This nationwide Swedish study is its first of its kind and revealed that 65% of children with LGEA utilized school-based support, reflecting their multifaceted daily needs in nutritional intake situations, education and medical management.
In this study, 42% of children with LGEA were reported to use “special” support in school, which was requisite of formal decision from the principal. “Special” support consisted of educational support to large extent. In comparison, 5.8% of the pupils in the Swedish compulsory school had an action plan as a part of “special” support in school[29]. Furthermore, the parents’ reports of their children’s school-based accommodations also reflected educational support. The proportion of children with LGEA who received educational support seems higher than in Swedish pupils in general and in earlier reports of children with EA which have been described that 22%-35% access special education[17–19]. In relation to other pediatric surgical malformations, 52% of children with anorectal malformations and 55% of children with Hirschsprung’s disease have been reported to receive special education and remedial teaching[37]. However, the definitions for “special” educational support and age of the study populations vary between studies[19].
Fifty-six percent of children with LGEA were recipients of school-based accommodations, all of whom had needed support regarding their nutrition intake. In our study, all children with LGEA and height ≤ 2SD for child age/sex at follow-up required this support. Growth retardation may be associated with weakened cognitive performance[38], in turn posing the need also for educational support. In this study, several children with LGEA needed school-based supports related to education/learning and nutritional intake. It is known that children with LGEA risk developing long-term esophageal complications[6, 10, 11, 39]. In agreement, we found more of them to having had antireflux treatments, esophageal dilations and nutritional intake support in school as opposed to children with PA who were expected to suffer from milder complications. This together implies that esophageal morbidity contributes to great care needs.
Furthermore, we observed that children with LGEA needed school-based support in relation to tube feeding and medical management, which can be provided based on the Swedish Act concerning Support and Service for Persons with Functional Impairments[40]. Parents may have described overlapping information to the questions of school-based “special” support and accommodation. Nevertheless, it together provides a more comprehensive understanding of their needs.
Special education in children with EA has been described to be more common in children with concomitant anomalies[19, 20], such as cardiovascular anomalies[21] or anorectal malformations[20]. In other studies, children with LGEA have been found to more commonly present with cardio-vascular malformations[7, 8] and genetic disorders[7] compared to short-gap EA with PA. However, in this study, prematurity and low birth weight, which are interrelated, were more commonly present. Low birth weight, even controlled for confounders, is associated with lower intelligence in the general population[41] and has earlier been shown to independently predict use of educational support in children with EA[19]. Moreover, the associated factors related to school-based support in children with LGEA were digestive and respiratory symptom burden, suggesting that there is a more disease burdened subgroup with multiple daily needs. In this study, use of school-based accommodations in children with LGEA was also related to young child age, but this was not found regarding “special” support. According to the Swedish definition, it could mean that children with LGEA at younger age needs less interventional support by ordinary school staff, which have not yet been included in an action plan with additional school resources and decided upon by the principal. However, school-based accommodations included support in nutritional intake issues in all cases. Previous research of coping abilities in children with EA[42] has shown that the child’s ability to deal with challenging nutritional intake situations increases with age, as their need for additional adult support at meals decreases. This could therefore help to explain our findings.
In this study, most parents reported to have received necessary school-based support for their child and almost all children with LGEA with abnormal levels of mental health according to SDQ were recipients of school-based support. In comparison to the known challenges children living with a rare disease may generally have in accessing inclusive and quality education[16, 43], our results could be viewed as encouraging. Perhaps this is attributable to the Swedish school systems/laws which provide these children right to school-based support[26–28]. The national follow-up care program for children with EA, does not yet formally include a standardized provision of psychological consultation to the child[44], but the pediatric surgeon can mandate the child to have school-based support, which seems to work adequately in most cases of children with LGEA in Sweden.
We found that 36% of children with LGEA were absent from school ≥ 1times/month the past year (equivalent to at least 12 times the past year). This concurs with previous studies showing that children with chronic conditions experience frequent periods of school absenteeism[45, 46]. In contrast to earlier studies suggesting school absenteeism to be related to disease severity[46], we found that high frequency of school absence did not differ between children with LGEA and PA. However, in agreement, children with LGEA were more frequently absent from school due to colds/airway infections and GI-specific morbidity. Both restrictive and obstructive lung dysfunction are observed as common in Swedish children with LGEA[14], which could contribute to these study findings. Respiratory morbidity is very common at young age[25, 39] and in this study school absence also correlated to young child age, suggesting an interrelationship. Knowledge of school absence in children with LGEA is important to recognize, as it has been described by parents of children with EA to impair social integration with peers and school achievement[19] and it is an independent factor that negatively influences family functioning[34].
We found that children with LGEA did not differ in scores of the school functioning domain in the PedsQL 4.0 compared to children with PA. This may be due to small sample size in LGEA, but it is also possible the provided school-based support has increased the level of school functioning. In comparison with other studies, children with chronic conditions have been found to functioning worse in school regarding peer relationships, academic achievement, and lack of engagement in school[45–47]. Previous research of children treated in advanced pediatric surgery has focused on academic achievement and found that a subgroup of children with gastroschisis (complex gastroschisis)[48], and children with congenital diafragmatic hernia (those with oxygen at discharge and longer initial hospital stay) perform worse academically[49]. In the whole group of children with EA, Burnett et al.[50] found a mixed picture regarding school functioning, with attention and working memory regarded as most vulnerable areas. Considering PedsQL 4.0 as a summative indicator of school functioning[32], we found that the subgroup of LGEA children with aerodigestive symptoms and those with school-based support risk poorer school functioning. It is possible that they need more intensive support in school than they currently receive. As LGEA is a congenital condition, it may be important to consider that children hospitalized for a chronic condition as early as during the preschool period are at risk of academic underperformance as they progress in school[51, 52].
Furthermore, most parents in this study rated their child’s school satisfaction as good, which was important as we earlier have found elevated levels of peer relationship problems in 31% of children with LGEA[22].
Study limitations
The study is limited by the small sample, heterogeneity related to anatomical subtypes, associated anomalies and surgical methods as well as by the number of non-participants in LGEA. The study population has a broad age-span, incorporating data from preschool children to upper secondary school. However, given the small sample size despite our nationwide recruitment of children with LGEA, investigations into narrower age subgroups would not be statistically feasible. Moreover, there is no standardized definition of problematic school absence, and we defined a cutoff for high frequency of school absence which had been previously used[19, 34]. Although we complied with terminology utilized by the Swedish National Agency for Education[28], the school questions, apart from those in the PedsQL 4.0, were not part of a valid scoring instrument and we collected data from parents. This study did not provide information on LGEA children’s academic achievement, total amount of time absent from school or compared school data with a general control group. The data on PA children were collected during a different time period.