Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services

Abstract Background Genetic counselling for adolescents necessitates an approach distinct from that used with adults. Developing best practices is crucial, considering the growing number of disabled adolescents worldwide and increasing use of genomic testing early in life. We investigated perception...

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Main Authors: Tasha Wainstein, Cyrus Boelman, Connie Ens, William T. Gibson, Kevin Gregory-Evans, Olubayo U. Kolawole, Sheila K. Marshall, Kathryn Selby, GenCOUNSEL Study, Alison M. Elliott, Jehannine Austin
Format: Article
Language:English
Published: BMC 2025-08-01
Series:Orphanet Journal of Rare Diseases
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Online Access:https://doi.org/10.1186/s13023-025-03968-x
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author Tasha Wainstein
Cyrus Boelman
Connie Ens
William T. Gibson
Kevin Gregory-Evans
Olubayo U. Kolawole
Sheila K. Marshall
Kathryn Selby
GenCOUNSEL Study
Alison M. Elliott
Jehannine Austin
author_facet Tasha Wainstein
Cyrus Boelman
Connie Ens
William T. Gibson
Kevin Gregory-Evans
Olubayo U. Kolawole
Sheila K. Marshall
Kathryn Selby
GenCOUNSEL Study
Alison M. Elliott
Jehannine Austin
author_sort Tasha Wainstein
collection DOAJ
description Abstract Background Genetic counselling for adolescents necessitates an approach distinct from that used with adults. Developing best practices is crucial, considering the growing number of disabled adolescents worldwide and increasing use of genomic testing early in life. We investigated perceptions of adolescents (10–19 years) who had been diagnosed with a genetic disorder in terms of how they describe receiving, understanding, and living with a genetic condition. We undertook a cross-sectional, qualitatively oriented mixed methods study underpinned by the pragmatic paradigm. Adolescents completed two self-report measures – the psychological adaptation scale (PAS) and the illness identity questionnaire (IIQ)—and participated in semi-structured interviews. Demographic, PAS, and IIQ data were analyzed using descriptive statistics. We used phronetic iterative analysis to interrogate interview data. Qualitative and quantitative components were integrated through abduction. Results Eighteen participants (median age: 15.5 years; 11/18 women/girls; 13/18 typical cognition; 8/18 de novo presentation) with a variety of genetic conditions participated. Participants had a mean PAS of 3.07 ± 0.84 indicating adequate adaptation. Their IIQ profiles indicated slightly better mean adaptive scores (3.10 ± 1.06) than mean maladaptive scores (2.85 ± 0.99). We developed a conceptual model that describes disability and genetic identity development and psychological adaptation among participants composed of three interacting components: internalizing processes; variability arising from contextual factors; and external factors associated with the processes. Adolescents generally moved among four internalizing processes (initiating, minimizing, exploring, and accepting). Movement across these processes took place frequently because of contextual factors like setting and disability type. Communication and engagement with caregivers, peers, and healthcare professionals, social interactions with others who have the same or a similar condition, and the impact of ableism constituted the main external factors with which adolescents engaged in the development of these identities. Conclusions Our findings present a foundation upon which to develop a care model optimized for the needs of adolescents with genetic conditions. Enhancing access to genetic counselling as a means of facilitating identity development is an important component of these care models.
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spelling doaj-art-879aa73acd49413297aabef00d200e012025-08-24T11:50:12ZengBMCOrphanet Journal of Rare Diseases1750-11722025-08-0120112010.1186/s13023-025-03968-xIdentity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics servicesTasha Wainstein0Cyrus Boelman1Connie Ens2William T. Gibson3Kevin Gregory-Evans4Olubayo U. Kolawole5Sheila K. Marshall6Kathryn Selby7GenCOUNSEL StudyAlison M. Elliott8Jehannine Austin9Department of Medical Genetics, Faculty of Medicine, The University of British ColumbiaDivision of Neurology, Department of Pediatrics, Faculty of Medicine, The University of British ColumbiaPediatric Partnership Heart Program, Pediatric BC Inherited Arrhythmia Program, BC Children’s Heart CentreDepartment of Medical Genetics, Faculty of Medicine, The University of British ColumbiaDepartment of Medical Genetics, Faculty of Medicine, The University of British ColumbiaDepartment of Ophthalmology and Visual Sciences, The University of British ColumbiaSchool of Social Work, University of British ColumbiaDivision of Neurology, Department of Pediatrics, Faculty of Medicine, The University of British ColumbiaDepartment of Medical Genetics, Faculty of Medicine, The University of British ColumbiaDepartment of Medical Genetics, Faculty of Medicine, The University of British ColumbiaAbstract Background Genetic counselling for adolescents necessitates an approach distinct from that used with adults. Developing best practices is crucial, considering the growing number of disabled adolescents worldwide and increasing use of genomic testing early in life. We investigated perceptions of adolescents (10–19 years) who had been diagnosed with a genetic disorder in terms of how they describe receiving, understanding, and living with a genetic condition. We undertook a cross-sectional, qualitatively oriented mixed methods study underpinned by the pragmatic paradigm. Adolescents completed two self-report measures – the psychological adaptation scale (PAS) and the illness identity questionnaire (IIQ)—and participated in semi-structured interviews. Demographic, PAS, and IIQ data were analyzed using descriptive statistics. We used phronetic iterative analysis to interrogate interview data. Qualitative and quantitative components were integrated through abduction. Results Eighteen participants (median age: 15.5 years; 11/18 women/girls; 13/18 typical cognition; 8/18 de novo presentation) with a variety of genetic conditions participated. Participants had a mean PAS of 3.07 ± 0.84 indicating adequate adaptation. Their IIQ profiles indicated slightly better mean adaptive scores (3.10 ± 1.06) than mean maladaptive scores (2.85 ± 0.99). We developed a conceptual model that describes disability and genetic identity development and psychological adaptation among participants composed of three interacting components: internalizing processes; variability arising from contextual factors; and external factors associated with the processes. Adolescents generally moved among four internalizing processes (initiating, minimizing, exploring, and accepting). Movement across these processes took place frequently because of contextual factors like setting and disability type. Communication and engagement with caregivers, peers, and healthcare professionals, social interactions with others who have the same or a similar condition, and the impact of ableism constituted the main external factors with which adolescents engaged in the development of these identities. Conclusions Our findings present a foundation upon which to develop a care model optimized for the needs of adolescents with genetic conditions. Enhancing access to genetic counselling as a means of facilitating identity development is an important component of these care models.https://doi.org/10.1186/s13023-025-03968-xAdolescentsDisability identityGenetic conditionsGenetic counsellingPsychological adaptation
spellingShingle Tasha Wainstein
Cyrus Boelman
Connie Ens
William T. Gibson
Kevin Gregory-Evans
Olubayo U. Kolawole
Sheila K. Marshall
Kathryn Selby
GenCOUNSEL Study
Alison M. Elliott
Jehannine Austin
Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services
Orphanet Journal of Rare Diseases
Adolescents
Disability identity
Genetic conditions
Genetic counselling
Psychological adaptation
title Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services
title_full Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services
title_fullStr Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services
title_full_unstemmed Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services
title_short Identity development and adaptation in adolescents with genetic conditions: a qualitatively oriented mixed-methods study to develop strategies for optimizing clinical genetics services
title_sort identity development and adaptation in adolescents with genetic conditions a qualitatively oriented mixed methods study to develop strategies for optimizing clinical genetics services
topic Adolescents
Disability identity
Genetic conditions
Genetic counselling
Psychological adaptation
url https://doi.org/10.1186/s13023-025-03968-x
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