uu.seUppsala University Publications
Change search
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • ieee
  • modern-language-association
  • vancouver
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf
Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
Insitutionen för kliniska vetenskaper, avd för pediatrik, Sahlgrenska akademin, Göteborgs universitet.
Institutionen för klinisk neurovetenskap, Karolinska Institutet, Stockholm.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Public Health and Caring Sciences. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
Show others and affiliations
(English)In: Article in journal (Refereed) Accepted
Abstract [en]

Purpose: VACTERL association is a rare and complex condition of congenital malformations, often requiring repeated surgery and entailing various physical sequelae. Due to scarcity of knowledge, the study aim was to investigate self-reported Health-Related Quality of Life (HRQoL), anxiety, depression and self-concept in children and adolescents with VACTERL association and self-reported anxiety and depression in their parents.

Methods: Patients aged 8-17 years with VACTERL association and their parents were recruited from three of four Swedish paediatric surgical centres during 2015-2019. The well-established validated questionnaires DISABKIDS, Beck Youth Inventories, Beck Anxiety Inventory and Beck Depression Inventory were sent to the families. Data were analysed using descriptives, t-tests and multivariable analysis. Results were compared with norm groups and reference samples.

Results: The questionnaires were returned by 40 patients, 38 mothers and 33 fathers. The mean HRQoL was M=80.4, comparable to children with asthma (M=80.2) and diabetes (M=79.5). Self-reported psychological well-being was comparable to the norm group of Swedish school children, and was significantly higher than a clinical sample. Factors negatively influencing children’s HRQoL and psychological well-being were identified. The parents’ self-reports of anxiety and depression were comparable to non-clinical samples.

Conclusions: Although children and adolescents with VACTERL association reported similar HRQoL to those of European children with chronic conditions, their psychological well-being was comparable to Swedish school children in general. Nevertheless, some individuals among both children and parents were in need of extra support. This attained knowledge is valuable when counselling parents regarding the prognosis for children with VACTERL association.

Keywords [en]
Congenital malformations, VACTERL association, Health-Related Quality of Life, psychological well-being, children and adolescents, parents
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-390576OAI: oai:DiVA.org:uu-390576DiVA, id: diva2:1343262
Available from: 2019-08-16 Created: 2019-08-16 Last updated: 2019-09-04
In thesis
1. Living with VACTERL association: From the perspectives of children, adolescents and their parents
Open this publication in new window or tab >>Living with VACTERL association: From the perspectives of children, adolescents and their parents
2019 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

VACTERL association is a rare and complex congenital condition often requiring repeated surgery and entailing various physical sequelae. Knowledge is scarce regarding experiences of the health condition and health care, need of support at school, health-related quality of life (HRQoL), and psychological well-being in children, adolescents and their parents.

This thesis aims to investigate various aspects of living with VACTERL association, from the perspectives of children, adolescents and their parents.

Ten children aged five to eight years were interviewed using the computer-assisted interview technique In My Shoes (Study I). They expressed awareness of their health history and felt proud but also different due to physical dysfunction. While happy to meet familiar staff in the hospital they voiced worries about medical procedures.

The nineteen parents interviewed described crisis reactions on the discovery of malformations in their child (Study II). Parental involvement in care was reported from the initial hospital admission until taking responsibility for treatments at home. Eventually the health condition became integrated in everyday life. Insufficient emotional and limited medical support were reported. Various levels of professionalism among healthcare professionals and discrepancies concerning knowledge and experience between the tertiary and local hospitals were described.

In ten evaluated pre-school children (Study III), intelligence measured by Wechsler-scales was within the normal range. Eight children had attention difficulties and two were later diagnosed with attention deficit hyperactivity disorder (ADHD). All children had physical dysfunctions affecting their nutrition, bowel or bladder functions. All needed extra support and adjustments at school.

Forty children and adolescents responded to validated questionnaires of DISABKIDS and Beck inventories (Study IV). The HRQoL was comparable to European children with chronic conditions. Their psychological well-being was similar to that of Swedish school children and significantly better than that of a clinical sample. Self-reported anxiety and depression in 38 mothers and 33 fathers were comparable to non-clinical samples.

In conclusion, regular follow-up by multi-professional team with continuity is crucial to optimise the physical function in children with VACTERL, to identify those in need of extra support at school and to detect reduced psychological well-being in children and parents. Fear of medical procedures may be reduced by carefully providing information and individual care strategies. For the parents psychological processing, support from medical experts and peers is essential to achieve self-confidence and adaptation. Transfer of knowledge and information between multi-professional teams at the local and tertiary hospitals could be improved by the use of video sessions.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2019. p. 72
Series
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 1591
Keywords
Congenital malformations, Experiences of health care, Health-related quality of life, Neurodevelopmental function, Physical dysfunction, Psychological well-being, VACTERL association
National Category
Medical and Health Sciences
Research subject
Pediatric Surgery
Identifiers
urn:nbn:se:uu:diva-389781 (URN)978-91-513-0731-2 (ISBN)
Public defence
2019-10-11, Rosénsalen, entrance 95/96, NBV, Akademiska sjukhuset, Uppsala, 13:15 (Swedish)
Opponent
Supervisors
Available from: 2019-09-19 Created: 2019-08-21 Last updated: 2019-10-15

Open Access in DiVA

No full text in DiVA

Authority records BETA

Kassa, Ann-MarieEngvall, GunnEngstrand Lilja, Helene

Search in DiVA

By author/editor
Kassa, Ann-MarieEngvall, GunnEngstrand Lilja, Helene
By organisation
Pediatric SurgeryDepartment of Public Health and Caring SciencesNeuropediatrics/Paediatric oncology
Medical and Health Sciences

Search outside of DiVA

GoogleGoogle Scholar

urn-nbn

Altmetric score

urn-nbn
Total: 97 hits
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • ieee
  • modern-language-association
  • vancouver
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf