One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution.
You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution.
So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
So, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 23 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 23 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 23 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 23 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 23 · 3:03
guidelineAn 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.↗
▶Ep 23 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 23 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 23 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 23 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 23 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 23 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 23 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 23 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 23 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 23 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 23 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 23 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 23 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 23 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 23 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
▶Ep 23 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 24 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 24 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 24 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 24 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 24 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 24 · 3:03
guidelineAn 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.↗
▶Ep 24 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 24 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 24 · 4:50
clinicalCauses 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).↗
▶Ep 24 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 24 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 24 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 24 · 5:45
clinicalMotility 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.↗
▶Ep 24 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 24 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 24 · 6:17
clinicalEnteropathies 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.↗
▶Ep 24 · 7:11
clinicalSome 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.↗
▶Ep 24 · 7:45
clinicalAlthough 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.↗
▶Ep 24 · 12:46
clinicalImproved 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.↗
guidelineCincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event.↗
▶Ep 4 · 1:09
quoteInstitutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event.↗
▶Ep 4 · 1:20
guidelineA 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event.↗
▶Ep 4 · 2:57
clinicalIn young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition).↗
▶Ep 4 · 3:30
clinicalPrematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors.↗
▶Ep 4 · 3:30
quoteNow, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that.↗
▶Ep 4 · 4:09
clinicalPrevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery.↗
▶Ep 4 · 7:00
clinicalTwo strategies for reversing cholestasis are dose restriction and change of lipid composition.↗
▶Ep 4 · 7:08
clinicalConventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized.↗
▶Ep 4 · 7:19
clinicalSMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver.↗
▶Ep 4 · 7:45
clinicalSMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency.↗
▶Ep 4 · 7:57
clinicalWhen SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency.↗
▶Ep 4 · 8:06
guidelineConventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day.↗
▶Ep 4 · 8:52
opinionA bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold.↗
▶Ep 4 · 8:52
quoteMy opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to.↗
▶Ep 4 · 12:11
clinicalDuring secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot.↗
▶Ep 4 · 12:53
clinicalLiver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits.↗
▶Ep 4 · 13:00
clinicalElastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis.↗
▶Ep 4 · 13:31
clinicalFollow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made.↗
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 112 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 112 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 112 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 112 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 112 · 3:03
guidelineAn 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.↗
▶Ep 112 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 112 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 112 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 112 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 112 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 112 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 112 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 112 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 112 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 112 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 112 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 112 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 112 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 112 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 112 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
▶Ep 112 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 113 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 113 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 113 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 113 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 113 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 113 · 3:03
guidelineAn 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.↗
▶Ep 113 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 113 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 113 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 113 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 113 · 4:50
clinicalCauses 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).↗
▶Ep 113 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 113 · 5:45
clinicalMotility 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.↗
▶Ep 113 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 113 · 6:17
clinicalEnteropathies 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.↗
▶Ep 113 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 113 · 7:11
clinicalSome 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.↗
▶Ep 113 · 7:45
clinicalAlthough 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.↗
▶Ep 113 · 12:46
clinicalImproved 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.↗
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 34 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 34 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 34 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 34 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 34 · 3:03
guidelineAn 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.↗
▶Ep 34 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 34 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 34 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 34 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 34 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 34 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 34 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 34 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 34 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 34 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 34 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 34 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 34 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 34 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 34 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
▶Ep 34 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 35 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 35 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 35 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 35 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 35 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 35 · 3:03
guidelineAn 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.↗
▶Ep 35 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 35 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 35 · 4:50
clinicalCauses 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).↗
▶Ep 35 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 35 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 35 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 35 · 5:45
clinicalMotility 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.↗
▶Ep 35 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 35 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 35 · 6:17
clinicalEnteropathies 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.↗
▶Ep 35 · 7:11
clinicalSome 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.↗
▶Ep 35 · 7:45
clinicalAlthough 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.↗
▶Ep 35 · 12:46
clinicalImproved 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.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1
▶Ep 36 · 0:51
clinicalThe outlook for preterm infants with short bowel syndrome has changed over the last two decades as a result of advances in medical and surgical care.↗
▶Ep 36 · 1:23
clinicalA more aggressive approach to surgical resection has accompanied improved outcomes in short bowel syndrome.↗
▶Ep 36 · 2:38
clinicalIntestinal transplant is not experimental therapy; it is part of the continuum of therapy for a child with short bowel syndrome.↗
▶Ep 36 · 4:10
clinicalIn the acute phase with infarcted gut, the clinical picture is usually one of overwhelming sepsis, and even after resection leaving ultra-short bowel, patients are rocky and unstable due to sepsis, SIRS, or septic experience.↗
▶Ep 36 · 4:34
clinicalIn the very acute time, the primary cause of death is sepsis and multi-organ failure.↗
▶Ep 36 · 4:44
clinicalIn the intermediate and longer term, complications include intestinal failure-associated liver disease, recurrent sepsis, or line problems, which historically led to death or transplant.↗
▶Ep 36 · 5:02
clinicalMuch of the success seen in the last two decades is because clinicians are way better at preventing sepsis and liver disease.↗
▶Ep 36 · 6:14
clinicalBabies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins, supporting the concept that an undecompressed foregut affects liver function.↗
▶Ep 36 · 6:42
clinicalLiver function is affected by multiple factors: prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition.↗
Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1
▶Ep 4 · 2:02
quoteThat remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 4 · 2:16
clinicalAdaptation is driven by intraluminal nutrients and their interaction with pancreatic biliary secretions and trophic gut peptides↗
▶Ep 4 · 2:42
clinicalStructural changes during adaptation include mucosal hypertrophy with increased villous length, increased blood supply through angiogenesis, bowel dilation, and gut lengthening in younger children, all increasing surface area for absorption↗
▶Ep 4 · 3:04
clinicalFunctional changes during adaptation include slowed motility to allow more contact time and upregulation of enterocyte transporters↗
▶Ep 4 · 3:18
quoteSo, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.↗
▶Ep 4 · 5:35
clinicalUntil recently there was no standardized definition for enteral autonomy↗
▶Ep 4 · 6:04
clinicalTPN complications include line infections, liver disease, and vascular thrombosis↗
▶Ep 4 · 6:20
clinicalIn the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; management of these complications has improved significantly↗
▶Ep 4 · 6:39
guidelineCurrent ASPEN guidelines define enteral autonomy as independence of parenteral support for 12 weeks with maintenance of adequate growth and hydration↗
▶Ep 4 · 6:53
quoteIt's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.↗
▶Ep 4 · 7:50
epidemiologicalA 2012 PIFCO paper by Squires showed 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% got transplanted↗
▶Ep 4 · 8:25
epidemiologicalRecent papers in the last 5-6 years show 60-80% of patients achieve enteral autonomy, with a higher proportion surviving to reach autonomy↗
▶Ep 4 · 9:55
clinicalSmall bowel length is an independently significant variable for adaptation capacity↗
▶Ep 4 · 10:18
clinicalThe ileum has a much greater capacity to adapt than the jejunum↗
▶Ep 4 · 10:40
clinicalAt 5 years old, a child has about 425 centimeters of small bowel, with the steepest growth rate between 35 weeks gestation to about 6 months postnatal↗
▶Ep 4 · 11:25
opinionThe ileocecal valve itself is not the important factor; most people who lose their ileocecal valve also lose their terminal ileum, which is the bigger factor for adaptive potential↗
▶Ep 4 · 11:53
clinicalIf a patient has the majority of their small bowel, it almost does not matter how much colon they have; probability of enteral autonomy is 85-100%↗
CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi
clinicalPatients with stomas, even those without intestinal failure, run the risk of sodium depletion, which has a significant impact on their growth.↗
▶Ep 5 · 6:19
clinicalStomas and high stool losses are a large source of sodium bicarbonate as well as magnesium loss.↗
▶Ep 5 · 6:26
clinicalSodium status needs to be thought of, especially in the setting of a baby experiencing poor weight gain in the exposure to what is believed to be adequate calories.↗
▶Ep 5 · 6:38
quoteIt's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth.↗
▶Ep 5 · 6:38
clinicalWhen sodium is replaced, it helps restore growth, but it doesn't allow catch up growth.↗
▶Ep 5 · 6:45
opinionIt's important to track these patients serially so that a trend can be detected and sodium depletion can be avoided in the first place.↗
▶Ep 5 · 7:12
opinionIf it becomes part of practice to order urine electrolytes at the time that someone's doing their TPN blood work, it's easy to calculate the fractional excretion of sodium ratio.↗
▶Ep 5 · 7:30
opinionHaving potassium, chloride, and osmolality added to the urine electrolytes in addition to just the sodium gives a better picture of whether someone is truly sodium depleted.↗
▶Ep 5 · 7:41
quoteIf we see their potassium level higher than their sodium level, that often will tell you that the aldosterone pathway is turned on.↗
▶Ep 5 · 7:41
clinicalIf potassium level is higher than sodium level in urine, that often indicates the aldosterone pathway is turned on.↗
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 8 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 8 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 8 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 8 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 8 · 3:03
guidelineAn 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.↗
▶Ep 8 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 8 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 8 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 8 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 8 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 8 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 8 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 8 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 8 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 8 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 8 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 8 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 8 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 8 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 8 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
▶Ep 8 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 9 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 9 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 9 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 9 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 9 · 3:03
guidelineAn 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.↗
▶Ep 9 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 9 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 9 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 9 · 4:50
clinicalCauses 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).↗
▶Ep 9 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 9 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 9 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 9 · 5:45
clinicalMotility 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.↗
▶Ep 9 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 9 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 9 · 6:17
clinicalEnteropathies 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.↗
▶Ep 9 · 7:11
clinicalSome 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.↗
▶Ep 9 · 7:45
clinicalAlthough 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.↗
▶Ep 9 · 12:46
clinicalImproved 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.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1
▶Ep 10 · 0:51
clinicalThe outlook for preterm infants with short bowel syndrome has changed over the last two decades as a result of advances in medical and surgical care.↗
▶Ep 10 · 1:23
clinicalA more aggressive approach to surgical resection has accompanied improved outcomes in short bowel syndrome.↗
▶Ep 10 · 2:38
clinicalIntestinal transplant is not experimental therapy; it is part of the continuum of therapy for a child with short bowel syndrome.↗
▶Ep 10 · 4:10
clinicalIn the acute phase with infarcted gut, the clinical picture is usually one of overwhelming sepsis, and even after resection leaving ultra-short bowel, patients are rocky and unstable due to sepsis, SIRS, or septic experience.↗
▶Ep 10 · 4:34
clinicalIn the very acute time, the primary cause of death is sepsis and multi-organ failure.↗
▶Ep 10 · 4:44
clinicalIn the intermediate and longer term, complications include intestinal failure-associated liver disease, recurrent sepsis, or line problems, which historically led to death or transplant.↗
▶Ep 10 · 5:02
clinicalMuch of the success seen in the last two decades is because clinicians are way better at preventing sepsis and liver disease.↗
▶Ep 10 · 6:14
clinicalBabies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins, supporting the concept that an undecompressed foregut affects liver function.↗
▶Ep 10 · 6:42
clinicalLiver function is affected by multiple factors: prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2
▶Ep 11 · 1:23
quoteThe goalposts have moved over the last two decades.↗
▶Ep 11 · 1:37
quoteLike in Toronto, we, we classified that as less than 20% of expected for age.↗
▶Ep 11 · 1:37
clinicalIn Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters.↗
▶Ep 11 · 1:52
epidemiologicalOverall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%.↗
▶Ep 11 · 2:07
clinicalUltra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters.↗
▶Ep 11 · 2:13
clinicalLong-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management.↗
▶Ep 11 · 2:13
quoteThere should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management.↗
▶Ep 11 · 2:26
clinicalUltra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants.↗
▶Ep 11 · 5:59
clinicalNeonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective.↗
▶Ep 11 · 6:10
opinionNot every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes.↗
▶Ep 11 · 6:10
quoteI don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.↗
▶Ep 11 · 6:29
opinionLong-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients.↗
▶Ep 11 · 7:20
opinionLipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers.↗
▶Ep 11 · 7:40
quoteYou know, that was never really a practice that we subscribe to.↗
▶Ep 11 · 11:53
opinionThe surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive.↗
▶Ep 11 · 11:53
quoteThe surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right?↗
▶Ep 11 · 12:04
quoteThose intraoperative decisions have a lifelong impact.↗
▶Ep 11 · 12:13
quoteSo, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't.↗
▶Ep 11 · 13:52
clinicalTraditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching.↗
▶Ep 11 · 14:20
clinicalSome of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse.↗
▶Ep 11 · 14:21
quoteOften, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.↗
Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1
▶Ep 12 · 2:02
clinicalIn short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 12 · 2:02
quoteThat remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 12 · 2:02
clinicalIn short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 12 · 2:02
quoteThat remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 12 · 2:16
quoteThe process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides.↗
▶Ep 12 · 2:16
clinicalThe adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides.↗
▶Ep 12 · 2:16
quoteThe process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides.↗
▶Ep 12 · 2:16
clinicalThe adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides.↗
▶Ep 12 · 2:42
clinicalStructural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption.↗
▶Ep 12 · 2:42
clinicalStructural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption.↗
▶Ep 12 · 2:58
quoteWhat all of those things have in common is that they increase surface area for absorption absorption of nutrients, right?↗
▶Ep 12 · 2:58
quoteWhat all of those things have in common is that they increase surface area for absorption absorption of nutrients, right?↗
▶Ep 12 · 3:04
clinicalFunctional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently.↗
▶Ep 12 · 3:04
clinicalFunctional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently.↗
▶Ep 12 · 3:18
quoteSo, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.↗
▶Ep 12 · 3:18
quoteSo, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.↗
▶Ep 12 · 5:35
clinicalUntil very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions.↗
▶Ep 12 · 5:35
quoteUp until very recently, there actually was no standardized definition for that.↗
▶Ep 12 · 5:35
clinicalUntil very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions.↗
▶Ep 12 · 5:35
quoteUp until very recently, there actually was no standardized definition for that.↗
▶Ep 12 · 6:10
clinicalHigher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis.↗
▶Ep 12 · 6:10
clinicalHigher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis.↗
▶Ep 12 · 6:20
clinicalIn the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications.↗
▶Ep 12 · 6:20
clinicalIn the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications.↗
▶Ep 12 · 6:39
quoteSo the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period.↗
▶Ep 12 · 6:39
quoteSo the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period.↗
▶Ep 12 · 6:39
guidelineThe current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period.↗
▶Ep 12 · 6:39
guidelineThe current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period.↗
▶Ep 12 · 6:53
clinicalA patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake.↗
▶Ep 12 · 6:53
quoteBecause it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.↗
▶Ep 12 · 6:53
quoteBecause it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.↗
▶Ep 12 · 6:53
clinicalA patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake.↗
▶Ep 12 · 7:57
epidemiologicalIn the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants.↗
▶Ep 12 · 7:57
epidemiologicalIn the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants.↗
▶Ep 12 · 8:13
quoteBasically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted.↗
▶Ep 12 · 8:13
quoteBasically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted.↗
▶Ep 12 · 8:25
epidemiologicalRecent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data.↗
▶Ep 12 · 8:25
epidemiologicalRecent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data.↗
▶Ep 12 · 8:35
quoteHas increased between, depending on who you read, anywhere between 60 to 80%.↗
▶Ep 12 · 8:35
quoteHas increased between, depending on who you read, anywhere between 60 to 80%.↗
▶Ep 12 · 9:55
clinicalSmall bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel.↗
▶Ep 12 · 9:55
clinicalSmall bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel.↗
▶Ep 12 · 10:18
quoteYou know, the ileum has a much greater capacity to adapt than the jejunum does.↗
▶Ep 12 · 10:18
clinicalThe ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy.↗
▶Ep 12 · 10:18
clinicalThe ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy.↗
▶Ep 12 · 10:18
quoteYou know, the ileum has a much greater capacity to adapt than the jejunum does.↗
▶Ep 12 · 10:34
clinicalA full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal.↗
▶Ep 12 · 10:34
clinicalA full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal.↗
▶Ep 12 · 10:40
quoteWhen you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal.↗
▶Ep 12 · 10:40
quoteWhen you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal.↗
▶Ep 12 · 11:13
opinionThe presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential.↗
▶Ep 12 · 11:13
opinionThe presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential.↗
▶Ep 12 · 11:34
quoteMost people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential.↗
▶Ep 12 · 11:34
quoteMost people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential.↗
▶Ep 12 · 11:53
clinicalWhen a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%.↗
▶Ep 12 · 11:53
quoteSo we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%.↗
▶Ep 12 · 11:53
quoteSo we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%.↗
▶Ep 12 · 11:53
clinicalWhen a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%.↗
▶Ep 12 · 12:06
clinicalWhen small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption.↗
▶Ep 12 · 12:06
clinicalWhen small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption.↗
CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi
clinicalPatients with stomas, even those without intestinal failure, run the risk of sodium depletion, which has a significant impact on their growth.↗
▶Ep 14 · 6:19
clinicalStomas and high stool losses are a large source of sodium bicarbonate as well as magnesium loss.↗
▶Ep 14 · 6:26
clinicalSodium status needs to be thought of, especially in the setting of a baby experiencing poor weight gain in the exposure to what is believed to be adequate calories.↗
▶Ep 14 · 6:38
quoteIt's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth.↗
▶Ep 14 · 6:38
clinicalWhen sodium is replaced, it helps restore growth, but it doesn't allow catch up growth.↗
▶Ep 14 · 6:45
opinionIt's important to track these patients serially so that a trend can be detected and sodium depletion can be avoided in the first place.↗
▶Ep 14 · 7:12
opinionIf it becomes part of practice to order urine electrolytes at the time that someone's doing their TPN blood work, it's easy to calculate the fractional excretion of sodium ratio.↗
▶Ep 14 · 7:30
opinionHaving potassium, chloride, and osmolality added to the urine electrolytes in addition to just the sodium gives a better picture of whether someone is truly sodium depleted.↗
▶Ep 14 · 7:41
quoteIf we see their potassium level higher than their sodium level, that often will tell you that the aldosterone pathway is turned on.↗
▶Ep 14 · 7:41
clinicalIf potassium level is higher than sodium level in urine, that often indicates the aldosterone pathway is turned on.↗
Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations
▶Ep 15 · 10:35
clinicalTraditional locking solutions are predominantly antibiotic locks, but they lead to development of bacterial resistance.↗
▶Ep 15 · 10:56
clinicalEthanol locks are antimicrobial without resistance development and kill both planktonic bacteria (floating in the lumen) and sessile bacteria (embedded in biofilm along the catheter wall).↗
▶Ep 15 · 10:56
quoteAntimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter.↗
▶Ep 15 · 11:24
clinicalKite lock is a 4% tetrasodium EDTA solution that is antimicrobial without resistance, and has antifibrinolytic and antithrombotic properties.↗
▶Ep 15 · 11:59
clinicalKite lock is licensed for pediatric use in Canada, Europe, and Australia, but availability varies by region.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
▶Ep 18 · 4:18
epidemiologicalThe majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.↗
▶Ep 18 · 5:42
quoteIf you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.↗
▶Ep 18 · 5:42
clinicalWhen reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent.↗
▶Ep 18 · 6:02
clinicalSize discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length.↗
▶Ep 18 · 7:08
epidemiologicalGastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes.↗
▶Ep 18 · 7:08
quoteIn general, gastroschisis, they don't tend to do as well regardless.↗
▶Ep 18 · 7:12
quoteIf, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.↗
▶Ep 18 · 9:09
quoteI don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.↗
▶Ep 18 · 9:09
clinicalSTEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile.↗
▶Ep 18 · 9:41
clinicalIn scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection.↗
▶Ep 18 · 10:45
clinicalThe STEP procedure was originally described by HP Kim and Tom Jackson.↗
▶Ep 18 · 11:02
clinicalFor STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility.↗
▶Ep 18 · 11:29
clinicalThe anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling.↗
▶Ep 18 · 11:58
clinicalBowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded.↗
▶Ep 18 · 12:20
opinionAn endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients.↗
▶Ep 18 · 12:34
clinicalStaple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°).↗
▶Ep 18 · 12:58
clinicalA vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience.↗
▶Ep 18 · 13:23
clinicalTarget caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility.↗
▶Ep 18 · 14:09
clinicalBefore firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length.↗
▶Ep 18 · 14:28
clinicalA U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point.↗
▶Ep 18 · 14:50
clinicalThe procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter.↗
▶Ep 18 · 15:08
clinicalAt the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time.↗
▶Ep 18 · 16:23
clinicalThe STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°).↗
▶Ep 18 · 16:56
clinicalThe duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum.↗
▶Ep 18 · 17:03
opinionAvoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak.↗
▶Ep 18 · 17:15
clinicalIf duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
▶Ep 19 · 4:18
epidemiologicalThe majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.↗
▶Ep 19 · 5:42
quoteIf you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.↗
▶Ep 19 · 5:42
clinicalWhen reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent.↗
▶Ep 19 · 6:02
clinicalSize discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length.↗
▶Ep 19 · 7:08
quoteIn general, gastroschisis, they don't tend to do as well regardless.↗
▶Ep 19 · 7:08
epidemiologicalGastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes.↗
▶Ep 19 · 7:12
quoteIf, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.↗
▶Ep 19 · 9:09
quoteI don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.↗
▶Ep 19 · 9:09
clinicalSTEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile.↗
▶Ep 19 · 9:41
clinicalIn scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection.↗
▶Ep 19 · 10:45
clinicalThe STEP procedure was originally described by HP Kim and Tom Jackson.↗
▶Ep 19 · 11:02
clinicalFor STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility.↗
▶Ep 19 · 11:29
clinicalThe anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling.↗
▶Ep 19 · 11:58
clinicalBowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded.↗
▶Ep 19 · 12:20
opinionAn endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients.↗
▶Ep 19 · 12:34
clinicalStaple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°).↗
▶Ep 19 · 12:58
clinicalA vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience.↗
▶Ep 19 · 13:23
clinicalTarget caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility.↗
▶Ep 19 · 14:09
clinicalBefore firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length.↗
▶Ep 19 · 14:28
clinicalA U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point.↗
▶Ep 19 · 14:50
clinicalThe procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter.↗
▶Ep 19 · 15:08
clinicalAt the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time.↗
▶Ep 19 · 16:23
clinicalThe STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°).↗
▶Ep 19 · 16:56
clinicalThe duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum.↗
▶Ep 19 · 17:03
opinionAvoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak.↗
▶Ep 19 · 17:15
clinicalIf duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 2
▶Ep 20 · 1:52
quoteI think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility.↗
▶Ep 20 · 1:52
clinicalThe biggest benefit of the STEP procedure is tapering the bowel and reestablishing a more normal caliber to improve motility.↗
▶Ep 20 · 3:04
quoteIt can take up to 6 months before you actually start to see a significant improvement in absorptive capacity.↗
▶Ep 20 · 3:04
clinicalIt can take up to 6 months before you actually start to see a significant improvement in absorptive capacity after STEP.↗
▶Ep 20 · 3:16
clinicalImprovements in absorptive capacity are measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and citrulline levels as they rise over time.↗
▶Ep 20 · 3:24
clinicalThe reason absorptive improvement takes time is that inflamed, sick, leaky mucosa in the setting of bacterial overgrowth needs to heal.↗
▶Ep 20 · 6:24
clinicalBleeding at STEP staple lines is an underreported complication that is really difficult to manage in some cases.↗
▶Ep 20 · 6:49
opinionStaple line bleeding is hypothesized to be a microbiome problem occurring in a pro-inflammatory environment.↗
▶Ep 20 · 6:49
quoteMy hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment.↗
▶Ep 20 · 6:55
clinicalStaple line bleeding tends to occur in type 2 anatomy, which is small bowel to colonic remnant in the absence of an intact colon ileocecal valve.↗
▶Ep 20 · 7:12
quoteIt's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult.↗
▶Ep 20 · 7:12
clinicalPathology of staple line ulcers shows non-specific inflammation with no vasculitis, no viral elements, and no obvious ischemia.↗
▶Ep 20 · 10:05
clinicalMost referrals for intestinal rehabilitation have had multiple operations before coming to the specialist center.↗
▶Ep 20 · 10:09
quoteKeep your eye on the horizon, which is what can we do to try to optimize this kid's anatomy. And if you think about that plan, it may not be conducive to just one operation today.↗
▶Ep 20 · 10:09
opinionOptimizing a child's anatomy may not be conducive to just one operation; you have to set yourself up sometimes planning for the next case.↗
Intestinal Rehabilitation, Episode 6: Cholestasis
▶Ep 21 · 1:09
quoteInstitutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event.↗
▶Ep 21 · 1:09
guidelineCincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event.↗
▶Ep 21 · 1:20
guidelineA 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event.↗
▶Ep 21 · 2:57
clinicalIn young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition).↗
▶Ep 21 · 3:30
quoteNow, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that.↗
▶Ep 21 · 3:30
clinicalPrematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors.↗
▶Ep 21 · 4:09
clinicalPrevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery.↗
▶Ep 21 · 7:00
clinicalTwo strategies for reversing cholestasis are dose restriction and change of lipid composition.↗
▶Ep 21 · 7:08
clinicalConventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized.↗
▶Ep 21 · 7:19
clinicalSMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver.↗
▶Ep 21 · 7:45
clinicalSMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency.↗
▶Ep 21 · 7:57
clinicalWhen SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency.↗
▶Ep 21 · 8:06
guidelineConventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day.↗
▶Ep 21 · 8:52
quoteMy opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to.↗
▶Ep 21 · 8:52
opinionA bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold.↗
▶Ep 21 · 12:11
clinicalDuring secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot.↗
▶Ep 21 · 12:53
clinicalLiver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits.↗
▶Ep 21 · 13:00
clinicalElastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis.↗
▶Ep 21 · 13:31
clinicalFollow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made.↗
Intestinal Rehabilitation, Episode 7: Refeeding in a neonatal patient
▶Ep 22 · 0:57
quoteEven if a kid has a complicated disease process, one of our goals as a team is to try to establish the most normal feeding habits we can that not only promote gut function and everything else, but also to optimize quality of life and the importance of the social aspects of eating that we all experience with our families and friends.↗
▶Ep 22 · 0:57
clinicalOne goal in intestinal failure management is to establish normal feeding habits that promote gut function and optimize quality of life including social aspects of eating.↗
▶Ep 22 · 2:22
clinicalWhen initiating feeds in patients with high stoma output, losses increase initially, but this must be pushed through in a hospital setting where volume replacement is available.↗
▶Ep 22 · 4:34
clinicalIn short bowel syndrome, protein absorption is fairly well preserved, so the benefit of completely broken-down protein formulas (free amino acids or hydrolysates) is primarily from an allergy perspective.↗
▶Ep 22 · 4:46
quoteIn the world of short bowel syndrome, protein absorption is actually fairly well preserved. So it seems like the benefit is from an allergy perspective.↗
▶Ep 22 · 5:04
clinicalMany formulas have moved from predominant long-chain fat to increasing MCT components, but long-chain fat is a stronger driver for adaptation.↗
▶Ep 22 · 5:04
quoteOver the last several years, a lot of the formulas have moved from predominant long chain fat to increasing in MCT components. But long chain fat is a stronger driver for adaptation, and I personally haven't really bought into that.↗
▶Ep 22 · 6:06
quoteYou're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution.↗
▶Ep 22 · 6:06
clinicalOne milliliter of parenteral nutrition is not isocaloric with one milliliter of formula, and advancing beyond 100-120 per kilo creates problems with not only calories and protein but also sodium and calcium due to compositional differences.↗
▶Ep 22 · 6:28
clinicalAt some point during feeding advancement, fortification is necessary.↗
▶Ep 22 · 7:21
clinicalFeeding options include bolus (oral or gastric via tube), continuous (post-pyloric tube or surgical J-tube), or a hybrid model with gastric bolus component plus continuous component (gastric or post-pyloric).↗
▶Ep 22 · 8:05
quoteBecause I like bolus feeds as a default, I'd like, I like to see a patient fail bolus feeds before they end up on continuous feeds as a sole delivery remote.↗
▶Ep 22 · 8:05
opinionBolus feeds are preferred as the default approach; patients should fail bolus feeds before being placed on continuous feeds as the sole delivery method.↗
▶Ep 22 · 8:17
clinicalContinuous feeds can be used as a supplemental approach, with bolus feeds during the day and continuous supplementation overnight.↗
▶Ep 22 · 8:40
clinicalEven when oral feeding is non-nutritive, it is important for skill development; children who never learn to suck, swallow, and process food by mouth will not eat solids later and will remain dependent on tube feeding.↗
▶Ep 22 · 8:40
quoteOne thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.↗
▶Ep 22 · 12:04
quoteYeah, and it, it goes to the point that we've made in a previous podcast about having a plan and and resisting the temptation to get it all done in one. Operation. It's all about optimizing the patient for the next step, but you don't have to do it all in one go.↗
▶Ep 22 · 12:12
clinicalThe technical benefit of distal feeding is that size discrepancy at anastomosis is significantly improved because the bowel has been used.↗
▶Ep 22 · 13:43
clinicalThe hole from a removed G-tube closes very quickly.↗
▶Ep 22 · 13:51
clinicalA G-tube provides versatility for nutrition supplementation, medication delivery, and venting for gassy or bloated children to improve enteral tolerance.↗
Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient
▶Ep 23 · 1:24
clinicalThe benefit and curse in older patients with bowel loss is that they already know how to eat and have established that behavior.↗
▶Ep 23 · 1:24
quoteThe benefit in the curse in a patient like that is that they do know how to eat. They've established that that behavior.↗
▶Ep 23 · 1:50
quoteSo even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can.↗
▶Ep 23 · 1:50
opinionEven if you cannot cure the patient and get them off TPN, it is important to make compromises to optimize quality of life.↗
▶Ep 23 · 2:14
quoteThe general strategy is you want to push the macronutrient modules of protein and fat are well tolerated. Most of these kids don't tolerate simple sugars very well.↗
▶Ep 23 · 2:14
clinicalThe general strategy is to push the macronutrient modules of protein and fat, which are well tolerated.↗
▶Ep 23 · 2:21
clinicalMost patients with short bowel do not tolerate simple sugars very well.↗
▶Ep 23 · 2:25
clinicalThe general concept of pushing solids and minimizing fluid intake helps prevent dumping.↗
▶Ep 23 · 2:25
quoteSo the general concept of pushing solids. And minimizing fluid intake helps. They don't tend to dump as much smaller meals more frequently of solids separated from their liquids to a certain extent, and minimization of simple sugars.↗
▶Ep 23 · 2:31
clinicalSmaller meals more frequently of solids separated from liquids, with minimization of simple sugars, reduces dumping tendency.↗
▶Ep 23 · 2:52
clinicalPatients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices, particularly increased sugars.↗
▶Ep 23 · 3:31
clinicalMinimizing sugars reduces symptoms related to bacterial overgrowth, making patients less bloated and gassy.↗
▶Ep 23 · 3:48
clinicalIn older children with fluid losses (by stoma or other source), if they are on parenteral support, some fluid can be replaced IV.↗
▶Ep 23 · 4:01
clinicalAs you try to get patients off IV support, keeping them hydrated by replenishing losses through enteral replacements is important.↗
▶Ep 23 · 4:11
clinicalTransport requires sodium and glucose, so the rehydration solution must contain some glucose and some salt.↗
▶Ep 23 · 4:11
quoteFor transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt.↗
▶Ep 23 · 4:39
clinicalThere are homemade recipes for oral rehydration solutions and over-the-counter preparations available.↗
▶Ep 23 · 4:48
quoteSome people will resort to things like Gatorade and stuff like that, but that doesn't always work that well. It's actually too much sugar in it.↗
▶Ep 23 · 4:48
clinicalGatorade does not always work well for rehydration because it has too much sugar in it.↗
▶Ep 23 · 6:23
opinionIt is all about normal growth parameters and getting patients through puberty.↗
▶Ep 23 · 6:28
clinicalSome kids end up back on parenteral support to get through puberty.↗
▶Ep 23 · 6:28
quoteIt is true some kids end up back on parental support, you know, to get through puberty. And then when you're not growing anymore as an adult, often that borderline or marginal gut function is enough to sustain them.↗
▶Ep 23 · 6:33
clinicalWhen patients are not growing anymore as adults, borderline or marginal gut function is often enough to sustain them.↗
▶Ep 23 · 7:22
clinicalPatients need to be monitored for micronutrient deficiencies after getting off TPN, as this is when they often get into trouble.↗
▶Ep 23 · 7:31
opinionLong-term growth and outcome need to be followed, highlighting the importance of multidisciplinary teams.↗
▶Ep 23 · 8:51
clinicalWeight is tracked as a major metric for growth, but it has to be balanced with height.↗
▶Ep 23 · 8:57
quoteWhat we commonly see in this type of population is. Round babies where their weight for height is elevated↗
▶Ep 23 · 8:57
clinicalWhat is commonly seen in this population is round babies where their weight for height is elevated.↗
▶Ep 23 · 9:01
clinicalThere is increasing data looking at quality of weight: how much is fat weight versus lean body mass.↗
Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes
▶Ep 28 · 5:23
quoteThey go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation.↗
▶Ep 28 · 5:23
clinicalLiver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation.↗
▶Ep 28 · 5:41
clinicalRisk 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.↗
▶Ep 28 · 5:41
quoteThere's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN.↗
▶Ep 28 · 6:08
clinicalThe term 'intestinal rehabilitation program' was coined in the year 2000.↗
▶Ep 28 · 6:08
quoteThe term intestinal rehab program was coined in the year 2000.↗
▶Ep 28 · 6:12
clinicalIntestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity.↗
▶Ep 28 · 6:12
quoteIntestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity.↗
▶Ep 28 · 6:34
quoteWe have a large reliance on speech, language, and occupational therapy.↗
▶Ep 28 · 6:34
clinicalIntestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists.↗
▶Ep 28 · 6:38
quoteWhile our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications.↗
▶Ep 28 · 6:38
clinicalWhile patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications.↗
▶Ep 28 · 6:45
quoteHaving a dedicated unit with dedicated staff all contributes to better outcomes.↗
▶Ep 28 · 6:54
quoteWhat we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma.↗
▶Ep 28 · 6:54
clinicalFamilies of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas.↗
▶Ep 28 · 7:11
quoteWe educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout.↗
▶Ep 28 · 7:11
clinicalHigh-quality families demonstrate evidence of burnout over time despite education and empowerment efforts.↗
▶Ep 28 · 7:25
quoteThere's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down.↗
▶Ep 28 · 7:25
epidemiologicalLiterature shows 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.↗
▶Ep 28 · 7:43
epidemiologicalMortality in intestinal failure has decreased with intestinal rehabilitation programs.↗
▶Ep 28 · 8:02
clinicalLipids 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.↗
▶Ep 28 · 8:02
quoteLipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways.↗
▶Ep 28 · 8:15
quoteHistorically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content.↗
▶Ep 28 · 8:15
clinicalHistorically, 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.↗
▶Ep 28 · 8:31
quoteIt is associated with deterioration of liver dysfunction, i.e., cholestasis.↗
▶Ep 28 · 8:31
clinicalProlonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis.↗
▶Ep 28 · 8:51
quoteThis is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease.↗
▶Ep 28 · 8:51
epidemiologicalFor 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.↗
▶Ep 28 · 9:15
quoteThe first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives.↗
▶Ep 28 · 9:15
clinicalFirst-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years.↗
▶Ep 28 · 9:32
quoteThe 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids.↗
▶Ep 28 · 9:32
clinicalThird-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids.↗
▶Ep 28 · 9:51
clinicalOmega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile.↗
▶Ep 28 · 9:51
quoteOmega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile.↗
▶Ep 28 · 10:01
clinicalOmega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile.↗
▶Ep 28 · 10:15
quoteOmega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function.↗
▶Ep 28 · 10:15
clinicalOmega-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.↗
▶Ep 28 · 10:32
clinicalThe two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition.↗
▶Ep 28 · 10:32
quoteOne is lipid minimization, and then the second strategy is a change in composition.↗
▶Ep 28 · 10:42
quoteThere's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less.↗
▶Ep 28 · 10:42
clinicalLiterature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less.↗
▶Ep 28 · 11:00
clinicalIn preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much.↗
▶Ep 28 · 11:00
quoteThe problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much.↗
▶Ep 28 · 11:22
clinicalDHA and arachidonic acid are important for retinal and brain development.↗
▶Ep 28 · 11:22
quoteDHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting.↗
▶Ep 28 · 11:26
clinicalNone of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings.↗
▶Ep 28 · 11:38
quoteWe're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid.↗
▶Ep 28 · 11:38
clinicalWork is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content.↗
▶Ep 28 · 12:03
clinicalCentral lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported.↗
▶Ep 28 · 12:03
quoteSo our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage.↗
▶Ep 28 · 12:10
clinicalCentral lines are at risk for complications including infections, blockages, thrombosis, and breakage.↗
▶Ep 28 · 12:24
quoteIn the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide.↗
▶Ep 28 · 12:24
epidemiologicalIn the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide.↗
▶Ep 28 · 12:48
quoteWe landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution.↗
▶Ep 28 · 12:48
clinicalThe team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain.↗
▶Ep 28 · 12:48
clinical4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution.↗
▶Ep 28 · 13:23
quoteIt's licensed in Europe, it's licensed in Australia. It's not available in the United States.↗
▶Ep 28 · 13:23
clinicalKitelock is licensed in Europe and Australia but not available in the United States.↗
▶Ep 28 · 13:29
clinicalA multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year.↗
▶Ep 28 · 13:29
quoteI'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States.↗
▶Ep 28 · 13:58
clinicalIn 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).↗
▶Ep 28 · 13:58
quoteIn short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon.↗
▶Ep 28 · 14:26
epidemiologicalThe most common anatomical subtype of short bowel syndrome in pediatrics is Type 2.↗
▶Ep 28 · 14:26
quoteThe most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum.↗
▶Ep 28 · 14:31
clinicalType 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum.↗
▶Ep 28 · 14:53
quoteThe problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection.↗
▶Ep 28 · 14:53
clinicalNative GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use.↗
▶Ep 28 · 14:58
clinicalGLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection.↗
▶Ep 28 · 15:23
epidemiologicalA multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period.↗
▶Ep 28 · 15:23
quoteWhat we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements.↗
▶Ep 28 · 15:32
epidemiological70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements.↗
▶Ep 28 · 15:48
quoteIt's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone.↗
▶Ep 28 · 15:48
guidelineTeduglutide is licensed for children greater than 1 year of age.↗
▶Ep 28 · 15:50
clinicalTeduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN.↗
▶Ep 28 · 16:02
clinicalAnother GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly.↗
▶Ep 28 · 16:02
quoteAnother GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide.↗
▶Ep 28 · 16:10
clinicalApraglutide works even better than teduglutide, producing more bowel lengthening.↗
▶Ep 28 · 16:36
quoteThese interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal.↗
▶Ep 28 · 16:36
epidemiologicalThese interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008.↗
▶Ep 28 · 16:41
opinionThe decrease in transplantation is in large part because of the successes of intestinal rehabilitation.↗
▶Ep 28 · 16:41
epidemiologicalOutcomes of intestinal transplant at 5 years are approximately 65%.↗
▶Ep 28 · 16:49
opinionThe goal is to avoid transplant and lifelong immunosuppression whenever possible.↗
▶Ep 28 · 17:19
quoteWe have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team.↗
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 46 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 46 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 46 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 46 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 46 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 46 · 3:03
guidelineAn 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.↗
▶Ep 46 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 46 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 46 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 46 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 46 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 46 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 46 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 46 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 46 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 46 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 46 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 46 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 46 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 46 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
▶Ep 46 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 47 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 47 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 47 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 47 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 47 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 47 · 3:03
guidelineAn 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.↗
▶Ep 47 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 47 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 47 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 47 · 4:50
clinicalCauses 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).↗
▶Ep 47 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 47 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 47 · 5:45
clinicalMotility 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.↗
▶Ep 47 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 47 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 47 · 6:17
clinicalEnteropathies 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.↗
▶Ep 47 · 7:11
clinicalSome 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.↗
▶Ep 47 · 7:45
clinicalAlthough 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.↗
▶Ep 47 · 12:46
clinicalImproved 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.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1
▶Ep 48 · 0:51
clinicalThe outlook for preterm infants with short bowel syndrome has changed over the last two decades as a result of advances in medical and surgical care.↗
▶Ep 48 · 1:23
clinicalA more aggressive approach to surgical resection has accompanied improved outcomes in short bowel syndrome.↗
▶Ep 48 · 2:38
clinicalIntestinal transplant is not experimental therapy; it is part of the continuum of therapy for a child with short bowel syndrome.↗
▶Ep 48 · 4:10
clinicalIn the acute phase with infarcted gut, the clinical picture is usually one of overwhelming sepsis, and even after resection leaving ultra-short bowel, patients are rocky and unstable due to sepsis, SIRS, or septic experience.↗
▶Ep 48 · 4:34
clinicalIn the very acute time, the primary cause of death is sepsis and multi-organ failure.↗
▶Ep 48 · 4:44
clinicalIn the intermediate and longer term, complications include intestinal failure-associated liver disease, recurrent sepsis, or line problems, which historically led to death or transplant.↗
▶Ep 48 · 5:02
clinicalMuch of the success seen in the last two decades is because clinicians are way better at preventing sepsis and liver disease.↗
▶Ep 48 · 6:14
clinicalBabies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins, supporting the concept that an undecompressed foregut affects liver function.↗
▶Ep 48 · 6:42
clinicalLiver function is affected by multiple factors: prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2
▶Ep 49 · 1:23
quoteThe goalposts have moved over the last two decades.↗
▶Ep 49 · 1:23
quoteThe goalposts have moved over the last two decades.↗
▶Ep 49 · 1:37
quoteLike in Toronto, we, we classified that as less than 20% of expected for age.↗
▶Ep 49 · 1:37
clinicalIn Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters.↗
▶Ep 49 · 1:37
clinicalIn Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters.↗
▶Ep 49 · 1:37
quoteLike in Toronto, we, we classified that as less than 20% of expected for age.↗
▶Ep 49 · 1:52
epidemiologicalOverall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%.↗
▶Ep 49 · 1:52
epidemiologicalOverall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%.↗
▶Ep 49 · 2:07
clinicalUltra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters.↗
▶Ep 49 · 2:07
clinicalUltra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters.↗
▶Ep 49 · 2:13
quoteThere should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management.↗
▶Ep 49 · 2:13
clinicalLong-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management.↗
▶Ep 49 · 2:13
quoteThere should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management.↗
▶Ep 49 · 2:13
clinicalLong-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management.↗
▶Ep 49 · 2:26
clinicalUltra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants.↗
▶Ep 49 · 2:26
clinicalUltra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants.↗
▶Ep 49 · 5:59
clinicalNeonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective.↗
▶Ep 49 · 5:59
clinicalNeonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective.↗
▶Ep 49 · 6:10
opinionNot every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes.↗
▶Ep 49 · 6:10
quoteI don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.↗
▶Ep 49 · 6:10
quoteI don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.↗
▶Ep 49 · 6:10
opinionNot every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes.↗
▶Ep 49 · 6:29
opinionLong-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients.↗
▶Ep 49 · 6:29
opinionLong-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients.↗
▶Ep 49 · 7:20
opinionLipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers.↗
▶Ep 49 · 7:20
opinionLipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers.↗
▶Ep 49 · 7:40
quoteYou know, that was never really a practice that we subscribe to.↗
▶Ep 49 · 7:40
quoteYou know, that was never really a practice that we subscribe to.↗
▶Ep 49 · 11:53
opinionThe surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive.↗
▶Ep 49 · 11:53
quoteThe surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right?↗
▶Ep 49 · 11:53
quoteThe surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right?↗
▶Ep 49 · 11:53
opinionThe surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive.↗
▶Ep 49 · 12:04
quoteThose intraoperative decisions have a lifelong impact.↗
▶Ep 49 · 12:04
quoteThose intraoperative decisions have a lifelong impact.↗
▶Ep 49 · 12:13
quoteSo, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't.↗
▶Ep 49 · 12:13
quoteSo, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't.↗
▶Ep 49 · 13:52
clinicalTraditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching.↗
▶Ep 49 · 13:52
clinicalTraditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching.↗
▶Ep 49 · 14:20
clinicalSome of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse.↗
▶Ep 49 · 14:20
clinicalSome of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse.↗
▶Ep 49 · 14:21
quoteOften, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.↗
▶Ep 49 · 14:21
quoteOften, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.↗
Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1
▶Ep 52 · 2:02
clinicalIn short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 52 · 2:02
quoteThat remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 52 · 2:02
clinicalIn short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 52 · 2:02
quoteThat remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 52 · 2:16
quoteThe process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides.↗
▶Ep 52 · 2:16
quoteThe process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides.↗
▶Ep 52 · 2:16
clinicalThe adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides.↗
▶Ep 52 · 2:16
clinicalThe adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides.↗
▶Ep 52 · 2:42
clinicalStructural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption.↗
▶Ep 52 · 2:42
clinicalStructural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption.↗
▶Ep 52 · 2:58
quoteWhat all of those things have in common is that they increase surface area for absorption absorption of nutrients, right?↗
▶Ep 52 · 2:58
quoteWhat all of those things have in common is that they increase surface area for absorption absorption of nutrients, right?↗
▶Ep 52 · 3:04
clinicalFunctional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently.↗
▶Ep 52 · 3:04
clinicalFunctional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently.↗
▶Ep 52 · 3:18
quoteSo, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.↗
▶Ep 52 · 3:18
quoteSo, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.↗
▶Ep 52 · 5:35
clinicalUntil very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions.↗
▶Ep 52 · 5:35
quoteUp until very recently, there actually was no standardized definition for that.↗
▶Ep 52 · 5:35
quoteUp until very recently, there actually was no standardized definition for that.↗
▶Ep 52 · 5:35
clinicalUntil very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions.↗
▶Ep 52 · 6:10
clinicalHigher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis.↗
▶Ep 52 · 6:10
clinicalHigher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis.↗
▶Ep 52 · 6:20
clinicalIn the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications.↗
▶Ep 52 · 6:20
clinicalIn the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications.↗
▶Ep 52 · 6:39
guidelineThe current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period.↗
▶Ep 52 · 6:39
quoteSo the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period.↗
▶Ep 52 · 6:39
quoteSo the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period.↗
▶Ep 52 · 6:39
guidelineThe current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period.↗
▶Ep 52 · 6:53
clinicalA patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake.↗
▶Ep 52 · 6:53
quoteBecause it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.↗
▶Ep 52 · 6:53
quoteBecause it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.↗
▶Ep 52 · 6:53
clinicalA patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake.↗
▶Ep 52 · 7:57
epidemiologicalIn the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants.↗
▶Ep 52 · 7:57
epidemiologicalIn the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants.↗
▶Ep 52 · 8:13
quoteBasically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted.↗
▶Ep 52 · 8:13
quoteBasically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted.↗
▶Ep 52 · 8:25
epidemiologicalRecent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data.↗
▶Ep 52 · 8:25
epidemiologicalRecent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data.↗
▶Ep 52 · 8:35
quoteHas increased between, depending on who you read, anywhere between 60 to 80%.↗
▶Ep 52 · 8:35
quoteHas increased between, depending on who you read, anywhere between 60 to 80%.↗
▶Ep 52 · 9:55
clinicalSmall bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel.↗
▶Ep 52 · 9:55
clinicalSmall bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel.↗
▶Ep 52 · 10:18
clinicalThe ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy.↗
▶Ep 52 · 10:18
quoteYou know, the ileum has a much greater capacity to adapt than the jejunum does.↗
▶Ep 52 · 10:18
clinicalThe ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy.↗
▶Ep 52 · 10:18
quoteYou know, the ileum has a much greater capacity to adapt than the jejunum does.↗
▶Ep 52 · 10:34
clinicalA full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal.↗
▶Ep 52 · 10:34
clinicalA full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal.↗
▶Ep 52 · 10:40
quoteWhen you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal.↗
▶Ep 52 · 10:40
quoteWhen you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal.↗
▶Ep 52 · 11:13
opinionThe presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential.↗
▶Ep 52 · 11:13
opinionThe presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential.↗
▶Ep 52 · 11:34
quoteMost people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential.↗
▶Ep 52 · 11:34
quoteMost people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential.↗
▶Ep 52 · 11:53
clinicalWhen a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%.↗
▶Ep 52 · 11:53
quoteSo we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%.↗
▶Ep 52 · 11:53
quoteSo we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%.↗
▶Ep 52 · 11:53
clinicalWhen a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%.↗
▶Ep 52 · 12:06
clinicalWhen small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption.↗
▶Ep 52 · 12:06
clinicalWhen small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption.↗
CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi
clinicalPatients with stomas, even those without intestinal failure, run the risk of sodium depletion, which has a significant impact on their growth.↗
▶Ep 58 · 6:19
clinicalStomas and high stool losses are a large source of sodium bicarbonate as well as magnesium loss.↗
▶Ep 58 · 6:26
clinicalSodium status needs to be thought of, especially in the setting of a baby experiencing poor weight gain in the exposure to what is believed to be adequate calories.↗
▶Ep 58 · 6:38
quoteIt's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth.↗
▶Ep 58 · 6:38
clinicalWhen sodium is replaced, it helps restore growth, but it doesn't allow catch up growth.↗
▶Ep 58 · 6:45
opinionIt's important to track these patients serially so that a trend can be detected and sodium depletion can be avoided in the first place.↗
▶Ep 58 · 7:12
opinionIf it becomes part of practice to order urine electrolytes at the time that someone's doing their TPN blood work, it's easy to calculate the fractional excretion of sodium ratio.↗
▶Ep 58 · 7:30
opinionHaving potassium, chloride, and osmolality added to the urine electrolytes in addition to just the sodium gives a better picture of whether someone is truly sodium depleted.↗
▶Ep 58 · 7:41
quoteIf we see their potassium level higher than their sodium level, that often will tell you that the aldosterone pathway is turned on.↗
▶Ep 58 · 7:41
clinicalIf potassium level is higher than sodium level in urine, that often indicates the aldosterone pathway is turned on.↗
Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations
▶Ep 63 · 10:35
clinicalTraditional locking solutions are predominantly antibiotic locks, but they lead to development of bacterial resistance.↗
▶Ep 63 · 10:56
clinicalEthanol locks are antimicrobial without resistance development and kill both planktonic bacteria (floating in the lumen) and sessile bacteria (embedded in biofilm along the catheter wall).↗
▶Ep 63 · 10:56
quoteAntimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter.↗
▶Ep 63 · 11:24
clinicalKite lock is a 4% tetrasodium EDTA solution that is antimicrobial without resistance, and has antifibrinolytic and antithrombotic properties.↗
▶Ep 63 · 11:59
clinicalKite lock is licensed for pediatric use in Canada, Europe, and Australia, but availability varies by region.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
▶Ep 66 · 4:18
epidemiologicalThe majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.↗
▶Ep 66 · 5:42
clinicalWhen reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent.↗
▶Ep 66 · 5:42
quoteIf you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.↗
▶Ep 66 · 6:02
clinicalSize discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length.↗
▶Ep 66 · 7:08
quoteIn general, gastroschisis, they don't tend to do as well regardless.↗
▶Ep 66 · 7:08
epidemiologicalGastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes.↗
▶Ep 66 · 7:12
quoteIf, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.↗
▶Ep 66 · 9:09
quoteI don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.↗
▶Ep 66 · 9:09
clinicalSTEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile.↗
▶Ep 66 · 9:41
clinicalIn scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection.↗
▶Ep 66 · 10:45
clinicalThe STEP procedure was originally described by HP Kim and Tom Jackson.↗
▶Ep 66 · 11:02
clinicalFor STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility.↗
▶Ep 66 · 11:29
clinicalThe anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling.↗
▶Ep 66 · 11:58
clinicalBowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded.↗
▶Ep 66 · 12:20
opinionAn endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients.↗
▶Ep 66 · 12:34
clinicalStaple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°).↗
▶Ep 66 · 12:58
clinicalA vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience.↗
▶Ep 66 · 13:23
clinicalTarget caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility.↗
▶Ep 66 · 14:09
clinicalBefore firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length.↗
▶Ep 66 · 14:28
clinicalA U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point.↗
▶Ep 66 · 14:50
clinicalThe procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter.↗
▶Ep 66 · 15:08
clinicalAt the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time.↗
▶Ep 66 · 16:23
clinicalThe STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°).↗
▶Ep 66 · 16:56
clinicalThe duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum.↗
▶Ep 66 · 17:03
opinionAvoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak.↗
▶Ep 66 · 17:15
clinicalIf duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
▶Ep 67 · 4:18
epidemiologicalThe majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.↗
▶Ep 67 · 5:42
clinicalWhen reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent.↗
▶Ep 67 · 5:42
quoteIf you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.↗
▶Ep 67 · 6:02
clinicalSize discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length.↗
▶Ep 67 · 7:08
epidemiologicalGastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes.↗
▶Ep 67 · 7:08
quoteIn general, gastroschisis, they don't tend to do as well regardless.↗
▶Ep 67 · 7:12
quoteIf, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.↗
▶Ep 67 · 9:09
clinicalSTEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile.↗
▶Ep 67 · 9:09
quoteI don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.↗
▶Ep 67 · 9:41
clinicalIn scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection.↗
▶Ep 67 · 10:45
clinicalThe STEP procedure was originally described by HP Kim and Tom Jackson.↗
▶Ep 67 · 11:02
clinicalFor STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility.↗
▶Ep 67 · 11:29
clinicalThe anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling.↗
▶Ep 67 · 11:58
clinicalBowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded.↗
▶Ep 67 · 12:20
opinionAn endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients.↗
▶Ep 67 · 12:34
clinicalStaple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°).↗
▶Ep 67 · 12:58
clinicalA vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience.↗
▶Ep 67 · 13:23
clinicalTarget caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility.↗
▶Ep 67 · 14:09
clinicalBefore firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length.↗
▶Ep 67 · 14:28
clinicalA U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point.↗
▶Ep 67 · 14:50
clinicalThe procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter.↗
▶Ep 67 · 15:08
clinicalAt the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time.↗
▶Ep 67 · 16:23
clinicalThe STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°).↗
▶Ep 67 · 16:56
clinicalThe duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum.↗
▶Ep 67 · 17:03
opinionAvoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak.↗
▶Ep 67 · 17:15
clinicalIf duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 2
▶Ep 68 · 1:52
quoteI think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility.↗
▶Ep 68 · 1:52
clinicalThe biggest benefit of the STEP procedure is tapering the bowel and reestablishing a more normal caliber to improve motility.↗
▶Ep 68 · 3:04
clinicalIt can take up to 6 months before you actually start to see a significant improvement in absorptive capacity after STEP.↗
▶Ep 68 · 3:04
quoteIt can take up to 6 months before you actually start to see a significant improvement in absorptive capacity.↗
▶Ep 68 · 3:16
clinicalImprovements in absorptive capacity are measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and citrulline levels as they rise over time.↗
▶Ep 68 · 3:24
clinicalThe reason absorptive improvement takes time is that inflamed, sick, leaky mucosa in the setting of bacterial overgrowth needs to heal.↗
▶Ep 68 · 6:24
clinicalBleeding at STEP staple lines is an underreported complication that is really difficult to manage in some cases.↗
▶Ep 68 · 6:49
quoteMy hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment.↗
▶Ep 68 · 6:49
opinionStaple line bleeding is hypothesized to be a microbiome problem occurring in a pro-inflammatory environment.↗
▶Ep 68 · 6:55
clinicalStaple line bleeding tends to occur in type 2 anatomy, which is small bowel to colonic remnant in the absence of an intact colon ileocecal valve.↗
▶Ep 68 · 7:12
clinicalPathology of staple line ulcers shows non-specific inflammation with no vasculitis, no viral elements, and no obvious ischemia.↗
▶Ep 68 · 7:12
quoteIt's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult.↗
▶Ep 68 · 10:05
clinicalMost referrals for intestinal rehabilitation have had multiple operations before coming to the specialist center.↗
▶Ep 68 · 10:09
opinionOptimizing a child's anatomy may not be conducive to just one operation; you have to set yourself up sometimes planning for the next case.↗
▶Ep 68 · 10:09
quoteKeep your eye on the horizon, which is what can we do to try to optimize this kid's anatomy. And if you think about that plan, it may not be conducive to just one operation today.↗
Intestinal Rehabilitation, Episode 6: Cholestasis
▶Ep 73 · 1:09
quoteInstitutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event.↗
▶Ep 73 · 1:09
guidelineCincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event.↗
▶Ep 73 · 1:20
guidelineA 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event.↗
▶Ep 73 · 2:57
clinicalIn young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition).↗
▶Ep 73 · 3:30
quoteNow, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that.↗
▶Ep 73 · 3:30
clinicalPrematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors.↗
▶Ep 73 · 4:09
clinicalPrevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery.↗
▶Ep 73 · 7:00
clinicalTwo strategies for reversing cholestasis are dose restriction and change of lipid composition.↗
▶Ep 73 · 7:08
clinicalConventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized.↗
▶Ep 73 · 7:19
clinicalSMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver.↗
▶Ep 73 · 7:45
clinicalSMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency.↗
▶Ep 73 · 7:57
clinicalWhen SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency.↗
▶Ep 73 · 8:06
guidelineConventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day.↗
▶Ep 73 · 8:52
quoteMy opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to.↗
▶Ep 73 · 8:52
opinionA bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold.↗
▶Ep 73 · 12:11
clinicalDuring secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot.↗
▶Ep 73 · 12:53
clinicalLiver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits.↗
▶Ep 73 · 13:00
clinicalElastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis.↗
▶Ep 73 · 13:31
clinicalFollow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made.↗
Intestinal Rehabilitation, Episode 7: Refeeding in a neonatal patient
▶Ep 79 · 0:57
quoteEven if a kid has a complicated disease process, one of our goals as a team is to try to establish the most normal feeding habits we can that not only promote gut function and everything else, but also to optimize quality of life and the importance of the social aspects of eating that we all experience with our families and friends.↗
▶Ep 79 · 0:57
clinicalOne goal in intestinal failure management is to establish normal feeding habits that promote gut function and optimize quality of life including social aspects of eating.↗
▶Ep 79 · 2:22
clinicalWhen initiating feeds in patients with high stoma output, losses increase initially, but this must be pushed through in a hospital setting where volume replacement is available.↗
▶Ep 79 · 4:34
clinicalIn short bowel syndrome, protein absorption is fairly well preserved, so the benefit of completely broken-down protein formulas (free amino acids or hydrolysates) is primarily from an allergy perspective.↗
▶Ep 79 · 4:46
quoteIn the world of short bowel syndrome, protein absorption is actually fairly well preserved. So it seems like the benefit is from an allergy perspective.↗
▶Ep 79 · 5:04
clinicalMany formulas have moved from predominant long-chain fat to increasing MCT components, but long-chain fat is a stronger driver for adaptation.↗
▶Ep 79 · 5:04
quoteOver the last several years, a lot of the formulas have moved from predominant long chain fat to increasing in MCT components. But long chain fat is a stronger driver for adaptation, and I personally haven't really bought into that.↗
▶Ep 79 · 6:06
clinicalOne milliliter of parenteral nutrition is not isocaloric with one milliliter of formula, and advancing beyond 100-120 per kilo creates problems with not only calories and protein but also sodium and calcium due to compositional differences.↗
▶Ep 79 · 6:06
quoteYou're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula. So if you keep going beyond 100 per kilo or 120 per kilo, you, you have a problem. And kids, not only will they take a calorie protein hit, they'll take, you start to get into problems with sodium and calcium as well, because the composition in the milk is not the same as what's in the parental solution.↗
▶Ep 79 · 6:28
clinicalAt some point during feeding advancement, fortification is necessary.↗
▶Ep 79 · 7:21
clinicalFeeding options include bolus (oral or gastric via tube), continuous (post-pyloric tube or surgical J-tube), or a hybrid model with gastric bolus component plus continuous component (gastric or post-pyloric).↗
▶Ep 79 · 8:05
quoteBecause I like bolus feeds as a default, I'd like, I like to see a patient fail bolus feeds before they end up on continuous feeds as a sole delivery remote.↗
▶Ep 79 · 8:05
opinionBolus feeds are preferred as the default approach; patients should fail bolus feeds before being placed on continuous feeds as the sole delivery method.↗
▶Ep 79 · 8:17
clinicalContinuous feeds can be used as a supplemental approach, with bolus feeds during the day and continuous supplementation overnight.↗
▶Ep 79 · 8:40
clinicalEven when oral feeding is non-nutritive, it is important for skill development; children who never learn to suck, swallow, and process food by mouth will not eat solids later and will remain dependent on tube feeding.↗
▶Ep 79 · 8:40
quoteOne thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.↗
▶Ep 79 · 12:04
quoteYeah, and it, it goes to the point that we've made in a previous podcast about having a plan and and resisting the temptation to get it all done in one. Operation. It's all about optimizing the patient for the next step, but you don't have to do it all in one go.↗
▶Ep 79 · 12:12
clinicalThe technical benefit of distal feeding is that size discrepancy at anastomosis is significantly improved because the bowel has been used.↗
▶Ep 79 · 13:43
clinicalThe hole from a removed G-tube closes very quickly.↗
▶Ep 79 · 13:51
clinicalA G-tube provides versatility for nutrition supplementation, medication delivery, and venting for gassy or bloated children to improve enteral tolerance.↗
Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient
▶Ep 80 · 1:24
clinicalThe benefit and curse in older patients with bowel loss is that they already know how to eat and have established that behavior.↗
▶Ep 80 · 1:24
quoteThe benefit in the curse in a patient like that is that they do know how to eat. They've established that that behavior.↗
▶Ep 80 · 1:50
opinionEven if you cannot cure the patient and get them off TPN, it is important to make compromises to optimize quality of life.↗
▶Ep 80 · 1:50
quoteSo even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can.↗
▶Ep 80 · 2:14
quoteThe general strategy is you want to push the macronutrient modules of protein and fat are well tolerated. Most of these kids don't tolerate simple sugars very well.↗
▶Ep 80 · 2:14
clinicalThe general strategy is to push the macronutrient modules of protein and fat, which are well tolerated.↗
▶Ep 80 · 2:21
clinicalMost patients with short bowel do not tolerate simple sugars very well.↗
▶Ep 80 · 2:25
clinicalThe general concept of pushing solids and minimizing fluid intake helps prevent dumping.↗
▶Ep 80 · 2:25
quoteSo the general concept of pushing solids. And minimizing fluid intake helps. They don't tend to dump as much smaller meals more frequently of solids separated from their liquids to a certain extent, and minimization of simple sugars.↗
▶Ep 80 · 2:31
clinicalSmaller meals more frequently of solids separated from liquids, with minimization of simple sugars, reduces dumping tendency.↗
▶Ep 80 · 2:52
clinicalPatients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices, particularly increased sugars.↗
▶Ep 80 · 3:31
clinicalMinimizing sugars reduces symptoms related to bacterial overgrowth, making patients less bloated and gassy.↗
▶Ep 80 · 3:48
clinicalIn older children with fluid losses (by stoma or other source), if they are on parenteral support, some fluid can be replaced IV.↗
▶Ep 80 · 4:01
clinicalAs you try to get patients off IV support, keeping them hydrated by replenishing losses through enteral replacements is important.↗
▶Ep 80 · 4:11
quoteFor transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt.↗
▶Ep 80 · 4:11
clinicalTransport requires sodium and glucose, so the rehydration solution must contain some glucose and some salt.↗
▶Ep 80 · 4:39
clinicalThere are homemade recipes for oral rehydration solutions and over-the-counter preparations available.↗
▶Ep 80 · 4:48
clinicalGatorade does not always work well for rehydration because it has too much sugar in it.↗
▶Ep 80 · 4:48
quoteSome people will resort to things like Gatorade and stuff like that, but that doesn't always work that well. It's actually too much sugar in it.↗
▶Ep 80 · 6:23
opinionIt is all about normal growth parameters and getting patients through puberty.↗
▶Ep 80 · 6:28
quoteIt is true some kids end up back on parental support, you know, to get through puberty. And then when you're not growing anymore as an adult, often that borderline or marginal gut function is enough to sustain them.↗
▶Ep 80 · 6:28
clinicalSome kids end up back on parenteral support to get through puberty.↗
▶Ep 80 · 6:33
clinicalWhen patients are not growing anymore as adults, borderline or marginal gut function is often enough to sustain them.↗
▶Ep 80 · 7:22
clinicalPatients need to be monitored for micronutrient deficiencies after getting off TPN, as this is when they often get into trouble.↗
▶Ep 80 · 7:31
opinionLong-term growth and outcome need to be followed, highlighting the importance of multidisciplinary teams.↗
▶Ep 80 · 8:51
clinicalWeight is tracked as a major metric for growth, but it has to be balanced with height.↗
▶Ep 80 · 8:57
clinicalWhat is commonly seen in this population is round babies where their weight for height is elevated.↗
▶Ep 80 · 8:57
quoteWhat we commonly see in this type of population is. Round babies where their weight for height is elevated↗
▶Ep 80 · 9:01
clinicalThere is increasing data looking at quality of weight: how much is fat weight versus lean body mass.↗
Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes
▶Ep 108 · 5:23
quoteThey go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation.↗
▶Ep 108 · 5:23
clinicalLiver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation.↗
▶Ep 108 · 5:23
quoteThey go anywhere from mild cholestasis to more profound steatosis, which we see in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis leading to death or the need for transplantation.↗
▶Ep 108 · 5:23
clinicalLiver disease associated with intestinal failure ranges from mild cholestasis to profound steatosis (in older children/adolescents/adults), with progression through inflammation, fibrosis, and ultimately cirrhosis leading to death or transplantation.↗
▶Ep 108 · 5:41
clinicalRisk 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.↗
▶Ep 108 · 5:41
quoteThere's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN.↗
▶Ep 108 · 5:41
quoteThere's some patient factors related to prematurity, uh, lack of enteral feeding, the inability to feed because of the anatomy or recurrent infections, sepsis, which can affect the liver directly and indirectly. And the components of TPN.↗
▶Ep 108 · 5:41
clinicalRisk 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.↗
▶Ep 108 · 6:08
quoteThe term intestinal rehab program was coined in the year 2000.↗
▶Ep 108 · 6:08
clinicalThe term 'intestinal rehabilitation program' was coined in the year 2000.↗
▶Ep 108 · 6:08
clinicalThe term 'intestinal rehabilitation program' was coined in the year 2000.↗
▶Ep 108 · 6:08
quoteThe term intestinal rehab program was coined in the year 2000.↗
▶Ep 108 · 6:12
clinicalIntestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity.↗
▶Ep 108 · 6:12
quoteIntestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity.↗
▶Ep 108 · 6:12
clinicalIntestinal rehabilitation teams improve outcomes through integration of care, improved communication, and better continuity.↗
▶Ep 108 · 6:12
quoteIntestinal rehab teams improve the outcome of children because of integration of care, improved communication, better continuity.↗
▶Ep 108 · 6:34
clinicalIntestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists.↗
▶Ep 108 · 6:34
quoteWe have a large reliance on speech, language, and occupational therapy.↗
▶Ep 108 · 6:34
quoteWe have a large reliance on speech, language, and occupational therapy.↗
▶Ep 108 · 6:34
clinicalIntestinal rehabilitation programs rely heavily on speech, language, and occupational therapy specialists.↗
▶Ep 108 · 6:38
quoteWhile our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications.↗
▶Ep 108 · 6:38
clinicalWhile patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications.↗
▶Ep 108 · 6:38
clinicalWhile patients are on TPN, the goal is to mitigate progression of liver disease, sepsis, and vascular complications.↗
▶Ep 108 · 6:38
quoteWhile our patients are on TPN, we're trying to mitigate the progression of liver disease, sepsis, vascular complications.↗
▶Ep 108 · 6:45
quoteHaving a dedicated unit with dedicated staff all contributes to better outcomes.↗
▶Ep 108 · 6:45
quoteHaving a dedicated unit with dedicated staff all contributes to better outcomes.↗
▶Ep 108 · 6:54
clinicalFamilies of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas.↗
▶Ep 108 · 6:54
quoteWhat we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma.↗
▶Ep 108 · 6:54
clinicalFamilies of intestinal failure patients manage significant tasks at home including running TPN pumps, adding vitamins, programming pumps, recording ins and outs, managing G-tubes, and managing stomas.↗
▶Ep 108 · 6:54
quoteWhat we ask our Families to do at home is significant. These patients go home on TPN. The families run the pump, add vitamins to the TPN, program the pump, record ins and outs, manage the G tube, manage the stoma.↗
▶Ep 108 · 7:11
quoteWe educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout.↗
▶Ep 108 · 7:11
clinicalHigh-quality families demonstrate evidence of burnout over time despite education and empowerment efforts.↗
▶Ep 108 · 7:11
clinicalHigh-quality families demonstrate evidence of burnout over time despite education and empowerment efforts.↗
▶Ep 108 · 7:11
quoteWe educate and empower our parents because we've all seen high-quality families that over time demonstrate evidence of burnout.↗
▶Ep 108 · 7:25
quoteThere's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down.↗
▶Ep 108 · 7:25
epidemiologicalLiterature shows 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.↗
▶Ep 108 · 7:25
epidemiologicalLiterature shows 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.↗
▶Ep 108 · 7:25
quoteThere's literature that shows the improvement of outcomes with programs in general. You see an improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and the patients that were listed for transplant are coming off the list because of improved status. Mortality has gone down.↗
▶Ep 108 · 7:43
epidemiologicalMortality in intestinal failure has decreased with intestinal rehabilitation programs.↗
▶Ep 108 · 7:43
epidemiologicalMortality in intestinal failure has decreased with intestinal rehabilitation programs.↗
▶Ep 108 · 8:02
quoteLipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways.↗
▶Ep 108 · 8:02
clinicalLipids 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.↗
▶Ep 108 · 8:02
quoteLipids are a source of essential fatty acids. They're a non-protein energy source important for growth. Fatty acids have a major role in cellular pathways.↗
▶Ep 108 · 8:02
clinicalLipids 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.↗
▶Ep 108 · 8:15
clinicalHistorically, 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.↗
▶Ep 108 · 8:15
quoteHistorically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content.↗
▶Ep 108 · 8:15
quoteHistorically, most of the lipid emulsions in North America are soybean-based, so they have a high phytosterol content. A high omega 6 long chain polyunsaturated fatty acid content and low antioxidant content.↗
▶Ep 108 · 8:15
clinicalHistorically, 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.↗
▶Ep 108 · 8:31
clinicalProlonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis.↗
▶Ep 108 · 8:31
quoteIt is associated with deterioration of liver dysfunction, i.e., cholestasis.↗
▶Ep 108 · 8:31
clinicalProlonged soybean-based lipid exposure is associated with deterioration of liver function, specifically cholestasis.↗
▶Ep 108 · 8:31
quoteIt is associated with deterioration of liver dysfunction, i.e., cholestasis.↗
▶Ep 108 · 8:51
epidemiologicalFor 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.↗
▶Ep 108 · 8:51
quoteThis is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease.↗
▶Ep 108 · 8:51
quoteThis is a paper that we wrote several years ago that showed that for every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease.↗
▶Ep 108 · 8:51
epidemiologicalFor 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.↗
▶Ep 108 · 9:15
clinicalFirst-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years.↗
▶Ep 108 · 9:15
quoteThe first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives.↗
▶Ep 108 · 9:15
quoteThe first generation were basically the soybean-based lipids, rich in omega 6UUFA. That would be intralipids, and that's been around for 40 years and it's saved a lot of lives.↗
▶Ep 108 · 9:15
clinicalFirst-generation lipid emulsions were soybean-based, rich in omega-6 PUFA (such as Intralipid), and have been in use for 40 years.↗
▶Ep 108 · 9:32
quoteThe 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids.↗
▶Ep 108 · 9:32
clinicalThird-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids.↗
▶Ep 108 · 9:32
quoteThe 3rd generation lipids attempt to not only reduce the omega 6 component, but to change the omega 6 to the omega 3 ratio with the addition of omega 3 lipids.↗
▶Ep 108 · 9:32
clinicalThird-generation lipid emulsions reduce omega-6 component and change the omega-6 to omega-3 ratio by adding omega-3 lipids.↗
▶Ep 108 · 9:51
clinicalOmega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile.↗
▶Ep 108 · 9:51
quoteOmega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile.↗
▶Ep 108 · 9:51
quoteOmega 6 lipids are metabolized to arachidonic acid. Which produce leukotrienes and prostaglandins of the more pro-inflammatory profile. The omega 3 lipids are metabolized through EPA and DHA and lead to the production of cytokines that have a less inflammatory profile.↗
▶Ep 108 · 9:51
clinicalOmega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile.↗
▶Ep 108 · 10:01
clinicalOmega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile.↗
▶Ep 108 · 10:01
clinicalOmega-3 lipids are metabolized through EPA and DHA, leading to production of cytokines with a less inflammatory profile.↗
▶Ep 108 · 10:15
clinicalOmega-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.↗
▶Ep 108 · 10:15
quoteOmega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function.↗
▶Ep 108 · 10:15
quoteOmega 3 lipids improve bile flow because they decrease lithogenicity of the bile. They decrease steatosis. They stimulate improved beta oxidation and clearance, and they lower oxidative stress and support immune function.↗
▶Ep 108 · 10:15
clinicalOmega-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.↗
▶Ep 108 · 10:32
clinicalThe two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition.↗
▶Ep 108 · 10:32
quoteOne is lipid minimization, and then the second strategy is a change in composition.↗
▶Ep 108 · 10:32
clinicalThe two main strategies for improving lipids in parenteral nutrition are lipid minimization and change in composition.↗
▶Ep 108 · 10:32
quoteOne is lipid minimization, and then the second strategy is a change in composition.↗
▶Ep 108 · 10:42
quoteThere's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less.↗
▶Ep 108 · 10:42
clinicalLiterature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less.↗
▶Ep 108 · 10:42
quoteThere's a lot of literature dating back even decades that show that if you restrict exposure to soybean lipid, you improve cholestasis. When you dose the lipid at 1 g per kilo per day or less.↗
▶Ep 108 · 10:42
clinicalLiterature dating back decades shows that restricting exposure to soybean lipid improves cholestasis, particularly when dosed at 1 g/kg/day or less.↗
▶Ep 108 · 11:00
quoteThe problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much.↗
▶Ep 108 · 11:00
clinicalIn preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much.↗
▶Ep 108 · 11:00
quoteThe problem with this in preterm babies is that fat is important for growth, especially for neurocognitive development. So there is the risk of essential fatty acid deficiency if we restrict lipid dosing too much.↗
▶Ep 108 · 11:00
clinicalIn preterm babies, fat is important for growth, especially for neurocognitive development, creating a risk of essential fatty acid deficiency if lipid dosing is restricted too much.↗
▶Ep 108 · 11:22
clinicalDHA and arachidonic acid are important for retinal and brain development.↗
▶Ep 108 · 11:22
quoteDHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting.↗
▶Ep 108 · 11:22
clinicalDHA and arachidonic acid are important for retinal and brain development.↗
▶Ep 108 · 11:22
quoteDHA and arachidonic acid are important for retinal and brain development. None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting.↗
▶Ep 108 · 11:26
clinicalNone of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings.↗
▶Ep 108 · 11:26
clinicalNone of the current lipid emulsions were designed for premature babies; they were all designed for adults in critical care settings.↗
▶Ep 108 · 11:38
quoteWe're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid.↗
▶Ep 108 · 11:38
clinicalWork is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content.↗
▶Ep 108 · 11:38
clinicalWork is currently underway with industry to create a lipid emulsion specifically tailored to preterm babies with higher arachidonic acid content.↗
▶Ep 108 · 11:38
quoteWe're currently in work with industry to create a lipid emulsion that's more specifically tailored to preterm babies that has a higher content of arachidonic acid.↗
▶Ep 108 · 12:03
clinicalCentral lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported.↗
▶Ep 108 · 12:03
quoteSo our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage.↗
▶Ep 108 · 12:03
quoteSo our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families. They are at risk for complications, infections, blockages, thrombosis, and breakage.↗
▶Ep 108 · 12:03
clinicalCentral lines are the lifeline for intestinal failure patients, and without venous access, families cannot be supported.↗
▶Ep 108 · 12:10
clinicalCentral lines are at risk for complications including infections, blockages, thrombosis, and breakage.↗
▶Ep 108 · 12:10
clinicalCentral lines are at risk for complications including infections, blockages, thrombosis, and breakage.↗
▶Ep 108 · 12:24
quoteIn the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide.↗
▶Ep 108 · 12:24
epidemiologicalIn the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide.↗
▶Ep 108 · 12:24
epidemiologicalIn the United States, there are approximately 240,000 central line-associated bloodstream infections (CLABSIs) annually, with each costing about $30,000, totaling approximately $2 billion per year nationwide.↗
▶Ep 108 · 12:24
quoteIn the United States, there's about 240,000 collapses in the United States on an annual basis. The cost of one of these is about $30,000 about $2 billion a year nationwide.↗
▶Ep 108 · 12:48
clinicalThe team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain.↗
▶Ep 108 · 12:48
clinical4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution.↗
▶Ep 108 · 12:48
quoteWe landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution.↗
▶Ep 108 · 12:48
clinical4% tetrasodium EDTA (Kitelock) is antithrombotic, anti-fibrinolytic, and antimicrobial, satisfying all three criteria important for a good lock solution.↗
▶Ep 108 · 12:48
clinicalThe team pivoted to 4% tetrasodium EDTA (Taurolock/Kitelock) when ethanol became too expensive and difficult to obtain.↗
▶Ep 108 · 12:48
quoteWe landed on 4% tetrosodium EDTA or kite lock as it's known in the trade name, which is an antithrombolytic, anti-fibrinolytic, and antimicrobial. So, it satisfies all three criteria that are important for a good lock solution.↗
▶Ep 108 · 13:23
quoteIt's licensed in Europe, it's licensed in Australia. It's not available in the United States.↗
▶Ep 108 · 13:23
clinicalKitelock is licensed in Europe and Australia but not available in the United States.↗
▶Ep 108 · 13:23
quoteIt's licensed in Europe, it's licensed in Australia. It's not available in the United States.↗
▶Ep 108 · 13:23
clinicalKitelock is licensed in Europe and Australia but not available in the United States.↗
▶Ep 108 · 13:29
quoteI'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States.↗
▶Ep 108 · 13:29
clinicalA multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year.↗
▶Ep 108 · 13:29
clinicalA multi-center randomized trial of Kitelock versus heparin went live 4 weeks ago and is actively recruiting patients, with results expected within the next year.↗
▶Ep 108 · 13:29
quoteI'm happy to say that we have finally launched our multi-center randomized trial of kitelock versus heparin, which went live 4 weeks ago, and we're actively recruiting patients. In the next year, we'll have the results of the trial, and we can get the kitelock into the United States.↗
▶Ep 108 · 13:58
quoteIn short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon.↗
▶Ep 108 · 13:58
clinicalIn 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).↗
▶Ep 108 · 13:58
quoteIn short bowel syndrome, we have 3 anatomical subtypes. Type 1 is the high L piginostomy. Type 2, where you've lost your distal small bowel, your ileum, and part of your colon, and there's a small bowel colonic anastomosis. And then type 3 where you've lost the mid-part of your small bowel, but you still have some retained ileum and an intact colon.↗
▶Ep 108 · 13:58
clinicalIn 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).↗
▶Ep 108 · 14:26
epidemiologicalThe most common anatomical subtype of short bowel syndrome in pediatrics is Type 2.↗
▶Ep 108 · 14:26
quoteThe most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum.↗
▶Ep 108 · 14:26
epidemiologicalThe most common anatomical subtype of short bowel syndrome in pediatrics is Type 2.↗
▶Ep 108 · 14:26
quoteThe most common anatomical subtype in pediatrics is type 2. These patients struggle. Type 1 and type 2 struggle because they lack an ileum.↗
▶Ep 108 · 14:31
clinicalType 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum.↗
▶Ep 108 · 14:31
clinicalType 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum.↗
▶Ep 108 · 14:53
quoteThe problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection.↗
▶Ep 108 · 14:53
clinicalNative GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use.↗
▶Ep 108 · 14:53
clinicalNative GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use.↗
▶Ep 108 · 14:53
quoteThe problem is that the native hormone has a half-life of 7 minutes. So it's not practical. So it's been altered, one amino acid alteration to an analog called taglutide. That has a half-life of 2 hours and it's given once a day subcutaneous injection.↗
▶Ep 108 · 14:58
clinicalGLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection.↗
▶Ep 108 · 14:58
clinicalGLP-2 has been altered by one amino acid to create an analog called teduglutide, which has a half-life of 2 hours and is given once daily by subcutaneous injection.↗
▶Ep 108 · 15:23
epidemiologicalA multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period.↗
▶Ep 108 · 15:23
quoteWhat we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements.↗
▶Ep 108 · 15:23
quoteWhat we see in patients that received taglutide, a 40% reduction in TPN fluid and calorie requirements over a six-month period. And 70% of patients that received the analog achieved the study endpoint, which was a 20% reduction in TPN requirements.↗
▶Ep 108 · 15:23
epidemiologicalA multi-center phase 3 study published in 2020 at Cincinnati Children's showed patients receiving teduglutide had a 40% reduction in TPN fluid and calorie requirements over a six-month period.↗
▶Ep 108 · 15:32
epidemiological70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements.↗
▶Ep 108 · 15:32
epidemiological70% of patients who received teduglutide achieved the study endpoint of a 20% reduction in TPN requirements.↗
▶Ep 108 · 15:48
guidelineTeduglutide is licensed for children greater than 1 year of age.↗
▶Ep 108 · 15:48
quoteIt's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone.↗
▶Ep 108 · 15:48
quoteIt's licensed for children that are greater than 1 year of age. Already, it's made a big difference in our practice and even kids with very extreme anatomy have been able to get off TPN as a result of being able to replace this hormone.↗
▶Ep 108 · 15:48
guidelineTeduglutide is licensed for children greater than 1 year of age.↗
▶Ep 108 · 15:50
clinicalTeduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN.↗
▶Ep 108 · 15:50
clinicalTeduglutide has made a big difference in practice, with even children with very extreme anatomy able to get off TPN.↗
▶Ep 108 · 16:02
clinicalAnother GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly.↗
▶Ep 108 · 16:02
quoteAnother GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide.↗
▶Ep 108 · 16:02
quoteAnother GLP-2 analog, a proglutide, has a longer half-life and can be given once a week. That's currently being studied. And what we've shown is that it actually works even better than taglutide. You get more bowel lengthening with a proglutide compared to glutide.↗
▶Ep 108 · 16:02
clinicalAnother GLP-2 analog, apraglutide, has a longer half-life and can be given once weekly.↗
▶Ep 108 · 16:10
clinicalApraglutide works even better than teduglutide, producing more bowel lengthening.↗
▶Ep 108 · 16:10
clinicalApraglutide works even better than teduglutide, producing more bowel lengthening.↗
▶Ep 108 · 16:36
quoteThese interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal.↗
▶Ep 108 · 16:36
epidemiologicalThese interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008.↗
▶Ep 108 · 16:36
quoteThese interventions have led to a decrease in transplantation since 2008. In large part because of the successes of intestinal rehabilitation, the outcomes of transplant at 5 years are about 65%. Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal.↗
▶Ep 108 · 16:36
epidemiologicalThese interventions (optimized lipids, central line infection prevention, GLP-2 analogs) have led to a decrease in transplantation since 2008.↗
▶Ep 108 · 16:41
opinionThe decrease in transplantation is in large part because of the successes of intestinal rehabilitation.↗
▶Ep 108 · 16:41
epidemiologicalOutcomes of intestinal transplant at 5 years are approximately 65%.↗
▶Ep 108 · 16:41
opinionThe decrease in transplantation is in large part because of the successes of intestinal rehabilitation.↗
▶Ep 108 · 16:41
epidemiologicalOutcomes of intestinal transplant at 5 years are approximately 65%.↗
▶Ep 108 · 16:49
opinionThe goal is to avoid transplant and lifelong immunosuppression whenever possible.↗
▶Ep 108 · 16:49
opinionThe goal is to avoid transplant and lifelong immunosuppression whenever possible.↗
▶Ep 108 · 17:19
quoteWe have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team.↗
▶Ep 108 · 17:19
quoteWe have lots of exciting medical and surgical options available to us, but this should all be done under the umbrella of a multidisciplinary team.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2
▶Ep 2 · 1:23
quoteThe goalposts have moved over the last two decades.↗
▶Ep 2 · 1:37
quoteLike in Toronto, we, we classified that as less than 20% of expected for age.↗
▶Ep 2 · 1:37
clinicalIn Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters.↗
▶Ep 2 · 1:52
epidemiologicalOverall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%.↗
▶Ep 2 · 2:07
clinicalUltra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters.↗
▶Ep 2 · 2:13
clinicalLong-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management.↗
▶Ep 2 · 2:13
quoteThere should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management.↗
▶Ep 2 · 2:26
clinicalUltra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants.↗
▶Ep 2 · 5:59
clinicalNeonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective.↗
▶Ep 2 · 6:10
quoteI don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.↗
▶Ep 2 · 6:10
opinionNot every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes.↗
▶Ep 2 · 6:29
opinionLong-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients.↗
▶Ep 2 · 7:20
opinionLipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers.↗
▶Ep 2 · 7:40
quoteYou know, that was never really a practice that we subscribe to.↗
▶Ep 2 · 11:53
quoteThe surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right?↗
▶Ep 2 · 11:53
opinionThe surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive.↗
▶Ep 2 · 12:04
quoteThose intraoperative decisions have a lifelong impact.↗
▶Ep 2 · 12:13
quoteSo, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't.↗
▶Ep 2 · 13:52
clinicalTraditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching.↗
▶Ep 2 · 14:20
clinicalSome of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse.↗
▶Ep 2 · 14:21
quoteOften, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.↗
Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient
▶Ep 4 · 1:24
clinicalThe benefit and curse in older patients with bowel loss is that they already know how to eat and have established that behavior.↗
▶Ep 4 · 1:24
quoteThe benefit in the curse in a patient like that is that they do know how to eat. They've established that that behavior.↗
▶Ep 4 · 1:50
opinionEven if you cannot cure the patient and get them off TPN, it is important to make compromises to optimize quality of life.↗
▶Ep 4 · 1:50
quoteSo even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can.↗
▶Ep 4 · 2:14
clinicalThe general strategy is to push the macronutrient modules of protein and fat, which are well tolerated.↗
▶Ep 4 · 2:14
quoteThe general strategy is you want to push the macronutrient modules of protein and fat are well tolerated. Most of these kids don't tolerate simple sugars very well.↗
▶Ep 4 · 2:21
clinicalMost patients with short bowel do not tolerate simple sugars very well.↗
▶Ep 4 · 2:25
clinicalThe general concept of pushing solids and minimizing fluid intake helps prevent dumping.↗
▶Ep 4 · 2:25
quoteSo the general concept of pushing solids. And minimizing fluid intake helps. They don't tend to dump as much smaller meals more frequently of solids separated from their liquids to a certain extent, and minimization of simple sugars.↗
▶Ep 4 · 2:31
clinicalSmaller meals more frequently of solids separated from liquids, with minimization of simple sugars, reduces dumping tendency.↗
▶Ep 4 · 2:52
clinicalPatients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices, particularly increased sugars.↗
▶Ep 4 · 3:31
clinicalMinimizing sugars reduces symptoms related to bacterial overgrowth, making patients less bloated and gassy.↗
▶Ep 4 · 3:48
clinicalIn older children with fluid losses (by stoma or other source), if they are on parenteral support, some fluid can be replaced IV.↗
▶Ep 4 · 4:01
clinicalAs you try to get patients off IV support, keeping them hydrated by replenishing losses through enteral replacements is important.↗
▶Ep 4 · 4:11
quoteFor transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt.↗
▶Ep 4 · 4:11
clinicalTransport requires sodium and glucose, so the rehydration solution must contain some glucose and some salt.↗
▶Ep 4 · 4:39
clinicalThere are homemade recipes for oral rehydration solutions and over-the-counter preparations available.↗
▶Ep 4 · 4:48
quoteSome people will resort to things like Gatorade and stuff like that, but that doesn't always work that well. It's actually too much sugar in it.↗
▶Ep 4 · 4:48
clinicalGatorade does not always work well for rehydration because it has too much sugar in it.↗
▶Ep 4 · 6:23
opinionIt is all about normal growth parameters and getting patients through puberty.↗
▶Ep 4 · 6:28
clinicalSome kids end up back on parenteral support to get through puberty.↗
▶Ep 4 · 6:28
quoteIt is true some kids end up back on parental support, you know, to get through puberty. And then when you're not growing anymore as an adult, often that borderline or marginal gut function is enough to sustain them.↗
▶Ep 4 · 6:33
clinicalWhen patients are not growing anymore as adults, borderline or marginal gut function is often enough to sustain them.↗
▶Ep 4 · 7:22
clinicalPatients need to be monitored for micronutrient deficiencies after getting off TPN, as this is when they often get into trouble.↗
▶Ep 4 · 7:31
opinionLong-term growth and outcome need to be followed, highlighting the importance of multidisciplinary teams.↗
▶Ep 4 · 8:51
clinicalWeight is tracked as a major metric for growth, but it has to be balanced with height.↗
▶Ep 4 · 8:57
quoteWhat we commonly see in this type of population is. Round babies where their weight for height is elevated↗
▶Ep 4 · 8:57
clinicalWhat is commonly seen in this population is round babies where their weight for height is elevated.↗
▶Ep 4 · 9:01
clinicalThere is increasing data looking at quality of weight: how much is fat weight versus lean body mass.↗
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 5 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 5 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 5 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 5 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 5 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 5 · 3:03
guidelineAn 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.↗
▶Ep 5 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 5 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 5 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 5 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 5 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 5 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 5 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 5 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 5 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 5 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 5 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 5 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 5 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 5 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
▶Ep 5 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 6 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 6 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 6 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 6 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 6 · 3:03
guidelineAn 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.↗
▶Ep 6 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 6 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 6 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 6 · 4:50
clinicalCauses 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).↗
▶Ep 6 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 6 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 6 · 5:45
clinicalMotility 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.↗
▶Ep 6 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 6 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 6 · 6:17
clinicalEnteropathies 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.↗
▶Ep 6 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 6 · 7:11
clinicalSome 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.↗
▶Ep 6 · 7:45
clinicalAlthough 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.↗
▶Ep 6 · 12:46
clinicalImproved 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.↗
Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2
▶Ep 9 · 1:23
quoteThe goalposts have moved over the last two decades.↗
▶Ep 9 · 1:37
clinicalIn Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age; in a term baby that corresponds to 20–30 centimeters.↗
▶Ep 9 · 1:37
quoteLike in Toronto, we, we classified that as less than 20% of expected for age.↗
▶Ep 9 · 1:52
epidemiologicalOverall survival in the ultra-short gut population in the current era of management is over 90%, actually 90–95%.↗
▶Ep 9 · 2:07
clinicalUltra-short gut patients who reached enteral autonomy required multiple nutritional supplements but were able to grow within normal parameters.↗
▶Ep 9 · 2:13
quoteThere should be more optimism in general with this population just because our ability to mitigate those long term, you know, risks. Of death from liver disease or sepsis have been transformed in the current era of management.↗
▶Ep 9 · 2:13
clinicalLong-term risks of death from liver disease or sepsis in ultra-short gut patients have been transformed in the current era of management.↗
▶Ep 9 · 2:26
clinicalUltra-short gut patients who did better tended to have some remnant ileum and longer colonic remnants.↗
▶Ep 9 · 5:59
clinicalNeonatologists see some of the survivors—ex-premature infants with intestinal failure—return to their follow-up clinics, which informs their perspective.↗
▶Ep 9 · 6:10
opinionNot every child needs to die with a laparotomy decision; the data will tell whether the unoperated septic/inflammatory response leads to negative outcomes.↗
▶Ep 9 · 6:10
quoteI don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.↗
▶Ep 9 · 6:29
opinionLong-term neurocognitive tracking of this population is part of the program's responsibility and is beneficial to patients.↗
▶Ep 9 · 7:20
opinionLipid restriction as a way of controlling liver disease was never a practice subscribed to by the speakers.↗
▶Ep 9 · 7:40
quoteYou know, that was never really a practice that we subscribe to.↗
▶Ep 9 · 11:53
opinionThe surgeon's intraoperative decisions—what is done and what is not done—have a lifelong impact and are critically important; these decisions can determine whether the child stays on or gets off TPN or whether they survive.↗
▶Ep 9 · 11:53
quoteThe surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right?↗
▶Ep 9 · 12:04
quoteThose intraoperative decisions have a lifelong impact.↗
▶Ep 9 · 12:13
quoteSo, those, those decisions that you make at the time that you see an intraabdominal catastrophe can make the difference between whether this kid will get, stay on or get off TPN or whether they survive or don't.↗
▶Ep 9 · 13:52
clinicalTraditional teaching is to remove obviously dead and infarcted bowel, leave suspicious or questionable bowel, and return in 24–48 hours to let it demarcate; the approach described is a departure from that teaching.↗
▶Ep 9 · 14:20
clinicalSome of these children look worse over the next 24–36 hours, then start to stabilize—they may not get better, but they stop getting worse.↗
▶Ep 9 · 14:21
quoteOften, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.↗
Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1
▶Ep 10 · 2:02
clinicalIn short gut syndrome, residual intestine undergoes adaptation to compensate and reestablish function to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 10 · 2:02
quoteThat remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.↗
▶Ep 10 · 2:16
quoteThe process is driven by The presence of intraluminal nutrients and their interaction with both gut secretions, you know, pancreatic biliary secretions, as well as the impact on the relationship with trophic gut peptides.↗
▶Ep 10 · 2:16
clinicalThe adaptive process is driven by the presence of intraluminal nutrients and their interaction with gut secretions (pancreatic, biliary) and trophic gut peptides.↗
▶Ep 10 · 2:42
clinicalStructural changes during adaptation include mucosal hypertrophy (increased villous length), increased blood supply through angiogenesis, bowel dilation, and in younger children, gut lengthening—all increasing surface area for nutrient absorption.↗
▶Ep 10 · 2:58
quoteWhat all of those things have in common is that they increase surface area for absorption absorption of nutrients, right?↗
▶Ep 10 · 3:04
clinicalFunctional changes during adaptation include slowed motility to allow more contact time and up-regulation of enterocyte transporters to move nutrients across cells more efficiently.↗
▶Ep 10 · 3:18
quoteSo, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.↗
▶Ep 10 · 5:35
clinicalUntil very recently, there was no standardized definition for enteral autonomy, and most intestinal failure outcomes have poor definitions.↗
▶Ep 10 · 5:35
quoteUp until very recently, there actually was no standardized definition for that.↗
▶Ep 10 · 6:10
clinicalHigher proportions of patients are now surviving to have the ability to reach enteral autonomy due to better management of TPN complications such as line infections, liver disease, and vascular thrombosis.↗
▶Ep 10 · 6:20
clinicalIn the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; current management is much better at preventing these complications.↗
▶Ep 10 · 6:39
guidelineThe current ASPEN guidelines define enteral autonomy as independence from parenteral support for 12 weeks with maintenance of adequate growth and hydration during that time period.↗
▶Ep 10 · 6:39
quoteSo the current Aspen guidelines that just came out with standardized definitions, the independence of uh parental support for 12 weeks and with maintenance of adequate growth and hydration during that time period.↗
▶Ep 10 · 6:53
clinicalA patient is not truly off TPN unless they can actually grow off TPN; stopping TPN without achieving growth is a mistake.↗
▶Ep 10 · 6:53
quoteBecause it's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.↗
▶Ep 10 · 7:57
epidemiologicalIn the 2012 Squires/PIFCO paper, 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% received transplants.↗
▶Ep 10 · 8:13
quoteBasically, 50% of patients achieved dental autonomy over 56 years, 25% died and 25% got transplanted.↗
▶Ep 10 · 8:25
epidemiologicalRecent papers from the last 5-6 years show that 60-80% of patients now achieve enteral autonomy, representing improved outcomes compared to historical data.↗
▶Ep 10 · 8:35
quoteHas increased between, depending on who you read, anywhere between 60 to 80%.↗
▶Ep 10 · 9:55
clinicalSmall bowel length is an independently significant variable predicting adaptive capacity, which is intuitive since the majority of nutrient digestion and fluid absorption occurs in the small bowel.↗
▶Ep 10 · 10:18
quoteYou know, the ileum has a much greater capacity to adapt than the jejunum does.↗
▶Ep 10 · 10:18
clinicalThe ileum has a much greater capacity to adapt than the jejunum; patients with predominant ileal anatomy do better than those with predominant jejunal anatomy.↗
▶Ep 10 · 10:34
clinicalA full-term baby is born with approximately 160 centimeters of small bowel, which grows to about 425 centimeters by age 5 years, with the steepest growth rate between 35 weeks gestation and 6 months postnatal.↗
▶Ep 10 · 10:40
quoteWhen you're 5 years old, you have about 425 centimeters of small bowel, and the rate of growth on the curve is steepest between 35 weeks gestation to about 6 months postnatal.↗
▶Ep 10 · 11:13
opinionThe presence or absence of the ileocecal valve is a predictor of adaptation, though the valve itself may not be the important factor; rather, loss of the valve typically accompanies loss of the terminal ileum, which is the bigger factor affecting adaptive potential.↗
▶Ep 10 · 11:34
quoteMost people that lose their ileocecal valve also lose their terminal ileum, and I think that is the bigger factor as far as, uh, adaptive potential.↗
▶Ep 10 · 11:53
quoteSo we've presented data that shows that if you've got the majority of your small bowel, then it almost doesn't matter how much colon you have, you have a probability of ventral autonomy somewhere between 85 to 100%.↗
▶Ep 10 · 11:53
clinicalWhen a patient has the majority of their small bowel, it almost does not matter how much colon they have—probability of enteral autonomy is 85-100%.↗
▶Ep 10 · 12:06
clinicalWhen small bowel remnant is less than 50% of expected length, the colon becomes vitally important, assuming an increasing role in energy absorption from short-chain fatty acids and fluid/salt absorption.↗
Intestinal rehabilitation: What is intestinal rehab? - Episode 1
▶Ep 3 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 3 · 1:56
clinicalUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 3 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 3 · 2:03
clinicalIntestinal failure is when gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 3 · 3:03
guidelineAn 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.↗
▶Ep 3 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 3 · 3:44
clinicalThe intestinal rehabilitation approach streamlines care and improves communication with families and between care providers.↗
▶Ep 3 · 4:39
clinicalCauses of intestinal failure divide into three categories: short bowel syndrome, motility disorders, and congenital enteropathies.↗
▶Ep 3 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 3 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 3 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 3 · 5:06
clinicalCauses of short bowel syndrome include congenital anomalies (intestinal atresia, malrotation, volvulus, gastroschisis, long segment Hirschsprung disease) and acquired neonatal diseases (necrotizing enterocolitis).↗
▶Ep 3 · 5:45
clinicalMotility disorders occur when abnormalities of intestinal muscle or the nerves controlling that muscle prevent coordinated propulsion of food and stool.↗
▶Ep 3 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 3 · 6:17
clinicalCongenital enteropathies are conditions where patients have all their bowel but the mucosal lining does not digest or absorb properly.↗
▶Ep 3 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 3 · 7:11
clinicalSome patients have elements of two or all three categories of intestinal failure in their presentation.↗
▶Ep 3 · 7:11
quoteThe reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.↗
▶Ep 3 · 7:45
epidemiologicalMost intestinal failure patients are infants or babies, but some older pediatric patients develop intestinal failure from inflammatory bowel disease, Crohn disease complications, trauma, malignancy, or vascular thrombosis.↗
▶Ep 3 · 12:46
clinicalLong-term survivors now develop 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 problems.↗
▶Ep 3 · 12:46
quoteBut now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.↗
Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?
▶Ep 4 · 1:46
quoteWell, let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.↗
▶Ep 4 · 1:56
guidelineUntil recently, there was no standardized definition for intestinal failure.↗
▶Ep 4 · 2:03
clinicalIntestinal failure is a functional problem where gut function is insufficient to absorb enough nutrients, fluids, and calories to support survival and, in children, growth.↗
▶Ep 4 · 2:03
quoteIt it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.↗
▶Ep 4 · 3:03
guidelineAn 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.↗
▶Ep 4 · 3:03
quoteSo, um, an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.↗
▶Ep 4 · 3:44
clinicalIntestinal rehabilitation streamlines care and improves communication between family and care providers through an amalgamation of experts providing holistic, comprehensive, coordinated care.↗
▶Ep 4 · 4:39
quotePatients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.↗
▶Ep 4 · 4:50
clinicalCauses 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).↗
▶Ep 4 · 4:50
epidemiologicalShort bowel syndrome is by far the most common category of intestinal failure in pediatric patients.↗
▶Ep 4 · 4:50
quoteThere's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure, and causes of short bowel syndrome are usually related to diagnoses of or, or disorders of the neonate.↗
▶Ep 4 · 5:45
quoteSo if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle. And the bowel is unable to push things through in a coordinated way.↗
▶Ep 4 · 5:45
clinicalMotility 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.↗
▶Ep 4 · 6:10
quoteAnd they are therefore dependent on intravenous support.↗
▶Ep 4 · 6:17
quoteEnteropathies or the congenital diarrheas, and these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.↗
▶Ep 4 · 6:17
clinicalEnteropathies 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.↗
▶Ep 4 · 7:11
clinicalSome 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.↗
▶Ep 4 · 7:45
clinicalAlthough 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.↗
▶Ep 4 · 12:46
clinicalImproved 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.↗
Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
▶Ep 5 · 4:18
epidemiologicalThe majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.↗
▶Ep 5 · 5:42
quoteIf you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.↗
▶Ep 5 · 5:42
clinicalWhen reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent.↗
▶Ep 5 · 6:02
clinicalSize discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length.↗
▶Ep 5 · 7:08
epidemiologicalGastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes.↗
▶Ep 5 · 7:08
quoteIn general, gastroschisis, they don't tend to do as well regardless.↗
▶Ep 5 · 7:12
quoteIf, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.↗
▶Ep 5 · 9:09
clinicalSTEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile.↗
▶Ep 5 · 9:09
quoteI don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.↗
▶Ep 5 · 9:41
clinicalIn scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection.↗
▶Ep 5 · 10:45
clinicalThe STEP procedure was originally described by HP Kim and Tom Jackson.↗
▶Ep 5 · 11:02
clinicalFor STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility.↗
▶Ep 5 · 11:29
clinicalThe anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling.↗
▶Ep 5 · 11:58
clinicalBowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded.↗
▶Ep 5 · 12:20
opinionAn endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients.↗
▶Ep 5 · 12:34
clinicalStaple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°).↗
▶Ep 5 · 12:58
clinicalA vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience.↗
▶Ep 5 · 13:23
clinicalTarget caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility.↗
▶Ep 5 · 14:09
clinicalBefore firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length.↗
▶Ep 5 · 14:28
clinicalA U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point.↗
▶Ep 5 · 14:50
clinicalThe procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter.↗
▶Ep 5 · 15:08
clinicalAt the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time.↗
▶Ep 5 · 16:23
clinicalThe STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°).↗
▶Ep 5 · 16:56
clinicalThe duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum.↗
▶Ep 5 · 17:03
opinionAvoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak.↗
▶Ep 5 · 17:15
clinicalIf duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail.↗
