Exam 2: GI

Question 1

Hirshprung’s Disease

Answer 1

• also known as congenital aganglionic megacolon
• Mechanical obstruction caused by inadequate motility of part of the intestine
• Lack the ganglion that are necessary for peristalsis
• Due to this aganglionic portion, you end up having an accumulation of stool proximal to the portion of the large intestine that is affected by the Hirshprung’s

Question 2

Areas affected by Hirshprung’s Disease

Answer 2

Usually includes rectum and proximal portion of the large intestine

• 15-20% of children can have it spread to the rest of the colon
• ~10% of children can have it spread to the entire intestines

Question 3

Incidence of Hirshprung’s Disease (6)

Answer 3

1. ¼ of all neonatal obstruction but may not be diagnosed until later in infancy or childhood
2. Is usually diagnosed at/around birth
3. More common in children with Downs Syndrome
4. 4x more common in males
5. 1 in 5,000 births
6. Familial component/genetic predisposition → if you have a child with Hirshprung’s then your next child has a slightly increased likelihood of getting it too

EXACT ETIOLOGY IS UNKNOWN

Question 4

Pathophysiology of Hirshprung’s Disease (4)

Answer 4

1st: Absence of ganglionic cells in one or more segments of the colon
2nd: Results in absence of propulsive movements (peristalsis) leading to accumulation of intestinal contents and distention of bowel proximal to defect (megacolon)
3rd: Internal anal sphincter fails to relax
4th: Intestinal distention and ischemia of bowel wall which leads to enterocolitis (inflammation of small bowel and colon) which is leading cause of death in children with Hirshprungs (worry about this if patient can’t have surgery)

Question 5

Number one complication of Hirshprung’s

Answer 5

Entercolitis then Peritonitis

Entercolitis: inflammation of intestine and colon

Peritonitis: perforation of bowel wall that will occur second to entercolitis

Question 6

Symptoms of Peritonitis (6)

Answer 6

1. severe abdominal pain,
2. very rigid and tender abdomen,
3. distended abdomen,
4. fever,
5. dyspnea,
6. no bowel sounds upon auscultation

Question 7

Newborn Signs and Symptoms of Hirshprung’s (4)

Answer 7

1. Failure to pass meconium (first stool) within 24 to 48 hrs. after birth
   - -Meconium is black and tarry and difficult to clean
   - -Failure to pass means you need further evaluation to see if the patient has Hirshprung’s
2. Reluctance to ingest fluids
3. Bile-stained vomitus
4. Abdominal distention

Question 8

Infancy Signs and Symptoms of Hirshprung’s (7)

Answer 8

1. Failure to thrive
2. Constipation due to lack of peristalsis
3. Abdominal distention
4. Episodes of diarrhea and vomiting
5. Explosive watery diarrhea
6. Fever
7. Severe exhaustion

Question 9

Childhood Signs and Symptoms of Hirshprung’s (6)

Answer 9

1. Constipation
2. Ribbon-like, foul-smelling stool that leaks around where the mega-colon is
3. Abdominal distention
4. Visible peristalsis in abdomen
5. Fecal masses easily palpable
6. Poorly nourished child and anemic

Very rare!

Question 10

Diagnosing Hirshprung’s (4)

Answer 10

1. Rectal exam –> tight internal sphincter and absence of stool
2. Barium enema
3. Anorectal manometry
4. Definitive diagnosis is rectal biopsy; there will be a lack of nerve fibers with Hirshprung’s disease

Question 11

Therapeutic Managment of Hirshprung’s

Answer 11

1. At birth, total surgery correction where they remove the diseased portion of the bowel and reinastimose the healthy parts (
2. At 9kg pull through procedure and colostomy closed
   * Depending on age of child, may have to do temporary ostomy after removal of aganglionic portion and once mega-colon area gets back to normal shape they reinastimose the healthy parts
3. Outcome of surgery is very good; 65-85% end up achieving normal bowel function

Question 12

Enterocolitis

Answer 12

Inflammation of intestine and colon; the biggest concern of pre-op patient’s with Hirshprung’s

*Will need to assess for signs and symptoms of perforated bowel (peritonitis)

Question 13

Assessing for Signs of Perforated Bowel (7)

Answer 13

1. Vital signs – looking for shock; tachycardia, increased RR, high fever, may have hypotension
2. Absent bowel sounds
3. Abdominal distention and tenderness
4. Vomiting
5. Irritable
6. Fever – DO NOT DO RECTAL TEMPS
7. Dyspnea and cyanosis

Question 14

Post-Op Care of Hirshprung’s

Answer 14

1. Colostomy care
2. Prevent contamination of wound with urine
3. Impaired skin integrity due to incontinence
4. NPO until bowel sounds return or flatus passed – IV fluids
5. Pain control
6. Strict I&O

Question 15

Gastroesophogeal Reflux

Answer 15

Very common, could be due to/in conjunction with something else such as a treatment

• Dysfunction of lower esophageal sphincter (LES)
• Delayed gastric emptying
• Poor clearance of esophageal acid
• Susceptibility of esophageal mucosa to acid injury

Question 16

Etiology of Gastroesophogeal Reflux (9 with most common cause)

Answer 16

1. Prematurity
2. Tracheal-esphogeal atresia
3. Neurological disorders; delay in the message from the brain to the sphincter to close after eating
4. Scoliosis
5. Asthma
6. Cystic Fibrosis
7. Partial or incomplete swallowing dysfunctions
8. Theophylline and caffeine
9. Increased abdominal pressure
10. Infants with short LES; Immature sphincter
    
    • **This is the most common cause!

Question 17

Tracheal-Esophogeal Atresia

Answer 17

When esophagus ends with a blind pouch; no connection to stomach

• Will have a fistula that is connected to a trachea
• When you try feeding them, they will present with cough, congestion, respiratory distress because the feed is going into the fistula then into trachea
• Surgical repair of this can put a child at risk for developing reflux

Question 18

Incidence of Gastroesophogeal reflux (3)

Answer 18

1. 3% of all newborns, but rare to see it in the newborn period
2. Peaks between 1-4 months of age, usually resolves by 6-12 months of age
   * **This is because regurgitation/irritability incidence decreases since they begin to sit up and are beginning to eat solids
3. Boys affected 3x more than girls

Question 19

Signs and Symptoms of Gastroesophageal Reflux (10)

Answer 19

1. Vomiting/may be constantly regurigtaing
2. Weight loss
3. Failure to thrive (may not be gaining weight)
4. Very Irritable because the mucosa is getting excoriated; every time they reflux it’s very painful (May increase after eating)
5. Respiratory illness
6. Coughing, choking, apnea, bradycardia (May be aspirating)
7. Hiccups
8. Recurrent weight loss
9. Heme (+) stools; blood in stool from bleeding in esophagus
10. Sandifer’s syndrome

Question 20

Apnea with Gastroesophogeal Reflux

Answer 20

Aspiration and regurgitation stimulates mucous production in the throat which can lead to apnea or bradycardia
*Near life threatening event: apneic episode at home and they need resuscitation

Question 21

Sandifer’s Syndrome

Answer 21

Neuro-behavioral syndrome; will see child arching and turning head to side

• While/after eating, they will have episodes where they exhibit this
• Baby will be irritable, trying to wiggle around and/or arch
• Similar traits could also be seen in patients with neurological disorders, such as seizure disorder; if you see a child exhibit this behavior, you may think something else is going on and may incorrectly give an anticonvulsant
• Very important to keep this in the back of your mind that it could be reflux

Question 22

Diagnostic Tests for GER (4)

Answer 22

1. Upper GI series to rule out something else like pyloric stenosis
2. Radionuclide tests
3. 24 hour esophageal ph monitoring
4. Endoscopy & esophageal biopsy; want to look and see if there is damage or inflammation in the esophagus

Question 23

24 hour esophageal pH monitoring test

Answer 23

This is the gold standard for diagnosing GERD

• *Will pass an NG tube with a monitor at the bottom that will assess different things such as
  1. pH of acid,
  2. How many episodes of reflux in 24 hours
  3. How long does it take for acid to clear

Question 24

Management of GER (two types)

Answer 24

Depends on the severity!!

1. Thriving infant without respiratory complications:
   A. Small frequent feedings
   B. Thickened feedings with rice cereal; only time you’ll put rice cereal in the bottle
   C. Positioning with HOB elevated
2. Failure to Thrive infants with severe reflux, who failed to respond to medical therapy or have an anatomic abnormality contributing to symptoms:
   A. NG feedings and/or surgery (Nissen Fundop)

Question 25

Nissen Fundoplication

Answer 25

Tx of severe GER Take the fundus of the stomach, wrap it around the lower part of the esophagus, and try to prevent the refluxing of food back into the esophagus * Not done unless necessary due to complications, such as: 1. Obstruction 2. Gas bloat syndrome; unable to burp and belch

Question 26

GERD Medications (3)

Answer 26

1. Antacids or H2 blockers (very rare to use antacids) 2. PPIs (better than H2 blockers) 3. Prokinetics

Question 27

Antacids or H2 Blockers for GERD (3)

Answer 27

1. cemetidine(Tagamet) 2. ranitidine (Zantac); Approved for children under age 1 3. famotidine (Pepcid); Approved for children under age 1 * It’s very rare to use antacids; not the choice of meds for children; can use H2 blockers

Question 28

PPIs for GERD (2)

Answer 28

1. omeprazole (Prilosec) 2. lansoprazole (Prevacid); Approved for children under age 1, but should mainly be 1 and above * *This is the drug of choice! * *Give prevacid about 30 minutes before eating - PPIs are better at healing than H2 antagonists - Less of developed tolerance than with H2 antagonists

Question 29

Prokinetics for GERD (2)

Answer 29

1. metoclopramide (Reglan) * *First choice in this category * *Give it 30 minutes before meal * *Give if they have decreased gastric emptying 2. bethanecol (Urecholine) **Decreased gastric emptying makes reflux worse; if they have decreased gastric emptying they will be on a prokinetic!!

Question 30

Nursing Diagnosis with Reflux (6)

Answer 30

1. Risk for aspiration 2. Impaired swallowing 3. Acute pain 4. Imbalanced nutrition 5. Knowledge deficit (Major one) 6. Parental anxiety (Major one) * *Major one since it’s a near life threatening event if resuscitation is necessary * *Always teach the parent to do CPR before they leave the hospital * *May need to use APNEIC MONITOR for first 6 months which helps with parental anxiety

Question 31

Crohn's Disease

Answer 31

A chronic inflammatory Disease * Can occur in any part of GI tract from mouth to anus, with ileum, colon & rectum most common affected areas * Transmural involving all 3 layers of mucosa Inflammation and ulceration of colon mucosa *There are healthy areas between the affected areas (“skip areas”)

Question 32

Crohn's Disease Etiology (7)

Answer 32

1. Unknown exact cause 2. May be a genetic factor 3. Triggers - -Viral & infectious agents - -Food allergies - -Increased intestinal permeability and immunological dysfunction 4. Stress 5. More common in whites, 3-6x more common in Jewish descent 6. Occurs equally in men and women 7. Runs in families

Question 33

Signs and Symptoms of Crohn's (7)

Answer 33

1. Abdominal pain with cramps 2. diarrhea 3. weight loss & poor growth * Can have delayed physical and sexual growth 4. Fever, 5. anorexia, 6. rectal bleeding, 7. perianal discomfort and fissures or fistulas to other loops of bowel, bladder, vagina or skin

Question 34

Extraintestinal Signs and Symptoms of Crohn's (8)

Answer 34

1. Erythema nosdosum (Red papular nodules on the legs) 2. Large jt. arthritis 3. Mouth ulcers 4. Liver disease 5. Renal calculi 6. Uveitis; inflammation of uvea (vascular area in eye beneath the sclera) 7. Anemia; Due to blood loss from the bleeding that can occur with Crohn’s 8. Elevated WBC & ESR & CRP (all indicating inflammation)

Question 35

Diagnosing Crohn's (6)

Answer 35

1. Lab data 2. History and physical; make sure nothing else is causing the symptoms 3. Stools -- blood, leukocytes & infectious agents 4. Upper GI series with small bowel follow through 5. CT scan & Endoscopy of upper and lower bowel 6. Mucosa biopsies

Question 36

Lab Data to check with Crohn's

Answer 36

1. CBC 2. ESR 3. C-reactive protein 4. pANCA 5. Measure all of the below because the disease is causing poor absorption; they may need to be on supplementation - Total protein - Albumin - Zinc - Magnesium - Vitamin B12 - Fat soluble vitamins

Question 37

pANCA

Answer 37

Done to decipher between ulcerative colitis and Crohn’s disease because the symptoms can be very similar * Will see a dramatic increase in pANCA in patient’s with ulcerative colitis * Will only have a minor increase in patient’s with Crohn’s disease

Question 38

Upper GI series with small bowel follow through

Answer 38

Used to dx Crohn's * Looking for bowel wall thickening or any complications that are occurring in the bowel from Crohn’s * Also looking for abscesses that can occur with the ulcerations

Question 39

Goals of Crohn's Management (4)

Answer 39

1. Control inflammatory process & reduce or eliminate symptoms 2. Obtain long term remission 3. Promote normal growth & development 4. Allow as normal lifestyle as possible

Question 40

Treatment of Crohn's (4)

Answer 40

Some can be controlled on diet alone, but some need medications 1. Corticosteroids 2. Aminosalicylates 3. Immunomodulators 4. Antibiotics (adjunct)

Question 41

Corticosteroid Treatment of Crohn's (with 8 side effects)

Answer 41

Used to mediate and control inflammation; can be oral, IV, or rectal *Enema of corticosteroid may decrease urgency of stools Side effects of corticosteroids: 1. Infection 2. Metabolic problems such as temporary hyperglycemia 3. Moon face 4. Acne 5. Hurstism (hair in places they aren’t normally) 6. Aggressive behaviors 7. Osteopenia 8. If a patient is on long term corticosteroids and needs surgery, their cortisol would go up because they are stressed but due to the corticosteroid their body won’t elevate cortisol * *Long term corticosteroids → body isn’t producing cortisol, this is why you wean people off of corticosteroids in order to give their adrenal glands time to start making cortisol * *Will need IV pre, during, and post doses of corticosteroids if they are having surgery

Question 42

Aminosalicylates tx for Crohn's

Answer 42

1. sulfasalizine (Azulfidine) 2. mesalamine (Asacol and Pentasa) * antinflammatory * Asacol and Pentasa are preferred * Tell patients that they are going to need extrafolic acid because these interfere with absorption of folic acid * Also causes an increase in sun sensitivity; may burn if out in the sun

Question 43

Immunomodulator tx for Crohn's

Answer 43

1. 6-mercaptopurine, 2. azathioprine, 3. methotraxate 4. cyclosporine *enhance effect of steroids * Methotraxate is used during chemo * Major concern is suppression of the bone marrow/immune system * Monitor WBC levels * Helps enhance the effects of steroids, may not need to be on it if they’re not on steroids

Question 44

Antibiotics with Crohn's

Answer 44

Used as adjunct therapy for complications such as abscesses Most commonly prescribed: Flagyl

Question 45

Other treatments for Crohn's

Answer 45

1. Anti-TNF – infleximab (Remicade) 2. Interleukin 10; helps reduce inflammation 3. Budesonide (Corticosteroid with fewer side effects than Prednisone) 4. Zinc (Helps remove free radicals that can contribute to inflammation)

Question 46

Anti-TNF tx for Crohn's

Answer 46

infleximab (Remicade) Used if the other medications are not working (last resort) * *TNF a protein that ends up getting produced by the immune system causing inflammation * *Remicade binds with TNF and excretes it in urine to help decrease inflammation * Has a lot of side effects * Need to come in once a month for monitoring * Only for children 6 and older

Question 47

Crohn's Nutrition (4)

Answer 47

Primary component of treatment!!; May not be on any meds but just diet management because due to issues of absorption they need a specific diet 1. High calorie, high protein 2. Multivitamins, iron & folic acid supplements 3. Enteral formulas by mouth or NG feedings * *These formulas are formulas that the protein is broken down into it’s simplest forms; it can be completely absorbed in the small intestine * *These formulas also help reduce remission 4. TPN

Question 48

TPN Treatment for Crohn's

Answer 48

1. Occurs when you can’t maintain weight or nutrition 2. Will be on intermittent TPN liquids for nutrition and weight maintenance 3. Broviac: central line that goes into the right atrium; used if child has Crohn’s and needs TPN treatment 4. Portocath is another option for this; acts as a tube that goes right into right atrium - These are considered central lines and are used for nutrition/TPN if necessary

Question 49

Surgery for Crohn's

Answer 49

*Not curative Drain abscesses, close fistulas, remove short segments of diseased bowel, repair perforations (reduce risk of peritonitis and enterocolitis), relieve obstructions or widen strictures Due to inflammation that occurs in bowel, these patients can develop enterocolitis then are at risk for peritonitis *Toxic megacolon: fever, acute abdominal pain and abdominal distention

Question 50

Prognosis of Crohn's (4)

Answer 50

1. Depends on how much of the bowel is involved, medication adherence, etc. 2. No cure 3. Outcome influenced by severity of GI involvement, the regions of bowel affected & appropriate therapeutic management 4. Main worry is colon cancer, which a long term complication - --> Children with Crohn’s will be getting colonoscopies in their teenage years to monitor this!!

Question 51

Nursing Care for Crohn's (8)

Answer 51

1. Home dietary management 2. Coping with factors that increase stress 3. Good mouth care * Important because they can develop oral ulcerations * Normal saline rinses * If ulcerations are very painful, use magic mouth wash (Nalox base with Benadryl and lidocaine) * Be careful with lidocaine because it can cause seizures * Sucking on ice also helps decrease the pain 4. Adjust to disease of remissions & exacerbations 5. Prepare for possible surgery, especially if they are on corticosteroids 6. NG or TPN instruction 7. Importance of continued drug therapy 8. Inform patients about Crohn’s and Colitis Foundation of America