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Living with VACTERL association: From the perspectives of children, 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.
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 [en]
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: urn:nbn:se:uu:diva-389781ISBN: 978-91-513-0731-2 (print)OAI: oai:DiVA.org:uu-389781DiVA, id: diva2:1344481
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
List of papers
1. Young children with severe congenital malformations (VACTERL) expressed mixed feelings about their condition and worries about needles and anaesthesia
Open this publication in new window or tab >>Young children with severe congenital malformations (VACTERL) expressed mixed feelings about their condition and worries about needles and anaesthesia
2017 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 106, no 10, p. 1694-1701Article in journal (Refereed) Published
Abstract [en]

Aim: Our knowledge of the perceptions that children with severe congenital malformations have of their health, treatment and how to improve hospital care is limited. This study focused on patients with vertebral defects, anal atresia, cardiac defects, tracheo-oesophageal fistula, renal anomalies and limb abnormalities (VACTERL).

Methods: We interviewed 10 children aged five to eight years with VACTERL association who were treated in a Swedish tertiary paediatric surgical centre, using a computer-assisted technique called In My Shoes. The interviews were analysed by qualitative content analysis.

Results: The children described their awareness of their health history and said they felt proud but different due to their physical dysfunction. They were happy to visit the hospital to meet familiar staff, but expressed negative feelings about missing normal life. They were afraid of needle-related procedures and not wakening up after anaesthesia. Various ways of coping with difficult situations were expressed, and suggestions to improve hospital care were voiced.

Conclusion: Careful follow-up of these children by multidisciplinary teams is crucial to optimise their health and functional status. Fear of medical procedures may be reduced by carefully delivered information, listening to the children, providing continuity of care and creating individual care strategies.

Place, publisher, year, edition, pages
WILEY, 2017
Keywords
Care strategies, Computer-assisted interview Fear of anaesthesia, Needle-related procedures, Severe congenital malformations
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:uu:diva-334743 (URN)10.1111/apa.13973 (DOI)000409348400024 ()28672091 (PubMedID)
Available from: 2017-11-29 Created: 2017-11-29 Last updated: 2019-08-21Bibliographically approved
2. From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation
Open this publication in new window or tab >>From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation
2019 (English)In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 14, no 4, article id e0215751Article in journal (Refereed) Published
Abstract [en]

Aim Knowledge is scarce regarding mothers' and fathers' experiences of being a parent of a child with VACTERL association-a complex malformation. The aim of the study was to describe experiences of being a parent of a child with VACTERL association. Method Semi-structured interviews were performed with ten mothers and nine fathers face-to-face or by telephone and analyzed by using Qualitative content analysis. Results The parents described crisis reactions at the discovery of malformations in their child. Involvement in care was reported from the initial hospital admission until actively taking responsibility for treatments at home. Eventually the health condition became an integrated part of everyday life. The parents expressed the importance of meeting other families with a child with VACTERL. Descriptions were given of more or less professionalism with perceived discrepancies of knowledge and experience between the healthcare professionals in the tertiary hospital and those in the local hospital. Difficulties in receiving medical support during the initial period at home were described. Furthermore, emotional support and practical arrangements regarding parental accommodation and transportation varied. Conclusion Being a parent of a child with VACTERL association involves crisis, mixed emotional reactions and shared responsibility for the child ' s treatment and care with the professional care providers. Psychological processing, good medical care and support from experts, and peer support from other parents is essential in the parents' struggle to reach self-confidence and adaptation. A care plan with individualized tailored care for each child including a training and support plan for the parents is warranted. To reduce the described discrepancies in knowledge and experience between the local and tertiary hospital, video sessions with the parents and responsible professionals at the local and tertiary hospital could be an appropriate mode of transferring information at discharge and follow up of the child.

Place, publisher, year, edition, pages
PUBLIC LIBRARY SCIENCE, 2019
National Category
Nursing
Identifiers
urn:nbn:se:uu:diva-383190 (URN)10.1371/journal.pone.0215751 (DOI)000465019900049 ()31002700 (PubMedID)
Available from: 2019-07-24 Created: 2019-07-24 Last updated: 2019-08-21Bibliographically approved
3. Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.
Open this publication in new window or tab >>Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.
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.

Keywords
Attention difficulties, Comorbidities, Congenital malformations, Neurodevelopmental function, Physical dysfunction
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-371838 (URN)10.1111/apa.14566 (DOI)30187514 (PubMedID)
Available from: 2019-01-02 Created: 2019-01-02 Last updated: 2019-08-21Bibliographically approved
4. Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents
Open this publication in new window or tab >>Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents
Show others...
(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
Congenital malformations, VACTERL association, Health-Related Quality of Life, psychological well-being, children and adolescents, parents
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:uu:diva-390576 (URN)
Available from: 2019-08-16 Created: 2019-08-16 Last updated: 2019-09-04

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