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Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.
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. Akademiska sjukhuset, sektionen för barnkirurgi. (Barnkirurgisk forskning)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health. (Barnneurologi/Barnonkologi)
Akademiska sjukhuset, Folke Bernadotte regionhabilitering.
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. Akademiska sjukhuset, sektionen för barnkirurgi. (Barnkirurgisk forskning)
2018 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227Article in journal (Refereed) Epub ahead of print
Abstract [en]

AIM: Knowledge on the neurodevelopmental and physical function in children with vertebral defects, anorectal malformations, cardiac defects, tracheo-oesophageal fistula, renal and limb malformations (VACTERL) is scarce. We evaluated Swedish preschool children with VACTERL and identified whether they would need extra support in school.

METHODS: From 2015 to 2017, we recruited children aged 5-7 with VACTERL association from the paediatric surgical centre at the University Children's Hospital at Uppsala. Neurodevelopmental function was assessed by age-appropriate intelligence and visual and auditory attention tests, and the children's behaviour and attention were observed by an experienced psychologist. Physical function was evaluated through parental interviews and examinations. Data on patient characteristics, including any surgery and anaesthesia, were extracted from medical records.

RESULTS: Of the 13 eligible families, 10 agreed to participate. Intelligence was within the normal range for all children, but attention difficulties were found in eight of the children, requiring adjustments at school, and two of these were later diagnosed with attention deficit hyperactivity disorder. All children had physical dysfunctions that affected their daily nutrition, bowel or bladder functions.

CONCLUSION: Attention difficulties and physical dysfunction were common in Swedish preschool children aged 5-7 with VACTERL and they would need support and adjustments when they started school.

Place, publisher, year, edition, pages
2018.
Keywords [en]
Attention difficulties, Comorbidities, Congenital malformations, Neurodevelopmental function, Physical dysfunction
National Category
Pediatrics
Identifiers
URN: urn:nbn:se:uu:diva-371838DOI: 10.1111/apa.14566PubMedID: 30187514OAI: oai:DiVA.org:uu-371838DiVA, id: diva2:1274629
Available from: 2019-01-02 Created: 2019-01-02 Last updated: 2019-08-21Bibliographically approved
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

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Kassa, Ann-MarieDahl, MargaretaEngstrand Lilja, Helene

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