Reference Models for Children and Young People with Epidermolysis Bullosa

Epidermolysis bullosa (EB), also known as Butterfly skin, is a term for a heterogeneous group of rare genodermatosis disorders resulting in painful mucocutaneous blisters and extreme fragility with minor trauma or friction. The low prevalence and heterogeneity of EB, clinically classified into 4 maj...

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Detalhes bibliográficos
Autores: Shin Kang, Ju Young, Gimenez-Llort, Lydia|||0000-0002-4091-489X
Formato: artículo
Fecha de publicación:2022
País:España
Recursos:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:308234
Acesso em linha:https://ddd.uab.cat/record/308234
https://dx.doi.org/urn:doi:10.3390/IECBS2022-12939
Access Level:acceso abierto
Palavra-chave:Reference models
Epidermolysis Bullosa (EB)
Rare diseases
Expert patient
Dystrophic epidermolysis bullosa (DEB)
Storytelling
Successful cases
Education
Coping strategies
Family and social support
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spelling Reference Models for Children and Young People with Epidermolysis BullosaFirst Case Report on the Challenges, Rewards, and Limiting Factors for Successful Academic and Professional AchievementsShin Kang, Ju YoungGimenez-Llort, Lydia|||0000-0002-4091-489XReference modelsEpidermolysis Bullosa (EB)Rare diseasesExpert patientDystrophic epidermolysis bullosa (DEB)StorytellingSuccessful casesEducationCoping strategiesFamily and social supportEpidermolysis bullosa (EB), also known as Butterfly skin, is a term for a heterogeneous group of rare genodermatosis disorders resulting in painful mucocutaneous blisters and extreme fragility with minor trauma or friction. The low prevalence and heterogeneity of EB, clinically classified into 4 major groups and over 30 subtypes, lead to significant psychological and social research constraints. An inverse correlation between disease severity and quality of life in patients and their families has been described. Psycho-social aspects in people with more severe subtypes of EB and body dysmorphia refer to loss of self-esteem and coping skills, low self-control, intense fear, anxiety, depression, and social isolation. On the other hand, acute, chronic, and procedural pain, common comorbidities in EB, can negatively affect cognition and interfere with academic and professional achievements. Here, we present the first case report of a current international project that searches for reference models for children and young people with EB and the creation of a guidebook with different life stories of strategies and skills proven to help achieve academic/professional success despite EB's condition. We analyze the semistructured interview with Sonia Ortiz Romero, alias 'Mariposa de cristal', an expert patient with dystrophic EB and co-founder of 'Son mis alas, I.A.P' ('They are my wings, NGO'), together with his brother Marcos Humberto. Her storytelling illustrates challenges, rewards, and limiting factors that make their Mexican association a successful case in helping other EB patients in Santiago Tianguistenco with their physical, psychological, and social well-being, as well as raising social awareness.Universitat Autònoma de Barcelona. Departament de Psiquiatria i de Medicina Legal 22022-01-0120222022-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/308234https://dx.doi.org/urn:doi:10.3390/IECBS2022-12939reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:3082342026-06-06T12:50:31Z
dc.title.none.fl_str_mv Reference Models for Children and Young People with Epidermolysis Bullosa
First Case Report on the Challenges, Rewards, and Limiting Factors for Successful Academic and Professional Achievements
title Reference Models for Children and Young People with Epidermolysis Bullosa
spellingShingle Reference Models for Children and Young People with Epidermolysis Bullosa
Shin Kang, Ju Young
Reference models
Epidermolysis Bullosa (EB)
Rare diseases
Expert patient
Dystrophic epidermolysis bullosa (DEB)
Storytelling
Successful cases
Education
Coping strategies
Family and social support
title_short Reference Models for Children and Young People with Epidermolysis Bullosa
title_full Reference Models for Children and Young People with Epidermolysis Bullosa
title_fullStr Reference Models for Children and Young People with Epidermolysis Bullosa
title_full_unstemmed Reference Models for Children and Young People with Epidermolysis Bullosa
title_sort Reference Models for Children and Young People with Epidermolysis Bullosa
dc.creator.none.fl_str_mv Shin Kang, Ju Young
Gimenez-Llort, Lydia|||0000-0002-4091-489X
author Shin Kang, Ju Young
author_facet Shin Kang, Ju Young
Gimenez-Llort, Lydia|||0000-0002-4091-489X
author_role author
author2 Gimenez-Llort, Lydia|||0000-0002-4091-489X
author2_role author
dc.contributor.none.fl_str_mv Universitat Autònoma de Barcelona. Departament de Psiquiatria i de Medicina Legal
dc.subject.none.fl_str_mv Reference models
Epidermolysis Bullosa (EB)
Rare diseases
Expert patient
Dystrophic epidermolysis bullosa (DEB)
Storytelling
Successful cases
Education
Coping strategies
Family and social support
topic Reference models
Epidermolysis Bullosa (EB)
Rare diseases
Expert patient
Dystrophic epidermolysis bullosa (DEB)
Storytelling
Successful cases
Education
Coping strategies
Family and social support
description Epidermolysis bullosa (EB), also known as Butterfly skin, is a term for a heterogeneous group of rare genodermatosis disorders resulting in painful mucocutaneous blisters and extreme fragility with minor trauma or friction. The low prevalence and heterogeneity of EB, clinically classified into 4 major groups and over 30 subtypes, lead to significant psychological and social research constraints. An inverse correlation between disease severity and quality of life in patients and their families has been described. Psycho-social aspects in people with more severe subtypes of EB and body dysmorphia refer to loss of self-esteem and coping skills, low self-control, intense fear, anxiety, depression, and social isolation. On the other hand, acute, chronic, and procedural pain, common comorbidities in EB, can negatively affect cognition and interfere with academic and professional achievements. Here, we present the first case report of a current international project that searches for reference models for children and young people with EB and the creation of a guidebook with different life stories of strategies and skills proven to help achieve academic/professional success despite EB's condition. We analyze the semistructured interview with Sonia Ortiz Romero, alias 'Mariposa de cristal', an expert patient with dystrophic EB and co-founder of 'Son mis alas, I.A.P' ('They are my wings, NGO'), together with his brother Marcos Humberto. Her storytelling illustrates challenges, rewards, and limiting factors that make their Mexican association a successful case in helping other EB patients in Santiago Tianguistenco with their physical, psychological, and social well-being, as well as raising social awareness.
publishDate 2022
dc.date.none.fl_str_mv 2
2022-01-01
2022
2022-01-01
dc.type.none.fl_str_mv Article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://ddd.uab.cat/record/308234
https://dx.doi.org/urn:doi:10.3390/IECBS2022-12939
url https://ddd.uab.cat/record/308234
https://dx.doi.org/urn:doi:10.3390/IECBS2022-12939
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Dipòsit Digital de Documents de la UAB
instname:Universitat Autònoma de Barcelona
instname_str Universitat Autònoma de Barcelona
reponame_str Dipòsit Digital de Documents de la UAB
collection Dipòsit Digital de Documents de la UAB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,81155

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