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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. - PowerPoint PPT Presentation
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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
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