Advances in pediatric intestinal failure management and rehabilitation
With Dr. Stephanie Bachi de Castro Oliveira & Dr. Paul Wales · hosted by Lizzy Lee, PA-C
Chapter 1 of 6
IF definition & rehab
Defining Intestinal Failure and Intestinal Rehabilitation
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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Dr. Stephanie Bachi de Castro OliveiraWhat the experts said
Intestinal failure is defined as reduction of functional intestinal mass below that which can sustain life, resulting in dependence on supplemental parenteral support for a minimum of 60 days within a 74 consecutive day interval.
More than 50% of intestinal failure cases are related to short bowel syndrome, with additional cases from mucosal enteropathies and dysmotility disorders.
Most short bowel syndrome cases in pediatrics occur during the neonatal period from gastroschisis, necrotizing enterocolitis, bowel atresia, and intestinal volvulus.
The field is now focusing on neurocognitive outcomes, quality of life, and caregiver burnout as more children survive intestinal failure.
The cause of chronic intestinal inflammation in intestinal failure survivors is not known.
The goal of intestinal rehabilitation is to optimize intestinal function and adaptation before the development of irreversible complications.
Intestinal adaptation is an active compensatory process that starts immediately after bowel resection.
Food, bacteria, and hormones in the gut enhance intestinal adaptation.
Enteral autonomy is defined as getting off TPN with all nutrition going through the gut, either by mouth or by feeding tube.
From 2010 to 2015, a repeat study with double the number of patients showed approximately 50% still reached enteral autonomy, but transplant rates and death rates significantly decreased.
Establishment of multidisciplinary teams is one factor that changed outcomes in intestinal failure.
Predictors of reaching enteral autonomy include greater residual bowel length, follow-up at an institution with an intestinal rehabilitation program, and no STEP procedure.
Patients should be referred to intestinal rehabilitation programs when not making progress, on prolonged TPN, or have significant comorbidities—sooner rather than later.
Patients should not be referred to intestinal rehabilitation programs when several complications have already occurred and transplant is the only remaining option.
Intestinal transplantation is considered when irreversible complications occur, but the number of transplants has significantly decreased over the last several decades.
Liver disease in intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation.
Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections/sepsis, and components of TPN.
The term 'intestinal rehabilitation program' was coined in the year 2000.
Intestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity.
Literature shows intestinal rehabilitation programs improve survival related to liver dysfunction, decrease septic episodes, reduce central line complications, reduce ICU admissions, and allow patients listed for transplant to come off the list due to improved status.
Lipids in parenteral nutrition are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways.
Historically, most lipid emulsions in North America are soybean-based with high phytosterol content, high omega-6 long-chain polyunsaturated fatty acid content, and low antioxidant content.
Prolonged soybean-based lipid exposure is associated with deterioration of liver dysfunction, specifically cholestasis.
For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g/kg/day, there is a 3% increase in the odds ratio of developing advanced liver disease.
First-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (intralipid), available for 40 years.
Third-generation lipid emulsions reduce omega-6 and change the omega-6 to omega-3 ratio by adding omega-3 lipids.
Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile.
Omega-3 lipids are metabolized through EPA and DHA and lead to production of cytokines with a less inflammatory profile.
Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta-oxidation and clearance, lowering oxidative stress, and supporting immune function.
Literature dating back decades shows that restricting soybean lipid exposure to 1 g/kg/day or less improves cholestasis.
In preterm babies, fat is important for growth, especially neurocognitive development, and there is risk of essential fatty acid deficiency if lipid dosing is restricted too much.
DHA and arachidonic acid are important for retinal and brain development.
None of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings.
Dr. Wales's team is working with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content.
Central lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported.
Central lines carry risks of complications including infections, blockages, thrombosis, and breakage.
In the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, costing about $30,000 per infection, totaling approximately $2 billion per year nationwide.
4% tetrasodium EDTA (Kitelock) is an antithrombotic, anti-fibrinolytic, and antimicrobial lock solution that satisfies all three criteria important for a good lock solution.
Kitelock is licensed in Europe and Australia but not available in the United States.
A multi-center randomized trial of Kitelock versus heparin launched 4 weeks ago and is actively recruiting patients, with results expected within the next year.
In short bowel syndrome, there are three anatomical subtypes: Type 1 (high jejunostomy), Type 2 (loss of distal small bowel/ileum and part of colon with small bowel-colonic anastomosis), and Type 3 (loss of mid-small bowel with retained ileum and intact colon).
Type 2 short bowel syndrome is the most common anatomical subtype in pediatrics.
Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum.
Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use.
Teduglutide is a GLP-2 analog with one amino acid alteration that has a half-life of 2 hours and is given once daily by subcutaneous injection.
Patients receiving teduglutide show a 40% reduction in TPN fluid and calorie requirements over a six-month period.
70% of patients receiving teduglutide achieved the study endpoint of a 20% reduction in TPN requirements.
Teduglutide is licensed for children greater than 1 year of age.
Teduglutide has enabled children with very extreme anatomy to get off TPN.
Apraglutide is another GLP-2 analog with a longer half-life that can be given once weekly and is currently being studied.
Apraglutide works even better than teduglutide, producing more bowel lengthening.
These interventions (lipid optimization, central line protection, GLP-2 analogs) have led to a decrease in transplantation since 2008, largely because of the successes of intestinal rehabilitation.
Five-year outcomes of intestinal transplant are approximately 65%.
The goal is to avoid transplant and lifelong immunosuppression whenever possible.
Chronic intestinal inflammation resembling Crohn's disease is now seen in intestinal failure patients and requires management with inflammatory bowel disease therapies like biologics.
A 2012 Pediatric Intestinal Failure Consortium study showed 50% of intestinal failure patients achieved enteral autonomy, while 25% either died or had intestinal transplant.
Studies from Canada, Michigan, and Spain show that multidisciplinary intestinal rehabilitation teams improve survival.
Second-generation lipid emulsions attempt to reduce omega-6 exposure by diluting the soybean component.
Omega-6 fatty acids promote inflammation while omega-3s lead to a more anti-inflammatory response.
In utero, babies receive significant amounts of DHA and arachidonic acid through the placenta, but standard lipid emulsions in TPN fall short of this.