Hirschsprung’s Disease: GI Implications & Nutritional Management

1. Hirschsprung’s Disease: GI Implications & Nutritional Management Presented by: Laura Kashtan

2. Outline • Anatomy of the Large Intestine • How Does the Gut Normally Function? • Hirschsprung’s Disease • Medical Nutrition Therapy • Patient Presentation • Summary

3. Large Intestines • Adult large intestines is about 5 feet long • Water and salt absorption • Rid body of waste product • Bacteria in gut is essential for normal gut function

6. Normal Gut Function • Peristalsis: a series of muscle contraction • Enteric Nervous System (ENS) • Miessner’s Plexus (submucosal) • Auerbach’s Plexus (myenteric) • Ganglion cells and nerve fibers line the muscle wall of the gastrointestinal (GI) tract

7. Importance of Peristalsis • Waste back up leading to bacterial overgrowth and infection • Malabsorption • What leads to lack of gut motility? • Diet • Obstructions • Nerve damage

8. Hirschsprung’s disease (HD) • HD occurs when the ganglion cells of the Auerbach’s plexus are absent in all or part of the large intestines. • Narrowing of the bowel • Short segmented vs. Long segmented HD

11. Epidemiology • Congenital disorder • Statistically occurs in about 1 of every 5000 births • 4:1 male to female ratio • Children with other congenital disorders are at higher risk of developing HD • Trisomy 21 • RET gene

12. Epidemiology • During fetal development ganglion cells begin to develop in the mouth and travel down the GI tract to the anus • 5th-12th week pregnancy is when these cells migrate • HD is usually detected in early infancy or childhood

13. Signs and Symptoms • 90% of infants with HD do not pass meconium in the first day of life • Constipation, poor appetite, abdominal distention, poor weight gain, pain • Slow growth and development within first 5 years of life • Enterocolitis is the most feared symptom of HD

14. Diagnostic Procedures Rectal Biopsy • Removes tissue from rectum to test for the presence of ganglion cells • Tests for inflammation, infections, tumors, and abscesses • 2 types: • Rectal Suction Biopsy • Rectal Punch Biopsy

15. Top: The arrows show ganglion cells in the rectum Bottom: Rectal biopsy shows an aganglionic nerve of the Auerbach’s plexus

16. Diagnostic Procedures • Barium Enema • X-ray of the large intestines • Barium is used as a contrast medium in the X-ray • Shows where the obstruction occurs

18. Diagnostic Procedures • Anorectal Manometry • Tests the sphincter muscles and muscle movement • A balloon is inserted into the anus and inflated • The sensors in the balloon measures pressure

19. Enemas, cathartics, laxatives Pain Medications Medications Zinc Oxide Flagyl (Metronidazole)

20. Treatment • Surgical intervention is the only method to treat HD • Colostomy/illeostomy • Indication in HD: Bowel perforation, malnutrition, massively dilated proximal bowel, HAEC • Pull through procedure • Swenson (rectosigmoidectomy) • Duhamel (retro rectal transanal) • Soave (endorectal)

22. Adult with HD “The patients have milder disease and go undiagnosed early in their lives because the proximal innervated colon can be hypertrophied and thus, compensates for the distal obstructed, aganglionic rectum.”

23. Adults with HD Misdiagnosis Ultra short segmented HD Symptoms Mortality rate

24. Medical Nutrition Therapy

25. Nutrition Assessment • Patient history • Nutrition focused physical findings • Lab values • Medications • Anthropometrics • Estimated energy and fluid needs

26. Nutrition Assessment

27. Oral Diet: PO intake • Ostomy care guidelines • Clear liquid  Full liquid  Soft/low residue Regular diet • High fiber diet and constipation • Fluid • Supplements

28. Low Fiber Diet: Recommended Foods • Milk & yogurt • Tender beef, poultry, fish • Refined grains • White bread, pasta, rice, cereal • Fruits and vegetables without skin, pulp, seeds

29. Low Fiber Diet: Foods to Avoid • Milk and milk products (lactose intolerance) • Yogurt with added fruit • Tough meat • Dried beans • Whole wheat bread, rice, pasta, high fiber cereals • Raw vegetables, high fiber vegetables • Dried fruit and fruit skin

30. Enteral Nutrition • Preferred route of alternate nutrition • Indications • Monitor for signs of intolerance • Refeeding Syndrome • Mechanical Implications • Formula composition

31. Parenteral Nutrition • Indications • Composition: • Dextrose • Amino Acids • Lipids • Electrolytes & Trace Minerals • Administration

32. Parenteral Nutrition Implications

33. Patient Presentation

34. G.A. • 29 yo Hispanic male • 5’6” • Usual body weight (UBW): 135-140 lbs • Lives with mother • Unemployed

35. Medical History • Hirschsprung’s disease • Gastroparesis • Iron deficiency anemia • Weight loss • Failure to thrive • Multiple laparoscopies • Allergies • Mild ileus and hernia- recent CT scan

36. G.A. Overview • January 2013 • Leakage around J-tube site • Abdominal wall cellulitis • Redness and chemical burns to the skin • 25 lb weight loss • Diet: Regular diet and Perative @ 65ml/hr x 14 hr • Treated for MRSA, J-tube removed

37. G.A. Overview • February 2013 • Redness around prior J tube site w/ leakage • J-tube reinserted • Diet: Vital AF 1.2 @ 75ml/hr x 14, Prostat 30 ml BID • Not tolerating TF’s, started on TPN • 350 g dextrose, 80 g amino acids, 40 g lipids (1910 calories)

38. G. A. Overview • April 23, 2013 • S/p removal of J and G tubes • Abdominal wall cellulitis • Air sounds around old G-tube site • “severe pain inside my stomach and throwing up blood since weekend” • Diet: full liquid, 5 small meals, TPN: 190 g dextrose, 27 grams amino acid, 15 grams lipids (904 calories)

39. April 30, 2013 Current diet order: Clear liquids and TPN meeting < ½ calorie needs, < 32% protein needs He reported weight has been stable at 135 since he has been on TPN. Ate PO as he when he felt like it Estimated needs: 1900 kcals (Mifflin St. Jeor x 1.25), 73-92 g protein (1.2-1.5 g/kg) Nut Rx: advance diet to soft/low residue, 6 small meals, Enlive TID, change TPN 370 g dextrose, 100 g AA, 15 g lipid (1808 calories)

40. May 2- May 8, 2013 • He received my TPN recommendations but the patient refused the supplement • Study showed + for an enterocutaneous fistula • Insertion of jejunostomy tube for drainage • Diet advancement, small c/o abd. pain • Discharged May 8 with surgery date in 1 week to remove fistula

41. The next day… • Re-admitted with acute intestinal fistula drain obstruction • “Severe pain on left side of stomach and throwing up”

42. Nutrition Assessment • Nutrition Focused Physical Findings: • + illestomy • + jejunostomy tube gravity drain • Vomiting, abdominal pain, “cant keep food down” • Current diet: NPO for procedure • Skin: Abdominal wall cellulitis

43. Anthropometrics • 5’6” (167 cm) • 140 lbs (63.6 kg) • BMI: 22.6

44. Patient Estimated Needs • 1500-1900 calories based on 25-30 kcals/kg • 82-100 grams protein based on 1.3-1.6 g/kg • 1500-1900 ml fluid

45. Nutrition Diagnosis Altered GI function related to bowel resection, intestinal fistula with obstruction, and HD as evidence by patient scheduled for bowel resection of fistula, pt NPO, patient reports “cant keep food down”

46. Nutrition Prescription & Goals • Nutrition prescription: • Advance diet as medically feasible/tolerated to soft/low residue • Ensure BID • TPN: 365 g dextrose, 100 g amino acids, 15 g lipids • Goals: • Modify diet  patient to tolerate PO diet • Commercial beverage intake  patient to consume > 75% • Initiate TPN if PO not feasible  patient to meet estimated needs

47. Monitoring and Evaluating • Indicator: • PO intake, as tolerated • Commercial beverage • TPN • Labs • Criteria: • Patient to consume > 75% meals • Patient to consume > 75% supplement • To meet patients estimated needs • To be within normal limits

48. Continued Hospital Course • Exploratory laparotomy, lysis of adhesions, takedown of enterocutaneous fistula • Segmental resection of jejunum with end to end anastomosis • Significant follow ups: • May 17, 2013 • May 24, 2013 • May 30, 2013

49. May 17, 2013 • Diagnosis: Altered GI Function - ongoing • Diet: NPO • Assessment: c/o abdominal pain, been NPO for 7 days • Nutrition Prescription: Initiate TPN as previously ordered to meet his needs, MVI to promote wound healing • Monitor and Evaluate: TPN, GI function, skin towards healing

50. Continued Hospital Course • J tube site draining serious fluid, c/o increased abdominal pain • Diet advanced to mechanical soft and recommendations to continue advancement per MD • Denies N/V, reports food would “leak out” • Cellulitis at old G-tube site • G.A. c/o increased pain and drainage at old G-tube site and MD noted he will most likely need closure of gastrocutaneous fistula