Tag Archives: short bowel syndrome

Enteral Autonomy in Pediatric Intestinal Failure

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A recent study (FA Khan et al. J Pediatr 2015; 167: 29-34 -thanks to Mike Hart for forwarding this reference) provides data from a multicenter retrospective cohort of 272 infants.  These infants had of IF were defined by requiring >60 days of PN; they were enrolled in the Pediatric Intestinal Failure Consortium.  The median followup was 33.5 months.  The most common etiologies of IF were necrotizing enterocolitis (NEC), gastroschisis, small bowel atresia, and volvulus. Key findings:

  • 43% achieved enteral autonomy (EA), defined as freedom from PN for >3 months, 13% remained dependent on PN, and 43% had died, undergone intestinal transplantation, or both.
  • Infants with EA were more likely to have had NEC, preserved ileocecal valve, longer preserved small bowel length, and care at a non-transplant center (with retrospective study, high likelihood of a selection bias).

The associated editorial by Valeria Cohran (pages 6-8) notes that pediatric intestinal transplants peaked in frequency in 2007, but in 2014 there only 56 performed.  She also notes that the care of these children with short bowel syndrome in the first year of life is approximately $500,000 ± $250,000!  The improved survival is attributed to minimizing cholestasis with new lipid strategies, minimizing blood stream infections with better care and ethanol locks, and the use of autologous bowel reconstruction surgery. Bottomline: This study and several others show that meticulous care and advances in the treatment of intestinal failure improve the likelihood of survival without the need for intestinal transplantation. FULL CITATION: Khan FA et al. Predictors of enteral autonomy in children with intestinal failure: A multicenter cohort study. J Pediatr 2015 Jul; 167:29-34. [Free full-text J Pediatr article PDF | PubMed® abstract] Related blog posts:

These windows were huge -Grand Tetons in background

These windows were huge -Grand Tetons in background

 

 

Stick with the (intestinal) rehab program?

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More data on the progress of treatment of short bowel syndrome (SBS) programs:

  • Avitzur J, et al. JPGN 2015; 61: 18-23

In this study, the researchers from Toronto and the Group for Improvement of Intestinal Function and Treatment (GIFT) retrospectively examine 84 patients over 3 time periods: 1999-2002, 2003-2005, and 2006-2009.

Key points:

  • Across those time periods, the authors find fewer SBS patients that needed to be listed for transplantation despite similar baseline characteristics.  In addition, many more patients in the late period were removed from the transplant waiting list due to clinical improvement.
  • Another important finding was a drop in mortality (15% vs >60%) and a shift from previous causes like liver failure and sepsis to death from other comorbid conditions.
  • “Since 2009, we have performed only 1 IT [intestinal transplant].”  They note this is a worldwide trend with ~50% reduction in pediatric IT since 2008.
  • Even with ultrashort bowel (small bowel length <30 cm), there are reports that “50% of these patients achieved PN independence within 2 years.”  As such, this is a declining indication for IT listing. In this study, ultrashort bowel was the reason for listing in 11% in the last period compared 21% in the first time period.

Why is this happening?

The authors credit the intestinal rehab program (IRP) for this impact along with specific management changes including new lipid emulsions/lipid minimization to reduce IFALD, use of ethanol locks to reduce bloodstream infections, and bowel reconstructive procedures (e.g. STEP).

Briefly noted: Merras-Salmio L, Pakarinen MP. JPGN 2015; 61: 24-9. This second retrospective study (n=48) from Finland reinforces the view of improvements in cholestasis  and prognosis from 1988-2014.  Similar strategies, as noted above, were implemented in SBS management protocols.

Bottomline: The outlook has improved for SBS.  While this is good news, at the same time, there will be less pediatric gastroenterologists with extensive intestinal transplantation experience.

In Wyoming often there are stretches of nearly deserted highways

In Wyoming often there are stretches of nearly deserted highways

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Iron Deficiency Common in Patients Requiring Long-Term Parenteral Nutrition

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A recent study (JPEN J Parenter Enteral Nutr May 13, 2015 0148607115587329) demonstates a high rate of iron deficiency anemia in patients requiring home parenteral nutrition (Thanks to Kipp Ellsworth for reference).

From Abstract:

Methods: Medical records of patients receiving HPN at the Mayo Clinic from 1977 to 2010 were reviewed. Diagnoses, time to IDA development, and hemoglobin, ferritin, and mean corpuscular volume (MCV) values were extracted. Response of iron indices to intravenous iron replacement was investigated.

Results: Of 185 patients (122 women), 60 (32.4%) were iron deficient…Of 93 patients who had sufficient iron storage, 37 had IDA development after a mean of 27.2 months (range, 2–149 months) of therapy. Iron was replaced by adding maintenance iron dextran to PN or by therapeutic iron infusion. Patients with both replacement methods had significant improvement in iron status. With intravenous iron replacement, mean ferritin increased from 10.9 to 107.6 mcg/L (P < .0001); mean hemoglobin increased from 11.0 to 12.5 g/dL (P = .0001); and mean MCV increased from 84.5 to 89.0 fL (P = .007).

Conclusions: Patients receiving HPN are susceptible to IDA. Iron supplementation should be addressed for patients who rely on PN.

Zoo Atlanta

Zoo Atlanta

 

N2U -Part 2: Poor Growth and Short Bowel Syndrome

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Chicago -from Lincoln Park

Chicago -from Lincoln Park

2015 N2U Syllabus & Presentations

Failure to Thrive –Praveen Goday

These sessions were case-based learning.

Case 1

2 mo birth weight 4.5 kg, taking 80 cal/kg/day –20 cal, formula-fed.  Taking 8 gm/day

What to do?

Point –If infant has a high birth weight (relative to height), there is a tendency to drop significant percentiles.  Often, careful observation is best approach. (Taal et al. Obesity. 2013;21:1261-8.)

Case 2: 14 mo birth weight 2.2 kg (at term), weight and length below the 3rd percentile but tracking. Weight-for-length is at the 25th percentile.

What to do?

For SGA babies, ensure adequate calories, avoid juice, ensure no GI symptoms, follow their growth

Case 3: Patient born at 36 weeks gestation, birth weight 3 lb. 14 oz. lbs., birth length 17 in.; Growth was a consistent problem throughout pregnancy; Dysmorphic; genetic workup – negative (Growth curves on pg 72-73 of syllabus).

More data: Taking 27 cal/oz, high-calorie baby foods, no GI symptoms, screening labs negative.  What are your options?  Make sure the length is accurate.  If the weight-for-length is really decreasing, then probably a trial of nasogastric feedings.  In Milwaukee, AMT bridle is often used to prevent dislodgement –youtube video (7:37 min), uses magnets.  Still, tubes need to be changed month.  The AMT bridle can work for tubes as small as 5 Fr.

Practical definition of Failure to Thrive:

  • Weight-for-length <2nd percentile (WHO growth chart for kids <2 yrs) or BMI ❤rd percentile. BMI more problematic in infants because of accuracy of length. If any inaccurate measurement, BMI value squares the length value; thus exponentially inflating any discrepancy.
  • Poor or no weight gain over a period of time that varies according to the age of the child
  • Significant downward trend in weight percentiles; however, 30% of full-term infants cross one percentile and 23% cross two percentiles between birth and 2 years of age
  • Keep in mind parental heights and correction for prematurity (where applicable).

Key points:

  • Large for gestational infants often have “catch-down” growth. Avoid overly aggressive nutritional intervention
  • In small infants who are growing steady and with good wt-for-ht, avoid overly aggressive nutritional intervention.
  • Older kids with poor growth –screening labs: TTG IgA, IgA, CBC, ESR, CMP, TSH, Urinalysis, and possibly fecal elastase.
  • Older kids with poor growth—1st steps: avoid juices, avoid grazing (no feeding outside mealtimes except water)/scheduled meals & snacks, and probably cyprohepatadine. Management: Have child sit at table for 20 minutes, feedings every 3 hours, and avoid force feeding.
  • In children with history of prematurity under 32 weeks gestation who do not catch up by ~6 months of age — usually never catch up.
  • In infants/children with highly selective diets, may be presentation of autism. Often, an approach in those with food selectivity is to start by offering only foods the child used to eat (for a day) and see if this will work (should be safe for at least one day).

Short Bowel Syndrome –Valeria Cohran (pages 9-20 in syllabus)

Case:  3 ½-year-old AAF who presents for a second option. She is a former 26-week infant who had NEC. She has approximately 45 cm of residual bowel anastomosed to the transverse colon.

  • TPN-dependent
  • Minimal oral intake
  • Diarrhea up to 60 ml/kg with Enfacare

 

GI Fluid losses –see page 15 of syllabus (Wessel et al Semin Perinat 2007; 31: 104-11).  Sodium losses ~140 mEq/L from stomach, 80-140 mEq/L from ileostomy –in comparison, normal stool with sodium of ~5 mEq/L.

Key points:

  • Normal intestinal length varies greatly by gestational age; so residual 45 cm length in a 26 week infant suggests much greater potential for improvement than 45 cm length in a full term infants (page 14 in syllabus).
  • Avoid probiotics in patients with central lines.
  • Sodium depletion (urine sodium <10) associated with poor growth. Probably urine sodium >20 is adequate. Though, if high urine potassium (more than double urine sodium), this could indicate that urine sodium is retained at the expense of spilling potassium (ie. May need more sodium) Related post: Don’t Forget to Check Urine Sodium | gutsandgrowth
  • Pectin (liquid) can be helpful: 1% of volume intake. Benefiber can be helpful –expensive. Related blog post: Green beans for short gut syndrome | gutsandgrowth
  • Bacterial overgrowth –treatment can help diarrhea. Try to minimize PPIs –6 months after resection (period of gastric hypersecretion). Cholestyramine is not a popular option due to trouble with usage. Related post: Rehabilitation for Short Bowel Syndrome | gutsandgrowth
  • Micronutrient/vitamin monitoring. Page 16 in syllabus lists the micronutrient concentration of parenteral products and RDAs of micronutrients. “Don’t take copper out of TPN” –unless high level. ‘Worry some about micronutrient deficiency while on TPN but perhaps worry even more when transitioning off.’ Ubesie et al J Pediatr 2013 162: 1692-96. 93% anemic in this study of transitioning off TPN (iron,copper, other causes –pg 18 in syllabus). Related blog posts:Missing ingredients in TPN -Case Report | gutsandgrowth and TPN Drug Shortages -A Useful Reference | gutsandgrowth
  • B12 deficiency. If high MMA (likely due to B12 deficiency), then B12 shots recommended. B12 important for cognition. Related posts: Are we missing Vitamin B12? | gutsandgrowth and What I Didn’t Know About Vitamin B12 and Crohn’s Disease …
  • Iron deficiency. Consider anastomotic ulcers/ulceration of STEP procedure.
  • Lipid minimization/fish oil lipid formulations
  • Follow kids even after coming off TPN –at least annually. These kids can develop problems many years later.

More related posts:

Disclaimer: This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

 

 

 

 

 

 

 

 

 

Update on Enteral Nutrition in Pediatric Intestinal Failure

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It is always nice to see how other centers manage clinical problems.  In a recent review (J Pediatr 2014; 165: 1065-90) from Boston Children’s, the authors provide details on how they use enteral nutrition in pediatric intestinal failure (IF) patients.  Prior to reviewing their approach, the authors provide a few definitions:

  • “IF occurs when there is a reduction of functional intestinal mass necessary for adequate digestion and absorption for nutrient, fluid, and growth requirements, resulting in the need for intensive nutritional support.”
  • “IF resulting from extensive intestinal resection is termed SBS” (short bowel syndrome)

The authors also discuss intestinal adaptation and factors that predispose to improvement.  Enteral nutrition (EN) stimulates adaptation and ‘gut rest’ results in atrophy of intestinal mucosa.

Key points:

  • “Prompt initiation of enteral feeding after bowel resection has been shown to decrease the duration of hospitalization”
  • “The optimal choice for EN in infants with IF seems to be human milk…If human milk is unavailable, amino acid-based formulas have been associated with improved outcomes.”
  • If intact colon with ileocecal valve, supplementation with dietary fiber (e.g.. green beans) at 2 g/kg/d may be helpful.
  • In this population, there is a high prevalence of micronutrient deficiencies while on partial PN support, TPN (depending on availability of components), and when on exclusive enteral feedings.
  • “We commonly employ an approach that uses … continuous feeding at night and bolus feeding during the day.
  • Outcomes of IF are reviewed (noted in previous blog entry –see below). Citrulline can be useful predictor of enteral autonomy.

Feeding Advancement Principles -Figure 1:

When feeds are held, usually held for 8 hours and then restarted at 75% of previous rate

Stool output:

  • If <10 mL/kg/d or < 10 stools/d —->advance rate by 10-20 mL/kg/d
  • If 10-20 mL/kg/d or 10-12 stools/d —>no change
  • If >20 mL/kg/d or >12 stools/d  —->reduce or hold feeds

Ostomy output:

  • If 2 mL/kg/h —> advance rate by 10-20 mL/kg/d
  • If 2-3 mL/kg/h –>no change
  • If >3 mL/kg/h  –>reduce or hold feeds

Also suggested to reduce or holding feeds when/if:

  • signs of dehydration
  • stool reducing substances >1%
  • gastric aspirates > four times previous hour’s infusion rate

Oral feeds:

  • When developmentally appropriate, offer one hour’s worth of continuous feeds BID-TID after 5 days of continuous feeds.  Hold tube feeds during oral feeds.
  • More than an hour’s worth of oral feeds once infant has reached full volume of feeds by continuous route and gaining weight.

Take-home message: Outcomes of IF have improved.  This review provides one approach towards optimizing enteral nutrition.

Related blog posts:

 

Enteral Fish Oil and Intestinal Adaptation in Premature Infants

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A provocative article (J Pediatr 2014; 165: 274-9) examines supplementation of enteral fat/fish oil in premature infants as a mechanism to reduce parenteral nutrition associated cholestasis (PNAC).  While the study’s limitations will prevent any dramatic conclusions, the article and associated editorial (pgs 226-27) do make several useful points.

Before discussing the limitations, the design of the study:

Infants were block randomized (block size of 8) into either a control group or treatment group.  While both groups received conventional PN, the treatment group received supplemental enteral fat as microlipid and fish oil after tolerating enteral feeds at 20 mL/kg/d.  Microlipid was started at 1 g/kg/d and advanced up to 2.5 g/kg/d; coinciding with microlipid increases, parenteral intralipid was decreased.  Fish oil was started at 0.2 g every 12 hours and was advanced to a maximum of 0.5 g every 6 hours.  The two fish oil products were Major Fish Oil 500 (Major Pharmaceuticals) and Rugby Sea Omega 50 (Rugby Laboratories).

The limitations include the following:

  • Small cohort of 18 patients in each arm
  • Due to the smell of fish oil, the study could not truly be blinded which introduces potential bias
  • Only 7 of the 36 patients could be considered to have short bowel syndrome as most of the infants had small amounts of intestine resected
  • Advancement of enteral feedings were halted if stoma output reached 20 mL/kg/d.  The editorialists note that 40 mL/kg/d would be more typical.  Thus, in both the treatment group and the control group, there was significant opportunity to reduce PN by more aggressive enteral nutrition advancement.

With these limitations in mind, there authors were able to show that supplemental fat (with fish oil) was associated with less parenteral intravenous lipid, and reduced conjugated bilirubin prior to anastomosis.  However, there was no significant difference in PN duration.  Growth parameters were similar prior to anastomosis, but improved in the treatment group after anastomosis.

In the editorial, it is noted that “enteral feeding with a high-fat diet has been demonstrated to enhance structural features of resection-associated adaptation, the underlying mechanisms for this phenomenon are still presently unknown.”

Take-home message: Enteral fat/fish oil supplementation helped decrease parenteral intravenous lipids in this study.  More broadly, advancing enteral nutrition by accepting higher ostomy outputs is likely the best strategy to avoid PNAC and other PN-associated complications.

Related blog posts:

 

Don’t Forget to Check Urine Sodium

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In patients with short bowel syndrome and excessive ostomy losses, salt depletion is associated with failure to thrive.  An easy way to screen for this is checking a “spot” urine sodium level; a urine sodium <10 mmol/L is too low.  While this has been recognized in infants for a long time, a recent case report shows that this can be an issue for older children (up to 19 years) as well (Nutr Clin Pract 2014 vol. 29 no. 397-401 -thanks to Kipp Ellsworth’s twitter feed for this reference & link: http://goo.gl/TkjKyd).

The authors conclusion: “We advise that patients of all ages with high stoma output have routine urine sodium levels checked, particularly in the setting of weight loss or poor gain. Furthermore, instances of TBSD [total body salt depletion] should be treated with sodium supplementation.”

Missing ingredients in TPN -Case Report

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Recently one of my radiology colleagues, Dr. Laura Hayes, put together (lead author) a presentation (poster) for an upcoming meeting.  The main focus of the presentation is a TPN-dependent toddler who presented with refusal to walk due to copper deficiency.

Attached is a link to the presentation: TPN Copper.  This link is a power point presentation with numerous radiographs and even bone scan images.

Key points:

  • All TPN components except dextrose have been in periods of shortage over the last few years.
  • TPN-dependent patients may not be receiving all the needed components and their physicians may not have been notified of the specific shortage(s).
  • Copper deficiency leads to reduced activity of numerous enzymes important for function of bone, blood, skin, nervous system and hair.
  • Subperiosteal hemorrhage leads to the periosteal thickening seen in this case and is associated with the bone pain our patient experienced.
  • Increased losses of bilious fluid can increase the risk of copper deficiency due to the excretion of copper in bile.
  • Other TPN-related deficiencies reviewed include thiamine deficiency (Wernicke’s encephalopathy), Vitamin D deficiency (Rickets), and Vitamin C deficiency (Scurvy).

Another recent case report:

Oestreich AE, Cole CR. Vigorous periosteal reaction secondary to copper deficiency in an infant on total parenteral nutrition. (2013) Pediatr Radiol 43:1411-1413.

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More STEP Experience

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A retrospective review of all serial transverse enteroplasty (STEP) procedures from Omaha provides more data on long-term outcomes (J Pediatr 2014; 164: 93-8).  In total 51 patients underwent a total of 68 STEP procedures.  Underlying bowel diseases: NEC (n=8), Gastroschisis (n=29), Intestinal Atresia (n=11), and Volvulus (n=4).  Median length prior to STEP: 30 cm.

Results:

  • Median bowel length gain of 54% (median 51 cm) was noted with first STEP.
  • Median age of 1 year at first STEP
  • Parenteral caloire requirement decreased to median <20 kcal/kg/day at 1 year post-op
  • Longer length gains had higher risk of stricture formation.  Six strictures developed which occurred in the first 22 procedures in the series.  Subsequently, the authors state that they have not reduced the luminal diameter below 2 cm at any point.
  • 7 children underwent transplantation; 60% of non-transplanted children were enterally independent.  The authors note that “no child has gone on to transplant following a STEP procedure since August 2009.”
  • 48 of 51 children are alive at a median of 39 months of followup.

Bottomline: The authors philosophy is that the STEP should create uniformity of luminal diameter as the first priority rather than increased length.  Based on their experience, there appears to be a learning curve to optimizing outcomes with STEP procedures.

Also noted: J Pediatr 2013; 163: 1692-6.  Retrospective study of prospectively collected data from 178 children with regard to micronutrient deficiency among intestinal failure patients transitioning to enteral feeds. Figures 1 and 2 along with Table 2 provide the prevalence of micronutrient deficiency while receiving supplemental parenteral nutrition (PN) and while on full enteral nutrition (FEN).  Iron deficiency was most common in both situations with prevalence of 84% and 61% respectively. With the exception of folate (0%), all of the vitamins and micronutrients had fairly high rates of deficiency.  While on FEN defiencies were  the following:

  • Vitamin A 19%
  • Vitamin B12 6.5%
  • Vitamin D 30%
  • Vitamin E 6%
  • Copper 8%
  • Iron 61%
  • Selenium 4%
  • Zinc 23%

A recent post on The Pediatric Nutritionist blog provides a suggested approach to the monitoring of vitamins and micronutrients based on the need for parenteral nutrition and on the need to consider inflammatory markers in the interpretation of these lab values: The Importance of Nutrition Lab Monitoring Protocols Featuring …

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What helps kids poop?

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While there are a number of answers to the above title, the answer that I’m looking for is physical activity (JPGN 2013; 57: 768-74).

With regard to the referenced study, a large prospective birth-cohort study (n=347 participants) in Rotterdam showed that preschool children with increased physical activity had about 1/3rd less frequency of functional constipation in the fourth year of life.  Activity measurements at the age of 2 years were accomplished by wearing ActiGraph accelerometers during 1 weekday and 1 weekend day.  Additionally, children who had physical activity of 60 min/day at age 4 had about 1/2 the likelihood of having functional constipation.  There are several limitations to the study; reduced activity and constipation could both be present in some individuals as a consequence of personality or psychologic attributes rather than physical activity having a causal relationship in causing constipation.

Bottomline: Another good reason to encourage physical activity –it might help with regular bowel habits.

Also, on a separate note, a recent blog post by Kipp Ellsworth is a useful reference for lab monitoring (micronutrients and vitamins) in children with short bowel syndrome:

Blog | The Pediatric Nutritionist | Covering the world of infant, child …

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