Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
Until recently, there was no standardized definition for intestinal failure.
guidelinePaul Wales1:56 ↗
Intestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.
clinicalPaul Wales2:03 ↗
New guidelines define intestinal failure as inadequate intestinal function requiring parenteral support for at least 60 days.
Host summaryRod Gerardo summarizing the discussion — not the host's own clinical position2:24 ↗
Earlier recognition and taking advantage of the gut's adaptive biology are time dependent.
clinicalMichael Helmrath2:40 ↗
An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and other associated needs, as defined in recent ASPEN guidelines.
guidelinePaul Wales3:03 ↗
Intestinal rehabilitation requires managing comorbidities including sepsis and liver disease that challenge infant growth.
Host summaryRod Gerardo summarizing the discussion — not the host's own clinical position3:33 ↗
Intestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.
clinicalPaul Wales3:44 ↗
Intestinal rehabilitation depends on key factors: nutrition in the gut, nutrition in the body, and healing.
clinicalMichael Helmrath4:07 ↗
Pattern recognition from multiple expert eyes observing a patient over time is essential in intestinal rehabilitation.
clinicalMichael Helmrath4:19 ↗
Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients.
epidemiologicalPaul Wales4:50 ↗
Causes of short bowel syndrome are usually related to neonatal disorders including congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) or acquired diseases (necrotizing enterocolitis).
clinicalPaul Wales4:50 ↗
Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves controlling that muscle prevent coordinated propulsion of food and stool, making patients dependent on intravenous support.
clinicalPaul Wales5:45 ↗
Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work, leading to hypersecretion and profuse fluid losses.
clinicalPaul Wales6:17 ↗
Some patients will have elements of two or all three categories of intestinal failure; for example, gastroschisis can involve short bowel, inflammation affecting absorption, and motility issues.
clinicalPaul Wales7:11 ↗
Although most intestinal failure patients are infants or babies, older pediatric patients can develop intestinal failure from inflammatory bowel disease, Crohn's disease complications, trauma, malignancy, or vascular thrombosis leading to gut loss.
clinicalPaul Wales7:45 ↗
Families reach intestinal rehabilitation programs at three time points: prenatal diagnosis (typically atresia with cystic fibrosis or gastroschisis), postnatal acquired problems (volvulus or NEC), and later diagnosis after discharge when patients fail to progress.
clinicalMichael Helmrath8:53 ↗
Innovation comes from multiple approaches to a problem and different visions; bringing in more expertise leads to better outcomes.
opinionMichael Helmrath10:15 ↗
Transitioning older children with intestinal failure into their late teen years and beyond is a major obstacle currently not being met.
clinicalMichael Helmrath10:51 ↗
The intestinal rehabilitation team includes dietitians (understanding nutritional needs), social work, nurse practitioners, pharmacy, interventional radiology, pathology, and subspecialties including endocrinology and nephrology.
clinicalMichael Helmrath11:52 ↗
Overall survival in big intestinal rehabilitation programs is usually over 90% long-term.
Host summaryRod Gerardo summarizing the discussion — not the host's own clinical position12:34 ↗
Improved survival has revealed chronic comorbidities not previously seen to the same extent because patients did not live long enough, including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life concerns.
clinicalPaul Wales12:46 ↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
Adrian Bianchi first reported the longitudinal intestinal lengthening procedure (Bianchi procedure) that divides bowel along its two leaves and tubularizes it.
clinicalMichael Helmrath1:23 ↗
In the 1980s and 1990s, babies with intestinal failure had poor outcomes primarily due to liver progression to inflammation and fibrosis associated with parenteral nutrition, lipids, and phytosterols.
Host summaryEllen Encisco summarizing the discussion — not the host's own clinical position1:50 ↗
Rising direct bilirubin was recognized as a sign that children with intestinal failure would not do well.
clinicalMichael Helmrath2:13 ↗
The first 4 months of life is when care for intestinal failure patients is most uncoordinated, and surgical decisions made during this period have the most profound effect on long-term outcome.
Host summaryTodd Ponsky summarizing the discussion — not the host's own clinical position2:29 ↗
The gut doubles in length during the last trimester and the first year of life, and this maturation process occurs when the baby is fed.
clinicalMichael Helmrath2:55 ↗
Healthy growth of the intestine requires nutrition; anything that disrupts this affects maturation of both absorptive/digestive capacity and peristalsis.
clinicalMichael Helmrath3:03 ↗
Absorptive surface area comes from the waves of peristalsis moving over the villi, not from the exposed length of bowel.
Host summaryTodd Ponsky summarizing the discussion — not the host's own clinical position3:32 ↗
Lengthening bowel that doesn't have peristalsis does not increase absorption; very short bowel children can sometimes come off TPN because their motility is good.
clinicalMichael Helmrath3:46 ↗
The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.
epidemiologicalPaul Wales4:18 ↗