Hirschsprung Disease - Case Study

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

 1.1 What is the condition, disease and problem(s)?

most likely condition: Hirschsprung Disease

• lack of ganglion cells results in lack of normal peristalsis = fecal obstruction

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

1.2 What are presenting signs and symptoms of the condition?

• chronic constipation since birth (congenital condition)

• poops only 2-3x a week

• abdominal distention (“lack of normal peristalsis”)

• hypoactive bowel sounds (“lack of normal peristalsis”)

• requires enemas, lactulose and plum juice. (“persistent constipation despite treatment)

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

1.3 provide your rationale. consider how many systems are affected. is it a cardiac problem? a respiratory ande GI problem?

Hirschsprung disease is most likely because consitpation began at birth, and is accompanied by abdominal distention. these findings do not confirm the diagnosis. a rectal biopsy is the diagnostic test

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

2.1 what other assessment do you want to include? provide your rationale. disease specific?

1. “has pt. passed meconium in the first 24 hours?”

= key sign of hirschsprung

2. stool characteristics. RIBBON-LIKE STOOL?

= key sign

3. focused abdominal assessment

• inspection → ausculation → percussion → palpation → measurement

• measure abdominal girth at level of umbilicus to assess distention

= assess progression of the cndition

4. ENTEROCOLITIS: “has pt. had fever or explosive watery diarrhea? “

= key complication

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

2.2 what other assessment do you want to include? provide your rationale. growth and development specific?

1. height, weight, and growth trends

= identifies FTT (failure to thrive)

2. assess nutrition & fluid intake

= appetite

= feeding tolerance

= get enough nutrients?

3. psychosocial development

= feelings towards bowel problems

= how it affecgts her daily activities

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

3. What are immediate priorities for the patient? Provide your rationale.

1. assess INTESTINAL OBSTRUCTION + ENTEROCOLITIS

= hirschsprung causes impaired intestinal motility and fecal obstruction.

• watch for FEVER + EXPLOSIVE DIARRHEA

= signs of ENTEROCOLITIS. this is life-threatening and can progress to sepsis. if found, NOTIFY DOCTOR to prevent further complications.

2. maintain fluid & electrolyte imbalance

• strict I&Os, monitor electrolyte serum, IV fluids as ordered

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

4 What are other priorities for the patient? Provide your rationale.

1. maintain adequate nutrition

= to prevent FTT

1. promote G&D

= asess whether bowel problems interferes with age-appropriate activites & toilet-training

3. emotional support

= reduces anxiety & discomfort associated w/ chronic constipation, enemas, and potential surgery.

4. educate parents

= condition

= diagnostic process

= warning signs of enterocolitis

= treatment plan

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

5.1 what are interventions for the patient? provide your raitonale. (disease-specific )

1. monitor abdominal girth and bowel sounds

= identifies worsening condition

2. monitor stool frequency and characteristics

= identifies constipation and abnormal stools

3. monitor for fever and explosive diarrhea

= enterocolitis

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

5.2 what are interventions for the patient? provide your raitonale. (immediate priorities )

1. maintain strict I&Os

= for hydration and fluid balance

2. maintain serum electrolytes

= identifies potential electrolyte imbalances

3. preapre for RECTAL BIOPSY

= diagnostic test for hirschsprung

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

5.3 what are interventions for the patient? provide your raitonale. (other priorities)

education and emotional support

= promotes understanding and reduce anxiety

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

5.4 what are interventions for the patient? provide your raitonale. (pre-op care)

1. administer prescribed enemas

= get rid of accumulated poop

2. Prepare the child and family for surgical treatment

= surgical removal of aganglionic portion

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

5.5 what are interventions for the patient? provide your raitonale. (post-op care )

no rectal temps or suppositories

prevents injuries to surgical sites

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

6. what are nursing dignoses that apply to the patient or condition? provide your rationale (observed nursing diagnosis)

constipation r/t impaired intestinal mobility AEB bowel movements 2-3 imes per week, abdominal distention, and the need for enemas and laxatives.

= the pt. has chronic constipation since birth. In Hirschsprung disease, the absence of ganglion cells prevents normal peristalsis, causing stool to accumulate in the intestines, making it difficult to poop

A 4-year-old female patient presented to the clinic with long-standing constipation, which afflicted her since birth. The mother reports that the patient is only able to defecate 2 to 3 times per week. The patient has been requiring the administration of rectal enemas, lactulose and plum juice. Upon physical examination, the patient has hypoactive bowel sounds, there is no abdominal tenderness on palpation, the abdomen is distended.

6. what are nursing dignoses that apply to the patient or condition? provide your rationale (risk

risk for deficient fluid volume r/t potential bilious vomiting, diarrhea, or decreased oral intake

hirschsprung disease can cause intestinal obstruction and enterocolitis, which lead to bilious vomiting, diarrhea, and decreased oral intake. these complications increase the pt’s risk for dehydration and fluid and electrolyte imbalane