Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents

Abstract

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.

Anxiety and Depression in Parents of Children Born with Esophageal Atresia: An International Online Survey Study

“…A literature review by van Oers et al (2014) found that child's medical characteristics, such as child's age or the severity of illness, were not related to parental anxiety or depression in parents of chronically ill children. However, studies specifically on EA have suggested that child's age (Faugli et al, 2009;Kassa et al, 2020;Witt et al, 2019), EA severity (Witt et al, 2019), associated anomalies (Rozensztrauch et al, 2020), and feeding problems (Dellenmark-Blom et al, 2015;Rozensztrauch et al, 2020) might have an impact on parental mental health or family functioning. The current study partly supported the findings by van Oers et al (2014), as statistically significant differences were not found in the levels of parental anxiety or depression between child's age, or EA complexity sub-groups.…”

“…Similarly, Witt et al (2019) found that the parents of younger children born with EA (aged 2-7 years) reported lower mental health compared to the parents of older children and adolescents. In another study, parents of older children and adolescents (aged 8-17) born with VACTERL association self-reported anxiety and depression at levels that were comparable to a non-clinical sample (Kassa et al, 2020). These findings would suggest that parental mental health might improve over time, whilst the child born with EA grows and develops through childhood into adolescence.…”

Schooling experiences in children with long-gap esophageal atresia compared with children with esophageal atresia and primary anastomosis: a Swedish study

“…In other Swedish studies, children with VACTERL (stands for Vertebral defects, Anal atresia, Cardiac defects, Tracheo-esophageal fistula, Renal anomalies, and Limb abnormalities. Individuals diagnosed with VACTERL association have at least three of these characteristic features) reported psychological well-being which was comparable to the norm group of Swedish school children [ 55 ] and intelligence was within the normal range [ 56 ]. However, attention difficulties were found in eight out of ten preschool children with VACTERL, requiring adjustments at school, and two of these were later diagnosed with attention deficit hyperactivity disorder [ 56 ].…”

Factors of family impact in a Swedish–German cohort of children born with esophageal atresia

“…However, after the multivariable regression analysis, these clinical variables were not retained as a part of the multifactorial model. Previous research has shown that associated anomalies in children with EA negatively impact family functioning [ 24 ] while parents of children with VACTERL association (i.e., with at least three of the following: vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities) self-report levels of anxiety and depression comparable to non-clinical samples [ 38 ].…”