Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes
With Dr. Stephanie Oliveira & Dr. Paul Wales · hosted by Dr. Lizzie Lee
Cued at 4:56 · stops at 5:41 · press play
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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What the experts said
Intestinal failure is defined as reduction of functioning 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 some mucosal enteropathies and dysmotility disorders
Most causes of short bowel syndrome in pediatrics happen during the neonatal period, including gastroschisis, necrotizing enterocolitis, bowel atresia, and intestinal volvulus
The field is starting to focus on neurocognitive outcomes, quality of life, and caregiver burnout
Chronic intestinal inflammation resembling Crohn's disease is now seen in intestinal failure patients, requiring management with inflammatory bowel disease therapies like biologics
The cause of chronic intestinal inflammation in intestinal failure patients is not known
Intestinal adaptation is an active compensatory process that starts happening right after bowel resection
Food, bacteria, and hormones in the gut are important for enhancing intestinal adaptation
Enteral autonomy is achieved when a patient gets off TPN and has all nutrition going to the gut, either by mouth or by feeding tube
A 2012 Pediatric Intestinal Failure Consortium report showed 50% of intestinal failure patients achieved enteral autonomy, and 25% either passed away or had an intestinal transplant
From 2010 to 2015, a repeat study with double the number of patients showed about 50% reached enteral autonomy, but the number of transplants and deaths significantly decreased
The establishment of multidisciplinary teams changed outcomes in intestinal failure
Studies from Canada, Michigan, and Spain show that a multidisciplinary intestinal rehab team improves survival
Predictors of reaching enteral autonomy include greater residual bowel length, follow-up at an institution with an intestinal rehabilitation program, and no-step procedure
The number of intestinal transplants has significantly decreased over the last several decades
Liver disease in intestinal failure ranges from mild cholestasis to profound steatosis in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis
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
Intestinal rehab programs improve outcomes through integration of care, improved communication, and better continuity
Literature shows improvement of outcomes with intestinal rehab programs including improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and patients coming off transplant lists
Lipids 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
Soybean-based lipid emulsions have 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 per kilo per day, there is a 3% increase in the odds ratio of developing advanced liver disease
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
Restricting soybean lipid exposure improves cholestasis when dosed at 1 g per kilo per day or less
In preterm babies, fat is important for growth, especially for 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 a critical care setting
There are approximately 240,000 central line-associated bloodstream infections in the United States annually, with each costing about $30,000, totaling about $2 billion per year nationwide
4% tetrasodium EDTA (kite lock) is an antithrombotic, anti-fibrinolytic, and antimicrobial that satisfies all three criteria important for a good lock solution
Kite lock is licensed in Europe and Australia but not available in the United States
A multi-center randomized trial of kite lock versus heparin went live 4 weeks ago and is actively recruiting patients
In short bowel syndrome, there are 3 anatomical subtypes: Type 1 is high jejunostomy, Type 2 has lost distal small bowel and ileum with small bowel-colonic anastomosis, and Type 3 has lost mid-small bowel but retains some ileum and intact colon
Patients with Type 3 short bowel syndrome have the best prognosis because they still have residual ileum
The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2
Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum, which is where GLP-2 is naturally produced
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
Apraglutide is another GLP-2 analog with a longer half-life that can be given once a week and works even better than teduglutide, producing more bowel lengthening
These interventions have led to a decrease in transplantation since 2008, largely because of the successes of intestinal rehabilitation
Outcomes of intestinal transplant at 5 years are about 65%