4 TREATMENT APPROACHES TO ADOLESCENT PATIENTS WITH FI AND DGBI
There is no defined pathway for managing DGBI-associated FI. Below is a suggested practical approach to treatment based on current evidence and expert opinion.
4.1 Effective communication and the positive diagnosis
Management begins with providing a positive diagnosis, education, and reassurance as part of demystifying these disorders, allowing patient and family acceptance of the diagnosis, collaborative treatment planning, building prognostic optimism, and enhancing motivation to participate in treatment (Figure
1). Education, including discussing the gut–brain axis and its role in DGBI, is recommended. Validation of the patient's pain and other symptoms is crucial, as physicians consistently underrate functional conditions leading to unintentional symptom intensification.
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4.2 Treatment options and personalization
As DGBI often relates to multiple co-occurring disturbances in both the gut and central nervous system, management should follow these mechanistic approaches and often requires addressing multiple therapeutic targets simultaneously. Treatment should focus on the most impairing symptoms to the patient, and it should generally start with education and dietary and lifestyle modifications and include other pharmacologic or nonpharmacologic strategies. The use of restrictive diets may put patients at risk for disordered eating and are contraindicated when a patient already exhibits disordered eating or an eating disorder.
Psychological therapies, including cognitive behavioral therapy and hypnotherapy, are indicated in patients with co-occurring psychological disorders and have some of the best evidence for the management of adolescent DGBI.
6 The diagnosing clinician, themselves, can promote resiliency and healthy coping as well as address problematic family dynamics. Recommending physical exercise, including stretching and yoga, mindfulness-based therapy, and diaphragmatic breathing, is helpful. Family members can be coached to simultaneously validate the patient's pain yet support expectations surrounding their treatment plan.
Data are lacking in the specific management of adolescent patients with DGBI-associated FI, but treatment to address symptoms, including pain, constipation, diarrhea, nausea, and bloating, may be personalized and helpful to the patient in tolerating nutrition.
7 Such treatments include enteric-coated peppermint, certain probiotics, anti-spasmodic medications, neuromodulators, and neurostimulation. There is a lack of data for anti-spasmodic medications and lacking or mixed data for most neuromodulators, and these are typically considered when psychiatric comorbidity is present. Neurostimulation has moderate evidence for adolescent abdominal pain and can be considered where available. Other treatments, including acupuncture, biofeedback, and aromatherapy, do not have evidence but can be considered based on patient-specific factors. For now, the best practice is an interdisciplinary team approach, partnering a primary care provider with a gastroenterology provider, dietician, and psychologist, and including additional experts as needed.
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4.3 Indications for enteral and PN and defining intestinal failure
There is a lack of guidance as to when and in which adolescent patients with DGBI-associated FI, enteral nutrition, or PN should be considered, as well as a misconception that these patients are experiencing pediatric intestinal failure (PIF). While gastric and jejunal feeding is sometimes used to allow a controlled infusion of nutrition to a patient who is declining oral intake due to symptoms, based on expert experience similar issues of FI can still arise with this approach, including ongoing feeding-related symptoms. This may lead to patients continuing to self-manage symptoms by reducing, pausing, or stopping enteral feeds and receiving inadequate nutrition. It is also important to consider the potential for secondary gain and a perceived tube dependency that may arise.
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PIF is broadly defined as “the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children.
10” The indications for PN in PIF typically include congenital or surgical reduction of the small intestine; neuromuscular GI disorders; and congenital enteropathies, amongst others (Table
1). The role of PN in DGBI-associated FI is not defined. Such patients with chronic FI and without PIF should avoid PN unless other nutrition options, including post-pyloric feeding, have been exhausted, particularly for those who have prognostic factors positively associated with achieving enteral autonomy.
11 Although there is a lack of evidence-based approach, if an adolescent with FI is receiving long-term PN, it is important to consider different treatment modalities, including inpatient hospitalization with the necessary expertise implementing the biopsychosocial care model for feeding advancement and avoiding prolonged hospitalization and over-medicalization when possible.
12 In these cases, it is important that there is alignment between the patient, their family, and an interdisciplinary clinical team (including an eating disorder expert), with shared goals surrounding nutrition and symptom rehabilitation.
Table 1. Indications for parenteral nutrition in pediatric intestinal failure.
Condition Example
Congenital short bowel syndrome Gastroschisis, omphalocele, intestinal atresias, malrotation, and volvulus
Acquired short bowel syndrome necrotizing enterocolitis, meconium peritonitis, inflammatory bowel disease, and traumatic bowel injury
Neuromuscular or gastrointestinal disorder Long segment Hirschsprung's disease, chronic intestinal pseudo-obstruction, dysmotility, and mitochondrial disease
Congenital enteropathies and enterocyte disorders Microvillous inclusion disease, tufting enteropathy, secretory or congenital diarrhea
Malignancies and bone marrow transplantation Radiation enteritis, chemotherapy associated with nausea and gastrointestinal dysfunction, and chronic graft-versus-host disease
Hypermetabolic states Severe burns and trauma