Nutrition Interventions for Pediatric Patients with Short Bowel Syndrome
Jackie CostantinoSodexo Dietetic
Intern
Austin Rath
“I just want to eat everything.”
Outline
▫Discussion of SBS and current treatments
▫Medical Nutrition Therapy
▫Case Study Patient
▫Questions
What is Short Syndrome?
Bowel
What is SBS? • Significant loss of bowel length leading to
malabsorption of fluid and nutrients • 7 out of 1,000 live births for neonates with birth
weights <1500g
• Risk with birth weight & gestational age
• Outcome based on many variables: length, anatomy of bowel resection, functional mass
• May be accompanied by intestinal failure (IF)
SBS Associated Intestinal Failure
•Definition in the pediatric population: ▫Insufficient intestinal mass to…
Absorb and digest fluid and nutrients Maintain fluid, protein-energy and
micronutrient balance for normal growth and development
▫Acute IF: Dependent on PN for 4-6 weeks▫Chronic IF: Dependent on PN >90 days
Etiologies
NECGastroschisisIntestinal atresiaVolulusAganglionosisCombinationOthers
Squires R et al . J. Pediatric. 2012
Gastroschisis
•Congenital defect when an infant's intestines protrude from the body through one side of the umbilical cord
http://www.cdc.gov/ncbddd/ birthdefects/Gastroschisis-graphic.html
Midgut Volvulus • Involves the entire
midgut twisting around the super mesenteric artery (SMA), cutting off the blood supply
• Midgut includes:▫ Distal duodenum▫ Ileum▫ Colon▫ Transverse colon
http://emedicine.medscape.com/article/411249-overview
Signs & Symptoms: Pre-resection
•Dependent on the etiology of SBS•Broad signs and symptoms
▫bilious vomiting▫abdominal pain ▫abdominal distention▫tachycardia▫tachypnea▫shock▫bloody stools
Complications Post-resection
• Intolerance and malabsoption ▫Diarrhea▫Steatorrhea
•Nutritionl deficiencies Weight loss (acute malnutrition) Growth stunting & head circumference
(chronic) Dry scaly skin Brittle hair and nails Poor wound healing
Absorption Of Nutrients Along the GI Tract Risk for specific nutritional deficiencies depend on the anatomy of the small bowel resection
Pathophysiology: 3 Phases
1. Immediate post-operative phase (1-7 days)
▫ Loss of communication between stomach and small intestine
▫ Poor absorption Loss of fluid and electrolytes
2. Adaptation ▫ Intestinal growth and morphological development ▫ EN is initiated critical to adaptation ▫ Can increase absorptive capacity by 4X the initial
capacity
3. Intestinal Autonomy▫ 100% EN is achieved
Labs & Tests •LFTs•BMP•CBC•Prealbumin & CRP•Tryglycerides •Calcium, phosphorus, magnesium•Fat soluble vitamins (ADEK) •Vitamin B12•Serum zinc levels•Endoscopy & colonoscopy
Treatment Options
•Surgical interventions ▫Intestinal transplantation ▫Intestinal lengthening procedures
•Substances indicated to promote adaptation ▫Growth hormone (GH)▫Glutamine▫Glucagon-like peptide 2 (GLP-2)
Intestinal Lengthening Procedures
Bianchi Procedure STEP Procedure
http://surgery.med.umich.edu/pediatric/chirp/clinical/treatments.shtml
Substances Indicated to Increase Adaptation•GH (FDA approved in adults)
▫Zorbtive® (somatropin rDNA origin for injection)
▫191 amino acid peptide hormone ▫GH + glutamine may stimulate intestinal
growth
•GLP-2 (not FDA approved)▫Gattex® (teduglutide) ▫33 amino acid peptide and growth hormone▫Adult studies show dependence on TPN
Medical Nutrition Therapy
Crucial Component to SBS Management
Role of the RD
•Evaluate nutritional status
• Identify malnutrition and growth failure
• Improve patients nutritional status through interventions
Goals of the RD
•Goals of the RD1. To ensure patient is receiving 100%
nutritional needs for proper growth and development
2. Initiate EN as soon as medically appropriate
3. Wean patient from TPN to reduce associated risks
4. End goal 100% EN
ADIME
•Assessment
•Diagnosis
•Interventions
•Monitoring and
•Evalulation
Assessment
•Patient’s history•Anthropometrics•“Ins and Outs” •Stool
characteristics •Feeding access
points•Food history
•Estimated needs•Physical
observations•Medications and
supplements•Laboratory and
diagnostic tests
Assessment
•Estimated Needs▫Pediatric Nutrition Care Manual:
Calories: Estimated Energy Requirement (EER) 1.2
Protein: DRI 1.3
▫Pediatric Reference Guide of Texas Children’s Hospital: Calorie needs: DRI x 1.0-1.5
Diagnosis
•Common problems for SBS:▫ Increased nutrient needs (NI-5.1) ▫Altered gastrointestinal function (NC-1.4) ▫ Impaired nutrient utilization (NC 2.1)
•Example PES statement SBS:▫Altered gastrointestinal function related to short
bowel syndrome (___cm remaining), as evidenced by inability to tolerate full enteral feeds and need for parenteral nutrition support.
Interventions
•Parenteral Nutrition ▫Cycling ▫Lipid Reduction Therapy ▫Omega-3 fatty acids for PN lipids▫Ethanol lock therapy
•Enteral Nutrition▫Nutrition source ▫Continuous vs. Bolus ▫Modulars
Total Parenteral Nutrition (TPN)•Essential when intestinal failure (IF) is
present
•Necessary for proper growth and development, but NOT ideal route for nutrition!
•Associated with 2 main causes of death among SBS▫PN-associated liver disease (PNALD) ▫Central line infections
PN-Association Liver Disease (PNALD)
▫Most prevalent and severe complication of long term PN
▫ 27% in children and 85% in neonates
▫Risk of death 8 fold when cholestasis is present
PN-Associated Liver Disease (PNALD)•Nutritional interventions to reduce risk of
PNALD:▫Wean from TPN (#1) ▫Cycling TPN ▫Lipid reduction therapy ▫Omega-3 fatty acids for PN lipids
Lipid Reduction Therapy
Reducing lipids to 1g/kg/day 3 times per week has shown to improve bilirubin levels
and resolve cholestasis in SBS patients without causing EFAD.
Lipid Reduction Therapy
•Prospective study at the University of Michigan ▫2005-2007▫31 NICU patients on PN with direct bili of
2.5 mg/dL▫Treatment group: 1g/kg/day 2 times per
week ▫Control group: 3/kg/day daily ▫EFAD monitored monthly
Results
• Treatment group: bili levels
• Control group: slight bili levels
• Treatment group developed
mild EFAD, but resolved when lipids increased to 1g/kg/d 3days/week
• No difference in growth
Omega-3 Fatty Acids • Use of omega-3 fatty acids as an alternative to
standard lipid emulsions may risk for PNALD
• Theory: omega-3 fatty acids have less pro-inflammatory effects and potential anti-inflammatory properties
• Omegaven® is the only current lipid emulsion made from 100% fish oil
Diamond et al. Changing the Paradigm: Omegaven for the Treatment of Liver Failure in Pediatric Short Bowel
Syndrome.
Central Line Infections•10-35% mortality associated with line
infections •More common in children
• risk for sepsis
•Can cause loss of central venous access for PNrisk for malnutrition
http://surgery.med.umcommon in children ich.edu/pediatric/clinical/patient_content/a-m/broviac_placement.shtml
Central Line Infections
•Ethanol lock therapy▫Dramatically reduces rate of a blood stream
infections▫Can be initiated in patients when weight is
>5kg and TPN cycling is achieved (at 22 hours)▫Most effect when given daily for at least 2 hours ▫NOT compatible with heparin ▫NOT compatible with polyurethane
catheters
Enteral Nutrition
• Introduce EN as soon as possible
•EN provides several beneficial effects on the GI tract▫Fuel for enterocytes ▫Stimulates hyperplasia▫Promotes peristalsis- decreases bacterial
overgrowth ▫Stimulates flow of GI secretions
Initiating EN
• Initiate trophic feeds of one of the following:
1. Mother expressed breast milk (MEBM) 2. Donor expressed breast milk (DEBM)3. Protein Hydrosylate formulas
Semi-elemental Elemental
Formulas
Semi-Elemental
Infant Pediatric
Alimentum Peptamen Jr.
Pregestimil Peptamen 1.5
Nutramigen Pediasure Peptide
Elemental
Infant Pediatric
Neocate Infant
Neocate Jr.
Elecare Infant
Elecare Jr.
Nutramigen Infant
Vivonex
Continuous vs. Bolus
Continuous▫ Preferred method in
infants and children with SBS
▫ Causes less stress and demand on intestinal function
▫ Provides constant saturation of intestinal wall may promote adaptation
Bolus▫ More physiological
▫ More often used in older children
▫ Less tolerated in infants
▫ Depends on the individual’s tolerance level
Modulars
•Pectin •Benefiber•Beneprotein•Duocal •Polycose•MCT oil•Human Milk Fortifier
Monitoring and Evaluation
Trend anthropometrics Monitor labs closely vitamin/mineral deficiencies for decreased liver function Monitor I/OsAdjust feeding regimen accordingly to meet 100% needs
Case Study
Presentation of Patient• CM
• 13 months old
• Full term, no significant history
• Twin brother
• Diagnosed with SBS at 15 weeks
CM’s Course of Care at SCHC
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
CM’s Hospital Course
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
• Admitted with abdominal distention • Diagnosed with midgut volvulus • 160 cm bowel resection• 16 cm remaining with ICV & colon• Broviac & G-tube placement• TPN & trophic feeds initiated
CM’s Hospital Course
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
May 1,2012Initial
Nutrition Assessment Age: 10 ½
mos
Chief Complaint: Broviac infection Medications: ELT, Gentamycin, Heparin Diet order: (G-tube) Elecare 20 @ 24ml/hr with 3tsp Benefiber Nutrition Support: D13P3.2L1 - 500mL HAL @ 32.2 mL/hr X 18Current Intake: (4/30) 495 mL HAL, 35mL IL, 596mL Elecare, 263mL NS with meds Anthropometrics:
• Weight: 9.8 kg (50th%ile)• Length: 79 cm (95th%ile) • Wt/Lgth: 10-25th%ile• Head circumference: 50 cm (>95th%ile)
Estimated Daily Needs:• 960 kcal (98 kcal/kg)- RDA• 16g pro (1.6g/kg)- RDA • 980mL fluid (100mL/kg)- Holiday-Segar
PES: Altered GI function related to short bowel syndrome as evidenced by 16cm remaining bowel and dependence on TPN/G-tube feeds to meet nutritional needs.
Recommended Interventions: • Continue D13P3.2L1 TFV of 550mL/day,
Lipids M/W/F• Provide HAL over 16 per home feeding regimen
(tapered) • 9.3mL/hr 1st and 16th hour, 18.5mL/hr 2nd and
15th hour, 37/hr 3rd-14th hour• Max GIR= 8.18
• Continue current G-tube feeding regimen• Daily weights, strict I/Os, monitor labs
Goals/evaluation: • Appropriate wt gain for age (11-12g/day) • Tolerates feeds
CM’s Hospital Course
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
May 1,2012Initial
Nutrition Assessment Age: 10 ½
mos
May 8, 2012F/U Nutrition AssessmentAge: 10 ¾
mos
CM’s Hospital Course
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
May 1,2012Initial
Nutrition Assessment Age: 10 ½
mos
May 8, 2012F/U Nutrition AssessmentAge: 10 ¾
mos
Wt: (5/7) 9.65kg, wt decreased 150g (21g/d X 7 days)TPN order: D13P3.2L1, TFV increased to 550ml/dayEN order: Elecare 20 with 3 tsp Benefiber: 20 oz @ 28mL/hr 672mL (69.6mL/kg), 448 kcal (46.4 kcal/kg), 13.8g pro (1.4g/kg) Intake (5/7): 712mL Elecare 20, 235mL D13P3.2, 19.5mL IL 670 kcal (69 kcal/kg), 27.8g Pro, 966mL (100mL/kg) Output (5/7): 1076mL (UOP= 4.665 mL/kg/hr), BM X2 Meds: Gentamycin, Ampicillin, ELT, Heparin
Diagnosis: Altered GI function related to SBS as evidenced by need for TPN/G-tube feeds
Interventions: • Continue current TPN regimen• Continue current EN order, increase per home schedule • T/C holding feeds for one hour and provide formula PO• Continue daily weights, strict I/Os, monitor labs • RD to follow
Monitoring/Evaluation:• Meet 100% needs • Wt gain 11-12g.day • Bowel movements WNL 5 BM/day • Tolerate TPN/G-tube feeds
CM’s Hospital Course
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
May 1,2012Initial
Nutrition Assessment Age: 10 ½
mos
May 13, 2012Readmitted
w/Central Line Infection
Age: 11 mos
May 8, 2012F/U Nutrition AssessmentAge: 10 ¾
mos
Chief Complaint: Fever with Broviac Medications: ELT, Cefotaxime, VancomycinDiet Order: Elecare 20 @ 28mL/hr via G-tube, Baby food PO ad lib Nutrition Support: D13P3.2 600mL x 19 (60mL/kg/d) @ 31.6mL (8AM-5PM) based on 10kg; L1 @5mL/hr x 20 M/W/F Current Intake: (5/13) 408.8 HAL, 672mL Elecare 20 ( I/O)= 1542.8/663Anthropometrics:
• Weight: 10.115 kg (50-75th%ile Wt/age) (5/1) 9.8kg, (4/7) 9.65kg
• Length/Height: 70 cm (~5th%ile Ht/age) • (4/26) 73.5, (5/1) 79cm inconsistency
• Wt/Ht: >95th%ile• Head circumference: 49 cm (>95th%ile HC/age)
Estimated Daily Needs: • 991 kcal (98 kcal/kg), 16.2g pro (1.6g/kg), 1012mL fluid (100mL/kg)
PES: Altered GI function related to SBS as evidenced by 16cm remaining small bowel and dependence on TPN/G-tube feeds to meet nutritional needs.
Recommended Interventions: • Continue current TPN with lipids M/W/F • Continue current EN regimen• T/C increasing Elecare 20 kcal/oz to 30mL/hr if BM WNL • Monitor daily weights, labs, I/Os and BM• Please re-check length (inconsistency)
CM’s Hospital Course
Oct 10- Nov 21, 2011
Diagnosis of SBSAge: 3 ¾ mos
Dec 5, 2011 – June 21, 2012
GI Outpatient VisitsAge: 5 ¾ mos- 12 mos
May 1,2012Initial
Nutrition Assessment Age: 10 ½
mos
May 13, 2012Readmitted
w/Central Line Infection
Age: 11 mos
May 8, 2012F/U Nutrition AssessmentAge: 10 ¾
mos
GI Outpatient Visits• Mom has gradually increased G-tube feeds 2mL/hr every
week as tolerated
• (start rate) 2mL/hr (current rate) 34mL/hr
• Gradually weaned from TPN
• Feeds held 2-3 times per day to allow PO
• Baby foods slowly introduced
• Benefiber consistently in feeds secondary to loose stools
Update on CM
•Current EN: ▫Elecare Jr. 37 kcal/oz @ 34mL/hr with
Benefiber•Current PN:
▫30g Dextrose per day (No amino acids or lipids)
•Plan: ▫To gradually concentrate Elecare Jr. by 2
kcal per week as tolerated to goal concentration of 30 kcal/oz
▫To continue to wean TPN
10/9
/07
10/2
6/07
11/1
2/07
11/2
9/07
12/1
6/07
1/2/
08
1/19
/08
2/5/
08
2/22
/08
3/10
/08
3/27
/08
4/13
/08
4/30
/08
5/17
/08
6/3/
08
6/20
/08
0
2
4
6
8
10CM’s Weight Progression
Date
Weig
ht
(kg
)
CM’s Progression from PN to EN
Date Age (mo) EN Regimen % Kcal from EN
PN Regimen % Kcal from PN
% KcalTOTAL
Oct 2011 4 ¼ None 0 D17 P3 L2.99 100 100
Nov 2011 5 2mL/hr 6 D16 P3 L2.5 94 100
Jan2012 7 ¼ 10mL/hr 27 *Lipids 3d/wk 73 100
April 2012 9 24ml/hr 50 D13 P3.2 L1 50 100
June 2012 12 34mL/hr 61 D13 P3.2 39 100
June 2012 12 ¼ 34mL/hr
*Elecare Jr. 22 73 50g D, 14g AA 27 100
Present 13 ¾ 34mL/hr*Elcare Jr. 27 90 30g D 10 100
Lipids reduced
Lipids D/C’d
AAs D/C’d
Critical Comments
•Anthropometrics- inconsistent height
•Estimated kcal needs
•Medications: ELT & heparin
•Laboratory values: suggestive of anemia
Summary
Key Points
Goal #1- Meet 100% needs for proper growth and development
Goal #2- Start EN as soon as medically appropriate
Goal #3- Reduce risk of PNALD and line infections
▫Wean TPN as EN increases▫Reduce lipids to 1g/kg/day 3X/week when
cholestasis is present
Austin’s Cupcake Fund
Questions?
References• Cole CR. Pathophysiology and Medical Management of Intestinal Failure in Childhood.
Cincinnati Children’s Hospital Medical Center 2012.• Beattie LM, Barclay AR, Wilson DC. Short bowel syndrome and intestinal failure in infants and
children. Paediatrics and Child Health 2010; 20:10.• Teitlbaun H. “Pediatric Intestinal Failure: Approaches to Optimize Care.” PASPEN (Philadelphia
Area Society for Parental and Enteral Nutrition) Spring Conference 2012.• Gastroschisis [CHOP]. Philadelphia: The Children’s Hospital of Philadelphia; c1996-2012
[updated 2012 Feb; cited 2012 June 10]. Available from http://www.chop.edu/service/fetal-diagnosis-and-treatment/fetal-diagnoses/gastroschisis.html.
• Intestinal Malrotation and Volvulus [Cincinnati Children’s]. Cincinnati: Cincinnati Children’s Hospital Medical Center; c1999-2012 (updated 2012 Aug; cited 2012 June]. Available from: http://www.cincinnatichildrens.org/health/i/intestinal-malrotation
• Bunting KD, Mills J, Phillips S, Ramsey E, Rich S, Trout S. Pediatric Nutrition Reference Guide. 9th ed. Houston: Texas Children’s Hospital; 2010.
• Pediatric Nutrition Care Manual. Short Bowel Syndrome. Available from: http://nutritioncaremanual.org/topic.cfm?ncm_heading=Nutrition%20Care&ncm_toc_id=144771
• McMellen M, Wakeman D, Longshore S, et al. “Growth Factors: Possible Roles for clinical Management of the Short Bowel Syndrome.” Semin Pediatr Surg 2010; 19 (1): 35-43.
• Tee C, Wallis K, Gabe S, et al. Emerging treatment options for short bowel syndrome: potential role of teduglutide. Clinical and Experimental Gastroenterology 2011:4 189-196.
Omegaven •Diamond et al.’s retrospective cohort study
•12 pediatric SBS patients with advanced PNALD
•All being considered for liver transplant
•Treatment: 1g/kg Intralipid, 1g/kg Omegaven (total lipids=2g/kg)
• Intralipid decreased or d/c’d if PNALD worsening
Results •9 out of 12 completely resolved
hyperbilirubinemia within a median of 24 weeks
•Out of those 9 patients:▫ 4 achieved resolution with combination of
Intralipid and Omegaven▫ 5 achieved resolution after Intralipids
discontinued
• All 12 patients were no longer considered for liver transplant
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