HIRSCHSPRUNG

[Type the document title] 1
I. INTRODUCTION

Hirschsprung's disease (HD) is a congenital illness in which there is a lack of
intrinsic nerves (ganglion cells) in the distal segments of the intestinal tract. These
abnormal segments produce mechanical obstruction because of failure to relax
during peristalsis. Hirschsprung's disease always starts in the anal verge, but the
length of the segment without ganglion cells (aganglionic) varies: it is limited to the
rectum and sigmoid in 75% of patients; involves the whole colon in 8%; and rarely
involves the small bowel.

Most babies with HSCR are born to mothers with a normal antenatal history
(92%) with the majority having good Apgar scores The incidence of Hirschsprung's
disease varies from 1 in 5,000 to 1 in 10,000 live births, highest incidence in Asian
populations (2.6 in 10,000), intermediate in Afro-Americans (2.1 in 10,000) and
Caucasians (1.5 in 10,000) and lowest in Hispanics (1 in 10,000). There appears to
be a male preponderance with a ratio of 3:1 to 5:1, particularly in those with short
segments. Approximately 30% of patients show an association with other disorders
such as chromosomal abnormalities. While possible environmental effects on
disease pathogenesis have not yet demonstrated, the genetic component has
been recognized since the 1960s.

Diagnosis of Hirschsprung's aims to confirm the absence of ganglionic cells.
The most widely used diagnostic procedures are barium enema, anorectal
manometry measurement of the pressures of the internal and external sphincter
with a rectal balloon) and rectal biopsies which is considered the gold standard
method.

The treatment of Hirschsprung's disease is surgical. Initial medical
management is important, however, in stabilizing the patient before surgical
therapy is undertaken. This includes the correction of fluid and electrolyte
imbalances, antibiotic therapy if enterocolitis is present and rectal decompression
with the use of rectal irrigations and rectal tubes until the time of surgery.
The basic principle for the definitive surgical therapy is resection of the
aganglionic segment followed by a pull-through of ganglionic bowel down to the
anus. There are different procedures that have been used, but the three most
common ones are: Swenson pull-through (rectosigmoidectomy), Duhamel pull-
through (retrorectal transanal pullthrough) and Soave pull- through (endorectal
pull-through). Recently the surgery is being performed in the newborn period using

minimally invasive surgical techniques, like laparoscopy.

In sum, as student nurses we play a vital role throughout the disease process.
Proper nursing management must be administered to help the patient cope with
his condition. Health teaching should be given in order for the patient to realize
the effects of her disease or condition. A student nurses we should check and
correct the lifestyle the patient has to lessen the occurrence of such disease

Reason for choosing the study
Diabetes Mellitus is one of the most prevalent cause of death and illness,
particularly among obese adolescents around the world. High incident rates
suggest that people still lack the knowledge and means on the prevention and
treatment.
The student nurses picked the case not only to enhance their awareness
about the disease, but also to utilize the knowledge obtained in the information
dissemination process. They believe that the growing concern on diabetes mellitus
should be taken with extended efforts. In the case of our patient, peripheral
vascular diseases and neuropathy resulted to the client to having diabetic foot
infections and later have lead to leg amputation. As we all know, the deaths from
Diabetes mellitus account from its devastating complications, specifically from
neuropathy. As student nurses, it is not enough that we are just equipped with the
theoretical competence but more importantly is our ability to integrate what we
have learnt through the practical application of skills. Hence, proper management
of the disease is pivotal in order to avoid occurrence of complications.


Objectives: Following the completion of the case study, the student nurses shall
have:
Cognitive:
Gain a significant amount of knowledge on Hirschsprungs disease
encompassing the definition, etiology, clinical signs and symptoms and how
it is diagnosed.
Discuss the anatomy and physiology of the Digestive system specifically the
connection of Enteric nerve system.
Explain the pathophysiologic nature of Hirschsprungs
Identify possible complications of Hirschsprungs disease
Determine the prognosis of the disease with the following criteria: duration of
illness, onset and precipitating factors,.
Interpret the findings from laboratory examinations with their clinical
significance.
Psychomotor:
Perform cephalo-caudal physical assessment thoroughly.
Relate nursing concepts learned in the management of a patient with
Hirschsprungs disease
Develop appropriate nursing care plans related to the potential and existing
problems effective for the improvement and management of disease.
Select the appropriate, immediate nursing management based on the
health deficit identified
Perform independent, dependent and collaborative nursing interventions
based on the nursing diagnosis listed.
Affective:
Express genuine concern for patients with Hirschsprungs disease.
Pay attention on the importance of developing a practice of performing
accurate and complete assessment findings.

Assert the role in the nursing profession of finding out appropriate ways to
promote patients relief and recovery.
Integrate the knowledge acquired to co-student nurses to increase their
awareness for future encounters with a client having the same condition.

II. NURSING HISTORY

A. BIOGRAPHIC DATA
SpongeBob is a 7-month old Filipino male born on December 4
th
2013.
He currently resides in Santo Rosario Candaba. SpongeBobs birthplace is San Jose

City, Nueva Ecija. On June 23, 2014, at around 7:00AM, the patient was admitted
to Jose B. Lingad Memorial Regional Hospital with the following chief complaint
upon admission: absence of defecation and the enlargement of his abdomen.

B. PAST MEDICAL HISTORY
Our patient Spongebob was a born prematurely at Malasin San Jose
City, Nueva Ecija; the mother was only pregnant for 7 months. The mother did not
undergo any newborn screening prior to Spongebobs delivery. Also during her
pregnancy she took medications that she could not remember for the doctor
diagnosed her with a Urinary Tract Infection which could have affected
Spongebob for she was only 2 months pregnant. During the interview SpongeBobs
mother said that once she gave birth to Spongebob he was immediately taken
away because he was to be transferred to another hospital that has a Neonatal
Intensive Care Unit for Spongebob was diagnosed to have pneumonia.
Spongebob stayed in the NICU for 1 week and the doctor advised Spongebobs
mother to have Spongebob undergo blood transfusion. The mother than took
Spongebob home against medical advice for she was told by family members to
not push through with the advice of the doctor and to just to take Spongebob
home. During the interview Spongebobs mother stated that when she brought
him home he was fine and showed no complications. Until May 15,2014 when
Spongebob was 4 months old he started not defecating for 3 days which alerted
the mother. She then took him to Cortez Hospital Arayat from May 18-23. The
doctor that handled Spongebob said that he had an inflammation of the
intestines which was preventing him from defecating. The doctor also said there
was a blockage in the intestines at the descending colon. Due to the bills they
incurred at Cortez Hospital Spongebobs mother decided to move Spongebob to
Arayat Hospital from May 23-June 6. The doctor in Arayat hospital then allowed
the mother to breastfeed Spongebob every 3 hours and in the following days
raised the proportion to 2 ounces every 2 hours. After a few days of close
monitoring on Spongebobs health status the doctor adviced Spongebobs

mother to transfer Spongebob to Manila where they have specialized pediatric
hospitals. Spongebobs mother thought about moving yet the following morning
Spongebob then passed stool and stayed that way for the following 3 days.
Afterwards the doctor said to Spongebobs mother that Spongebob has no
obstruction in the intestines and that his stomach was no longer distended so the
doctor sent them home. Spongebob and his mother left Arayat hospital on June 6,
yet on June 7-9 Spongebob did not defecate which then alerted his mother. She
was going to bring him to the hospital the following day yet he finally defecated
from June 10-17. Unfortunately from June 17-23 Spongebob did not defecate
thereby distending his stomach and giving him complications. This is when they
brought Spongebob to Jose B. Lingad Memorial Regional Hospital. The patient
does not have any allergies noted.

C. HISTORY OF PRESENT ILLNESS
One week prior to admission the SO noticed that the patients stomach was
starting to enlarge and he has not been defecating for 6 days. This is when the SO
rushed the patient to Jose B. Lingad Memorial Regional Hospital on June 23, 2014
at 7:00AM with the diagnosis of to consider Hirschsprungs disease.

Characteristic:
Onset:
Location:
Duration:
Severity:
Pattern:
Associated factors:.
Related symptoms:

51 years old 60 years old
54 years
old
58 yrs old

D. LIFESTYLE
The patient rests all through the day and his mother provides him with
nutrition by breastfeeding. ADLs during disease process: The patient showed signs
of weakness due to many days of being placed on NPO status. Also he would cry
whenever any people get near him for he is traumatized from all the procedures
performed on him.

E. Family History of Illness


19 years
old
27 years old
30 years
old
30 years
old old
36 years
old
35 years
old
26 years
old
28 years
old
7 months old

LEGEND:

The Genogram above portrays the immediate and branch family
members including the living and the deceased with their causes of death. The
patient does not have any familial history of Hirschsprungs disease. Also the
patient told us that he has no familial history of any chronic diseases. The patient
has is the first born child and his maternal and paternal grandparents are still alive.

III. PHYSICAL ASSESSMENT
June 23, 2014 (LIFTED FROM THE CHART)
Cardiac rate: 110 bpm
Respiratory rate: 32 cpm
Temperature: 37
O
C

Skin (-) Cyanosis; (-) Jaundice; Cold skin
Head:
EENT: AS, PPC
Lymph nodes: No lymph adenopathy

Chest:
MALE
FEMALE
Spongebob
DECEASED

Lungs: SCE ; CBS
Cardiovascular: AP, MRRR

Abdomen: Globular, Distended abdomen
Musculoskeletal: Good muscle tone
Neurologic: Awake; coherent
Admitting impression: T/C Hirschsprung Disease

July 03, 2014
General Appearance
Received patient on bed with an ongoing IVF of PLRS 1L x 125 ugtts/minute
@ 900cc level, infusing well at the right foot; with intact NGT draining on bedside
bottle; with intact rectal tube.

Vital Signs:
PR: 114 bpm
RR: 39 cpm
T: 36.5C

Skin:
Brown skin complexion
Warm to touch
Presence of pallor
poor skin turgor
(+) dry cracked, scaly skin

Head
normocephalic, proportion to the body
no lesions, masses palpated
Hair

black hair
hair is evenly distributed
(+) oily hair
(+) dandruff
Eyes
eyebrows and eyelashes are evenly distributed
(+) PERRLA
anicteric sclera
pale conjunctiva
Ears
symmetrical
both ears can hear well when spoken softly
(+) dry cerumen
(-) discharge
(-) tenderness
Nose
Nose located midline
(-) discharges
both nares are patent
nasal mucosa is pinkish
Mouth
pink tonsils
(+) dental carries
hoarseness of voice
(+) halitosis
(+) gag reflex
(-) lesions

Neck
trachea located midline
palpable bilateral cervical lymph nodes
with full range of motion
(-) stiffness
(-) pain
Chest and Lungs
with symmetrical chest expansion
with clear breath sounds on both lung field
with no chest retractions
intercostal spaces are even and relaxed
(-) pain during inhalation and exhalation
(-) adventitious sounds
RR is 25cpm

Upper Extremities
Muscle weakness
unable to perform a full range of motion due to reported weakness

Abdomen
soft
rounded contour
no lesions, nodules and masses upon palpation
(-) tenderness
presence of striae

Lower Extremities

amputated left leg
(+) wounds on the right foot
(+) edema
absence of the 2
nd
,4
th
and 5
th
digits of the right foot
(+) weakness, leg muscles
limited range of motion
Intake and Output

Date Intake Output

March 6, 2014
8:00am 2:00pm

ORAL INTAKE: 200ml
IV FLUIDS: 750ml
TOTAL INTAKE: 950 ml

TOTAL URINE OUTPUT:
1,200 ml


IV. DIAGNOSTICS AND LABORATORY PROCEDURES

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Indications or Purpose Results Normal
Values
Analysis and
Interpretation

1. Complete
Blood Count

A. Hemoglobin
(Hgb)

February 25, 2014
Hemoglobin is a protein
inside red blood cells that
carries oxygen throughout
the body. It is used to
screen, diagnose, or
monitor a number of
conditions and diseases
that affect red blood cells
(RBCs) and/or the amount
of hemoglobin in blood.

118

125 175 g/L

Hemoglobin level
is decreased. This
indicates a
decrease oxygen
supply in the
body.

B. Hematocrit
(Hct)

February 25, 2014
Hematocrit is the
proportion of your total
blood volume that is
composed of red blood

0.36

0.40 0.52
The result showed
DECREASED Hct
level which may
indicate anemia,

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

cells. A hematocrit (Hct)
test indicates whether you
have too few or too many
red blood cells
conditions that can occur
as the result of certain
diseases.
malnutrition,
nutritional
deficiencies of
iron, folate,
vitamin B12 and
vitamin B6, and
overhydration.
C. White Blood
Cells (WBC)

February 25, 2014
White blood cells (WBCs),
also called leukocytes, are
an important part of the
immune system. These
cells help fight infections
by attacking bacteria,
viruses, and germs that
invade the body.

11

5 10 x 10
9/L
WBC is within
normal range.
D. Neutrophils/
Segmenters

February 25, 2014
Neutrophils are a type of
white blood cell. They are

.70

0.45 0.65
Since it is
attracted to sites

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

the most abundant type
of white blood cells. They
are a part of the body's
immune system and are
the first cells to increase in
case of an infection,
especially bacterial
infection.
of injury and
infection, an
increased in
amount may
indicate
metabolic
disorder such as
diabetic acidosis.
E. Lymphocytes
February 25, 2014
Lymphocytes are an
integral component of
blood in human beings.
They are also an important
part of the human
immune system. This test
measures the number of
lymphocytes (a type of
white blood cell) in blood.
It is used to evaluate and

0.30

0.20 0.35

Within normal
range

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

manage disorders of the
blood or the immune
system.
F. Platelet count
February 25, 2014
Platelet count is a
diagnostic test that
determines the number of
platelets in the patient's
blood. Platelets, which are
also called thrombocytes,
are small disk-shaped
blood cells produced in
the bone marrow and
involved in the process of
blood clotting.

275

150 400 x
10 9/L

Within normal
range

Nursing Responsibilities:
Before:
1. Explain the test procedure and purpose.
2. Explain that slight discomfort may be felt when the skin is punctured.
3. Obtain a history of the patient's complaint, which includes a list of known allergens.
4. Obtain a list of the patient's current medications, including herbs, nutritional supplements, and nutraceuticals.
5. Note any recent procedures that can affect with test results.
6. Encourage to avoid stress if possible because altered physiologic status influences and changes normal hematologic
values.
7. Be sensitive to social and cultural issues, as well as concern for modesty, are important in providing psychological support

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

before, during, and after the procedure.

During:
1. Instruct the patient to follow and cooperate with the given directions.
2. Instruct the patient to breathe normally and to avoid unnecessary movement.
3. Observe standard precautions, and follow the general guidelines.
4. Identify the patient, and label the appropriate tubes with the corresponding patient demographics, date, and time of
collection.
5. Perform a venipuncture and collect the specimen.
6. Apply manual pressure and dressings over puncture site to stop bleeding
7. Transport the specimen to the laboratory for processing and analysis.
After:
1. Monitor the puncture site for oozing or hematoma formation.
2. Evaluate test results in relation to the patient's symptoms and other tests performed.

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

1. Blood
Chemistry

A. Random Blood
Sugar (RBS)

February 25, 2014
This blood test measures the
amount of glucose in the
blood. Because it is a

19.92

3.85 9.0
mmol/L
There is not
enough insulin, or
if the body stops

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

random test, it means that it
can be done at any time
responding to
insulin, sugar
builds up in the
blood.
B. Blood Urea
Nitrogen (BUN)

February 25, 2014
BUN test reveals important
information about how well
your kidneys and liver are
working. A BUN test
measures the amount of
urea nitrogen that's in your
blood.

5.2

1.7 8.3
mmol/L

Normal values
indicate that the
kidneys are still
functioning
properly
C. Creatinine
February 25, 2014
Creatinine is a waste
product that forms when
creatinine breaks down.
Creatinine is a substance
found in muscle. Creatinine
levels help determine how
well your kidneys function.

114.4

60 120
umol/L

Results are within
normal range.

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

Your kidneys are responsible
for eliminating creatinine
from the body when you
urinate.
D. Sodium
February 25, 2014
Sodium is a mineral essential
to the body. Sodium is
particularly important for
nerve and muscle function.
Blood sodium testing is used
to detect abnormal
concentrations of sodium

140.9

135 145
mmol/L
The results
showed a normal
sodium level
which indicates
normal fluids and
electrolytes in the
body.

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

E. Potassium
February 25, 2014
Potassium test checks how
much potassium is in the
blood. Potassium is both
an electrolyte and a
mineral. It helps keep the
water (the amount of fluid
inside and outside the
body's cells) and electrolyte
balance of the body.
Potassium (K+) also helps
nerves and muscles
communicate.

4.42

3.5 5.5
mmol/L
Result is within
normal range;
indicative of
normal renal
functioning.
F. Ionized
Calcium

February 25, 2014
Ionized calcium test checks
the amount of calcium that
is not attached to protein in
the blood.

1.3

1.1 1.4
mmol/L

It is within normal
range.

Nursing Responsibilities:
Before:

Diagnostic/
Laboratory
Procedure
Date Ordered
And
Date Performed
Values
Analysis and
Interpretation

1. Define and explain the test.
2. State the specific purpose of the test.
3. Explain the procedure.
During:
4. Ensure that the patient is sitting or lying comfortably.
5. Note that when the needle is inserted to draw blood, the patient may feel moderate pain.
After:
6. Monitor the puncture site for oozing or hematoma formation.
7. Safely dispose of any waste.


V. THE PATIENT AND HER ILLNESS

A. ANATOMY AND PHYSIOLOGY

GASTROINTESTINAL
SYSTEM
If a human adults digestive tract
were stretched out, it would be 6 to 9 m
(20 to 30 ft) long. In humans, digestion
begins in the mouth, where both
mechanical and chemical digestion
occur. The mouth quickly converts food
into a soft, moist mass. The muscular
tongue pushes the food against the
teeth, which cut, chop, and grind the
food. Glands in the cheek linings secrete
mucus, which lubricates the food, making
it easier to chew and swallow. Three pairs
of glands empty saliva into the mouth
through ducts to moisten the food. Saliva
contains the enzyme ptyalin, which
begins to hydrolyze (break down)
starcha carbohydrate manufactured
by green plants.
The Esophagus
The presence of food in the pharynx stimulates swallowing, which squeezes the
food into the esophagus. The esophagus, a muscular tube about 25 cm (10 in)
long, passes behind the trachea and heart and penetrates the diaphragm
(muscular wall between the chest and abdomen) before reaching the stomach.
The Stomach
The stomach, located in the upper abdomen just below the diaphragm, is a
saclike structure with strong, muscular walls. The stomach can expand significantly
to store all the food from a meal for both mechanical and chemical processing.
The stomach contracts about three times per minute, churning the food and
mixing it with gastric juice. This fluid, secreted by thousands of gastric glands in the

lining of the stomach, consists of water, hydrochloric acid, an enzyme called
pepsin, and mucin (the main component of mucus). Hydrochloric acid creates the
acidic environment that pepsin needs to begin breaking down proteins. It also kills
microorganisms that may have been ingested in the food. Mucin coats the
stomach, protecting it from the effects of the acid and pepsin.
The Small Intestine
Most digestion, as well as absorption of digested food, occurs in the small
intestine. This narrow, twisting tube, about 2.5 cm (1 in) in diameter, fills most of the
lower abdomen, extending about 6 m (20 ft) in length. Over a period of three to six
hours, peristalsis moves chyme through the duodenum into the next portion of the
small intestine, the jejunum, and finally into the ileum, the last section of the small
intestine. During this time, the liver secretes bile into the small intestine through the
bile duct. Bile breaks large fat globules into small droplets, which enzymes in the
small intestine can act upon. Pancreatic juice, secreted by the pancreas, enters
the small intestine through the pancreatic duct.
The Large Intestine
The colon is approximately five feet long in length, begins in the ileocecal
valve, and ends at the rectosigmoid junction. Arterial Blood supply to the colon
from cecum to splenic flexure is through the superior mesenteric artery which gives
rise to the ileocolic, right colic, and middle colic arteries. The left and sigmoid
colon is supplied by the inferior mesenteric artery which gives rise to left colic and
sigmoidal arteries. There can be several anatomic variations in the colic arteries
including absent middle colic artery, absent right colic artery, common trunk for
right and ileocolic artery, and the presence of an arc of riolin between the middle
and left colic artery. The colonic wall histologically rom lumen and outward
consists of (1.) a simple columnar epithelium which forms crypts, (2.) lamina
propria, (3.) muscularis mucosa, (4.) submucosa, (5.) mascularis propria formed by
an inner circular and outer longitudinal layer of smooth muscle, and (6.) serosa.

Rectum
The rectum is the terminal portion of the large intestine beginning at the
confluence of the three tenia coli of the sigmoid colon and ending in the anal
canal.


PHYSIOLOGY OF THE DIGESTIVE SYSTEM
The digestive system is responsible for taking whole foods and turning them into
energy and nutrients to allow the body to function, grow, and repair itself. The six
primary processes of the digestive system include:
1) Ingestion of food
2) Secretion of fluids and digestive enzymes
3) Mixing and movement of food and wastes through the body
4) Digestion of food into smaller pieces
5) Absorption of nutrients
6) Excretion of wastes

The most important mechanism in which food is transported throughout the
digestive system is called peristalsis. Peristalsis is a muscular wave that travels the
length of the GI tract, moving partially digested food a short distance down the
tract. It takes many waves of peristalsis for food to travel from the esophagus,
through the stomach and intestines, and reach the end of the GI tract. The
peristalsis movement is governed by another body system, which acts in concert
with the digestive system; this is the Enteric Nervous system.

ENTERIC NERVOUS SYSTEM
The digestive system is
sometimes termed as the Gut
Brain, it is because of the
presence of neurons along the
tract. Thee ENS is the nervous
system intrinsic to the digestive
system. It consists of thousands of
interconnected small ganglia
embedded in the walls of the
entire digestive tract. Each
ganglion is composed of
neurons and associated glial
cells. As summarized below, the
extensive neural circuits of the ENS are capable of local autonomous function. In
addition, the ENS works in hand in hand with the central nervous system (CNS)
through widespread two-way connections, to control the digestive system in the
context of local and whole body physiological demands These connections are
provided by both vagal and sympathetic fibers along mesenteric vessels

The gut wall is composed of a mucosal layer, a smooth muscle layer, and a
serosal layer. The muscle layer consists of at least two sheets of parallel muscle
fibers typically arrayed at right angles to one another: a circular layer wrapping
around the gut and a longitudinal layer running along it. Most neurons of the ENS
are found in the mucosal and muscle layers, forming two main networks of nerve
ganglia extending from esophagus to anus, and known as the submucosal plexus
and the myenteric plexus, respectively (Fig. 1a). The myenteric plexus lies between
the longitudinal and circular muscle sheets of the gut wall and exerts control
primarily over digestive tract motility. The submucosal plexus, as its name implies, is
buried underneath the mucosa. Its principal role is in sensing the environment
within the lumen, regulating gastrointestinal blood flow and controlling epithelial
cell function.
Neural crest cells: the multipotent precursors of the ENS
Cells forming the ENS are derived from a
transient and vertebrate specific
population of multipotent cells called neural
crest cells (NCC). These vagal NCC initially
migrate in a dorsal to ventral direction to
colonize the foregut (future esophagus and
stomach), where they are now named
enteric (e) NCC. Gut colonization by eNCC
occurs during a relatively long period of time
and is performed in parallel with gut tissue
growth and rearrangement. Such timing appears important for proper colonization
of the hindgut since a subpopulation of eNCC crosses from the prospective small
intestine to the future colon via the mesentery during a developmental time
period in which these gut regions are transiently juxtaposed. These trans-
mesenteric eNCC constitute a large part of the hindgut
This minor contingent of NCC colonizes the hindgut up to the cecum in a caudal
to rostral direction. During and following migration, eNCC will undergo extensive
proliferation and differentiation up to one month after birth in order to form the
mature ENS



B. PATHOPHYSIOLOGY
a) BOOK-BASED (SCHEMATIC DIAGRAM)

Modifiable Factors

Non-modifiable factors
Gene mutation
Familial predisposition (sibling
who has Hirschsprungs Disease)
Other congenital defects( Cardiac
problems, Down syndrome)
Age (0-24 months)
Gender (Male)
Race (Non white)
Failure of ganglion cells to migrate
craniocaudally via neural crest during gestation
Absence of Ganglion cells in the myenteric
plexuses of the large intestines
Depressed parasympathetic innervation
Inadequate expression of PNS stimulation (Increased
acetylcholinesterase and decreased Nitrous Oxide synthase
Ineffective peristaltic movement
Obstruction
Failure to pass meconium with 48 hours
following birth
Constipation

Proximal intestine markedly
dilated with feces

Bowel distention secondary
to fecal stagnation and gas
Decreased
colonic water
re-absorption
Increased
water in stool
Protuberant abdomen
Bacterial
overgrowth
Mucosal
irritation
Perforation
Passage of
watery stool
(overflow
diarrhea)
Bile stained
vomiting/
fecal vomiting
Abdominal Pain
Lack of interest in
feeding
Irritability
Dehydration
Electrolyte
Imbalance
Excessive crying
Inhibits relaxation of contracted segment
Mutation of RET Proto-oncogene
Inflammatory
response
Increased
leukocytes
Fever

Obstruction Ineffective peristaltic movement Constipation
dilated with feces
Decreased
water
absorption

Increased
intraluminal
pressure

Abdominal
Pain
Increased
water in stool
Protuberant abdomen
Bacterial
overgrowth
Mucosal
irritation
Perforation
Passage of
loose watery
stool
Enterocolitis
Poor nutrition
Decline in
weight
Growth delay
Easy
fatigability,
lethargy
Peritonitis
SHOCK
Failure to thrive
Anemia
Wasted
extremities,
loss of
subcutaneous
tissues


i. SYNTHESIS OF THE DISEASE
1. DEFINITION OF THE DISEASE

Hirschsprungs disease, a congenital disorder also known as aganglionic
megacolon, is characterized by absence or marked reduction of
parasymphatetic ganglionic innervation to the muscle of a section of the
bowelin most instances, the lower portion of the sigmoid colon just above the
anus which is termed as rectosigmoid (Dasgupta & Langer, 2008). During normal
fetal development, cells from the neural crest migrate craniocaudally from the
foregut, midgut and lastly into the hindgut of the large intestine (colon) to form
the networks of nerves called Auerbach's plexus and Meissner's plexus. In
Hirschsprung's disease, the migration is not complete and part of the colon lacks
these nerve bodies that regulate the activity of the colon. The absence of these
nerve cells means there are no peristaltic waves in this section to move fecal
material through the segment of intestine. This results in chronic constipation or
ribbonlike stools (stools passing through such a small, narrow segment look like
ribbons). The portion of the bowel proximal to the obstruction dilates, distending
the abdomen.

A. PREDISPOSING AND PRECIPITATING FACTORS WITH RATIONALE

Non-modifiable Factors:
Familial Predisposition
o Studies show that the susceptibility of an individual to acquire
Hirschsprungs Disease is Increased with affected sibling. Recurrence
rate within families varies from 1% to 33% for subsequent pregnancies as
follows:
If first child is male and has short segment Hirschsprung: 5% for male
siblings, 1% for female siblings.

If first child is female and has short segment Hirschspung: 5% for male
If first child is male and has long segment Hirschsprung: 17% for male
If first child is female and has long segment Hirschsprung: 33% for
male siblings, 9% for female siblings. (Washington University in St.
Louis, 2014)

Gene Mutation
o The major gene of Hirschsprung disease was identified in this chromosomal
10 region, it was the RET proto-oncogene (Roncheto et al., 2001). It was
estimated that RET mutations account for approximately 50% of HSCR
cases and EDNRB mutations account for approximately 5%. Short-segment
HSCR occurs in about 25% of RET-caused cases and in more than 95% of
EDNRB-related cases (Chakravarti, 1996).

Other congenital defects
o In 12% of cases, HSCR is associated with chromosomal anomalies such as
trisomy 21, and in 18% of cases, it is part of a syndrome in recurrent
combination with other defects like congenital heart defects (Bergeron et
al., 2012)
Age
o Nearly all children with Hirschsprung disease are diagnosed during the first 2
years of life. Approximately one half of children affected with this disease
are diagnosed before they are aged 1 year. A small number of children
with Hirschsprung disease are not recognized until much later in childhood
or adulthood.
o (Black and Hawks, 2009).

Gender

o It has long been recognized that males are more commonly affected than
females with a male:femate ratio of 4:1. The male preponderance is less
evident in long-segment HD, where the male:female ratio is 1:1-2:1 and is
even reversed in total colonic aganglionosis, where the male:female ration
is 0.8:1. The reason for these skewed ration is unclear; no X-linked factor
have been described in HD (Puri & Montedonico, 2008).

Race
o The California Birth defects Monitoring Program have found he highest
incidence of HD among Asians with a frequency of 2.8 in 10,000 live births
followed by African-Americans with a frequency of 2.1 in 10,000 live births
(Puri & Montedonico, 2008). Previous epedimiological studies suggest that
the ratio for non-whites to whites was 1.67: 1. Non-white males had the
highest rate, 37.6 per 100000 livebirths. (Goldberg, 1984)

Modifiable Factors:
Essentially, Hirschsprungs disease appears to result at a molecular level
from disruption of normal signaling during development. As a result, the cues of its
development all point out on gene defect in which there are no known
modifiable factors associated to it. Very little is known about micro-environmental
factors in relation to the pathogenesis of Hirschsprungs Disease and this would
seem to be the area for future emphasis in research (Lantieri et al., ). Among
environmental factors studied, there was no time trend and no relationship with
socioeconomic status found also. (Goldberg, 1984). Hence, there are no guidelines
for the prevention of Hirschsprung's.

.

Signs and Symptoms with rationale (Book-based):

Failure to pass meconium within 24-48 hours
o Since ganglion cells failed to migrate into the distal portion of the large
intestines, no peristalsis will take place resulting to inability to propel
intestinal contents forward.

Constipation
o The hallmark of Hirschsprungs disease is the presence of prolonged
constipation. In the absence of signaling nerves, the portion of the colon
affected will remain contracted and narrow, which leads to difficulty
passing stool.

Abdominal distention
o Protuberant abdomen will be evident on the patient secondary to
retention of gas, intestinal contents and bowel obstruction. The
accumulation of intestinal contents and obstruction will be seen on the
portion proximal to the aganglionic colon, behind the affected part. This is
termed as megacolon. Fecal masses are easily palpated from the
retention of stool.

Vomiting
o Bile stained or fecal vomiting is a mechanism of the stomach to empty itself
due to feeling of fullness caused by intestinal obstruction and increased
intraluminal pressure.

diarrhea
o Diarrhea may happen as a normal consequence of mucosal irritation
brought by bacterial growth in the obstructed colon. In Hirschsprungs

disease, colonic water reabsorption is diminished. In effect, more water
accumulates the colon. Because of its form, it may leak despite the narrow
and contracted colon giving rise to overflow diarrhea.

Dehydration
o Dehydration may happen in Hirschsprungs disease in three ways. First as a
result of excessive vomiting and second from the water leakage
happening in diarrhea and lastly related to subsequent feeding difficulties
and inability to ingest adequate liquids. These mechanisms can displace
the normal hydration status of the body thats why it is imperative to watch
out for signs and symptoms of dehydration and electrolyte imbalances.

Abdominal Pain
o The discomfort felt by the patient is induced by bowel distention, bacterial
overgrowth and increased intraluminal pressure. It can be manifested as
Irritability and excessive crying as seen among infants.

Increased white blood cells
o Because of the bacterial overgrowth in the colon, the body reacts by
increasing circulating white blood cells to fight off infection.

FEVER
o As a normal mechanism of the body to ward off the growth of bacteria,
inflammatory response occurs such as increased in body temperature.

Loss of appetite
o Poor food intake is noticed among patients with Hirschsprungs disease. This
is associated to resultant abdominal distention, retention of intestinal
contents and the discomfort felt after eating.

Failure to thrive

o Marked malnutrition is apparent as a result of subsequent feeding
difficulties and inability to ingest adequate fluids. In essence, proper
nourishment of the body is compromised in which growth delay and
weight loss can be noted.

Weakness, fatigue and lethargy
o In severe cases, a patient with Hirschsprungs disease may appear floppy
due to the decrease glucose intake by the cells leading to decrease
energy production. It can also be a resultant of low blood count level
(RBC) brought by undernourishment.

Loss of subcutaneous tissues
o Along with failure to thrive muscle wasting will take place secondary to
impaired intestinal motility and its effects on nutrition and intake.

Complications (Book-based):

Perforation
o Perforation of the intestines happens as a comsequence of inability of the
body to evacuate stools leading to fecal impaction. The impacted stool
will be a good medium for bacterial growth and invasion. If not diagnosed
promptly, the bacteria may cause mucosal lining irritation leading to
bleeding, perforation and shock.

Enterocolitis
o Enterocolitis is a clinical codition whith symptoms including diarrhea,
abdominal distention, pyrexia, colicky pain, lethargy and passage of blood
stained stool. The incidence of enterocolitis in Hirschsprungs disease ranges
from 20% to 58%. Fortunately, mortality has decreased from 6.5% to 0.7%.

Decreased mortality is related to early detection and prompt treatment
through rectal decompression and antibiotic therapy.


C. PATHOPHYSIOLOGY
b) BOOK-BASED (SCHEMATIC DIAGRAM)

Modifiable Factors

Non-modifiable factors
Age (6 months)
Gender (Male)
Race (Asian)
Failure of ganglion cells to migrate
craniocaudally via neural crest during gestation
Absence of Ganglion cells in the myenteric
plexuses of the large intestines
Depressed parasympathetic innervation
Inadequate expression of PNS stimulation
(Increased acetylcholinesterase and decreased
Nitrous Oxide synthase
Ineffective peristaltic movement
Obstruction
Failure to pass meconium with 48 hours
following birth
Constipation

dilated with feces

Decreased
colonic water
re-absorption
Increased
water in stool
Protuberant abdomen
Date:
Abd Girth:
Bacterial
overgrowth
Increased
WBC
Date:
Passage of
watery stool
(overflow
diarrhea)
Bile stained
vomiting/
fecal vomiting
Abdominal Pain
Lack of interest in
feeding
Irritability
Dehydration
Electrolyte
Imbalance
Excessive crying
Mutation of RET Proto-oncogene
Hypokalemia
Fever

Poor nutrition
Decline in
weight
Growth delay
Easy
fatigability,
lethargy
Failure to thrive
Anemia
Wasted
extremities,
loss of
subcutaneous
tissues



ii. SYNTHESIS OF THE DISEASE
2. DEFINITION OF THE DISEASE

Hirschsprungs disease, a congenital disorder also known as aganglionic
megacolon, is characterized by absence or marked reduction of
parasymphatetic ganglionic innervation to the muscle of a section of the bowel
in most instances, the lower portion of the sigmoid colon just above the anus which
is termed as rectosigmoid (Dasgupta & Langer, 2008). During normal fetal
development, cells from the neural crest migrate craniocaudally from the foregut,
midgut and lastly into the hindgut of the large intestine (colon) to form the
networks of nerves called Auerbach's plexus and Meissner's plexus. In
Hirschsprung's disease, the migration is not complete and part of the colon lacks
these nerve bodies that regulate the activity of the colon. The absence of these
nerve cells means there are no peristaltic waves in this section to move fecal
material through the segment of intestine. This results in chronic constipation or
ribbonlike stools (stools passing through such a small, narrow segment look like
ribbons). The portion of the bowel proximal to the obstruction dilates, distending
the abdomen.

B. PREDISPOSING AND PRECIPITATING FACTORS WITH RATIONALE

Non-modifiable Factors:
Age
o The patients age predisposed him to develop Hirschrprungs disease. Higher
percentage of infants are diagnosed before reaching 1 year old. The
patient was four months old when he started to manifest abdominal
distention and constipation.


Gender
o Males are more prone to develop the disease which is true in the case of our
patient.

Race
o Higher chances of developing Hirschprungs were attributed to the Asian
race. Since our patient is an Asian, he is prone to acquire the disease.

Signs and Symptoms with rationale (Client-based):

Constipation
o The client has been experiencing extreme constipation at the age of four
months.

Abdominal distention
o Abdominal swelling was prominent in our patient due to retention of gas,
intestinal contents and bowel obstruction. The abdominal girth continually
increase from ___ since admission and _____cm as of DATE. Fecal masses
are also easily palpated from the retention of stool.

Vomiting
o The patient experienced bilious vomiting on the third day after admission
through the NGT draining into a bedside bottle.

diarrhea
o On the second week following confinement, the patient was observed with
increased passage of loose watery stool in which foul smell was noted.

Electrolyte imbalances

o The NPO diet of the patient and diarrhea precipitated the occurrence of
hypokalemia. This was evident on the serum test result of Potassium below
the normal value.

Abdominal Pain
o The patient appeared very irritable and cries even with minimal stimulation.

Increased white blood cells
o Laboratory results of the patient showed increased in WBC.

FEVER
o The patient experienced fever on June _____ with a temperature of _____

Failure to thrive
o The patients weight has decreased dramatically from ____________.

Loss of subcutaneous tissues
o Along with failure to thrive muscle wasting will take place secondary to
impaired intestinal motility and its effects on nutrition and intake.

Weakness, fatigue and lethargy
o Decreased food intake of the patient lead to undernourishment and
subsequently low blood count level (RBC) as evidenced by Hemoglobin
level of _______. Physiologically the patient was noted with body weakness,
decrease sensorium and easy fatigability.

VI. PATIENT AND HIS CARE
A. MEDICAL MANAGEMENT

A. Intravenous Fluids
Medical
Management

General Description

Indications

DATE

Clients
Response

D5 0.3 NaCl
500cc x 8
62-63
ugtts/min.

*KCl x 7
67 ugtts/min x
2 doses

D5 0.3 NaCl
500cc x 7
50 ugtts/hr.

D5 0.3 NaCl
29 ugtts/min.

PLRS 1L x 8
125 ugtts/min.

D5 0.3NaCl is a
hypertonic solution
owing to the higher
than normal amount
of Na and Cl ions. It
pulls fluid and
electrolytes from the
intracellular and
interstitial
compartments in to
the intravascular
compartments.

Plain Lactated
Ringers is a sterile
solution that is used
to replace fluid lost
by the body. It is
commonly used for
fluid resuscitation,
meaning that the
patient needs
aggressive fluid
replacement for
their injury or illness.

To
compensate
cellular
dehydration
and corrects
moderate fluid
loss, prevents
alkalosis and
provides
calorie and
NaCl.

Plain Lactated
Ringers, USP is
indicated as a
source of
water and
electrolytes or
as an
alkalinizing
agent.

#1
06/24/14

#3
06/26/14

#4
06/28/14

#5
06/29/14

#6
06/30/14

#2
06/24/14

The client
maintained
a normal
hydration
status as
evidence
by good
skin turgor
and moist
skin when
assessed by
the student
nurses.

Client
manifested
no adverse
reactions to
the
treatment,
but it
helped by
rehydrating
the body
and
providing
electrolytes.

D5IMB 500cc x
17-18
ugtts/min

Lactated Ringers
looks like water, but
it contains additives
including calcium,
potassium, lactate,
sodium and
chloride.

D5imb is a solution
that consists of 5%
dextrose and water
level. It is usually
given to patients
that could
potentially become
ill through high
sodium levels or low
blood sugar levels.

At 5% there will be 5
grams of dextrose
within each 100ml
measurement of the
solution. The
dextrose delivers
fluid to the body
and it contains
around 170 calories
per liter. Due to the
fact that the
dextrose solution is
essentially made up

The D5imb
solution can
be used for
both adult and
child patients.
The indications
for usage
would be
when the
patient needs
an increase in
calories,
hydration and
electrolytes in
the body.

#7
07/01/14

Client
manifested
no adverse
reactions to
the
treatment,
but it
helped by
rehydrating
the body
and
providing
electrolytes
and
calories.


NURSING RESPONSIBILITIES:
Before the Procedure
Check the doctors order regarding to what type of IVF to be used and also
its volume and rate.
Explain the procedure to the patient.
Gather all materials needed for the insertion of IVF to save time and not to
waste time for looking for other materials.
Wash hands before and after the procedure to prevent contamination from
insertion site.

During the Procedure
Place patient in a comfortable position to facilitate easy insertion of IV line
and to decrease patients fear about the procedure.
Make sure that we give the proper IV fluid and drop rate accurately
because patient may experience fluid overload or dehydration.
Check for its patency by observing the backflow of blood upon insertion.
After the Procedure
Press the site where the needle was inserted and secure it with micropore.
Check the site of hand where the needle is inserted if bulging is not visible. If
so, reinsertion is to be undertaken.
of basic sugar, it is
metabolized
extremely quickly by
the body. This leaves
behind pure water
that can travel
through the
membranes in the
body at ease.


Advice patient to avoid scratching the site less movement of the hand
where the needle was inserted to keep it in place.
Instruct patient and significant others to inform the nurse on duty if bulging of
the site is visible, if there is back flow of blood of if IVF is not infusing well.
Observe the IV site at least every hour for signs of infiltration or other
complications fluid or electrolyte overload and air embolism.
IVF regulation should be checked and monitored upon receiving patient.
Always check the doctors order for new orders regarding the IVF
supplement of the patient.
Always check if the IVF is infusing well and intact.

B. Nasogastric Tube Feeding
Type of
Procedure
Description Indication Contraindication Nursing
Responsibilities

Nasogastric
tube (NGT)
Nasogastric
(NG)
intubation is a
procedure
during which a
thin, plastic
tube is inserted

Aspiration
of gastric
fluid
content.

Absolute
contraindications
Severe midface
trauma
Recent nasal
surgery
Before
Check for
medical
order
Determine
right patient

through the
nostril, down
the
esophagus,
and into the
stomach.
Once an NG
tube is in
place,
healthcare
providers can
deliver food
and medicine
directly to the
stomach or
remove
substances
from it.

NG tube may
be used to
remove
contents of
the stomach
for testing or
analysis. The
contents of
the stomach
can tell
doctors how
well the
gastrointestinal
tract is
functioning.
An NG tube
can also be
used to
remove an
intestinal
obstruction or
blockage that
may be
causing pain,
discomfort,
and swelling.

Relative
contraindications
Coagulation
abnormality
Esophageal
varices or stricture
Recent banding
or cautery of
esophageal
varices
Alkaline ingestion
Gain consent
Explain the
procedure
and reasons
for the need
of tube
insertion
Explain
possible
effects

During
Maintain
aseptic
technique
Insert the
nasogastric
tube and
confirm it is
correctly
sited

After
Report
residual and
record as
appropriate.


C. Rectal Lavage
Type of
Procedure
Description Indication Contraindication Nursing
Responsibilities

Rectal
Lavage

Rectal
Bowel
washouts
are
performed
to
decompress
the bowel
and deflate
the
abdomen
by
removing
gas and
stool using
small
amounts of
Normal
Saline 0.9%.

Performed in
babies with
Hirschsprung's
Disease (HD)
or to relieve
low intestinal
obstruction
due to
meconium
plug,
meconium
ileus or
intestinal
dysmotility of
prematurity.

Used as a
mode of
temporary
management
in proven
cases of HD till
definitive
surgery. (This
may be for 4-
12 weeks
depending on
each case)

Rectal biopsies
taken in
preceding 24 hr
Rectal
bleeding
(relative
contraindication)
Severe anal
stenosis
Anus not
clearly identified
Known surgical
patient (without
discussion with
surgical team)

Before
Check for
medical
order
Determine
right patient
Gain consent
Explain the
procedure
and the
need for
rectal
washout
Explain
possible
effects

During
Maintain
aseptic
technique
Do not use
excessive
force if
resistance is
felt.
After
Report and
record as
appropriate.


B. PHARMACOLOGICAL MANAGEMENT

Generic Name
(brand name)

Mechanism
of Action

Date
Ordered/A
dministere
d

Indications

Contraindi
cations

Clients
Response
to
Treatment

NURSING RESPONSIBILITIES

PARACETAM
OL
(Acetaminop
hen)

47 mg IV q4
rtc

ANTIPYRETIC,
ANALGESICS
(NON-
OPIOID)

Decreases
fever by a
hypothalami
c effect
leading to
sweating
and
vasodilation.
Inhibits
pyrogen
effect on
the
hypothalami
c-heat-
regulating

06/24/14

For relieving
fever

.

Hypersens
itivity to
paraceta
mol

The patient
did not
manifest
any
allergic
reaction to
Paracetam
ol. The
fever
subsided
from
(TEMP.)
degrees
Celsius
Before the administration of drug
Check for medical order
Determine if patient is allergic to
the drug
Explain the procedure and
reasons for giving the drug, to
gain patient cooperation
Explain possible side effects

During drug administration
Maintain aseptic technique
Check medication, right route,
dosage, storage, etc
Do not exceed the
recommended dosage


centers
Inhibits CNS
prostaglandi
n synthesis.

After the administration of drug
Monitor any untoward effects of
the drug
Instruct SOs to report to the
attending nurse if any unusual
effects occur
Provide comfort for the patient.
Report and record as
appropriate.


Generic Name
(brand name)

Mechanism
of Action

Date
Ordered/Ad
ministered

Indications

Contraindications

Clients
Response to
Treatment


RANITIDINE
HCL
(Zantac)
5mg IV q8

HISTAMINE-2
(H2)
ANTAGONIST

Competitive
ly inhibits the
action of
histamine at
the H2
receptors of
the parietal
cells of the
stomach,
inhibiting
basal gastric
acid
secretion
that is
stimulated
by food,
insulin,
histamine,

06/24/14

Prevention
for gastric
ulceration
since
patient is
placed
under NPO
status.

Hypersensitivity to
ranitidine

The patient
did not
manifest
any allergic
reaction to
Ranitidine.
No sign of
ulceration
was
reported
until
changed
from NPO
status.
Before the administration
of drug
Check for medical
order
Determine if patient is
allergic to the drug
Explain the procedure
and reasons for giving
the drug
Explain possible side
effects

Maintain aseptic
technique
Check medication,
right route, dosage,
storage, etc
Stay with the patient

cholinergic
agonists,
gastrin and
pentagastrin
.
while she takes in the
drug
Do not exceed the
recommended
dosage

After the administration of
drug
Monitor any untoward
effects of the drug
Instruct SOs to report
to the attending nurse
if any unusual effects
occur.
appropriate.


Generic Name
(brand name)

Mechanism
of Action

Date
Ordered/A
dministere
d

Indications

Contraindications

Clients
Response to
Treatment


CEFOXITIN
(Mefoxin)

78.33mg IV
q8

ANTIBIOTIC,
CEPHALOSP
ORIN

Bactericidal;
Inhibits
synthesis of
bacterial
cell wall
causing
death.

06/24/14

Treatment
for possible
bacterial
growth
caused by
bowel
obstruction
and
retention of
intestinal
contents.

Contraindicated in
patients who have
shown
hypersensitivity to
Cefoxitin and the
cephalosporin
group of antibiotics.
.
The patient
did not
manifest
any allergic
reaction to
Cefoxitin
and
responded
well to drug;
decreased
WBC from
(INITIAL LAB)
to (CURRENT
LAB).
of drug
Check for medical
order
the drug, to gain
patient cooperation
effects

Maintain aseptic
technique
Check medication,
right route, dosage,
storage, etc

Stay with the patient
while she takes in the
drug
Do not exceed the
recommended
dosage

drug
Monitor any untoward
effects of the drug
occur.
appropriate.


Generic Name
(brand name)

Mechanism
of Action

Date
Ordered/A
dministere
d

Indications

Contraindications

Clients
Response to
Treatment


METRONIDAZ
OLE
(Flagyl IV)

35 mg IV q6

AMEBECIDE,
ANTIBACTERI
AL,
ANTIBIOTIC,
ANTIPROTOZ
OAL

Disrupts DNA
and protein
synthesis in
susceptible
organisms.
Metronidazo
le is used to
treat serious
bacterial
infections in
different
areas of the
body. It is
also used to
prevent
infections in

06/27/14

Use for
patients with
anaerobic/
bacterial
infections.

Hypersensitivity to
Metronidazole

The patient
did not
manifest
any allergic
reaction to
Metronidazo
le and
responded
well to drug;
(INITIAL LAB)
to (CURRENT
LAB).

of drug
Check for medical
order
the drug
effects

Maintain aseptic
technique
Do not exceed the
recommended
dosage

drug

the
intestines
before and
after surgery
for some
patients.

occur.
appropriate.


C. DIET
Type of
Activity
General
Description
Indications/
Purpose
Date
Ordered/Perf
ormed
Clients Response
NPO

Type of diet
wherein the
client is not
allowed to eat
anything for a
certain period of
time, either fluids
or foods. Medical
instruction
meaning, to
withhold oral
food and fluids
from a patient for
various reasons.

Since the
patient has just
been admitted
and is to be
subjected to a
series of
observations. It
is necessary
that NPO order
be carried out
to assure
accuracy of
findings of tests.
Typical reasons
for NPO
instructions are
the prevention
of aspiration
pneumonia,
e.g. in those
who will
undergo
general
anesthetic, or
those with
weak
swallowing
musculature, or
in case of
gastrointestinal
bleeding or
gastrointestinal
06/24/14

The patient was
able to comply
with the diet, no
gastric irritation
noted.


NPO
NURSING RESPONSIBILITIES BEFORE, DURING, AND AFTER

Breast
Feeding with
SAP

Aspiration
precautions are
measures taken
to prevent a
person from
aspirating, or
choking. Choking
can occur in
debilitated
patients who
have a
diminished
swallowing reflex,
and the
condition can
even cause a
type of
pneumonia
called aspiration
pneumonia.
blockage.

Breastfeeding is
important until
2 years of
ageand that
food
supplement
can be given
only after 6
months of
breastfeeding.
SAP is
indicated to
patients with
diminishedor
weak
swallowing
reflex.

06/28/14

The patient did
not experience
aspiration and
was fed well.

Check the doctors order.
Check the right client.
Be sure that the diet is properly instructed.
Explain the reason for type of diet
Monitor if the client complies with the given diet.
Be sure patient is nottaking any food or liquid.
After the Procedure
Assess for patients condition; how he responded to the diet.

BREASTFEEDING WITH STRICT ASPIRATION PRECAUTION
NURSING RESPONSIBILITIES BEFORE, DURING, AND AFTER

Check the doctors order.
Check the right client.
Be sure that the diet is properly instructed.
Explain the reason for type of diet
For SAP instructions:
Positioning the person in an upright position or elevated
head while feeding
Positioning the person to maintain an upright position for
one hour after eating
Providing instruction and direction to staff to immediately report to
nursing staff all
changes to the persons baseline especially :
Increased coughing

Increased secretions or changes to secretions (color, texture)
Change in respiratory status
Fever
Instruct SO the need for prescribed diet.

Encourage the patient to follow the diet program.
Inform other caretakers including SO that the patient is under the diet
therapy.
Emphasize strict compliance on the diet regimen.
Make sure that the client complies with the given order.

After the Procedure
Assess for patients condition; how he responded to the diet



VII. NURSING CARE PLANS

NURSING PROBLEM: Ineffective tissue perfusion 2 to Diabetes Mellitus Type 2.
CUES
NURSING
DIAGNOSIS
SCIENTIFIC
EXPLANATION
OBJECTIVES
NURSING
INTERVENTIONS
RATIONALE

EVALUATION

S >
O> The patient
manifested
Pallor
Presence of
gangrenous
foot
Dry skin
Body
Weakness
Decreased
sensations
Increased
respiratory
rate (25cpm)
Decreased
amount of
Ineffective
tissue
perfusion
2 to
Diabetes
Mellitus Type
2.
Diabetes
Mellitus Type 2
causes an
abnormally
Increased
blood glucose
level. Chronic
elevations
lead to
increased
blood viscosity
that causes
poor circulation
because the
more viscous
blood is, the
slower is its
movement. DM
Short Term:
After 2 of
nursing
interventions,
the patient
will be
able to
identify and
demonstrate
ways to
improve
Circulation.

Long term:
After 1-2
weeks of
nursing
intervention,

1. . Take and
record vital
signs.

2. Assess pt.s
condition

3. Instruct patient
to increase
intake of
vitamin C like
orange juice,

1. To obtain
baseline
data.

2. To be able
to prepare
for
appropriat
e
interventio
ns.

3. to boost
immune
system

Short Term: The
patient shall
have verbalized
understanding
of condition
and
therapeutic
regimen.

Long Term: The
patient shall
have
demonstrated
increased
perfusion as
effectively
available.

Hct: 0.36%
Hgb: 118g/L
Delayed
wound
healing
fatigue

also affects the
kidneys
particularly its
renin-
angiotensin
system.
Aldosterone is
being
produced
which causes
fluid retention
and eventually
hypertension.
Due to impaired
circulation, less
oxygen is being
delivered to the
different
systems
especially to
the periphery.
Oxygen delivery
is also
the patient
will
demonstrate
Increased
perfusion AEB
absence of
pallor
dyspnea, and
VS within
Normal
range.
calamansi juice

4. Emphasize the
recommended
diet

5. Promote good
hand washing
by staff and
patient.

6. Position
properly.

4. to prevent
further
complicati
ons of the
disease
condition

5. Reduces
risk of cross
contamina
tion

6. This
promotes
optimal
lung
ventilation
and

specifically
decreased in
the capillary
level. A
decreased in
oxygen supply
also causes
weakness and
difficulty of
breathing.

perfusion.
The patient
will
experience
optimal
lung
expansion
in upright
position.


NURSING PROBLEM: Activity Intolerance r/t muscle weakness as evidenced by verbal reports of weakness
CUES
NURSING
DIAGNOSIS
SCIENTIFIC
EXPLANATION
OBJECTIVES
NURSING
INTERVENTIONS
RATIONALE

EVALUATION

Subjective:
Masaki tang
mga
kasukasuhan
ko,
nahihirapan
ako
gumalaw as
verbalized by
patient.

Objective:
body
weakness
restlessnes
s
increased
RR
(25cpm)
Activity
Intolerance
r/t muscle
weakness as
evidenced
by verbal
reports of
weakness
Activity
Intolerance is
insufficient
physiological or
psychological
energy, poor
endure or
complete
required or
desired daily
activities.
Because of low
hct level there
will be decrease
oxygen being
delivered to the
tissues of the
body since the
hgb is responsible
for the
After 2-3
hours
of nursing
interventions
and giving
health
teachings,
the patient
will be able
to :
Follow
energy
conservati
on
technique
s to lessen
fatigue
Perform
ADL as
tolerated.
Assess patients
level
of mobility.

Assess ability to
stand and
move about
and the
degree
of assistance
necessary.

This aids
defining
what pati
ent is
capable
of which is
necessary
before
setting
realistic
goals.

To
determine
current
status and
needs
associate
d
with partic
Goal met.
After 2-3 hours
of nursing
interventions the
patient was able
to perform
comfort measure
to minimize
energy
consumption like
refraining from
doing non
essential
procedures and
placing
frequently used
items within
reach.

low Hct:
0.36%
Hgb:
118g/L
fatigue
prefers to
lie down
on bed
oxygenation of
tissue. As a
compensatory
mechanism, the
body will
increase its
demand of
oxygen by
increasing
respiratory rate of
the patient
which results then
to fatigue.
Because of this
there will be fast
consumption of
ATP leading to
weaker
contractions thus
causing muscle
weakness and if
the patient has
muscle weakness

Provided
adequate rest
periods,
especially
before meals,
other ADLs,
and
ambulation.

Instruct patient
to eat nutritious
foods and
drink adequate
fluid intake.

Teach comfort
ipation in
needs
or desired
activities.

Rest
between
activities p
rovides
time
energy
conservati
on and
recovery.

Promotes
well-being
and
maximizes
energy
production.

This

there will be
activity
intolerance.
measure to
conserve
energy by: 1.)
Changing
position
frequently; 2.)
Placing
frequently used
items within
reach; 3)
Bedside
commode

Instruct patient
to promote /
have
ambulation
and reposition
as necessary.

distributes
work to
the
different
muscles to
avoid
fatigue

To prevent
skin
breakdow
n and
maximizes
energy
productio
n.
NURSING PROBLEM: Fluid volume deficit r/t osmotic pressure AEB secondary to increase blood glucose levels

ASSESSMENT
NURSING
DIAGNOSIS
SCIENTIFIC
EXPLANATION
PLANNING INTERVENTION RATIONALE EVALUATION
Subjective:
Parang
nanunuyo lagi
ang lalamunan
ko, as
verbalized by the
patient

Objectives:

o increased
urine output
(1200ml for 6
hours)
o sweating of
the skin
o thirst sensation
o exhaustion
o dry skin
o poor skin
turgor
o hyperglycemia
RBS: 19.92
mmol/L

Fluid volume
deficit r/t
osmotic
pressure AEB
secondary
to increase
blood
glucose
levels.
Glucose
appears in the
urine
(glycosuria)
because the
kidney excretes
the excess
glucose to
make the blood
glucose level
normal. Glucose
excreted in the
urine acts as
osmotic diuretic
and causes
excretion of
increased
amount of
water, resulting
in fluid volume
deficit
Short term:
After 4 hours
of nursing
intervention,
Patient will
demonstrate
proper
hydration
through good
skin turgor
and moist,
pink mucous
membranes.

1. Take and
record vital
signs.

2. Assess skin
turgor and
mucous
membranes
for signs of
dehydration.

3. Measure
and record
urine output
hourly; report
urine output
less than
30ml for 2
consecutive
hours.

1. To obtain
baseline
data.

2. Dry skin
and
mucous
membran
es are
signs of
dehydrati
on.

3. Fluid
volume
deficit
reduces
glomerular
filtration
and renal
blood flow
causing
oliguria. T
he patient
in DM may
also be
undergoin
g osmotic
diuresis
and have
o The patient
achieved
and
demonstrate
d evidences
of adequate
hydration
such as good
skin turgor,
moist lips,
and moisture
on skin.

or polyuria.

4. Assess for
signs of
hypokalemia
and
hyponatremi
a

5. Encourage
the patient
to increase
fluid intake

6. Administer
IVF as
ordered by
the Doctor
with
adequate
regulation

excessive
outputs.

4. Osmotic
diuresis
causes
increased
excretion
of
potassium.

5. To replace
fluid loss
and
prevent
dehydrati
on

6. To replace
electrolyte
s and fluid
loss

NURSING PROBLEM: Hype

ASSESSMENT
NURSING
DIAGNOSIS
SCIENTIFIC
EXPLANATION
Subjective:
Paano ba ung
diabetes?
Normal naman
ung sugar ko, di
naman
mataas, as
verbalized by
the patient
Objective:
> unfamiliarity
to disease
condition
>misconception
about real
disease
> inaccurate
follow through
of instruction

> incompliance
to the
treatment
regimen
Deficient
Knowledge
r/t
unfamiliarity
with disease
condition as
evidenced
by verbal
reports of
request of
information
Deficient
Knowledge is
the absence or
deficiency of
cognitive
information
necessary for
the client/SOs to
make informed
choices
regarding the
condition,
treatment or
lifestyle
changes.
Short Term:

After 2 hours of
nursing
interventions,
the pt. will be
able to Exhibit
increased
interest/assume
responsibility
for own
learning and
begin to look
for information
and ask
question.

Long Term:

After 2 days of
Nursing
Interventions,
the pt. will be
able to initiate
necessary
lifestyle
changes and
participate in
treatment
regimen.

Monitor,
Assess and
Record the
Vital Signs of
the patient.

Ascertain
level of
knowledge,
including
anticipatory
needs.

Determine
clients
ability to
learn

To
become
aware of
any
abnormaliti
es and
irregularitie
s of the
patients
vital signs

To know
what is the
level of
understan
ding of the
person to
know what
information
should be
reinforced.

Right
timing is
important
in giving
information
, knowing
the clients
ability
gives the
nurse idea
Short Term:
The pt. shall have
exhibited
increased
interest/assume
responsibility for
own learning and
begin to look for
information and
ask question.

Long Term:

The pt. shall have
initiated
necessary lifestyle
changes and
participate in
treatment
regimen.

o

>poorly
controlled
diabetes
mellitus

Provide
general
information
about
diabetes
mellitus such
as the cause,
signs and
symptoms
and
prognosis

Determine
patients
most urgent
need

on what
way will
he/she
present the
information
.

To let the
client know
about the
present
situation.

Knowing to
prioritize
the
patients
learning
needs
increases
the
effectivity
of the
teaching

Recognize level
of
achievement,
time factors,
and short term
and long term
goals
plan

To know
what are
the
purpose of
the patient
teaching


NURSING PROBLEM: Risk for spread infection
ASSESSMENT
NURSING
DIAGNOSIS
SCIENTIFIC
EXPLANATION
Subjective:
Matagal na
yung sugat ko
hindi
gumagaling,
as verbalized
by the patient

Objectives:
presence of
diabetic
ulcer on
the first and
third digits
of the right
foot
Erythema
on wound

Risk for
spread
infection
Risks for infection is
an increased
probability of
invasion
of pathogenic
organisms for a pt.
with DM wound
is possible in the
future. Clients with
diabetes are
susceptible to
infections because
of
polymorphonuclear
leukocyte function,
diabetic
neuropathies, and
vascular
insufficiency as a
result is a
Short term:
After 4 hours
of nursing
interventions,
the risk
factors of
occurrence
of infection
will be
reduced or
control to a
manageable
level by a
clean bed
and maintain
skin intact.

1. Take and
record vital
signs.

2. Promote good
hand washing
by staff and
patient.

3. Encourage
patient to
increase fluid
intake.

4. Advise to
increase Vitamin
C in the diet and
increase CHON
intake if not
1. To obtain
baseline data.

2. Reduces risk
of cross
contamination.

3. To replace
fluid loss and
prevent
dehydration.

4. To boost
immune
system and
promote
collagen
formation for
tissue repair.
Goal Met
o After 4 hours
of nursing
intervention
s, the
patient was
able to
demonstrat
e ways on
how to
reduce
occurrence
of infection
such as
hand
washing
and
keeping the
dressing
clean and
dry..

poor glycemic
control; thus
making a wound to
heal slowly
because the
damaged of the
vascular system
cannot carry
sufficient oxygen,
WBC, nutrients, and
antibodies to the
injured site. Thereby
infections increase
and enhance
possibility of further
complications.
contraindicated.

5. Maintain the
dressing clean
and dry.

6. Take due
medications on
time such as
clindamycin

5. To promote
healing and
prevent
contamination
on the wound.

6. Clindamycin
acts as an
antibacterial
therefore
preventing
infection of
wound

VIII. CONCLUSION

The success of any diabetic regimen depends upon the patients willingness
to adhere to the care plan given. Essentially, learning to live with diabetes is like
any other form of learning. Patients must obtain a grasp of unfamiliar factual
material (nature of the disease), learn to perform certain procedures (insulin
injection) and permanently change certain behavior patterns (eating habits,
lifestyle, recreational activities). Like a student, patient needs scheduled classes,
planned instruction, reading materials which are geared to educational level,
demonstrations of procedures and the opportunity to perform these procedures
with supervision.
Learning is a continuing process and patients are given with the most basic
facts regarding diabetes. As student nurses, it is recommended to encourage
patients to continuously read and learn about their disorder and to keep abreast
of new developments in the field. Knowledge and confidence go hand in hand.
The more the patient knows about diabetes, the easier it will be for them to
accept the condition, control the disorder and live a normal productive life.
As for us student nurses, we are tasked to learn the different interventions
that should be given in a client who has diabetes mellitus in order for us to provide
our clients with the necessary care that they need.
Studies about different treatment modalities with regards with this disease do
not cease as members of the medical and research fields exert effort to always

find ways to alleviate the modes of living of the people. But this has always been a
challenge. We may be too young to do such sensitive researches, yet it does not
follow that we are excused of the responsibility. We need to provide intensive and
good nursing care for our clients. They may be very difficult to handle but let us not
deprive them of the care, respect and compassion that they all deserve.

IX. RECOMMENDATION

To other student nurses, that they may learn more what Diabetes Mellitus is,
its signs and symptoms, risk factors, laboratory and diagnostic procedures that can
be done to detect this disease, its origin and how it can be acquired and the
nursing management that we have to consider regarding this disease condition.

To the DOH, that they may conduct seminars and other programs in order
for the public to be aware of what Diabetes Mellitus is and its cause and effect,
and on how to reduce the risk of acquiring it.
To the government, that they may provide adequate financial budget for
health and allotting free or low priced medicines for the treatment of this disease
and to give people proper information regarding Diabetes Mellitus.
To the different members of the Health Care Team, that they may perform
adequate skills in taking care of the patients afflicted with this illness and that they
may know the nursing considerations and managements prior, during and after
conducting procedures that the patients will undergo and to prevent further
complications.
To the patients afflicted with this illness and to the public, that they may
understand the factors that will predispose them to acquire such illness, the reason
for performing such diagnostic procedures to determine the progress of their
condition, the importance of medications that they are taking, the ways on how
to prevent this illness and that they may demonstrate techniques and procedures
on how to promote maximum health and participate in the process of preventing
complications in their health.

X. BIBLIOGRAPHY

Black, J.M. & Hawks, J.H. (2009). Medical-Surgical Nursing: Clinical Management for
Positive Outcomes (8th ed.). Philadelphia: Elsevier/Saunders.

Goldberg E L (Johns Hopkins University, School of Hygiene and Public Health, 615 North
Wolfe Street, Baltimore, Maryland 21205, USA). An epidemiological study of
Hirschsprungs Disease. International Journal of Epidemiology 1984, 13: 479485.


Chakravarti, A. Endothelin receptor-mediated signaling in Hirschsprung disease. Hum.
Molec. Genet. 5: 303-307, 1996. [PubMed: 8852653, related citations] [Full Text: HighWire
Press]

Romeo G,Ronchetto P, Luo Y, Barone V, Seri M, Ceccherini I, Pasini B, Bocciardi R,
Lerone M, Kriinen H & Martucciello G (1994). "Point mutations affecting the tyrosine
kinase domain of the RET proto-oncogene in Hirschsprung's disease". Nature 367 (6461):
377378.doi:10.1038/367377a0. PMID 8114938.

Washington University in St. Louis. (2014). Electronic references. Retrieved from
http://research.peds.wustl.edu/labs/heuckeroth_robert_o/Home/Hirschsprungdisease/tabid
/961/Default.aspx

Dasgupta R, Langer JC. Evaluation and management of persistent problems after surgery
for Hirschsprung disease in a child. J Pediatr Gastroenterol Nutr. 2008;46(1):13-19.

Puri P., Montedonico S. (2008) 'Pathophysiology of Hirschsprung's Disease' In:
Hirschsprungs Disease and Allied Disorders, 3rd Edition. Germany: Springer. Retrieved
from: http://link.springer.com/chapter/10.1007/978-3-540-33935-9_8.

Bergeron KF, Silversides DW, Pilon N. The developmental genetics of Hirschsprung's
disease. Clin Genet. 2013 Jan;83(1):15-22. doi: 10.1111/cge.12032. Epub 2012 Nov 7.
PubMed PMID: 23043324.

F. Lantieri, P. Griseri, J. Amiel, G. Martucciello, I. Ceccherini, G. Romeo & S. Lyonnet.
(2008). Hirschsprungs Disease and Allied Disorders, 3rd Edition. Germany: Springer.
Retrieved from: http://link.springer.com/chapter/10.1007/978-3-540-33935-9_5.

http://www.thirdage.com/hc/c/hirschsprungs-disease-photos

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