Interdisciplinary transition of care for congenital gastrointestinal malformations: analysis of a standardized program

Introduction

Transition of care (TOC) programs are mandatory to ensure the continuity of care as pediatric patients with chronic and complex conditions have to be transferred to adult healthcare systems. Research shows that up to 40 % of pediatric patients are lost to follow-up during this transitional phase, but structured programs can reduce this figure to as low as 10 % [1]. Despite advances in neonatal surgery, up to 85 % of esophageal atresia (EA) patients [2], [3], [4] and one-third of anorectal malformation (ARM) patients [5], [6], [7], [8] continue to experience significant complications, affecting their quality of life and mental health. Thus, in patients with rare congenital malformations such as EA (incidence 1:3.000–5.000 live births [9], 10]), anorectal malformation (incidence 1:3.000–5.000 live births [11]), and Hirschsprung’s Disease (HD, incidence 1:5.000 live births [12]) and persistence of chronic sequelae into adulthood, the need for such programs is evident [13], [14], [15], [16].

Despite an increasing recognition of the importance of transitional care over the last two decades, there is still a lack of standardization and evidence [17], [18], [19]. While a structured transition to adult healthcare for children with congenital heart disease has already been recommended by the American Heart Association in 2011 [20], standardization for children with congenital gastrointestinal malformations has yet to be addressed in many places [21], [22], [23]. Recent findings from a survey involving the EUPSA Network Office, ERNICA, and eUROGEN revealed that, despite awareness of the importance of TOC programs, today, they are implemented in less than 50 % of the participating centers. Furthermore, there is substantial heterogeneity in the content and timing of these programs [19].

In our institution we have implemented an interdisciplinary standardized TOC program for patients affected by congenital gastrointestinal malformations since 2014. The aim of this study was to evaluate the efficacy of our interdisciplinary transitional care program using dedicated questionnaires that assess patients’ well-being, gastro-intestinal quality of life, transition competence and subjective patient satisfaction with the TOC program, indicating its efficacy.

Materials and methods

Study design

This prospective observational study evaluated the effects of our interdisciplinary TOC-program for patients with congenital gastrointestinal malformations on their autonomy and quality of life. The study was approved by our local ethics committee (reference number 10817_BO_K_2023).

Our interdisciplinary TOC-program involved regular outpatient clinic visits with a multidisciplinary team, including pediatric surgeons, adult gastroenterologists with expertise in pediatric endoscopy, nutrition experts, and child and adolescent psychologists. During these visits, the patient’s medical status, quality of life, transition competence, and satisfaction with the transition process were documented (Figure 1).

Figure 1:

Structure of our TOC-program.

Patients with congenital gastrointestinal malformations, including ARM, Hirschsprung’s Disease, and EA who received their primary or secondary treatment at Hannover Medical School (MHH) and participated in regular follow-up care were included in the interdisciplinary transition care program during adolescence (ages >14 years). Additionally, adolescents with gastrointestinal malformations who were not part of any regular follow-up care but voluntarily chose to participate after referral from family doctors or support groups were also included.

Results

Demographics and contact to transition (Tables 1a, 1b and 2)

Of 202 patients enrolled in the TOC-program, 63 participated to the survey. Table 1a outlines their demographics at the first visit. Among participants, 63.4 % (n=40) were ≥18 years old, and 61.9 % (n=39) had EA (out of which n=28 isolated). Additionally, 47.6 % (n=30) had colorectal malformations, with 11 having both conditions. Surgical interventions are listed in the Supplementary Material (Table 1b). Most participants were referred by the department of pediatric surgery or from parent support groups (Table 2).

Table 1a:

Patients’ demographics. Age of the patients at start of transition of care. n=number of patients

Age at start of transition (in years)
Age	<14	14–15	16–17	18–19	>19	Sum
EA (n)	0	3	6	6	13	28
ARM/HD (n)	1	6	3	6	3	19
Combined (n)	0	1	2	6	2	11
Other (n)	0	1	0	3	1	5
Overall (n)	1	11	11	21	19	63

Table 2:

Contact to transition care program. MHH=Hannover medical school.

Specialists and diagnostic work-up during transition (Tables 3a and 3b)

During the study period as part of the transition program, patients received different types of specialists’ support and diagnostic work-up, depending on their symptoms and needs.

Patients received predominantly support from general (69.8 %) and pediatric (31.8 %) practitioners, along with internal medicine specialists (20 %) (Table 3a).

Among 63 patients (108 visits), diagnostic endoscopies were the most common procedure (68 instances, 69.8 %). Seven patients required corrective surgery, and 50 cases needed consultations with other medical subspecialties (Table 3b).

WHO-5 (Figure 2)

Regarding the WHO-5 well-being index, 59 out of 63 patients returned the questionnaire during their first visit to our program. The WHO´s scores were significantly lower as compared to healthy controls (15.92, SD 4.52 vs. 18.36, SD: 4.80; p=0.00011). Two patients (1.18 %) scored less than seven points, falling in the category “depression most likely”. Figure 2. A detailed division per entity may be found in the Supplement Material (Table 4).

Figure 2:

Box-plot with WHO-5 graphical representation. X=average score; _= median score.

During the second visit to the TOC-program, only 20 out of 59 patients returned the questionnaire. Since the sample size was small (n<30), the Kolmogorov-Smirnov test was used to ensure the data represented the general population. The test confirmed a normal distribution (critical value=0.294, t=0.166).

These 20 patients reached an average of 16.65 points (SD: 3.84) at their first visit, slightly below healthy controls, but the difference was not statistically significant (p=0.061). On the second visit, their average score decreased to 16.45 (SD: 4.89) (p=0.097). No significant change of WHO5-scores occurred between first and second visit (p=0.857). Only patients with combined morbidity showed an improvement between first and second visit, however not statistically significant (details in Supplement Table 4).

GIQLI (Figure 3 and Table 5)

At the first visit, 60 patients returned their GIQLI scoring in average significantly lower compared to healthy controls (106.23, SD:24.34 vs. 125.8, SD:13.0; p<0.00001). (Figure 3). A detailed division per pathology is visible in the Supplementary Material (Table 5).

Figure 3:

Box-plot with graphical representation of the GIQLI score. X=average score; _= median score.

At the second visit, 20 patients returned the GIQLI, they had normal distribution (KS-test: critical value=0.294, t=0.096). These patients scored an average of 112.55 points (SD: 17.38) at the first and 111.55 (SD: 23.09) at the second visit. GIQLI was in both visits significantly lower than healthy controls (respectively p=0.0036 at the first and p=0.0125 at the second visit). No significant changes have been revealed between first and second visit (p=0.849).

TCS (Figure 4)

At the first visit, 60 patients completed the TCS, averaging 27.43 out of 44 points (SD: 9.82), significantly lower than healthy controls (p<0.00001). At the second visit, 20 patients returned the TCS questionnaire, and normal distribution was confirmed (critical value=0.294, t=0.151). For these patients the TCS score improved significantly from 27.35 (SD: 10.40) to 31.80 (SD: 7.73) points (p=0.01509), demonstrating the educative impact of the transition care program (Figure 4).

Figure 4:

Box-plot with graphical representation of the transition competence scale (TCS). Considering the overall visits of the patients an increasing improvement in subjective patients’ competence could be observed, with statistical significance between first and second vist. X=average score; _= median score *=p<0.05 n.s.=non statistical significance.

ZAP (Figure 5)

54 ZAP questionnaires were evaluable. Compared to reference data for “organization,” “information,” “personal competence,” and “interaction,” our TOC-program was scored highest in “interaction” (93.1 %, 21.95/24 points, SD: 4.77) and “organization” (91.3 %, 8.22/9 points, SD: 1.04). “Professional competence” scored 86.1 % (5.07/6 points, SD: 1.33), while “information” scored lowest at 81.3 % (19.27/24 points, SD: 4.48). Categories without reference comparisons were “trust” (87.65 %, 2.58/3 points, SD: 0.71) and “quality of treatment” (85.8 %, 2.53/3 points, SD: 0.78). Overall satisfaction was 59.62/69 points (SD: 9.07). Our TOC-program achieved significantly higher satisfaction-scores than in the literature available reference groups [27] (p<0.00001).

Figure 5:

Diagram illustrating satisfaction with the program. Each category was compared to similar assessments described in the literature. A value of 100 % represent the maximum possible score for each category. Satisfaction scores in category “Interaction” and “Organisation” were statistically significant better compared to ZAP-scores for other programs (respectively p=0.015 and p=0.0003) *= p < 0.05 and **= p< 0.001 [27].

Discussion

TOC-programs are crucial in preventing the loss of follow-up of pediatric patients during their transfer to adult care, reducing the risks associated with unmanaged chronic symptoms and reducing healthcare costs [28], 29].

In our study we identified several key aspects of TOC for patients with gastrointestinal congenital malformations, emphasizing both challenges and achievements. Patients in our cohort reported significantly lower mental health well-being and gastrointestinal quality of life compared to healthy controls. These findings reflect the chronic burden faced by these patients and emphasize the necessity comprehensive transitional care programs to address the complexity of their conditions. Our results are consistent with long standing evidence of low menlat health in this population, according a recent multicentric study, in which patients with ARM and HD were interviewed from 11 to 26 years [30]. The authors reported on a spectrum of challenges extending beyond the pathology itself, including profound physical, mental and emotional impacts, effects on family dynamics, concerns about future relationships and careers [30]. These findings emphasize the several challenges faced by adolescents with congenital gastrointestinal conditions, reinforcing the need for holistic approaches in their care.

Psychological well-being is intricately linked to patients’ overall quality of life, and addressing the complex interplay of physical, mental, and emotional health is necessary. This complexity also enhances the necessity of integrating psychologists as core members of interdisciplinary care teams. Evidence from other studies also supports this approach, pointing to the benefits of a multidisciplinary model in improving patient outcomes [31].

In our TOC- program, we involved an interdisciplinary team, led by a pediatric surgeon and an adult gastroenterologist, the program brings together specialists from various fields to address patients’ diverse medical, surgical and psychological needs. This approach fosters coordinated decision-making and comprehensive care, essential for optimizing outcomes in patients with complex conditions [32]. Although not statistically significant, patients with combined conditions such as EA and ARM showed a promising trend toward improved well-being and gastrointestinal quality of life. These findings suggest that structured, multidisciplinary support programs may be beneficial particularly for patients. However, the limited scope of our analysis, which included only the first two visits and a relatively small number of patients completing the questionnaires, likely contributed to these modest results. Moreover, the integration of psychological care continues to represent a systemic challenge, as emerged from both our findings and the broader studies [33]. Despite national guidelines, such as those in the United States, emphasizing the importance of addressing psychosocial and developmental needs, psychologists are still not consistently integrated into TOC teams [34]. Expanding psychological support within the transition framework is crucial to address the unmet emotional and mental health needs of this population [41].

Our TOC-program demonstrated a significant improvement in patients’ transition competence, with measurable gains in their ability to manage healthcare and navigate adult medical systems between the first and second visit. These results suggest that the program can improve essential self-management skills; however, they must be interpreted with caution, as no statistically significant changes were observed in psychological well-being or gastrointestinal quality of life. Furthermore, our patients expressed high satisfaction with the program, particularly in areas such as interaction, organization, and the structured, patient-centered approach. Finally, although patient satisfaction with our program was high, claims of its superiority over other transition models require additional comparative evidence. Notably, the existing programs have not been directly evaluated using standardized scales or questionnaires. However, the category of “information” received the lowest scores, pointing to a need to improve communication strategies and ensure that patients fully understand their conditions and care plans.

An important element supporting the program’s effectiveness was the early implementation of an interdisciplinary, structured approach, as suggested from north American society of Pediatric Gastroentorology, Hepatology and Nutrition [35]. In our program we additionaly complemented by the involvement of patient and parent support groups. Close interdisciplinary collaboration ensured that adult medicine teams were already familiar with patients’ medical histories from childhood, which in turn facilitated the establishment of a dedicated transition clinic for adolescents during the transition phase. Furthermore, in recent years, contact with patients/parents support groups has become significantly more important for both patients and their parents. The goal of these organizations is to improve treatment quality providing patients and families peer connections, education, and resources to navigate the transition process [36], 37]. Approximately one-quarter of our cohort of patients was referred through patients/parents support groups, emphasizing the fundamental role in disseminating information about the program and creating awareness about the importance of transitional care [38], 39]. These groups not only help patients gain independence but also assist families [40] in adapting to their new roles during the transition [39], 41], ultimately improving outcomes and reducing stress [42].

Despite the implementation of a standardized transition program represents an important initiative, several limitations of our study warrant consideration. Over a 10-year period, 63 of 202 eligible patients participated, and only 20 completed follow-up assessments. This small sample size, corresponding to approximately six patients per year and two patients per year with longitudinal follow-up, necessarily limits the generalizability of our findings. Consequently, any conclusions regarding the overall benefit of the program should be interpreted with caution. Notably, the possibility of selection bias must be acknowledged. It remains unclear whether patients who did not return for follow-up visits were highly satisfied with their initial care and therefore felt no need to continue participation, or conversely, whether they disengaged due to dissatisfaction. Differentiating between these groups was not feasible in the present study.

Furthermore, patients invited from other centres may have had different backgrounds and previous medical experiences, contributing to a mixed starting point for all patients at the beginning of the program. These limitations evidence the complexity of evaluating transition programs and point up the challenges in interpreting our findings.

Conclusions

We presented the outcomes and patient perceptions of the first standardized transition of care program for individuals with congenital gastrointestinal malformations in Germany. Our findings suggest improvements in patients’ transition competence and high levels of satisfaction with the program’s supportive and patient-centered approach. However, given the small sample size and limited follow-up data, these results should be interpreted with caution. While the study emphasizes the potential benefits of structured transition care and the need for broader implementation of standardized programs, further research with larger cohorts and longer follow-up is recomended.