Hirschsprung's Disease

Hirschsprung's disease is a birth defect in which part of the large intestine lacks nerve cells. Without nerve cells, the muscles in
that part of the intestine can't work properly. The symptoms of Hirschsprung's disease usually begin when a child is very young.
Hirschsprung's disease may occur by itself or as part of a syndrome or disorder that affects other parts of the body, such as Down
syndrome (Trisomy 21). It is four times more common in males than in females, and occurs in about 1 in 5,000 births.
Symptoms of Hirschsprung's Disease
Newborns

difficulty with their first bowel movement after birth

vomiting
swelling of the abdomen
discomfort from gas and constipation
Infants

constipation
diarrhea
swelling of the abdomen
difficulty growing and developing
Childhood

constipation
ribbon-like, foul-smelling bowel movements
swelling of the abdomen
anemia, if there is blood in the bowel movements

Diagnosing Hirschsprung's Disease

Several types of tests may be used to tell if an individual has Hirschsprung's disease:

Barium enema x-ray

Barium is put through the anus into the intestine via an enema; the barium makes the inside of the intestine show up better on
the x-ray

Anorectal manometric examination

A small balloon is inflated inside the rectum to check muscle pressures

Rectal biopsy
A small sample of the intestine is removed and examined under a microscope to see if the nerve cells are present.
The biopsy is the most accurate test for Hirschsprung's disease and confirms the diagnosis.
Treatment of Hirschsprung's Disease
Since part of the intestine is lacking nerve cells and will never work properly, that part of the intestine is surgically removed. The
remaining healthy intestine on either side is then connected. This is called a pull-through operation.
Some individuals, especially those who have been sick, may first have an operation called an ostomy. The diseased part of the
intestine is removed, but the top part of the intestine is connected to the outside of the abdomen through an opening (called a
stoma). The ostomy lets the intestine work normally and allows the individual to become healthy prior to the pull-through
operation. When the pull-through is done, the ostomy is closed up because it is no longer needed.

With an incidence of 1/5000 births, the most cited feature is absence of ganglion cells: notably in males,
75% have none in the recto-sigmoid and 8% with none in the entire colon. The enlarged section of the
bowel is found proximally, while the narrowed, aganglionic section is found distally. The absence of
ganglion cells results in a persistent over-stimulation of nerves in the affected region, resulting in
contraction.
1. Delayed passage of meconium
2. Abdominal distension
3. Constipation

Diagnosis
Hirschsprung's disease is suspected in a baby who has not passed meconium within 48 hours of delivery.
Normally, 90% of babies pass their first meconium within 24 hours, and 99% within 48 hours. Other
symptoms include, green or brown vomit, explosive stools after a doctor inserts a finger into the rectum,
swelling of the abdomen, lots of gas and bloody diarrhea. Definitive diagnosis is made by suction biopsy
of the distally narrowed segment.

[12]

[13]

Diagnostic techniques involve anorectal manometry,

Radiologic findings may also assist with diagnosis.

barium enema, and rectal biopsy.

[14

Treatment
Treatment of Hirschsprung's disease consists of surgical removal (resection) of the abnormal section of
the colon, followed by reanastomosis. There used to be two steps typically used to achieve this goal.

The first stage used to be a colostomy. When a colostomy is performed, the large intestine is cut and
an opening is made through the abdomen. This allows bowel contents to be discharged into a bag.

Later, when the childs weight, age, and condition is right, a pull-through procedure is performed.

Orvar Swenson, who discovered the cause of Hirschsprungs, first performed it in 1948.

[15]

The pull-

through procedure repairs the colon by connecting the functioning portion of the bowel to the anus. The
pull-through procedure is the typical method for treating Hirschsprungs in younger patients. Swenson
devised the original procedure, and the pull-through surgery has been modified many times.
The Swenson, Soave, Duhamel, and Boley procedures all vary slightly from each other:

The Swenson procedure leaves a small portion of the diseased bowel.

The Soave procedure leaves the outer wall of the colon unaltered. The Boley procedure is a small
modification of the Soave procedure. The term "Soave-Boley" procedure is sometimes used.

[16][17]

The Duhamel procedure uses a surgical stapler to connect the good and bad bowel.

Of those 15% of children who do not obtain full control, other treatments are available. If constipation is
the problem then usually laxatives or a high fiber diet will overcome the problem. If lack of control is the
problem then a stoma may be necessary. The Malone ACE is also an answer. This is where a tube goes
through the abdominal wall to the appendix or, if available, to the colon. Then once a day the bowel is
flushed. Children as young as 6 do fine with administering this on their own. Details of ostomical sugery
and its results can be found in the book Unwanted Baggage by P. and E. Prosser.
If the affected portion of the lower intestine is restricted to the lower portion of the rectum, other surgical
procedures, such as the posterior rectal myectomy, can be performed.

ARELLANO UNIVERSITY
College of Nursing
Legarda, Manila

HEALTH ASSESSMENT FORM

DEMOGRAPHIC DATA

NAME: ____________________________ ADDRESS: __________________________

AGE: _____ SEX: ________ NATIONALITY: _________ OCCUPATION: _________
RELIGION: ________________ INFORMAT: _________________________________
DATE OF ADMISSION: ______________ TIME: _______ WARD ______ BED: ____
NO. OF ADMISSIONS: _____________MODE OF ARRIVAL: ( ) AMBULATORY
( ) WHEELCHAIR ( ) STRETCHER
MEDICAL DIAGNOSIS: ___________________ PHYSICIAN: ___________________
CHIEF COMPLAINTS: ___________________________________________________

PART 1: HEALTH HISTORY

1. HEALTH PERCEPTION-HEALTH MANAGEMENT PATTERN
A. PAST HEALTH STATUS
Allergies:

FOOD ( ) None ( ) Yes specify: _______________________

________________________________________________________________________
Manifestations: _____________________________________________________
Management done: __________________________________________________

Previous Hospital Experience

( ) None

( ) Yes

When: ____________________________________________________________
Reasons: __________________________________________________________
Childhood Illness: ________________________________________________________
Immunization Received: ___________________________________________________
____________________________________________________________
Description of Health: _____________________________________________________
( ) Excellent
( ) Good

( ) Fair
( ) Poor

B. PRESSENT HEALTH STATUS

Description of Health:
( ) Excellent
( ) Good

( ) Fair
( ) Poor

History of Present Illness

Precipitating Factors: _________________________________________
________________________________________________________________________
________________________________________________________________________
________________________________________________________________________
________________________________________________________________________
________________________________________________________________________
Aggravating factors: ___________________________________________
____________________________________________________________
Alleviating factors: ____________________________________________
____________________________________________________________
Other existing Health Problems: _______________________________________
____________________________________________________________
____________________________________________________________

Current Medication in use: ____________________________________________

____________________________________________________________
____________________________________________________________
C. FAMILY HEALTH STATUS:
Family History: ( ) Diabetes Mellitus

( ) Cancer

( ) Hypertension

( ) Arthritis

( ) Cardiac Diseases
( ) CVA / Stroke

( ) Respiratory Disease
( ) Hemophilia

( ) Kidney Disease

( ) Allergy

( ) Nervous System Dses ( ) STD

Others: Specify __________________________________
_______________________________________________
_______________________________________________
D. DAILY HEALTH PRACTICES:
Dental:

How many times do you brush your teeth in a day?

( ) once ( ) twice

( ) thrice

( ) never

How many times do you visit your dentist in a year?

( ) once ( ) twice( ) monthly

( ) never

Do you have dental carriers?

( ) none ( ) Yes

Location: _____________________________________________
( ) Loose teeth: _________________________________________
( ) Malpositions: ________________________________________
Do you have prosthesis? ( ) none
Use of Cigarette:

( ) no

( ) yes

( ) yes

Type: ______

Brand: __________________

Sticks used everyday: ____________________________________

Use of Alcohol: ( ) no

( ) yes

Brand: __________________

Frequency and amount per day: ____________________________

Use of Drugs:

( ) over the counter

( ) prescribed drugs

Specify: ______________________________________________
______________________________________________
What are the health practices to prevent occurrence of illness?
( ) adequate nutrition

( ) physical examination

( ) weight control

( ) immunization

( ) exercise

( ) self-examination

( ) adequate rest/sleep
Home Health Management: _________________________________________________
__________________________________________________________________
__________________________________________________________________
__________________________________________________________________
2. NUTRITIONAL-METABOLIC PATTERN
A. NUTRITIONAL PATTERN
Usual Foods Taken: _________________________________________________
__________________________________________________________________
Frequency Preferences: ______________________________________________
Food Dislikes: _____________________________________________________
Ability to Eat:
Swallow solid foods

( ) Yes

Swallow Liquids ( ) Yes

( ) No
( ) No

Reason: ___________
Reason: ___________

Chew

( ) Yes

( ) No

Reason: ___________

Ability to Feed Self

( ) Yes

( ) No

Reason: ___________

How is your appetite? _______________________________________________

__________________________________________________________________
Do you take food supplements? Specify: _________________________________
__________________________________________________________________

B. METABOLIC PATTERN
Previous Weight: __________________

Present Weight: ________________

Weight Loose: ____________________

Reason: _______________________

Height: __________________________

3. SLEEP-REST PATTERN
Usual number of hours in sleeping: ________________
Naps: ( ) No

( ) Yes

Number of Hours: ____________

__________________________________________________________________
Sleeping Aids:

( ) Yes

( ) No

Medications: _________________________________________________
Foods: ______________________________________________________
Others: _____________________________________________________

4. ELIMINATION PATTERN
A. URINATION PATTERN
Color:

( ) Yellow amber

( ) cloudy

( ) Bloody

other: ______

Frequency per day: ________________ Amount each voiding: ____________

Odor: __________________________________________________________
Difficulty when voiding specify: ____________________________________

Assistive voiding devices: __________________________________________

B. BOWEL ELIMINATION:
Color: ___________________

Odor: _______________________

Consistency: ______________

Pattern: ______________________

Difficulties in bowel movement: ____________________________________

___________________________________________________________

5. ACTIVITY-EXERCISE PATTERN
ACTIVITIES OF DAILY LIVING
Self-Care Ability:
( ) Grooming

( ) Cooking

( ) Bathing

( ) Home maintenance

( ) Toileting

( ) Exercise

( ) Dressing

( ) Leisure activity

( ) Bed mobility

( ) General mobility

( ) Occupational Activities
Reason of inability to perform above activities: _____________________
___________________________________________________________
____________________________________________________________
FUNCTIONAL LEVEL CODES
0 Full self care
1 Requires use of devices

2 Requires assistance / supervision from others

3 Requires use of devices and supervision others
4 Dependent to care giver and does not participate

6. COGNITIVE PERCEPTUAL PATTERNS

SENSORY PERCEPTUAL DEFICITS
( ) Hearing
( ) Sight

( ) Smelling
( ) Taste

( ) Touch
Use of PROSTHESIS
( ) Eyeglasses
COMPLAINTS OF:
( ) Vertigo
( ) insensitivity to superficial pain
( ) sensitivity to cold and heat
ABILITY TO READ:

( ) Yes

( ) No

( ) Yes

( ) No

7. SELF PERCEPTION PATTERN

What are your most concerned about? ___________________________________
__________________________________________________________________
What are your present health goals? ____________________________________
_________________________________________________________________
8. ROLE RELATIONSHIP PATTERN
Language / dialect spoken: ____________________________________________

__________________________________________________________________
Speech: ( ) clear

( ) slurred

( ) relevant

( ) others: specify ____________________________________________

_________________________________________________________________
_________________________________________________________________

Ability to express self:

( ) Oral

( ) Written

( ) Gesture

Family life:
Do you live alone?

( ) Yes

( ) No

If No, with whom? __________________________________________________

Reason: ___________________________________________________________
How do you manage your finances? ____________________________________
How is your culture background? ______________________________________
__________________________________________________________________
Difficulty with:

( ) marital

( ) parenting

( ) relatives

( ) abuse specify

Describe: _________________________________________________________
_________________________________________________________________

9. SEXUALITY SEXUAL FUNCTION PATTERN

Any change of sexual function because of illness? If yes describe:
____________________________________________________________
____________________________________________________________
____________________________________________________________

Do you have difficulties in: ( ) libido

( ) erection

( ) fertility

Describe: ___________________________________________________
___________________________________________________________
Do you use contraceptives?

( ) No

( ) Yes

Methods used: ______________________ Duration of Use: __________

10. COPING STRESS MANAGEMENT PATTERN

Decision Making:

( ) Death of love ones

( ) Job

( ) others, specify ___________________________

How do you manage these losses in life? ________________________________
___________________________________________________________
___________________________________________________________
What do you do when you are under stress of tensed? ______________________
____________________________________________________________

11. VALUE BELIEF SYSTEM PATTERN

Who is / are your source/s of strength of life? _____________________________
____________________________________________________________
____________________________________________________________
What are religious practices? __________________________________________
____________________________________________________________
Is there any religious practices you desire during hospitalization? _____________
____________________________________________________________
____________________________________________________________
____________________________________________________________