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A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder.

ABSTRACT: Lysosomal storage disorders are rare multiorgan, degenerative conditions requiring invasive treatment. Rare disorders pose unique challenges; therefore, exploring their impact is crucial for understanding family needs. This novel review aimed to understand the psychosocial outcomes for parents of children with lysosomal storage disorders. Five electronic databases were systematically searched. Thirty-eight (23 qualitative, 10 qualitative and 5 mixed methods) studies were included, analysed using a sequential explanatory narrative synthesis and appraised for their methodological quality. Quantitative data revealed the multifaceted impact on parents' psychological and social wellbeing. Qualitative data informed the challenges that these parents faced which were expressed within three main themes: (a) Uncertainty and the unknown, (b) All-encompassing impact and (c) Finding a way forward. The synthesis demonstrated that factors associated with the condition (symptoms, behaviour and severity) had a substantial negative impact on parental outcomes, upheld by concurrent loss (deterioration and poor prognosis) and uncertainty. This substantive integrated review revealed considerable unmet parental psychosocial needs.

SUBMITTER: Hassall S

PROVIDER: S-EPMC9305282 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder.

Hassall Sadie S Smith Debbie Michelle DM Rust Stewart S Wittkowski Anja A

Journal of inherited metabolic disease 20220224 3

Lysosomal storage disorders are rare multiorgan, degenerative conditions requiring invasive treatment. Rare disorders pose unique challenges; therefore, exploring their impact is crucial for understanding family needs. This novel review aimed to understand the psychosocial outcomes for parents of children with lysosomal storage disorders. Five electronic databases were systematically searched. Thirty-eight (23 qualitative, 10 qualitative and 5 mixed methods) studies were included, analysed using ...[more]

PMID: 35124835

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Project description:BackgroundEvaluation of complex interventions should include a process evaluation to give evaluators, researchers, and policy makers greater confidence in the outcomes reported from RCTs. Implementation fidelity can be part of a process evaluation and refers to the degree to which an intervention is delivered according to protocol. The aim of this implementation fidelity study was to evaluate to what extent a dialogue-based psychosocial intervention was delivered according to protocol. A modified conceptual framework for implementation fidelity was used to guide the analysis.MethodsThis study has an explanatory, sequential two-phase mixed methods design. Quantitative process data were collected longitudinally along with data collection in the RCT. Qualitative process data were collected after the last data collection point of the RCT. Descriptive statistical analyses were conducted to describe the sample, the intervention trajectories, and the adherence measures. A scoring system to clarify quantitative measurement of the levels of implementation was constructed. The qualitative data sources were analyzed separately with a theory-driven content analysis using categories of adherence and potential moderating factors identified in the conceptual framework of implementation fidelity. The quantitative adherence results were extended with the results from the qualitative analysis to assess which potential moderators may have influenced implementation fidelity and in what way.ResultsThe results show that the core components of the intervention were delivered although the intervention trajectories were individualized. Based on the composite score of adherence, results show that 80.1% of the interventions in the RCT were implemented with high fidelity. Although it is challenging to assess the importance of each of the moderating factors in relation to the other factors and to their influence on the adherence measures, participant responsiveness, comprehensiveness of policy description, context, and recruitment appeared to be the most prominent moderating factors of implementation fidelity in this study.ConclusionsThis evaluation of implementation fidelity and the discussion of what constitutes high fidelity implementation of this intervention are crucial in understanding the factors influencing the trial outcome. The study also highlights important methodological considerations for researchers planning process evaluations and studies of implementation fidelity.Trial registrationClinicalTrials.gov , NCT02338869; registered 10/04/2014.

Project description:BackgroundMental health crisis presentations are common in those who have a diagnosis of borderline personality disorder (BPD), and psychosocial interventions should be provided. However, there is limited evidence outlining what a crisis-focused psychosocial intervention for this population should include.AimsTo conduct a systematic review and narrative synthesis of crisis-focused psychosocial interventions for people diagnosed with BPD.MethodThree databases (MEDLINE, Embase, PsycInfo) were searched for eligible studies. Studies were included if they were quantitative studies comparing a crisis-focused intervention with any control group and they included adults (18+ years of age) who were diagnosed with BPD (or with equivalent experiences). A narrative synthesis was undertaken to analyse results.ResultsA total of 3711 papers were initially identified, 95 full texts were screened and 5 studies were included in the review. Two of five studies reported on the same trial, so four individual trials were included. Overall moderate risk of bias across studies was identified. The review tentatively demonstrated that crisis-focused psychosocial interventions are feasible and acceptable to people with BPD and that they have potential impacts on outcomes such as self-harm and number of days spent in hospital. There is limited consensus on what outcome measures should be used to assess the impact of interventions.ConclusionsThere is presently insufficient data to recommend any specific psychosocial crisis intervention for people with BPD. Given the relationship between BPD and the high frequency of crises experienced by this group, further large-scale trials examining crisis-focused psychosocial interventions are required.Statement on languageWe acknowledge that the term personality disorder can be controversial and stigmatising. As the term borderline personality disorder has been retained in DSM-5 and is used in the research evidence base we have decided to use this term throughout this review. However, we recognise that this term may not be acceptable to all.

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A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder.

Publications

A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder.

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