Suppr超能文献

养育复杂性和罕见病患儿:探索普拉德-威利综合征的叙事研究。

Mothering a Child With Complexity and Rarity: A Narrative Inquiry Exploring Prader-Willi Syndrome.

机构信息

School of Nursing and Midwifery, Faculty of Health, Community and Education, Mount Royal University, Calgary, AB, Canada.

Faculty of Nursing, University of Calgary, Calgary, AB, Canada.

出版信息

Qual Health Res. 2024 Jul;34(8-9):742-755. doi: 10.1177/10497323231225412. Epub 2024 Jan 28.

Abstract

Daily experiences of mothers caring for children with Prader-Willi syndrome (PWS) are largely unknown and unvoiced. Knowledge of PWS has generally focused on pathology of the disorder. This emphasis overlooks the challenging moments of everyday life caring for children with PWS. Storied accounts of mothers caring for children with PWS offer expanded narratives to medicalized descriptions of experience. An understanding of everyday challenges in managing physical and mental health issues of PWS including hyperphagia and anxiety may create shifts in social and clinical perspectives. This understanding could improve practices in health and social care for families with PWS. This narrative inquiry studied everyday experience using storied accounts. Participants were mothers caring for children aged 3-17 years with genetically confirmed PWS who were experiencing hyperphagia. Four participants were recruited, and each interviewed 8-12 times over 12 months. Field texts and narrative accounts were co-composed through a collaborative process of analysis. Engaging with participants' day-to-day experiences offered insights into their work of nurturing, caring, and contributing to the care of a child with PWS. Narrative threads focused on complexity and rarity and include the desire to be normal, how ordinary becomes extraordinary, isolation, behaviors and normative standards, and alternative stories of mothering. Recommendations for practice and policy include (a) challenges of mothering a child with complexity, (b) moving beyond functionality and impairment to participation and quality of life, (c) re-storying narratives and supports for families, and (d) engaging with mothers to determine care priorities.

摘要

母亲照顾患有普拉德-威利综合征(PWS)孩子的日常经历在很大程度上是未知的和未被提及的。对 PWS 的了解通常集中在该疾病的病理学上。这种重点忽视了照顾患有 PWS 的孩子日常生活中的挑战时刻。母亲照顾患有 PWS 的孩子的故事性叙述为经验的医学化描述提供了扩展的叙述。对管理 PWS 的身心健康问题(包括贪食症和焦虑症)的日常挑战的理解可能会改变社会和临床观点。这种理解可以改善 PWS 家庭的健康和社会保健实践。这项叙事研究使用故事性叙述来研究日常经验。参与者是照顾 3-17 岁患有经基因确认的 PWS 且正在经历贪食症的孩子的母亲。招募了 4 名参与者,每位参与者在 12 个月内接受了 8-12 次访谈。实地文本和叙事叙述通过分析的协作过程共同构成。参与参与者的日常经验提供了对他们养育、照顾和为照顾患有 PWS 的孩子做出贡献的工作的深入了解。叙事线索集中在复杂性和稀有性上,包括渴望正常、平凡变得非凡、孤立、行为和规范标准,以及母性的替代故事。实践和政策建议包括:(a)照顾患有复杂性孩子的挑战,(b)超越功能和损伤,走向参与和生活质量,(c)重新叙述家庭的叙事和支持,以及(d)与母亲合作确定护理重点。

相似文献

1
Mothering a Child With Complexity and Rarity: A Narrative Inquiry Exploring Prader-Willi Syndrome.
Qual Health Res. 2024 Jul;34(8-9):742-755. doi: 10.1177/10497323231225412. Epub 2024 Jan 28.
2
: Experiences of Mothers Caring for Children With Prader-Willi Syndrome.
Glob Qual Nurs Res. 2024 Mar 28;11:23333936241242929. doi: 10.1177/23333936241242929. eCollection 2024 Jan-Dec.
3
The impact of Prader-Willi syndrome on the family's quality of life and caregiving, and the unaffected siblings' psychosocial adjustment.
J Intellect Disabil Res. 2013 Sep;57(9):861-73. doi: 10.1111/j.1365-2788.2012.01634.x. Epub 2012 Oct 12.
6
Characteristics and relationship between hyperphagia, anxiety, behavioral challenges and caregiver burden in Prader-Willi syndrome.
PLoS One. 2021 Mar 25;16(3):e0248739. doi: 10.1371/journal.pone.0248739. eCollection 2021.
7
Life Satisfaction Among Mothers of Individuals with Prader-Willi Syndrome.
J Autism Dev Disord. 2016 Jun;46(6):2126-2137. doi: 10.1007/s10803-016-2741-5.
8
Parent Recommendations for Family Functioning With Prader-Willi Syndrome: A Rare Genetic Cause of Childhood Obesity.
J Pediatr Nurs. 2016 Jan-Feb;31(1):47-54. doi: 10.1016/j.pedn.2015.11.001. Epub 2015 Dec 10.
9
Caregiver priorities for endpoints to evaluate treatments for Prader-Willi syndrome: a best-worst scaling.
J Med Econ. 2018 Dec;21(12):1230-1237. doi: 10.1080/13696998.2018.1528980. Epub 2018 Oct 16.
10
A disease specific questionnaire for assessing behavior in individuals with Prader-Willi syndrome.
Compr Psychiatry. 2015 Apr;58:189-97. doi: 10.1016/j.comppsych.2014.12.005. Epub 2014 Dec 30.

引用本文的文献

1
The burden of illness in Prader-Willi syndrome: a systematic literature review.
Orphanet J Rare Dis. 2025 Jul 24;20(1):374. doi: 10.1186/s13023-025-03787-0.
2
: Experiences of Mothers Caring for Children With Prader-Willi Syndrome.
Glob Qual Nurs Res. 2024 Mar 28;11:23333936241242929. doi: 10.1177/23333936241242929. eCollection 2024 Jan-Dec.

本文引用的文献

1
: Experiences of Mothers Caring for Children With Prader-Willi Syndrome.
Glob Qual Nurs Res. 2024 Mar 28;11:23333936241242929. doi: 10.1177/23333936241242929. eCollection 2024 Jan-Dec.
2
Gender and burden differences in family caregivers of patients affected by ten rare diseases.
Ann Ist Super Sanita. 2023 Apr-Jun;59(2):122-131. doi: 10.4415/ANN_23_02_05.
3
Quality of Life in Children with Prader-Willi Syndrome and the Impact of the Disease on the Functioning of Families.
Int J Environ Res Public Health. 2022 Dec 6;19(23):16330. doi: 10.3390/ijerph192316330.
4
Parenting a Child with a Neurodevelopmental Disorder.
Curr Dev Disord Rep. 2021;8(4):212-218. doi: 10.1007/s40474-021-00240-2. Epub 2021 Nov 20.
5
Building a culture of engagement at a research centre for childhood disability.
Res Involv Engagem. 2021 Nov 6;7(1):78. doi: 10.1186/s40900-021-00319-5.
6
Designing a tool to support patient and public involvement in research projects: the Involvement Matrix.
Res Involv Engagem. 2020 Jun 16;6:30. doi: 10.1186/s40900-020-00188-4. eCollection 2020.
8
The War on Prevention II: Battle Metaphors Undermine Cancer Treatment and Prevention and Do Not Increase Vigilance.
Health Commun. 2020 Nov;35(13):1698-1704. doi: 10.1080/10410236.2019.1663465. Epub 2019 Sep 9.
10
Requirements for improving health and well-being of children with Prader-Willi syndrome and their families.
J Paediatr Child Health. 2019 Sep;55(9):1029-1037. doi: 10.1111/jpc.14546. Epub 2019 Jun 30.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验