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“I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood

vFor youth with life-limiting chronic illnesses, transitioning to adulthood in line with age-norms may be difficult due to symptom severity and shortened survival. This study explores whether individuals with Barth syndrome (BTHS), a condition uniquely characterized by extreme prognostic uncertainty...

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Detalles Bibliográficos
Autores principales: Mazar, Iyar, Moorman, Sara M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8640324/
https://www.ncbi.nlm.nih.gov/pubmed/34869844
http://dx.doi.org/10.1177/23743735211060783
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author Mazar, Iyar
Moorman, Sara M.
author_facet Mazar, Iyar
Moorman, Sara M.
author_sort Mazar, Iyar
collection PubMed
description vFor youth with life-limiting chronic illnesses, transitioning to adulthood in line with age-norms may be difficult due to symptom severity and shortened survival. This study explores whether individuals with Barth syndrome (BTHS), a condition uniquely characterized by extreme prognostic uncertainty, experience similar or different challenges compared to youth with other conditions. During focus groups with adults with BTHS (n = 12) and caregivers (n = 13), participants reported that the ability to independently manage one’s health condition, the social/emotional impacts of BTHS, and the ability to set goals in the context of future uncertainty challenge their transition to adulthood. This aligns with prior research, indicating that prognostic uncertainty may hinder long-term goal setting in BTHS. Implications of these findings include providing strategies for identifying meaningful alternative goals for individuals with chronic illnesses to target, promoting increased autonomy earlier in youth, and fostering coping strategies to manage non-disease related impacts.
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spelling pubmed-86403242021-12-04 “I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood Mazar, Iyar Moorman, Sara M. J Patient Exp Research Article vFor youth with life-limiting chronic illnesses, transitioning to adulthood in line with age-norms may be difficult due to symptom severity and shortened survival. This study explores whether individuals with Barth syndrome (BTHS), a condition uniquely characterized by extreme prognostic uncertainty, experience similar or different challenges compared to youth with other conditions. During focus groups with adults with BTHS (n = 12) and caregivers (n = 13), participants reported that the ability to independently manage one’s health condition, the social/emotional impacts of BTHS, and the ability to set goals in the context of future uncertainty challenge their transition to adulthood. This aligns with prior research, indicating that prognostic uncertainty may hinder long-term goal setting in BTHS. Implications of these findings include providing strategies for identifying meaningful alternative goals for individuals with chronic illnesses to target, promoting increased autonomy earlier in youth, and fostering coping strategies to manage non-disease related impacts. SAGE Publications 2021-11-23 /pmc/articles/PMC8640324/ /pubmed/34869844 http://dx.doi.org/10.1177/23743735211060783 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Research Article
Mazar, Iyar
Moorman, Sara M.
“I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood
title “I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood
title_full “I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood
title_fullStr “I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood
title_full_unstemmed “I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood
title_short “I Want That Life a Lot…How on Earth Do I Get That?” Examining Challenges for Men With Barth Syndrome in Their Transitions to Adulthood
title_sort “i want that life a lot…how on earth do i get that?” examining challenges for men with barth syndrome in their transitions to adulthood
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8640324/
https://www.ncbi.nlm.nih.gov/pubmed/34869844
http://dx.doi.org/10.1177/23743735211060783
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