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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 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 NEC Gastroschisis Intestinal atresia Volulus Aganglionosis Combination Others 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: Preresection • 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 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 1. 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/am/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 Elemental Infant Pediatric Infant Pediatric Alimentum Peptamen Jr. Neocate Infant Neocate Jr. Pregestimil Peptamen 1.5 Elecare Infant Elecare Jr. Nutramigen Pediasure Peptide Nutramigen Infant Vivonex Continuous vs. Bolus Continuous ▫ Preferred method in infants and children with SBS ▫ Causes less stress and demand on intestinal function Bolus ▫ More physiological ▫ More often used in older children ▫ Less tolerated in infants ▫ Provides constant saturation of intestinal wall may promote adaptation ▫ 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’s Course 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 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 SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos PES: GI function to short bowel ChiefAltered Complaint: Broviacrelated infection syndrome as evidenced by 16cm remaining bowel Medications: ELT, Gentamycin, Heparin and on TPN/G-tube feeds to meet Dietdependence order: (G-tube) nutritional needs. Elecare 20 @ 24ml/hr with 3tsp Benefiber Nutrition Support: Recommended Interventions: D13P3.2L1 - 500mL HAL @ 32.2 mL/hr X 18 •Continue D13P3.2L1 TFV of 550mL/day, Current Intake: Lipids (4/30) M/W/F 495 mL HAL, 35mL IL, 596mL Elecare, 263mL •Provide HAL over 16 per home feeding regimen NS with meds (tapered) Anthropometrics: •9.3mL/hr 1st thand 16th hour, 18.5mL/hr 2nd •Weight: 9.8 kg (50 %ile) th hour,th37/hr 3rd-14th hour and79 15cm •Length: (95 %ile) •Max10-25 GIR=th8.18 •Wt/Lgth: %ile •Continue current G-tube feeding •Head circumference: 50 cm (>95thregimen %ile) •Daily weights, I/Os, monitor labs Estimated Dailystrict Needs: • 960 kcal (98 kcal/kg)- RDA Goals/evaluation: •16g pro (1.6g/kg)- RDA •Appropriate wt gain for age (11-12g/day) •980mL fluid (100mL/kg)Holiday-Segar •Tolerates feeds 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 Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos Altered GI function150g related to SBS evidenced by need Wt:Diagnosis: (5/7) 9.65kg, wt decreased (21g/d X 7 as days) fororder: TPN/G-tube feedsTFV increased to 550ml/day TPN D13P3.2L1, Monitoring/Evaluation: EN•Meet order:100% Elecare 20 with 3 tsp Benefiber: 20 oz @ 28mL/hr 672mL needs Interventions: (69.6mL/kg), 448 kcal (46.4 kcal/kg), 13.8g pro (1.4g/kg) •Wt gain 11-12g.day •Continue currentElecare TPN Intake (5/7): 712mL 235mL D13P3.2, 19.5mL IL 670 kcal •Bowel movements WNLregimen 20, 5 BM/day •Continue current EN966mL order, (100mL/kg) increase per home schedule (69 kcal/kg),TPN/G-tube 27.8g Pro, •Tolerate feeds •T/C holding feeds for one hour provide formula Output (5/7): 1076mL (UOP= 4.665and mL/kg/hr), BM X2 PO •Continue daily weights, strict I/Os, monitor labs Meds: Gentamycin, Ampicillin, ELT, Heparin •RD to follow 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: Chief Complaint: Fever with Broviac •991 kcal (98 kcal/kg), 16.2g pro (1.6g/kg), 1012mL fluid (100mL/kg) Medications: ELT, Cefotaxime, Vancomycin Oct 10Nov 21, 2011 Diet Order: PES: Diagnosis SBS via G-tube, Baby food PO ad lib Elecare 20 @of 28mL/hr Altered to SBS as evidenced by 16cm remaining Age:GI3function ¾ mosrelated Nutrition Support: D13P3.2 600mL x 19 (60mL/kg/d) @ 31.6mL (8AMsmall bowel and dependence on TPN/G-tube feeds to meet nutritional 5PM) based on 10kg; L1 @5mL/hr x 20 M/W/F needs. Current Intake: (5/13) 408.8 HAL, 672mL Elecare 20 ( I/O)= 1542.8/663 Recommended Interventions: Anthropometrics: •Continue current TPN withthlipids M/W/F •Weight: 10.115 kg (50-75 %ile Wt/age) •Continue current EN regimen (5/1) 9.8kg, (4/7) 9.65kg •T/C increasing Elecare 20 kcal/oz to 30mL/hr if BM WNL •Length/Height: 70 cm (~5th%ile Ht/age) •Monitor daily weights, labs, I/Os and BM May 8, 2012 1,2012 •(4/26) 73.5, (5/1)May 79cm inconsistency •Please re-check length (inconsistency) F/U Nutrition •Wt/Ht: >95th%ile Initial Nutrition Assessment •Head circumference: 49 cm (>95th%ile HC/age) Assessment Age: 10 ¾ mos Age: 10 ½ 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 May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos Dec 5, 2011 – June 21, 2012 GI Outpatient Visits Age: 5 ¾ mos- 12 mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos May 13, 2012 Readmitted w/Central Line Infection Age: 11 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 CM’s Weight Progression Weight (kg) 10 8 6 4 2 0 Date CM’s Progression from PN to EN Date Age (mo) EN Regimen % Kcal from EN PN Regimen % Kcal from PN % Kcal TOTAL Oct 2011 4¼ None 0 D17 P3 L2.99 100 100 Nov 2011 5 2mL/hr 6 D16 P3 L2.5 94 100 Jan2012 7¼ 10mL/hr 27 *Lipids 3d/wk 73 April 2012 9 24ml/hr 50 D13 P3.2 L1 50 June 2012 12 34mL/hr 61 D13 P3.2 39 June 2012 12 ¼ 34mL/hr *Elecare Jr. 22 73 50g D, 14g AA 27 Present 13 ¾ 90 30g D 10 34mL/hr *Elcare Jr. 27 Lipids 100 reduced 100 Lipids 100 D/C’d 100 AAs D/C’d 100 Critical Comments • Anthropometrics- inconsistent height • Estimated kcal needs • Medications: ELT & heparin • Laboratory values: suggestive of anemia 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 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/fetaldiagnoses/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. 9 th 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