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Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience

BACKGROUND: CLN3 disease is a neurodegenerative condition presenting in the first decade of life typically leading to death in the third decade. The earliest symptom is rapidly progressive visual impairment followed by intellectual and motor impairments, epilepsy and behavioural disturbances. There...

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Autores principales: Krantz, Mattias, Malm, Emma, Darin, Niklas, Sofou, Kalliopi, Savvidou, Antri, Reilly, Colin, Boström, Petra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9541062/
https://www.ncbi.nlm.nih.gov/pubmed/35233790
http://dx.doi.org/10.1111/cch.12993
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author Krantz, Mattias
Malm, Emma
Darin, Niklas
Sofou, Kalliopi
Savvidou, Antri
Reilly, Colin
Boström, Petra
author_facet Krantz, Mattias
Malm, Emma
Darin, Niklas
Sofou, Kalliopi
Savvidou, Antri
Reilly, Colin
Boström, Petra
author_sort Krantz, Mattias
collection PubMed
description BACKGROUND: CLN3 disease is a neurodegenerative condition presenting in the first decade of life typically leading to death in the third decade. The earliest symptom is rapidly progressive visual impairment followed by intellectual and motor impairments, epilepsy and behavioural disturbances. There are limited data on how the condition affects the family system or the role of family resilience in paediatric neurodegenerative diseases. METHODS: Semi‐structured interviews were conducted with eight parents (five mothers and three fathers) of five children with CLN3. Interview questions focused on the experience of having a child with CLN3, its impact on the family system as well as the concept of family resilience. Data were analysed via thematic analysis. RESULTS: The thematic analysis resulted in four main themes. The theme ‘recurring losses’ included the feeling of losing a healthy child, the child's loss of abilities and loss of relationships. The theme ‘disruption to the family system’ included that siblings could be ‘side‐lined’, the potential negative impact on romantic relationships and difficulties finding time to oneself. The theme ‘Society is not developed for a progressive disease’ highlighted the difficulties parents faced with respect to contacts with the health and/or social insurance system. The paediatric health care system was seen as supportive, but the adult health care system was not seen as fit for the purpose. Regarding family resilience, parents felt that the disease forced them to reconsider what was important in life. Several parents described that they learned to value small moments of joy and create deep connections through involvement in family routines and rituals. CONCLUSIONS: CLN3 places a very significant burden on the family system including parental feelings of loss, impact on family relationships and lack of understanding within the health/social insurance systems. The concept of family resilience may be useful in understanding the experiences of families affected by paediatric neurodegenerative conditions.
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spelling pubmed-95410622022-10-14 Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience Krantz, Mattias Malm, Emma Darin, Niklas Sofou, Kalliopi Savvidou, Antri Reilly, Colin Boström, Petra Child Care Health Dev Research Articles BACKGROUND: CLN3 disease is a neurodegenerative condition presenting in the first decade of life typically leading to death in the third decade. The earliest symptom is rapidly progressive visual impairment followed by intellectual and motor impairments, epilepsy and behavioural disturbances. There are limited data on how the condition affects the family system or the role of family resilience in paediatric neurodegenerative diseases. METHODS: Semi‐structured interviews were conducted with eight parents (five mothers and three fathers) of five children with CLN3. Interview questions focused on the experience of having a child with CLN3, its impact on the family system as well as the concept of family resilience. Data were analysed via thematic analysis. RESULTS: The thematic analysis resulted in four main themes. The theme ‘recurring losses’ included the feeling of losing a healthy child, the child's loss of abilities and loss of relationships. The theme ‘disruption to the family system’ included that siblings could be ‘side‐lined’, the potential negative impact on romantic relationships and difficulties finding time to oneself. The theme ‘Society is not developed for a progressive disease’ highlighted the difficulties parents faced with respect to contacts with the health and/or social insurance system. The paediatric health care system was seen as supportive, but the adult health care system was not seen as fit for the purpose. Regarding family resilience, parents felt that the disease forced them to reconsider what was important in life. Several parents described that they learned to value small moments of joy and create deep connections through involvement in family routines and rituals. CONCLUSIONS: CLN3 places a very significant burden on the family system including parental feelings of loss, impact on family relationships and lack of understanding within the health/social insurance systems. The concept of family resilience may be useful in understanding the experiences of families affected by paediatric neurodegenerative conditions. John Wiley and Sons Inc. 2022-03-04 2022-09 /pmc/articles/PMC9541062/ /pubmed/35233790 http://dx.doi.org/10.1111/cch.12993 Text en © 2022 The Authors. Child: Care, Health and Development published by John Wiley & Sons Ltd. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Articles
Krantz, Mattias
Malm, Emma
Darin, Niklas
Sofou, Kalliopi
Savvidou, Antri
Reilly, Colin
Boström, Petra
Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
title Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
title_full Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
title_fullStr Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
title_full_unstemmed Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
title_short Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
title_sort parental experiences of having a child with cln3 disease (juvenile batten disease) and how these experiences relate to family resilience
topic Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9541062/
https://www.ncbi.nlm.nih.gov/pubmed/35233790
http://dx.doi.org/10.1111/cch.12993
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