1. 1 Pediatric Enteral Nutrition in Short Bowel Syndrome

2. 2 Definition of SBS  Malabsorptive state occurring as a result of the loss of a significant portion of the intestine  Based on the need for intervention, such as need for TPN for longer than 1-3 months  Amount of resection/remaining bowel resulting in this degree of malabsorption varies BASED ON FUNCTION FUNCTION MORE IMPORTANT THAN LENGTH

3. 3 Causes of Short Bowel Syndrome Necrotizing enterocolitis Volvulus Atresia Gastroschisis Aganglionosis Other 436 patients from 13 series (1972-2000) 29 % 27 % 23 % 10 % 4 % 7 %

4. 4 Factors Contributing to Outcome  Age at time of injury  Amount and site of remaining bowel  Function and motility of residual intestine  Adaptation  Other complicating factors  cholestatic liver disease  infections  further injury to remaining bowel

5. 5 Age at Time of Injury  Intestine will grow as the infant grows  Potential for growth is greatest in premature infant  19 to 27 weeks gestation: 115 + 21 cm  27 to 35 weeks gestation: 172 + 29 cm  over 35 weeks gestation: 248 + 40 cm (length of normal jejunum and ileum at autopsy) Touloukian. J Ped Surg 1983

6. 6 Amount and Site of Remaining Bowel

7. Intrinsic factor, pepsin, HCl CCK, secretin iron carbohydrates, protein, fats, vitamins, minerals, trace elements CCK, secretin, GIP, VIP water, electrolyte, vitamin B 12, bile salt, fat, fat soluble vitamins enterglucagon, GLP-2, peptide YY water, electrolytes, SCFA, oxalates

8. 8 Loss of Any Bowel  Decreased surface area for absorption  Shorter transit time  Hypergastrinemia  decreased pancreatic enzyme activity  precipitation of bile acids  damage to epithelium of proximal small bowel  stimulates intestinal motility

9. 9 Loss of Jejunum  Initial marked decrease in nutrient absorption  Generally better tolerated because of adaptive capacity of ileum  Decreased CCK and secretin results in decreased pancreatic enzyme activity

10. 10 Loss of Ileum  Large fluid and electrolyte losses  Sodium loss can contribute to poor growth  Zinc depletion can worsen diarrhea  Loss of ileal brake  Malabsorption of bile acids impairing fat and fat soluble vitamin absorption  Lack of absorption of Vitamin B 12

11. 11 Loss of Ileocecal Valve  Promotes small bowel bacterial overgrowth

12. 12 Loss of Colon  Loss of colonic brake  Loss of water and electrolyte resorptive capacity  Loss of ability to salvage calories from malabsorbed carbohydrates

13. 13 Function and Motility of Residual Intestine

14. 14 Adaptation  Hyperplasia increased surface area  Increased crypt cell production  Increased crypt depth  Increased length of villi  Increased number of transporters per cell  Increase in enzyme activity

15. 15 hyperplasia dilatationelongation Increased surface area

16. 16 Promoted by Luminal nutrients Endogenous secretions Humoral factors Adaptation

17. 17 Post-Operative Management  Use PN to maintain hydration and adequate nutrition for growth and development  Promote adaptation with the use of trophic feedings

18. 18 Luminal Nutrients-Enteral Nutrition  Fuel for enterocytes – stimulating hyperplasia  Promote peristalsis – decreases overgrowth  Stimulate flow of gastrointestinal secretions and secretion of humoral factors

19. 19 “The main goal of treatment in SBS is intestinal adaptation while optimizing weight gain and linear growth while trying to maximize enteral nutrition while minimizing parenteral support.” “This is as much an art as it is a science” Quiros-Teijeira et al. J Pediatr 2004

20. 20 Type of Feeding  Breast milk  Standard formula  Protein hydrolysate formula  Amino acid formula

21. 21 Breast Milk  Bolster immune system  Contain growth factors  Induce protective colonic flora  Shorter duration of parenteral nutrition (Andorsky et al, 2001)

22. 22 Standard Formula  Increased permeability to intact proteins with mucosal injury  SBS - dilated intestine, poor motility, bacterial overgrowth  Allergic reactions to cow’s milk or soy protein is common  Carbohydrate source (lactose)

23. 23 Protein Hydrolysate Formula  Lower antigenicity  Contain MCT oil – does not require bile acids or micelles for absorption  Lower peak bilirubin (Andorsky et al, 2001 )

24. 24 Amino Acid Based Formula  Two infants weaned from TPN using a dilute elemental formula - Christie and Ament J Pediatr 1975  Four patients were able to wean from TPN after change to amino acid based formula - Bines et al JPGN 1998  Shorter duration of TPN - Andorsky et al 2001

25. 25 EleCare ®  Protein (15% of Kcal)  Amino acid-based medical food and infant formula  Amino acid profile based on breast milk  “Clinically” hypoallergenic (Sicherer et al, J Pediatr, 2001)

26. 26 Amino Acid Profile per 70 Kcal Breast Milk and EleCare

27. 27 EleCare  Fat (42% of Kcal)  33% of fat as MCT  30% MCT diet increased (MCT + LCT) absorption from: 23 to 58% preserved colon 46 to 58% no colon (Jeppesen and Mortense Gut, 1998)

28. 28 EleCare  Fat (42% of Kcal)  Provides essential fatty acids Linoleic (LA) - 8% of total energy Linolenic (LNA) - 0.9% of total energy  Dietary Reference Intakes (DRI’s) LA Infants 0- 6 mos8% of Kcal Infants 7 - 12 mos6% of Kcal Children 1-18 yr5-10% of Kcal LNA Infants 0- 6 mos1% of Kcal Infants 7 - 12 mos1% of Kcal Children 1-18 yr0.7-1.2% of Kcal

29. 29 EleCare  Carbohydrate (43% of Kcal)  Corn syrup solids  Osmolality at 20 Kcal/oz = 350 mOsm/kg water  Used in chronic diarrhea due to SBS (Saavedra, et al, 2000)

30. 30 Route of Delivery/Advancing  Continuous drip  continuous saturation of transporters  Advance (slowly) as long as  output < 25-50 ml/kg/day  perianal area intact

31. 31 Other Strategies to Improve Feeding Tolerance  Addition of soluble fibers to the feeding  Acid blockade  Zinc  Sodium chloride  Loperamide

32. 32

33. 33 ProductCost per 100 calCost savings per month compared to EleCare EleCare ® $1.60NA Neocate ® Infant Formula $1.904 month old- $67 Neocate ® 1+ $2.1515 month old- $214 3 year old- $280 Neocate ® Jr. $1.8215 month old- $86 3 year old- $112 Pediatric EO28 ® $1.6715 month old- $27 3 year old- $36 Vivonex ® Pediatric $1.8015 month old- $78 3 year old- $102

34. 34 Summary  Enteral nutrition is the key to bowel adaptation  Breast milk and amino acid formula are associated with shorter duration of PN  EleCare is the only infant amino acid formula with MCT oil.