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Nutrition Interventions for Pediatric Patients with Short Bowel Syndrome. Jackie Costantino Sodexo Dietetic Intern. Austin Rath. “I just want to eat everything.” . Outline. Discussion of SBS and current treatments Medical Nutrition Therapy Case Study Patient Questions.
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Nutrition Interventions for Pediatric Patients with Short Bowel Syndrome Jackie Costantino Sodexo 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 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
MidgutVolvulus • 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 • Immediate post-operative phase (1-7 days) • Loss of communication between stomach and small intestine • Poor absorption Loss of fluid and electrolytes • Adaptation • Intestinal growth and morphological development • EN is initiated critical to adaptation • Can increase absorptive capacity by 4X the initial capacity • 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® (somatropinrDNA 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 RD • To ensure patient is receiving 100% nutritional needs for proper growth and development • Initiate EN as soon as medically appropriate • Wean patient from TPN to reduce associated risks • 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: • Mother expressed breast milk (MEBM) • Donor expressed breast milk (DEBM) • Protein Hydrosylate formulas • Semi-elemental • Elemental
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/Os Adjust feeding regimen accordingly to meet 100% needs
Presentation of Patient • CM • 13 months old • Full term, no significant history • Twin brother • Diagnosed with SBS at 15 weeks
CM’sCourse of Care at SCHC Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos
CM’s Hospital Course Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos • Admitted with abdominal distention • Diagnosed with midgutvolvulus • 160 cm bowel resection • 16 cm remaining with ICV & colon • Broviac & G-tube placement • TPN & trophic feeds initiated
CM’s Hospital Course • 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 • Chief Complaint: Broviac infection • Medications:ELT, Gentamycin, Heparin • Diet order: (G-tube) • Elecare20 @ 24ml/hr with 3tsp Benefiber • Nutrition Support: • D13P3.2L1- 500mL HAL @ 32.2 mL/hr X 18 • Current 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 Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos
CM’s Hospital Course Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos
CM’s Hospital Course • Monitoring/Evaluation: • Meet 100% needs • Wt gain 11-12g.day • Bowel movements WNL 5 BM/day • Tolerate TPN/G-tube feeds • 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 • Wt:(5/7)9.65kg, wt decreased 150g (21g/d X 7 days) • TPN order: D13P3.2L1, TFV increased to 550ml/day • EN 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 Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos
CM’s Hospital Course • 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) • Chief Complaint: Fever withBroviac • Medications:ELT, Cefotaxime, Vancomycin • Diet Order: • Elecare20 @ 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)= 1542.8/663 • Anthropometrics: • Weight: 10.115 kg (50-75th%ileWt/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) Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos May 13, 2012 Readmitted w/Central Line Infection Age: 11 mos
CM’s Hospital Course Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos Dec 5, 2011 – June 21, 2012 GI Outpatient Visits Age: 5 ¾ mos- 12 mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos May 13, 2012 Readmitted w/Central Line Infection Age: 11 mos