identified 11 problematic items; two had clear solutions, while consultation continued for the remaining items. Mental health workers reported that the tool was a positive, strengths-based experience. The Qanuippit aided in forming connections with clients, which positively impacted their practice. Conclusions: The Qanuippit has proven to be a good fit to assess the health and well-being of Inuit children in Iqaluit.

Background: Current requirements for ethical research in Canada, specifically the standard of active or signed parental consent, can leave Indigenous children and youth with inequitable access to research opportunities or health screening. Our objective was to examine the literature to identify culturally safe research consent processes that respect the rights of Indigenous children, the rights and responsibilities of parents or caregivers, and community protocols. Methods: We followed PRISMA guidelines and Arksey and O’Malley’s approach for charting and synthesizing evidence. We searched MEDLINE, PsycINFO, ERIC, CINAHL, Google Scholar, Web of Science, Informit Indigenous Collection, Bibliography of Native North Americans, and Sociological Abstracts. We included peer-reviewed primary and theoretical research articles written in English from January 1, 2000, to March 31, 2022, examining Indigenous approaches for obtaining informed consent from parents, families, children, or youth. Eligible records were uploaded to Covidence for title and abstract screening. We appraised the findings using a Two-Eyed Seeing approach. These findings were inductively coded using NVivo 12 and analyzed thematically. Results: We identified 2,984 records and 11 eligible studies were included after screening. Three key recommendations emerged: addressing tensions in the ethics of consent, embracing wise practices, and using relational approaches to consent. Tensions in consent concerned Research Ethics Board consent requirements that fall short of protecting Indigenous children and communities when culturally incongruent. Wise practices included allowing parents and children to consent together, land-based consenting, and involving communities in decision-making. Using relational approaches to consent embodied community engagement and relationship building while acknowledging consent for Indigenous children cannot be obtained in isolation from family and community. Conclusions: Very few studies discussed obtaining child consent in Indigenous communities. While Indigenous communities are not a monolith, the literature identified a need for community-driven, decolonized consent processes prioritizing Indigenous values and protocols. Further research is needed to examine nuances of Indigenized consent processes and determine how to operationalize them, enabling culturally appropriate, equitable access to research and services for all Indigenous children.

Introduction: Though numerous research pursuits in Indigenous communities have been undertaken, very few have consistently addressed community priorities, or collaborated with Indigenous peoples throughout the research process. This scoping review protocol proposes to explore the existing wise consent processes that respect the rights of Indigenous families (parents, children), and Indigenous community protocols. Methods and analysis: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for conducting scoping reviews will be followed closely. All primary and theoretical studies of any design written in English from January 1st 2000 to March 31st 2022 examining Indigenous approaches to obtaining informed consent among parents or families and/or children and youth, will be included. Two reviewers will independently review the literature in order to apply the inclusion and exclusion criteria. Data from studies will be extracted and charted in NVivo, following the Arksey and O’Malley’s (2005) framework. The Critical Appraisal Skills Programme (CASP) checklists, depending on the study design of each included study, and the original and modified Aboriginal and Torres Strait Islander Quality Appraisal Tool (ATSI) versions, will be used to assess study quality. A narrative synthesis of the informed consent literature will be reported. Dissemination: This scoping review will evaluate the existing informed consent processes, barriers to consent, and alternative consent processes in the literature. Results will be shared via conferences, reports and social media with our Indigenous communities, and disseminated through a peer-reviewed publication. This scoping review may prove useful to others who are investigating informed consent processes among Indigenous families in research.

Introduction: In many countries, there is a shift from standard half‐life (SHL) to extended half‐life (EHL) clotting factor concentrates (CFCs).Aim: To describe the experience of switching from SHL to an EHL FVIII CFC and the impact of this on frequency of infusions, factor consumption, bleeding rates and HRQoL using the Canadian Hemophilia Kids’ Life Assessment Tool (CHO‐KLAT).Methods: A retrospective chart review was conducted at a single haemophilia treatment centre in 2018 that included boys (ages: 4‐18 years) with moderate/severe haemophilia A, without inhibitors, who switched from a SHL to an EHL FVIII CFC in the previous 2 years and for whom HRQoL data were available.Results: The study cohort comprised 38 boys [mean (SD) age: 11.0 (3.4) years] with moderate (n = 5)/severe (n = 33) haemophilia A. The switch was associated with a 33% reduction in the number of weekly infusions from a median of 3.5 to 2.3 (P < .0001) and a 17% reduction in median FVIII consumption from 103 IU/kg/wk to 85.5 IU/kg/wk (P = .004). There was no significant change in annualized joint bleed rates or in CHO‐KLAT scores.Conclusions: Despite documenting several benefits of switching to EHL FVIII (less infusions, lower factor consumption with no increase in bleeding), our study did not demonstrate any improvement in HRQoL. We conclude that either the current CHO‐KLAT tool is not optimized to measure burden of treatment administration in boys with low bleed rates switching from SHL to EHL FVIII CFCs or that a reduction of 1.2 infusions/week does not result in a meaningful change in HRQoL.

Background: The Aboriginal Children’s Health and Well-Being Measure (ACHWM) was developed to enable Aboriginal health leaders to gather information on the health of children at a local community level. This paper aims to describe the typical health profiles of First Nation children living on traditional territory as a reference to assist in the interpretation of ACHWM scores. Methods: Three First Nations in Ontario, Canada, gathered health data from children using the ACHWM administered on Android tablets between 2013 and 2015. The survey data were previously analyzed to inform local health planning. These survey data were pooled to describe the distribution of ACHWM summary and quadrant scores from a larger sample and inform interpretation of ACHWM scores. Results: ACHWM data from 196 participants (aged 7.6 to 21.7 yr) across 3 communities were included in the pooled sample. ACHWM summary scores ranged from 39.8 to 98.7 with a mean of 74.1 (95% confidence interval [CI] 72.5–75.7) and a maximum of 100. Strengths were reported in the spiritual (mean 78.7, 95% CI 76.7–80.8), physical (mean 77.1, 95% CI 75.1–79.0) and emotional (mean 74.4, 95% CI 72.5–76.3) quadrants. The greatest opportunity for improvement was in the mental (cognition) quadrant (mean 61.6, 95% CI 56.9–63.4). Interpretation: This paper presents initial estimates for child health scores based on self-report from a large sample of First Nations children living on reserve. These results establish benchmarks to aid interpretation of the ACHWM scores in these and other communities and contexts in the future.