“An urgent need for transition of care programs for adolescents and young adults with neurogastroenterology and motility disorders”

1 |. INTRODUCTION

Pediatric Neurogastroenterology and Motility disorders (NGM) are complex, prevalent, and often debilitating conditions with substantial health care burden that negatively impact quality of life (QoL),¹ deeply influencing a child’s physical and mental development. Children with congenital or acquired NGM disorders including esophageal and gastric motor disorders, aerodigestive disorders, pediatric intestinal pseudo-obstruction (PIPO), anorectal malformations, Hirschsprung disease and disorders of the gut-brain interaction (DGBIs), benefit from specific transition focus depending on the condition.² As NGM disorders affect children of all ages and extend into adulthood, the transition of adolescents and young adults (AYA) to adult care is a crucial moment in managing these chronic conditions. Improved understanding, medical treatment and availability of newer diagnostic, therapeutic and surgical techniques have improved the prognosis of pediatric NGM disorders, prolonging survival, thus making the transition to adulthood a timely and imperative endeavor to undertake despite a notable paucity of NGM transition programs across North America.

The transition from the pediatric to the adult care can be a challenging time, not only for patients, but also their parents and caretakers, not to mention their health care team, including the adult NGM experts. Delayed transition leads to worse outcomes, unpreparedness and difficulty settling into an independent care model in adulthood. Incomplete and hasty transition processes can result in dissatisfaction for patients and health care personnel, worse symptoms, and potentially increased hospitalizations during the transition phase. The organization of transition is a dynamic process, aiming at ensuring continuity, coordination, flexibility, and sensitivity in a multidisciplinary context, to meet the adolescent’s biopsychosocial and educational needs to enhance clinical outcomes.

Multiple factors lead to delayed or inefficient transition of AYA with NGM disorders. Some of these include a lack of clear guidance for transition of care, coupled with difficulties in gauging readiness, absence of NGM specific validated transition of care tools and shortages of NGM experts. Developmental competence is an important milestone achieved during adolescence and is necessary to be deemed, “transition ready.” It is important to acknowledge that neurodivergence and varying developmental abilities can impact this. Youth experience increased distress during transition. However, psychometrically validated transition ready tools are limited and not widely used to streamline this process. Pediatric programs care for children up to 21 years of age and sometimes longer for those with complex medical conditions (i.e., NGM disorders), developmental and behavioral needs. This creates a turmoiled phase between 18 and 21 years of age where there is a higher influence of psychosocial factors on Gl symptoms and sometimes secondary gains from hospitalizations for patients with DGBI.³ Adolescents also go through numerous changes such as moving away for college and changing medical insurance coverage, and these factors need to be taken into consideration in optimizing timing of transition and selection of adult care personnel.⁴ Due to lack of availability and resources or being lost to follow up, patients with NGM disorders are sometimes plugged back in general gastroenterology or primary care practices rather than specialty clinics.⁴ This could often lead to confusion with diagnosis, repetitive diagnostic testing and add to patient and family frustration.

Various transition of care models, detailed in the literature, cater to the unique needs of patients with special needs and chronic diseases requiring multidisciplinary care.^5,6 Robust evidence underscores superior outcomes associated with these models, involving diverse health care personnel such as specialty and primary care physicians, nurses, psychologists, dietitians, and social workers.

There are multiple standardized approaches for assessing a patient’s transition readiness. The Transition Readiness Assessment Questionnaire (TRAQ), a 29-point patient-reported measure intricately designed to gauge transition readiness across domains of self-management and self-advocacy, emerges as a valuable tool.⁷ Rigorously scrutinized, TRAQ demonstrates applicability across the vast spectrum of chronic conditions. Other available questionnaires for assessing readiness include the UNC TRxANSITION Index, the AM I ON TRAC questionnaire, and disease-specific instruments such as the Readiness for Adult Care in Rheumatology (RACER).⁷

There is convincing evidence supporting the effectiveness of an earlier and meticulously structured transition of care model for chronic disease conditions. A comprehensive systematic review, exploring outcomes like hospital readmissions, mortality rates, health care costs, and QoL metrics, unequivocally affirms the positive impact of a judiciously executed transition, particularly in terms of health care cost savings.⁸ Several studies focused on AYA with inflammatory bowel disease (IBD) underscore the positive influence of early, structured transition protocols on QoL. Notably, a strong correlation has been shown between the TRAQ and Short IBD Questionnaire (SIBDQ) scores, elucidating their impact on emotional well-being and social activity limitations.⁹ In a comparative study evaluating transition versus transfer of care models in IBD, the structured transition approach exhibited considerable advantages. This included a noteworthy reduction in surgical complications, enhanced economic benefits through decreased hospitalizations, and superior sexual health outcomes when compared to the transfer model.¹⁰ These findings highlight the pivotal role of early, systematic transitions in fostering optimal health care outcomes for young individuals with IBD, emphasizing the need for comprehensive strategies to address both medical and psychosocial aspects in the transition process. Similarly structured transition of care models in other conditions such as cystic fibrosis also show better outcomes and increase ability to cope with disease in adulthood.¹¹

Published survey data documents special considerations and barriers of care AYA with chronic pain syndromes face as they transition to adult experts.⁴ These focused on helping AYA develop transition-readiness skills and feeling adequately supported by their pediatric and new adult experts during this process. Similarly, AYA with NGM disorders not only require an understanding of their diagnoses and the medical system, but also must be able to have intact management strategies and a level of acceptance and understanding of the expected fluctuations in symptoms that will allow them to appropriately advocate for themselves when meeting new adult experts. They must also have access to experts with experience in managing complex, multi-factorial diagnoses requiring interdisciplinary management approaches in non-judgmental or stigmatized fashions. Patients with PIPO may also have an ileostomy, gastrojejunostomy tubes and parenteral nutritional dependance making the transition process more complex as adult primary care and general gastroenterologists may not be comfortable managing these. There are a variety of approaches that can be used to educate adolescents about their diagnoses, navigating the health care system as an adult, and self-advocacy. Overall, offering structured educational opportunities has demonstrated benefit in other chronic diseases- whether this is through remote technological approach, or directed personalized education in transition clinic models.¹²

We propose a transition model which emphasizes pediatric and adult NGM expert collaborative efforts and patient transition-readiness (Figure 1). This layered transition process, starting as early as 12 years old with patient tracking and transition readiness, allows AYA to develop independent decision-making abilities. Later in the transition process, the pediatric and adult NGM experts have an opportunity to discuss the patient while both are briefly technically managing care. This process culminates with the transfer of care to adult providers. We recommend a somewhat flexible model which considers patient age as well as developmental stage, health and nutrition status, transition-readiness, and adult health care services needed, with the goal for transition completion by 21 years of age. At the time of transition, AYA should be prepared to manage their care, and pediatric NGM expert team should ensure that patient’s health and nutritional status are optimized. Pediatric experts should provide a summary document that includes relevant Gl studies (including imaging, motility testing, endoscopies with pathology results), as well as a list of previous and current medications (including side effects), a list of prior interventions [i.e., abdominal surgeries, feeding tube placements and/or removals, Botox injections, total enteral nutrition initiations, long-term intravenous access placement and/or removal, total parenteral nutrition initiation, etc.].

FIGURE 1.

Transition of care model for patients with NGM disorders. Created with BioRender.com. NGM, neurogastroenterology and motility.

AYA with NGM often have complex and broad health care utilization and needs, which may need to be provided in a different structure in the adult health care system. Some of these are outlined in Figure 2. For example, while the pediatric NGM expert may provide centralized care, with an in-clinic psychologist and dietitian, within the adult health care model, the internist may provide centralized care with consultation support from an adult psychologist specialized in chronic Gl symptoms, dietitian, and adult NGM expert. Understanding how local adult NGM experts are prepared to meet each AYA’s medical needs and educating the patient about these differences is vital for successful transfer completion.

FIGURE 2.

Special considerations for AYA with NGM disorders transitioning to adult care. AYA, adolescent and young adult; Gl, gastrointestinal; NGM, neurogastroenterology and motility.

2 |. CALL TO ACTION

1. Currently, there are no well-developed NGM transition programs in North America.

2. There is an urgent need to establish streamlined care models based on efficient partnerships and communication between pediatric and adult NGM experts that could be adopted nationally and globally.

3. Transition care models include: Shared clinics between pediatric and adult NGM experts, or serial clinics with written handoffs.

4. Clear criteria for transition readiness and involved steps in transition should be outlined to AYA and their families in a timely manner. This ensures sufficient time to navigate important decisions related to this process.

5. Outcomes for transition success include tracking number of patients “lost to follow up,” patient and family satisfaction, emergency department visits, university and/or work attendance, and QoL scores.

6. Integrating an effective model allows for timely and efficient transition of NGM patients that would set them up for success and improve outcomes in the long run.

Key Points.

• Pediatric neurogastroenterology and motility (NGM) disorders are common, negatively impact quality of life (QoL) and daily functioning and often continue into adulthood.

• Thus, structured transition of care programs for adolescents and young adults with pediatric NGM disorders are urgently needed.

• Successful establishment of transition of care programs should lead to decreased hospitalizations, improved follow up with NGM experts, and improved QoL and daily functioning measures, such as work or school attendance.

Abbreviations:

AYA

adolescents and young adults

DGBIs

disorders of the gut-brain interaction

Gl

gastrointestinal

IBD

inflammatory bowel disease

NGM

neurogastroenterology and motility disorders

QoL

quality of life

TEN

total enteral nutrition

TPN

total parenteral nutrition

TRAQ

The Transition Readiness Assessment Questionnaire