hirschsprung’s disease: gi implications & nutritional management

Hirschsprung’s Disease: GI Implications & Nutritional

Management

Presented by: Laura Kashtan

Outline

• Anatomy of the Large Intestine

• How Does the Gut Normally Function?

• Hirschsprung’s Disease

• Medical Nutrition Therapy

• Patient Presentation

• Summary

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

Water Biotin

Vitamin K

Short Chain Fatty Acids

NaClK

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

Importance of Peristalsis

• Waste back up leading to bacterial overgrowth and infection

• Malabsorption

• What leads to lack of gut motility?• Diet• Obstructions• Nerve damage

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

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

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

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

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

Top: The arrows show ganglion cells in the rectum

Bottom: Rectal biopsy shows an aganglionic nerve of the Auerbach’s plexus

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

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

Medications

Enemas, cathartics, laxatives

Pain Medications

Zinc OxideFlagyl

(Metronidazole)

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)

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.”

Adults with HD

Misdiagnosis

Ultra short segmented HD

Symptoms

Mortality rate

Medical Nutrition Therapy

Nutrition Assessment

• Patient history

• Nutrition focused physical findings

• Lab values

• Medications

• Anthropometrics

• Estimated energy and fluid needs

Nutrition Assessment

Comparative Standards:

Maintenance Goals:

Repletion Goals:

Calories 25-30 kcal/kg 30-40 kcal/kg

Protein 0.8-1.2 g/kg 1.5-2.5 g/kg

Fluid ~1 ml/kg

Oral Diet: PO intake

• Ostomy care guidelines

• Clear liquid Full liquid Soft/low residue Regular diet

• High fiber diet and constipation

• Fluid

• Supplements

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

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

Enteral Nutrition

• Preferred route of alternate nutrition

• Indications

• Monitor for signs of intolerance

• Refeeding Syndrome

• Mechanical Implications

• Formula composition

Parenteral Nutrition

• Indications

• Composition:• Dextrose• Amino Acids• Lipids• Electrolytes & Trace Minerals

• Administration

Parenteral Nutrition

Implications

Psycological

Medical

•IV dependence•Social Life•Lack of enjoyment for eating

•Line Sepsis•Bacterial Translocation•Cholestasis•Gut Atrophy•Refeeding Syndrome

Patient Presentation

G.A.

• 29 yo Hispanic male

• 5’6”

• Usual body weight (UBW): 135-140 lbs

• Lives with mother

• Unemployed

Medical History• Hirschsprung’s disease

• Gastroparesis

• Iron deficiency anemia

• Weight loss

• Failure to thrive

• Multiple laparoscopies

• Allergies

• Mild ileus and hernia- recent CT scan

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

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)

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)

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)

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

The next day…

• Re-admitted with acute intestinal fistula drain obstruction

• “Severe pain on left side of stomach and throwing up”

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

Anthropometrics

5’6” (167 cm)140 lbs (63.6

kg)BMI: 22.6

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

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”

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

Monitoring and Evaluating

• Indicator:1 PO intake, as tolerated

2 Commercial beverage

3 TPN

4 Labs

• Criteria:1 Patient to consume > 75% meals

2 Patient to consume > 75% supplement

3 To meet patients estimated needs

4 To be within normal limits

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

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

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

May 24, 2013

• Diagnosis: Altered GI function - ongoing

• Diet: Soft/Low residue, snacks between meals

• Assessment: pt reports “okay intake, but pain after meals” MD noted he was tolerating diet, cellulitis at PEG improving

• Nutrition Prescription: continue as ordered

• Monitor and Evaluate: PO intake, supplement intake, GI function

May 30, 2013

• Diagnosis: Altered GI function - improving

• Diet: Soft/Low residue with snacks between meals

• Assessment: skin improved, patient reports good intake with slight pain after meals

• Nutrition Prescription: continue diet as ordered

• Monitor and Evaluate: PO intake, GI function

Medication (brand name)

Action Indication Food Interactions

Benadryl Antihistamine

Ciprofloxacin (Cipro)

Antibiotic Cellulitis Take 2 hrs before, 6 hrs after consuming milk, yogurt, Ca fortified foods, Mg, Fe, AlAvoid/limit caffeine

Metronidazole(Flagyl)

Antibiotic, Antiemetic

Tx: bacteria in GI, abdomen

Decrease Na

Pantoprazole(Protonix)

PPI, AntiGERD Gastroparesis, acid secretion

May B12May need Ca supp.

Zinc Oxide Skin Irritation Cellulitis

Odanestron(Zofran)

Antiemetic, Antinauseant

Nausea

Metoclopramide(Reglan)

Antiemetic, AntiGERD,

Gastroparesis Take ½ hr before meals and HS

Lab Values

Labs 4/29 5/9 5/17 5/24 5/30

Albumin (g/dL) 3.7 4.8 3.3

Glucose (mg/dL) 106 134 96 90 89

Na (mEq/L) 140 137 135 138 139

K (mEq/L) 4.8 3.7 4.0 4.1 4.1

Cl (mmpl/L) 99 101 96 101 103

Hgb (g/dL) 10.1 8.8 7.2 9.2

Hct (g/dL) 31.7 27.3 22.4 28.9

Critical Comments

• Following up with orders

• Lipid shortage

• Patient’s compliance

Summary

• HD is a congenital disorder where all or part of the large intestines lack ganglion cells

• Adults with HD is rare due to a probable misdiagnosis or ultra short segmented HD

• G.A. is a 29 y/o male treated for HD 3 years ago and since then has had several GI complications

• Since discharge he has not been back

Capital Health Hospital

Gayanne & Kristen

My Fellow Interns

Resources. McKeown, Sonja; Stamp, Lincon; Hao, Marlene; Young, Heather. Hirschsprung disease: a

developmental disorder of the enteric nervous system. Wiley Interdisciplinary Reviews: Developmental Biology. April 24, 2012; Volume 2 113-129. Available form: DOI: 10.1002/wdev.57, June 2013.

Langer, Jacob. Hirschsprung Disease. Division of General and Thoracic Surgery Hospital for Sick Children. June 2013. Volume 25 pages 368-374.

 Children’s Hospital of Pittsburg, Organs: Small and Large Intestines, Available at: http://www.chp.edu/CHP/organs+intestine. May 2013.

 Sasselli, V.; Pachnis, V.; Burns, A.. The Enteric Nervous System. National Institute for Medical Research. Elivisier Journal: Developmental Biology. June 2012; Volume 366 pages 64-73. Available from: 10.1016, May 2013.

National Institute of Health. MedlinePlus. Available at: http://www.nlm.nih.gov/medlineplus/medlineplus.html, June 2013.

Bowen, R. The Enteric Nervous System. Available at: http://www.vivo.colostate.edu/hbooks/pathphys/digestion/basics/gi_nervous.html. May 2013.

Lucille Packard Children’s Hospital at Stanford. Hirschsprung’s Disease. Available: http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/digest/hirschpr.htm l. May 2013.

Chen, F. MD; Winston, J. MD; Jain, S. MD; Frankel, W. MD. Hirschsprung’s Disease in a young a young adult: report of a case a review of the literature. Annals of Diagnostic Pathology. Volume 10 pages 347-351. Available from: http://www.surgery.uthscsa.edu/faculty/pubs/hirschprungscase.pdf.

•  

ResourcesEvaluation and Treatment of Constipation in Infants and Children: Recommendations of North American

Society for Pediatric Gastroenterology, Hepatology. J Pediat Gastroenterol Nutr. Volume 43. May 2013.  

Lin, Y; Chang, T; Chiu, H; Liu T; Ying, T. Hirschsprung’s Disease in an Adult with a 37 year Bistory of Colostomy. J Soc Colon Rectal Surgeon. June 2010. Volume 21 pages 111-113. 21: 111-114. May 2013.

 Nurko, S MD. Hirschsprung’s Disease. American Motility Society. Available at: http://www.motilitysociety.org/pdf/Hirschsprung's%20disease%208.28a.2006.pdf. May 2013.

 Kim, H MD; Kim A MD; Lee, C MD et al. Hirschsprung’s Disease and Hypoaganglionosis in Adults: Radiologic Findings andDifferentiation. Radiology. May 2008. Volume 247 pages 428-434. DOI: 10.1148/radiol.2472070182. May 2013.

 Riley Hospital for Children. Motility Tests. Available at: http://iuhealth.org/riley/. May 2013.

Gastroenterology Consultants. Anorectal Manometry. Available at: http://www.gastroconsultantsqc.com/services/procedures/anorectal-manometry/. May 2013

United Ostomy Associated of America, Inc.. Diet and Nutrition Guide. Available at: http://www.ostomy.org/ostomy_info/pubs/OstomyNutritionGuide.pdf. June 2013.

Children’s Hospital St. Louis. Hirschsprung’s Disease. Available at: http://www.stlouischildrens.org/diseases-conditions/hirschsprung-disease. June 2013.

ResourcesJohns Hopkins Medicine. Hirschsprung’s Disease. Available at:

http://www.hopkinsmedicine.org/geneticmedicine/Clinical_Resources/Hirschsprung/WhatisHirschsprung.html. May 2013.

Merck Manual. Merck Sharp and Dohme Corp. Place of publication: publisher; 2011.

Ansel, Karen. The Food and Nutrition Expert: What a Registered Dietitian Can Do for You. Available at: www.eatright.org. May 2013

Parrish, Carol RD, MS; Elemental and Semi Elemental Formulas: Are They Superior to Polymeric Formula?. Nutrition Issues in Gastroenterology. Volume 34 pages 59-72. Available at: http://www.medicine.virginia.edu/clinical/departments/medicine/division s/digestive-health/nutrition-support-team/nutrition-articles/MakolaArticle.pdf

Martindale, R. MD PhD; McClave, S. MD; Vanek, V. MD; et al. Guidelines for the provision and assessment of nutrition support therapy in the adult critically ill patient: Society of Critical Care Nutrition Medicine and American Society for Parenteral and Enteral Nutrition: Executive Summary. Crit Care Med. 2009. Volume 37 pages 1757-1760. DOI: 10.1097/CCm.On013e3181a40116. May 2013.

Kraft, M PharmD; Btaiche, I PharmD; Sacks, G PharmD. Review of the Refeeding Syndrome. Nutrition in Clinical Practice. Volume 20 pages 625-633. DOI:10.1177/01154265050200006625. June 2013

 Uklejia, A MD; Freeman, K PharmD; Gilbert, K RN CNSC, MSN, CRNP; et al. Standards for Nutrition Support in Adult Hospitalized Patients. Nutrition in Clinical Practice. August 2010. Volume 25 pages 403-414. DOI: 10.1177/0884533610374200. June 2013.

Resources

Ziegler, T MD. Parenteral Nutrition in the Critically Ill Patient. N Engl J Med. September 2009. Volume 361 pages 1088-1097. DOI: 10.1056. June 2013.

 Singer, P; Berger M; Van den Berghe G; et al. ESPEN Guidelines on Parenteral Nutrition: Intensive Care. Clinical Nutrition. May 2009. Volume 28 pages 387-400. DOI: 10.1016. May 2013.

ADAM Medical Encyclopedia. National Library of Medicine. Hirschsprung’s Disease. Available at: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002125/.

Adult Nutrition Care Manual. Low-Fiber Nutrition Therapy. Available at: http://nutritioncaremanual.org. May 2013

 Hokkam, E.; Adel, M.; El-Labban, G.; et al. Evaluation of Anorectal Myectomy in Managing Ultrashort Segment of Hirschsprung’s Disease. Egyptian Journal of Surgery. July 2009. Volume 28. Available at: http://www.ess- eg.org/pages/journal/allpdf/338.pdf. June 2013.

 Pronsky, Z. MD RD LDN RADA; Crowe, J. PharmD RPH; Food Medication Interactions 16 th edition. Food-Medication Interactions. 2010.