C - Hirschprung Disease

Question 1

What’s hirschprung disease?

Answer 1

• Congenital condition
• nerves from some parts of colon are missing -> blocked colon -> enlarged

Question 2

Cause & pathology of Hirschprung?

Answer 2

Mutations in either RET/EDNRB genes -> no parasympathetic ganglionic cells in rectum/parts of colon -> unopposed sympathetic stimulation of intestines = always contracted -> NO peristalsis -> poop stuck :(((

Question 3

Symptoms of Hirschprung for neonatal, infants & older children?

Answer 3

Neonatal:
- Never pass meconium within first 2 days of life
- Digital Rectal Examination: use glove to assess rectum -> explosive gas & watery stools (squirt or blast sign)
- excessive constipation -> massive colon dilation -> abdominal distention -> ↑ risk of rupturing bowel

Infants
- bilious vomiting,
- poor feeding & refusal to feed
- failure to gain weight

Older children
- chronic constipation

Question 4

Whats the nursing care you’ll provide?

Answer 4

1. Promote adequate bowel elimination, monitor for complications, provide supportive care
2. Keep NBM, maintain NGT,
   - administer IV + antibiotics + pain medications
3. Monitor Vitals, i/o, measure abdominal circumference
4. Immediately report if your client presents signs of enterocolitis, including fever, abdominal distension, or explosive, foul-smelling diarrhoea

If temporary colostomy was performed
- Assess site
- Report to HCP if ↑ bleeding / swelling OR signs of ischemia (dusky / blue stoma) OR signs of retraction (flattening of the stoma or if it starts to move below the level of the skin)

No colostomy performed
- monitor output from bowel
- provide care for the skin around anus

When peristalsis returns
- Discontinue NGT, start on clear liquids
- Closely monitor colour, consistency & amount of stool
- report if client presents signs of an obstruction, including vomiting, abdominal distension, or an absence of gas or stool

Question 5

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Diagnosis of HD?

Answer 5

1. client history & physical examination
2. Abdominal X-Ray
   - very big megacolon full of stool + check length
3. Barium / water-soluble contrast enema
   - will show dilated colon proximal to aganglionic region
4. Anorectal manometry
   - evaluates fxn of rectal & anal muscles
   - show NO relaxation of the internal anal sphincter
5. Confirmed with a rectal suction biopsy of the narrowed area in the colon
   - obtains tissue sample to show NO ganglion cells

Question 6

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What Client & Family Teaching will you provide?

Answer 6

● Explain pathology

● Colostomy care
○ let the parents or caregivers know that the colostomy will be needed for several months, and that the paediatric surgeon will let them know when it is no longer needed
○ Teach them how to care for colostomy + how to protect surrounding skin from irritating effects of stool with prescribed barrier cream
○ ensure they know how to remove, empty, and apply the collection pouch
○ Instruct them to contact their healthcare provider if they see excessive bleeding of the stoma, recurrent leaking, persistent diarrhoea or blood in the stool, pain, fever, or nausea and vomiting

● For children without colostomy
○ let the parents or caregivers know that their child will have a lot of diarrhoea initially, and teach them how to protect the skin around the anus using the prescribed barrier cream
○ stress the importance of monitoring for skin breakdown with each diaper change, and prompt them to let their healthcare provider know if excessive irritation occurs, or a rash develops that does not get better with cleaning and application of the prescribed creams
○ remind them of the importance of ensuring their child is well hydrated until the diarrhoea resolves

● Teach parents how to administer prescribed paediatric acetaminophen as needed for pain

● Wound care
○ Show them the steri-strips covering the incisions, and let them know that it is okay to bathe their child with them in place, and that they usually fall off on their own in a couple of weeks
○ Explain that there may be some redness and swelling present while the incisions are healing, and stress the importance of contacting their healthcare provider right away if they notice worsening redness or swelling; or if their child develops a fever

● Teach parents that opening in their child’s anus may not be large enough for stool to pass easily, and that the anus may need to be gently dilated, or stretched, for several weeks after surgery
○ Reassure them that the home healthcare nurse will assist them so they can learn how to perform the procedure on their own as needed

Review signs like
○ change in the number of bowel movements the child has each day
○ foul-smelling bowel movements
○ abdominal distension
○ Vomiting

• Remind the parents or caregivers to keep all their child’s follow-up appointments
• Reassure them that although children with Hirschsprung disease might be prone to constipation or faecal incontinence, that they can often live normal lives.

Question 7

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Types of treatment for HD? (6)

Answer 7

Non-surgical options
○ Chronic constipation — stool softeners
○ Rectal irrigation with NaCl
○ Hydration maintained with IV fluids and electrolytes
○ Abdominal distention → NGT placement
○ IV antibiotics to limit risk of infections

Surgical options
○ Surgical resection of aganglionic part of colon → then connect to healthy end of anus