Pediatric intestinal failure management and rehabilitation strategies
With Dr. Stephanie Bachi de Castro Oliveira & Dr. Paul Wales · hosted by Lizzy Lee, PA-C
Chapter 1 of 5
IF basics & rehab
Intestinal Failure Definition, Causes, and Rehabilitation Fundamentals
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Dr. Stephanie Bachi de Castro OliveiraWhat the experts said
Intestinal failure is defined as the reduction of functional intestinal mass below 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 dismotility disorders.
Most causes of short bowel syndrome in pediatrics occur during the neonatal period, including gastroschisis, necrotizing enterocolitis, bowel atresias, 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 patients 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 are important factors that enhance intestinal adaptation.
Intestinal rehabilitation strategies to avoid transplant include enteral nutrition, parenteral nutrition, medication, surgical procedures, feeding therapy, and central line locks.
Enteral autonomy is defined as getting off TPN with all nutrition going into 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 the number of transplants and deaths significantly decreased.
The establishment of multidisciplinary teams is one factor that changed outcomes in intestinal failure.
Predictors of reaching enteral autonomy include residual small bowel length (more bowel is better), follow-up at an institution with an intestinal rehabilitation program, and no STEP procedure.
Patients should be referred to intestinal rehabilitation programs when they are not making progress, are on prolonged TPN, or have significant comorbidities, and referral should occur 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 there are irreversible complications.
The number of intestinal transplants has significantly decreased over the last several decades due to multiple interventions available to avoid transplantation.
Liver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (seen in older children, adolescents, and adults), with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or need for transplantation.
Risk factors for intestinal failure-associated liver disease include patient factors related to prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections, sepsis (which affects the liver directly and indirectly), 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 that intestinal rehabilitation programs improve survival related to liver dysfunction, decrease septic episodes, reduce central line complications, reduce ICU admissions, and enable patients listed for transplant to come off the list due to improved status.
Mortality in intestinal failure has decreased with intestinal rehabilitation programs.
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 grams per kilogram per day, there is a 3% increase in the odds ratio of developing advanced liver disease.
First-generation lipid emulsions were soybean-based lipids rich in omega-6 polyunsaturated fatty acids, exemplified by Intralipid, which has been in use for 40 years.
Third-generation lipids attempt to reduce the omega-6 component 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, and stimulating improved beta oxidation and clearance.
The main goals of improving lipids in parenteral nutrition are lipid minimization and change in composition.
Literature dating back decades shows that restricting exposure to soybean lipid improves cholestasis when lipid is dosed at 1 gram per kilogram per day or less.
In pre-term 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 critical care settings.
Dr. Wales' team is currently working with industry to create a lipid emulsion specifically tailored to pre-term 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.
There are approximately 240,000 central line-associated bloodstream infections (CLABSIs) in the United States annually.
The cost of one CLABSI is approximately $30,000, totaling about $2 billion per year nationwide.
When ethanol became too expensive and difficult to obtain, Dr. Wales' team pivoted to 4% tetrasodium EDTA (Kitelock), which is antithrombolytic, anti-fibrinolytic, and antimicrobial.
Kitelock satisfies three criteria important for a good lock solution: addressing organisms, clot thrombosis, and biofilm.
Kitelock is licensed in Europe and Australia but not available in the United States.
A multi-center randomized trial of Kitelock versus Heparin launched four weeks ago and is actively recruiting patients, with results expected within the next year.
In short bowel syndrome, there are three anatomical subtypes: type one is high output jejunostomy; type two involves loss of distal small bowel (ileum) and part of colon with small bowel-colonic anastomosis; type three involves loss of mid small bowel with retained ileum and intact colon.
The most common anatomical subtype of short bowel syndrome in pediatrics is type two.
Type one and type two short bowel syndrome patients struggle because they lack an ileum.
Native GLP-2 hormone has a half-life of seven minutes, making it impractical for clinical use.
Teduglutide is a GLP-2 analog with one amino acid alteration that has a half-life of two hours and is given once daily by subcutaneous injection.
In a multi-center phase three study, patients who received Teduglutide showed a 40% reduction in TPN fluid and calorie requirements over a six-month period.
70% of patients who received Teduglutide achieved the study endpoint of a 20% reduction in TPN requirements.
Teduglutide is licensed for children greater than one 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 better than Teduglutide, producing more bowel lengthening.
These interventions (optimizing lipids, preventing line infections, and using peptide therapies) have led to a decrease in transplantations since 2008, largely because of the successes of intestinal rehabilitation.
The outcomes of intestinal transplant at five years are approximately 65%.
The goal is to avoid transplant and lifelong immunosuppression whenever possible.