Short Bowel Syndrome: Transition of Pediatric Patients to Adult Gastroenterology Care

The transition of pediatric short bowel syndrome (SBS) patients to an adult gastroenterology (GI) practice presents substantial challenges to patients, their families, and their providers.¹ Although many of these challenges are shared by young patients with other chronic diseases, some are unique to the SBS patient population. There are few studies of the effect of transition on the long-term outcomes of pediatric SBS patients. In this review, we will identify the problems faced during transition and propose some potential solutions to provide a framework for successful management by adult gastroenterologists in a typical office setting.

Challenges for Transition to Adult Care

• A.
  General challenges:

  • 1.
    Ensuring timely follow-up:

    • Avoiding gaps in care is critical for pediatric patient transition to adult care. For example, a study of congenital heart disease (CHD) patients showed that 42% of patients with CHD have gaps in care of more than 3 years.^2,3 This study also reported a high incidence of anxiety and mood disorders in CHD.¹ Recognition of this problem led to the development of a 2-year fellowship in subspecialty care of adults with CHD, as it became clear that formalized physician training was needed to provide the best care for these patients. Kyrana et al⁴ found in a survey study that one-third of responding practitioners noted “psychological problems” in their adolescent/young adult transition intestinal failure patients.
  • 2.
    Finding appropriate adult practitioners:

    • For many pediatric patients with complex chronic diseases, finding the appropriate physicians for follow-up may be difficult, especially for patients in rural areas. The framework is not in place in most institutions for identifying practitioners who have the required experience and who are willing to begin caring for transitioning young adult patients. This has been problematic for young adult patients with CHD and we have observed the same for patients with SBS. Just as each CHD patient may be unique,² so may each SBS patient. In many geographic areas, there is a shortage of adult medical providers with expertise in managing patients with SBS.⁵ A study by Mundi et al⁵ found that most providers that prescribe parenteral nutrition (PN) prescribed it for less than 4 patients. Many patients traveled over 100 miles to see their provider.
    • Often, the onus is on the patient and their family to find the adult practitioners, which can be a daunting task. Also, parents may become less involved as their child approaches adulthood, leaving the responsibility to the emerging adult. Allergy-immunology subspecialty leaders recognized these issues, particularly for pediatric patients with immune disorders, and the American Board of Allergy and Immunology requires fellows to cross-train in pediatric and adult care. Programs for transition for patients with asthma and allergies are under investigation internationally and a position paper by the European Academy of Allergy and Clinical Immunology has just been published.⁶
  • 3.
    Ensuring adherence with medications and evaluation (testing such as blood work, imaging, etc.).

    • The patient has previously attended medical visits with family members and may now either be expected to or wish to become independent from family during doctor visits.
  • 4.
    Moving out of a supportive home environment:

    • New responsibilities for self-care can be overwhelming as patients leave for college or move out of the home. They may face new financial challenges due to loss of insurance coverage from their parents and also be unprepared for being responsible for taking/refilling their medications.
• B.
  Challenges for transition of care specific to SBS:

  • 1.
    Patients dependent on PN:

    • The family is familiar with a large team approach for care and the patient has likely been seen in a tertiary center, especially if PN-dependent or recently weaned off PN (surgeon, dietitian, nurse, physician assistant/medical doctor, pharmacist, social worker). Depending on where the patient lives, they may transition to an adult GI office which is not part of an SBS team and/or has little experience in SBS care.
    • It may be difficult to find an adult GI provider who is willing to accept an SBS young adult, especially if PN-dependent.
    • The patient and family are used to very frequent follow-up in the pediatric GI office as well as in the general pediatric office, and quick access to pediatric surgeons if needed (eg for PN line replacement, g-tube issues, etc.) This is often not the practice pattern in adult care, with reliance on the patient to phone in or message when needed in place of frequent, regularly scheduled appointments.
    • Social services/social work support is not generally available in an adult GI clinic.
    • If dependent on PN, the patient and family have generally received weekly calls from pharmacists and dietitians as orders are reviewed each week. If insurance has changed, these caregivers may also change in concert with a change in the PN provider and home health company.
    • If needed, the pediatric emergency room environment is generally supportive; adult emergency rooms are usually larger and if at a tertiary care center, may be much busier and intimidating for the patient and family.
    • Details of original surgery/surgeries may be difficult to find and present anatomy, bowel length, etc., may be unknown or unclear.
  • 2.
    Patients dependent on PN and/or enterally fed:

    • New responsibilities for self-care can be overwhelming as patients leave for college or move out of the home. They may face new financial challenges due to loss of insurance coverage from their parents and also be unprepared for being responsible for taking/refilling their medications.
    • Dietary guidelines and restrictions especially for patients who are newly weaned or who have a colon in continuity may be difficult to follow, in the absence of parental supervision. Financial restraints may also limit food choices and may result in increased ingestion of fast food which is higher in fat and lower in general quality.

Plan for Transition With Participation of Pediatric and Future Adult Providers: (a “Do it Yourself” Office Model)

• 1.Identify a provider:

• There should be a structured handoff between the pediatric gastroenterologist and adult gastroenterologist. Ideally, this would be a joint appointment with both providers. However, billing issues may make this arrangement difficult for some centers. Telehealth options make this more feasible. If possible, this should be attended by all team members from pediatrics (nursing, dietitian, social worker, and surgeon if still actively involved). At a minimum, a written summary of the medical history and treatment course should be provided to the accepting physician (see below). A UK study found that patients with inflammatory bowel disease who underwent a structured transition from pediatric to an adult gastroenterologist had fewer disease flares and emergency room visits.⁷

• 2.Transition appointments for adult MDs for all other medical problems including pending, recent (unsolved) issues.
• 3.Surgical summary note:

• Record details of all surgeries/surgical records prior to transition (pediatric surgical records may be very old and hard for the adult provider to obtain)

• 4.
  Generate a transition summary note to include the following:

  • a.
    Amount of remaining small bowel/colon in continuity/present bowel pattern and weight/when was last use of PN/what complications from PN or from SBS has this patient experienced/recent liver function tests and last imaging studies.
  • b.
    All active problems, pending evaluations, and present medications.
  • c.
    Summary of medications used for diarrhea that have failed as well as present medication regimen/use of teduglutide in the past. If no teduglutide for PN patients, include an explanation for why this had not been considered for patients who have not yet weaned.
  • d.
    Complete set of nutritional labs prior to transfer to avoid care gaps.
• 5.Identification of cultural and linguistic diversity and appropriate resources for robust support.
• 6.Engage present social worker to identify a social worker in the adult arena who can provide continuity of care.
• 7.Consider use of technology including electronic notifications and use of video visits to increase frequency and rigor of follow-up.
• 8.Identify and communicate full insurance information.
• 9.Inform patients about support groups that may help facilitate the patient’s transition to adult care.

• These include The Oley Foundation, shortbowelsyndrome.com, shortbowelfoundation.org and for patients with inflammatory bowel disease, Crohn’s and Colitis Foundation ccfa.org.

For additional information from national guidance and guidelines for transitions: See https://www.gottransition.org/six-core-elements/

Six core elements have been identified as required for a successful transition of care (see www.gottransition.org for details). This website describes a general process for both pediatric and adult offices to prepare patients, families, and providers for transition of care.

The 6 core elements include (1) Developing a transition of care policy/guide; (2) Develop a process for identifying young adults in transition (tracking and monitoring); (3) Transition readiness for pediatric office/orientation to adult practice for adult office; (4) Transition planning for pediatric office/integration into adult practice for adult office; (5) Transfer of care (pediatric office)/initial visits for adult office; and (6) Transfer completion (pediatrics)/ongoing care (adult practice).

Additional educational resources can be found at https://www.gottransition.org/resources-and-research/clinician-education-resources.cfm.

Future Plans: Ensure a Successful Transition

• 1.
  Support specific, special training in health care transitions during residency/ fellowship:

  • Combined medicine/pediatrics programs have developed a program with 20 hours of formal training in issues of transition planning. Consider cross-training pediatric and adult care of SBS during GI fellowship to expand the knowledge base of future practitioners.
• 2.
  Consider establishing a center for healthcare transition⁸

  • At Lurie Children’s Hospital in Chicago, a center for health care transition has been established which coordinates all aspects of the patient’s care as transition occurs. They are not a primary care clinic, but a referral clinic staffed by an medical doctor and a social worker. They provide a holistic assessment and plan for the patient to ensure successful transition, including social issues such as financial security, education, employment, housing, and family readiness and skills to participate in care for the patient.