nutrition interventions for pediatric patients with short bowel syndrome jackie costantino sodexo...

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