Fatigue in adults with cerebral palsy: A three-year follow-up study.

OBJECTIVES: To describe the course of fatigue over a 3-year follow-up period in adults with cerebral palsy and to investigate the association of known determinants of fatigue (i.e. demographic characteristics and/or body composition) with change in fatigue. METHODS: Forty-one adults with cerebral palsy from a previous study of fatigue were invited to participate in a follow-up study. Twenty-three adults with cerebral palsy (Gross Motor Function Classification System (GMFCS) levels I-V; mean age 38 years 2 months, standard deviation (SD) 14 years 1 month)) agreed to participate (convenience sample). Fatigue was measured with the Fatigue Impact and Severity Self-Assessment (FISSA, range 31-157) questionnaire. The course of fatigue is described at group, subgroup (GMFCS) and individual levels. RESULTS: The mean FISSA score for all participants was 84.0 (SD 27.7) at baseline and 91.7 (SD 26.7) at follow-up. Despite variations among individuals in the change of fatigue, there was no statistically significant difference in FISSA score over time (p = 0.087, 95% confidence interval (95% CI) -16.7 to 1.22). No known determinants of fatigue predictive of change in FISSA scores were found. DISCUSSION: Fatigue appears to be relatively stable within adults with cerebral palsy over time, with a variable presentation between individuals and across GMFCS levels. Care providers should monitor and discuss fatigue in young individuals with cerebral palsy in order to attenuate fatigue later in life

The formula for health and well-being in individuals with cerebral palsy: Cross-sectional data on physical activity, sleep, and nutrition

Objective To determine physical activity, sleep, and nutrition patterns in individuals with cerebral palsy (CP) and investigate the association of Gross Motor Function Classification System (GMFCS) and age with these health behaviors. Methods A cross-sectional study was conducted in an outpatient setting. Participants included adolescents and adults with CP (n=28; GMFCS level I-V; mean age 35.1±14.4 years). An Exercise Questionnaire or Leisure Time Physical Activity Questionnaire was used to measure physical activity in adolescents and adults, respectively. Sleep quality was measured using the Pittsburgh Sleep Quality Index (PSQI). An adapted version of the PrimeScreen questionnaire was used to assess nutrition. Linear regression analyses were performed to investigate the association between GMFCS and age with physical activity, sleep, and nutrition. Results The average total physical activity was 29.2±30.0 min/day. Seventy-five percent of the participants had poor sleep quality (PSQI score >5). Seventy-one percent reported "fair" eating behaviors; none reported "excellent" eating behaviors. Neither GMFCS nor age were significantly associated with PSQI score, PrimeScreen score, or total physical activity. A negative correlation existed between sleep quantity (hr/night) and PSQI score (r=-0.66, p=0.01). Conclusion The triad of health components, consisting of physical activity, sleep, and nutrition, was not associated with GMFCS or age in our sample of 28 individuals with CP, suggesting that these three health behaviors should be assessed during clinical encounters of CP in adolescents and adults at all levels of the GMFCS

Harmonizing data on correlates of sleep in children within and across neurodevelopmental disorders: lessons learned from an Ontario Brain Institute cross-program collaboration

There is an increasing desire to study neurodevelopmental disorders (NDDs) together to understand commonalities to develop generic health promotion strategies and improve clinical treatment. Common data elements (CDEs) collected across studies involving children with NDDs afford an opportunity to answer clinically meaningful questions. We undertook a retrospective, secondary analysis of data pertaining to sleep in children with different NDDs collected through various research studies. The objective of this paper is to share lessons learned for data management, collation, and harmonization from a sleep study in children within and across NDDs from large, collaborative research networks in the Ontario Brain Institute (OBI). Three collaborative research networks contributed demographic data and data pertaining to sleep, internalizing symptoms, health-related quality of life, and severity of disorder for children with six different NDDs: autism spectrum disorder; attention deficit/hyperactivity disorder; obsessive compulsive disorder; intellectual disability; cerebral palsy; and epilepsy. Procedures for data harmonization, derivations, and merging were shared and examples pertaining to severity of disorder and sleep disturbances were described in detail. Important lessons emerged from data harmonizing procedures: prioritizing the collection of CDEs to ensure data completeness; ensuring unprocessed data are uploaded for harmonization in order to facilitate timely analytic procedures; the value of maintaining variable naming that is consistent with data dictionaries at time of project validation; and the value of regular meetings with the research networks to discuss and overcome challenges with data harmonization. Buy-in from all research networks involved at study inception and oversight from a centralized infrastructure (OBI) identified the importance of collaboration to collect CDEs and facilitate data harmonization to improve outcomes for children with NDDs

Multimorbidity risk assessment in adolescents and adults with cerebral palsy: a protocol for establishing a core outcome set for clinical research and practice

Background: Estimates of multimorbidity, defined as the presence of at least two chronic conditions, some of which attributable to modifiable behaviours, are high in adults with cerebral palsy (CP). An assessment protocol evaluating multimorbidity risk is needed in order to develop and evaluate effective interventions to optimize lifelong health in individuals with CP. The aim of this protocol paper is to describe the development of a core outcome set (COS) for assessing multimorbidity risk in adolescents and adults with CP, to be used in clinic and research. Methods: The expert consortium will first define the target population and outcomes to be measured. Through a process of literature review and an international Delphi survey with expert clinicians and researchers, we will then determine which outcome measurement instruments (OMIs) can best measure those outcomes. The resulting OMIs will be used in a feasibility study with adolescents and adults with CP from an international clinical research network. Finally, a face-to-face stakeholder meeting with adolescents and adults with CP, their families/caregivers and researchers and clinicians who are experts in CP, will be organized to reach final agreement on the COS. Discussion: This COS will guide clinicians and researchers in assessing multimorbidity risk in adolescents and adults with CP. The inclusion of experts and individuals with CP from international locations for establishing the COS lends strong support to its generalizability. Evidence of its feasibility and approval from all stakeholders will enable implementation in clinical practice, and guide future research using the COS in individuals with CP

Molecular mechanisms of cell death: recommendations of the Nomenclature Committee on Cell Death 2018.

Over the past decade, the Nomenclature Committee on Cell Death (NCCD) has formulated guidelines for the definition and interpretation of cell death from morphological, biochemical, and functional perspectives. Since the field continues to expand and novel mechanisms that orchestrate multiple cell death pathways are unveiled, we propose an updated classification of cell death subroutines focusing on mechanistic and essential (as opposed to correlative and dispensable) aspects of the process. As we provide molecularly oriented definitions of terms including intrinsic apoptosis, extrinsic apoptosis, mitochondrial permeability transition (MPT)-driven necrosis, necroptosis, ferroptosis, pyroptosis, parthanatos, entotic cell death, NETotic cell death, lysosome-dependent cell death, autophagy-dependent cell death, immunogenic cell death, cellular senescence, and mitotic catastrophe, we discuss the utility of neologisms that refer to highly specialized instances of these processes. The mission of the NCCD is to provide a widely accepted nomenclature on cell death in support of the continued development of the field

Global incidence, prevalence, years lived with disability (YLDs), disability-adjusted life-years (DALYs), and healthy life expectancy (HALE) for 371 diseases and injuries in 204 countries and territories and 811 subnational locations, 1990–2021: a systematic analysis for the Global Burden of Disease Study 2021

Background: Detailed, comprehensive, and timely reporting on population health by underlying causes of disability and premature death is crucial to understanding and responding to complex patterns of disease and injury burden over time and across age groups, sexes, and locations. The availability of disease burden estimates can promote evidence-based interventions that enable public health researchers, policy makers, and other professionals to implement strategies that can mitigate diseases. It can also facilitate more rigorous monitoring of progress towards national and international health targets, such as the Sustainable Development Goals. For three decades, the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) has filled that need. A global network of collaborators contributed to the production of GBD 2021 by providing, reviewing, and analysing all available data. GBD estimates are updated routinely with additional data and refined analytical methods. GBD 2021 presents, for the first time, estimates of health loss due to the COVID-19 pandemic. Methods: The GBD 2021 disease and injury burden analysis estimated years lived with disability (YLDs), years of life lost (YLLs), disability-adjusted life-years (DALYs), and healthy life expectancy (HALE) for 371 diseases and injuries using 100 983 data sources. Data were extracted from vital registration systems, verbal autopsies, censuses, household surveys, disease-specific registries, health service contact data, and other sources. YLDs were calculated by multiplying cause-age-sex-location-year-specific prevalence of sequelae by their respective disability weights, for each disease and injury. YLLs were calculated by multiplying cause-age-sex-location-year-specific deaths by the standard life expectancy at the age that death occurred. DALYs were calculated by summing YLDs and YLLs. HALE estimates were produced using YLDs per capita and age-specific mortality rates by location, age, sex, year, and cause. 95% uncertainty intervals (UIs) were generated for all final estimates as the 2·5th and 97·5th percentiles values of 500 draws. Uncertainty was propagated at each step of the estimation process. Counts and age-standardised rates were calculated globally, for seven super-regions, 21 regions, 204 countries and territories (including 21 countries with subnational locations), and 811 subnational locations, from 1990 to 2021. Here we report data for 2010 to 2021 to highlight trends in disease burden over the past decade and through the first 2 years of the COVID-19 pandemic. Findings: Global DALYs increased from 2·63 billion (95% UI 2·44–2·85) in 2010 to 2·88 billion (2·64–3·15) in 2021 for all causes combined. Much of this increase in the number of DALYs was due to population growth and ageing, as indicated by a decrease in global age-standardised all-cause DALY rates of 14·2% (95% UI 10·7–17·3) between 2010 and 2019. Notably, however, this decrease in rates reversed during the first 2 years of the COVID-19 pandemic, with increases in global age-standardised all-cause DALY rates since 2019 of 4·1% (1·8–6·3) in 2020 and 7·2% (4·7–10·0) in 2021. In 2021, COVID-19 was the leading cause of DALYs globally (212·0 million [198·0–234·5] DALYs), followed by ischaemic heart disease (188·3 million [176·7–198·3]), neonatal disorders (186·3 million [162·3–214·9]), and stroke (160·4 million [148·0–171·7]). However, notable health gains were seen among other leading communicable, maternal, neonatal, and nutritional (CMNN) diseases. Globally between 2010 and 2021, the age-standardised DALY rates for HIV/AIDS decreased by 47·8% (43·3–51·7) and for diarrhoeal diseases decreased by 47·0% (39·9–52·9). Non-communicable diseases contributed 1·73 billion (95% UI 1·54–1·94) DALYs in 2021, with a decrease in age-standardised DALY rates since 2010 of 6·4% (95% UI 3·5–9·5). Between 2010 and 2021, among the 25 leading Level 3 causes, age-standardised DALY rates increased most substantially for anxiety disorders (16·7% [14·0–19·8]), depressive disorders (16·4% [11·9–21·3]), and diabetes (14·0% [10·0–17·4]). Age-standardised DALY rates due to injuries decreased globally by 24·0% (20·7–27·2) between 2010 and 2021, although improvements were not uniform across locations, ages, and sexes. Globally, HALE at birth improved slightly, from 61·3 years (58·6–63·6) in 2010 to 62·2 years (59·4–64·7) in 2021. However, despite this overall increase, HALE decreased by 2·2% (1·6–2·9) between 2019 and 2021. Interpretation: Putting the COVID-19 pandemic in the context of a mutually exclusive and collectively exhaustive list of causes of health loss is crucial to understanding its impact and ensuring that health funding and policy address needs at both local and global levels through cost-effective and evidence-based interventions. A global epidemiological transition remains underway. Our findings suggest that prioritising non-communicable disease prevention and treatment policies, as well as strengthening health systems, continues to be crucially important. The progress on reducing the burden of CMNN diseases must not stall; although global trends are improving, the burden of CMNN diseases remains unacceptably high. Evidence-based interventions will help save the lives of young children and mothers and improve the overall health and economic conditions of societies across the world. Governments and multilateral organisations should prioritise pandemic preparedness planning alongside efforts to reduce the burden of diseases and injuries that will strain resources in the coming decades. Funding: Bill & Melinda Gates Foundation

Establishing and sustaining authentic organizational partnerships in childhood disability research: lessons learned

Abstract There is an increased interest from both researchers and knowledge users to partner in research to generate meaningful research ideas, implement research projects, and disseminate research findings. There is accumulating research evidence to suggest the benefits of engaging children/youth with disabilities and their parents/families in research partnerships; however, less is known about the benefits of, and challenges to, engaging organizations as partners in research. The purpose of this commentary is to reflect on successful organizational partnership experiences from the perspectives of researchers at an internationally-recognized childhood disability research centre (CanChild), and to identify and share key ingredients for developing partnerships between organizations and academic institutions. A companion study is underway to examine partnership experiences with CanChild from the partners’ perspective. Four CanChild researchers and two co-facilitators participated in a collaborative auto-ethnography approach to share experiences with organizational research partnerships and to reflect, interpret, and synthesize common themes and lessons learned. The researchers and facilitators met virtually via Zoom for 105 min. Researchers were asked to discuss the following: the formation of their organizational partnerships; if/how partnerships evolved over time; if/how partnerships were sustained; and lessons learned about benefits and challenges to building research partnerships with organizations. The meeting was recorded, transcribed verbatim, and analyzed by the facilitators to identify and synthesize common experiences and reflections. Multiple rounds of asynchronous reflection and feedback supported refinement of the final set of analytic themes. Researchers agreed that partnerships with organizations should be formed through a mutual interest, and that partnerships evolved by branching to include new organizations and researchers, while also involving trainees. Researchers identified the importance of defining roles and responsibilities of key individuals within each partnering group to sustain the partnership. Lessons learned from organizational partnerships included reciprocity between the partnering organization and academic institution, leveraging small pockets of funds to sustain a partnership over time, and building a strong rapport with individuals in a partnership. This commentary summarized lessons-learned and provided recommendations for researchers and organizations to consider when forming, growing, and sustaining research partnerships over time

Difficultés fonctionnelles chez les enfants et les jeunes atteints d’un trouble du spectre de l’autisme : une analyse de l’Enquête canadienne sur la santé des enfants et des jeunes de 2019

IntroductionCette étude porte sur la prévalence des difficultés fonctionnelles et des facteurs connexes chez les enfants et les jeunes canadiens de 5 à 17 ans ayant reçu un diagnostic de trouble du spectre de l'autisme (TSA). MéthodologieNous avons analysé les données tirées de l’Enquête canadienne sur la santé des enfants et des jeunes (ECSEJ) de 2019, une enquête nationale représentative de la population canadienne d’enfants et de jeunes, qui utilisait l’ensemble abrégé de questions sur le fonctionnement du Groupe de Washington (Washington Group Short Set on Functioning, WG-SS) pour évaluer le fonctionnement dans le cadre de six tâches quotidiennes. Pour chaque domaine fonctionnel, des résultats binaires ont été générés (aucune difficulté/quelques difficultés, beaucoup de difficultés/n’y parvient pas du tout). Nous avons ensuite utilisé une régression logistique pour déterminer les associations entre, d’une part, les caractéristiques sociodémographiques, les expériences d’apprentissage et la santé générale et mentale perçue et, d’autre part, les difficultés fonctionnelles les plus courantes, celles liées à la mémoire/concentration, à la communication et aux soins personnels. Toutes les estimations ont été pondérées de façon à être représentatives de la population cible. Nous avons utilisé la méthode « bootstrap » pour établir les estimations de la variance. RésultatsL’analyse des données des 660 enfants et jeunes atteints d’un TSA a révélé que les difficultés fonctionnelles les plus courantes concernaient la mémoire/concentration (22 %; IC à 95 % : 18 à 27), la communication (19 %; IC à 95 % : 15 à 23) et les soins personnels (13 %; IC à 95 % : 10 à 17). Une moins bonne santé mentale perçue était associée à des difficultés fonctionnelles accrues liées à la mémoire/concentration. Un diagnostic de TSA à un plus jeune âge et une moins bonne santé générale perçue étaient associés à des difficultés fonctionnelles accrues liées à la communication. Le fait que les parents avaient des attentes en matière d’études postsecondaires était associé à des difficultés fonctionnelles réduites liées aux de soins personnels. ConclusionL’étude a révélé que 39 % des enfants et des jeunes canadiens de 5 à 17 ans ayant reçu un diagnostic de TSA présentaient au moins une difficulté fonctionnelle citée dans l’ensemble de questions WG-SS, les difficultés fonctionnelles liées à la mémoire/ concentration, à la communication et aux soins personnels étant les plus fréquentes

Functional difficulties in children and youth with autism spectrum disorder: analysis of the 2019 Canadian Health Survey on Children and Youth

IntroductionThis study examined the prevalence of functional difficulties and associated factors in Canadian children/youth aged 5 to 17 years diagnosed with autism spectrum disorder (ASD). MethodsWe analyzed data from the 2019 Canadian Health Survey on Children and Youth (CHSCY), a nationally representative survey of Canadian children/youth that used the Washington Group Short Set on Functioning (WG-SS) to evaluate functioning in six daily tasks. For each functional domain, binary outcomes were derived (no/some difficulty, a lot of difficulty/no ability). We used logistic regression to identify associations between demographic characteristics, educational experiences, and perceived mental and general health and the most common functional difficulties, namely those related to remembering/concentrating, communication and self-care. All estimates were weighted to be representative of the target population. The bootstrap method was used to calculate variance estimates. ResultsAnalysis of the records of 660 children/youth with ASD revealed that the most common functional difficulties were remembering/concentrating (22%; 95% CI: 18–27), communicating (19%; 95% CI: 15–23) and self-care (13%; 95% CI: 10–17). Lower perceived mental health was associated with increased functional difficulties with remembering/concentrating. ASD diagnosis at a lower age and lower perceived general health were associated with increased functional difficulty with communication. Parental expectations for postsecondary education were associated with decreased functional difficulty for self-care. ConclusionOne or more functional difficulties from the WG-SS was present in 39% of Canadian children/youth aged 5 to 17 years with ASD. Functional difficulties with remembering/concentrating, communication and self-care were most common

Multimorbidity risk assessment in adolescents and adults with cerebral palsy: a protocol for establishing a core outcome set for clinical research and practice

Background: Estimates of multimorbidity, defined as the presence of at least two chronic conditions, some of which attributable to modifiable behaviours, are high in adults with cerebral palsy (CP). An assessment protocol evaluating multimorbidity risk is needed in order to develop and evaluate effective interventions to optimize lifelong health in individuals with CP. The aim of this protocol paper is to describe the development of a core outcome set (COS) for assessing multimorbidity risk in adolescents and adults with CP, to be used in clinic and research.Methods: The expert consortium will first define the target population and outcomes to be measured. Through a process of literature review and an international Delphi survey with expert clinicians and researchers, we will then determine which outcome measurement instruments (OMIs) can best measure those outcomes. The resulting OMIs will be used in a feasibility study with adolescents and adults with CP from an international clinical research network. Finally, a face-to-face stakeholder meeting with adolescents and adults with CP, their families/caregivers and researchers and clinicians who are experts in CP, will be organized to reach final agreement on the COS.Discussion: This COS will guide clinicians and researchers in assessing multimorbidity risk in adolescents and adults with CP. The inclusion of experts and individuals with CP from international locations for establishing the COS lends strong support to its generalizability. Evidence of its feasibility and approval from all stakeholders will enable implementation in clinical practice, and guide future research using the COS in individuals with CP