TRINITY UNIVERSITY OF ASIA

St. Luke’s College of Nursing

IN PARTIAL FULFILLMENT OF THE REQUIREMENTS

In RELATED LEARNING EXPERIENCE

A Grand Case Study of

PEUTZ-JEGHERS SYNDROME

SUBMITTED BY:

4NU07
Group 3
MASCAREÑAS, Mary Denn
MATIGNAS, Kathrine Elaine
MATIONG, Jose Ramon
MENDERO, Mariel

Group 4
MENDEVIL, Irene
MENDOZA, Anne Carmina
MENDOZA, Audrey
MENDOZA, Kim Moana
MENDOZA, Lizette
 OBJECTIVES
Part I

General Objective
The study about Peutz-Jeghers Syndrome was made to imply knowledge and information for
health care professionals and other people about the said disease. The study was also made ito
prevent or reduce its the occurrence of its possible complications by means of health awareness.
Lastly, it was made to allocate proper treatment during occurrence of PJS to prevent further
progression to other diseases.

Specific Objectives

Knowledge
1. To improve the awareness, diagnosis, and treatment of health care professionals in patients
with Peutz-Jeghers Syndrome;
2. To determine the pathophysiology of Peutz-Jeghers Syndrome; and
3. To develop and implement programs to promote efforts to reduce the occurrence of the
complications of the disease.

Skills
1. To identify proper management for the manifestation of Peutz-Jeghers Syndrome.
2. To verify Peutz-Jeghers Syndrome by analyzing findings from the laboratory findings and
diagnostic procedures, and appropriate nursing management for different diseases; and
3. To provide further support to patients with Peutz-Jeghers Syndrome and to their relatives
by therapeutic communications.

Attitude
1. To strengthen collaboration within health care professionals in obtaining comprehensive
comparable information to manage Peutz-Jeghers Syndrome;
2. To help health care professionals impose discipline and lifestyle modifications to their
patients with Peutz-Jeghers Syndrome; and
3. To develop and promote effective clinical and professional practices.
 INTRODUCTION
Part II

Background of the Case

Ms . G.R., a 21 years old female, admitted to St. Luke’s Medical Center, June 21, 2010. She was
admitted to the said hospital with the chief complaints of difficulty of breathing, abdominal pain
and vomiting. This was later on diagnosed as Peutz-Jegher’s Syndrome.

The group decided to choose this case due to it low incidence, thus it is rarely encountered
in the ward by student nurses and health care professionals. The group gathered up research about
Peutz-Jegher's Syndrome to bring knowledge about the signs and symptoms, diagnostic tests and
management of Peutz-Jegher's syndrome. This will serve a guiding tool in proper management of
the disease.

Definition of the Case

Peutz-Jeghers syndrome is an autosomal dominant inherited disorder. It is characterized by
intestinal hamartomatous polyps in association with mucocutaneous melanocytic macule. It has a
high risk of developing cancer in the intestinal and extraintestinal sites. The polyps develop mainly
in the small intestine, but also in the colon. It will show the polyps through colonoscopy or barium
x-ray or a small camera that is swallowed and then take multiple pictures as it travels through the
small bowel (capsule endoscopy).

Peutz-Jeghers syndrome's gastrointestinal polyps are typically hamartous or scattered. It is

characterized by extensive smooth muscle arborization throughout the polyp in Histology. Since the
epithelial cells is usually from benign glands and surrounded by the smooth muscle it would give
the lesion the appearance of pseudoinvasion.

General Signs and Symptoms

General
 Brownish or bluish-gray pigmented spots on the lips, gums, inner lining of the mouth, and
skin
 Clubbed fingers or toes
 Cramping pain in the belly area
 Dark freckles on and around the lips of a newborn
 Blood in stool
 Vomiting

Signs and Tests

 Intussuseption (part of the intestine folded in on itself)
 Noncancerous tumors in the ear (exostoses)
 Complete bood count may show anemia
Etiology
There are two types of Peutz-Jeghers Syndrome, the familial and the sporadic. Familial is due to the
mutation of gene STK11. This genetic defect is passed down through families as an autosomal
dominant trait meaning if one of the parents has familial type of Peutz-Jeghers Syndrome, there will
be a 50:50 chance of inheriting the gene and having the disease. While Sporadic cannot be
inherited and appears unrelated to the STK11 gene mutation.

Incidence
International
The frequency of cases of Peutz-Jeghers syndrome is rare but quantitatively it is simillar with the
incidence of the United Staes. Its frequency is 1 case per 60,000 population. Also according to
National Institutes of Health, it was estimated that it affects about 1 in 25,000 to 300,000 births.
50% of patients diagnosed with Peutz-Jeghers syndrome (PJS) develop and die from cancer by age
57 years old and there is 93% risk for developing cancer at the age 0f 15-64 years old associated
with Peutz-Jeghers syndrome.

Sex and Age

The occurrence of Peutz-Jeghers syndrome in males and females is equally the same. It is common
to 23 years old in men and 26 years old in women. Pigmented lesions are present in the first years
of life and may fade at puberty, except for lesions on the buccal mucosa, making the diagnosis
possible in pediatric patients with a high level of suspicion
 PATIENT PROFILE
Part III

Demographic Data
Name: G. R.
Sex: Female
Age: 21
Birthday: August 19, 1988
Race: Filipino

Marital Status: Single

Occupation: None
Religious Affiliation: Roman Catholic

Health Care Financing/Source of Medical Care: N/A

Date and Time of Admission: June 21, 2010 at 7:25am

Mode of Admission: Wheelchair borne
Accompanied by: Mother (E.R.)

Chief Complaint
Difficulty of breathing
Abdominal pain
Vomiting

Physical Examination

Health History
None

General
Height: 5’5
Weight: 40.5 kg
BMI: 14.8 (gain 13.63kg to fall within the healthy weight range)

Vital Signs
Blood Pressure: 110/80
Temperature: 36.8⁰C
Pulse Rate: 103
Respiratory Rate: 21
Pain Rate: 10 (according to the patient)

Skin
Skin Integrity/Condition: Normal
Color: Pale
Temperature: Normal
Moisture: Normal
Head, Eyes, Ears, Nose, Throat
Vision: Both eyes 20/20, Normal PERRLA(Pupil Equally Rounded Reactive to Light
Accommodation
Hearing: Normal
Mouth: Mucocutaneous lesions
Lips: Mucocutaneous lesions
Teeth: Normal
Nasal Discharge: None
Headache/Head Injury: None

Cardiovascular
Neck Veins: Strong L/R
Apical Pulse: Strong L/R
Radial Pulse: Strong L/R
Brachial Pulse: Strong L/R
Pedal Pulse: Strong L/R
Edema: None
Numbness: Level 1 (10 being the highest)

Respiratory
Retraction: None
Cough: None
Stridor: None
Ability to Clear Secretion: N/A
Tracheostomy: None
Chest Tube: None
Oxygen: None

Gastrointestinal
Abdomen: Soft
Tenderness: Non-tender
Bowel Sounds: Normoactive
Constipation: Yes
Diarrhea: Yes
Appetite: Poor
Feed Independently: Yes
Nasogastric Tube: None

Neurological
Eyes Open: 4
Best Verbal Responses: 5
Best Motor Responses: 6
Total Glasgow Scale: 15
Pupils: Normal

Neuromuscular
Weakness: Back portion of the body
Deformities: None
Wasting: None
 Pressure Ulcer: None

Urogenital
Discharge: None
Soreness: None
Catheter: None
Color: Light yellow to yellow
Smell: comparable to the odor of a medicine

Allergies
No known allergy.

GORDON’S FUNCTIONAL HEALTH PATTERN

Organizing Data According to Gordon’s 11 Functional Health Patterns

Functional Health Pattern
Health Perception/ Health
Management
Nutritional-Metabolic
Elimination
Before Admission During Admission
The patient is compliant with Patient is compliant with her
her medications that she takes medications and cooperates
at home. She is aware of the with nursing interventions. She
nature of her illness and this remains in bed in most of her
causes her to limit her hospital stay.
activities of daily living.
Patient eats meat, vegetables Patient was not able to finish
and rice but only in little her meals but she still eats
amounts. She is used in eating small, frequent meals but she is
small, frequent meals. She does also receiving Total Parental
not prefer eating chocolates or Nutrition. She still does not
any chocolate-flavored drinks prefer chocolates or any
because the patient perceives chocolate-flavored drinks
that it gives her diarrhea. which is why she does not like
Fiber drink which is chocolate
flavored.
The bowel movement of the Since she was admitted, there
patient is alternating diarrhea was no recorded diarrhea but
and constipation. The the patient noted constipation.
appearance of the and the stool But she still has the same urine
is watery and brownish while output before she was
she urinates 4 to 6 times a day admitted.
and the color of her urine is
clear yellow.

Activity-Exercise
Cognitive-Perceptual
Sleep-Rest
Self-Perception/ Self Concept
Role-Relationship
Sexuality-Reproductive
Coping/Stress Tolerance
Patient does not exercise. Her Patient stayed in bed most of
activity is only limited to her hospitals stay. She mainly
house-hold chores that does slept while she was at the
not require her to exert too hospital.
much effort because she
perceives that if she over exerts
herself, she will be easily tired.
According to the patient, her The patient’s Cognitive-
senses appear to be normal. Perceptual remains the same
She states that she does not before hospitalization.
have any change in taste.
The patient states that she gets According to her, she had
adequate sleep, with 8 hours of enough rest and sleep since she
sleep per day. She normally is already asleep at 6pm and
sleeps at 8-9pm and wakes up she normally stays at bed.
at around 6-7am. She also get
adequate rest and stays at
home.
According to her, she is a very She does not have activities
shy person and does not mingle while she was at the hospital.
with other people, even to her She just stays at bed and sleep.
siblings. Her activities are
usually watching television and
drawing.
She stopped studying after she During her hospital stay, her
graduated from high school. parents are the one who
Then, she entered the convent watches over her.
but she left due to her illness.
She now stays at home with her
family.
She is not sexually active and During her hospitalization, she
she states that she had never did not had her menstruation.
had any boyfriend.
She had her menarche at the
age of 14 and she usually has
her menstruation for 4 to 7
days.
Before she was admitted, she Since she was admitted, she
copes with her stress by does not make any art works
drawing and doing different art and she does not draw
works. anymore. She just sleeps in the
hospital.
 She is a Roman Catholic. She
used to go to the church, reads
Value-Belief
the bible and prays every night
before going to sleep.
Due to her confinement, she
has not been able to go to
church to attend mass but she
still reads the bible and pray.

Past History
Medical: None
Surgical: 2004 – Ileocecal resection with an end-to-end anastomosies
Obstetric/Gynecologic: 2008 – Dilatation and Curettage due to heavy menses
Psychiatric: None
Last Medical Visit: June 9, 2010
Previous Diagnostic Examination: None

Present History

1 month prior to Presence of abdominal pain, crampy, non-radiating, VAS of 7/10,

consultation associated with nausea, postprandial vomiting, changes in bowel
movement claimed to have anorexia and weight loss, regurgitate
Still with aforementioned symptom consult with surgery, referred to
GI, given Omeprazole (Omepron) 20mg/tab
1 week prior to Increase in the severity of abdominal pain and increase in frequency of
consultation postprandial vomiting, presence of chest pain radiating to back, VAS
7/10, aggravated by deep respiration, alleviated by sitting up, as
radiation to left arm or jaw, no fever

Course in the ward

22 June 2010
 With IVF of #3 PNSS + 20meq KCL x 40cc on the left hand with a side drip of Kabiven x
40cc/hr and Nexium 40mg + 90cc PNSS x 20cc/hr
 Responsive
 Not in distress
 V/S taken and recorded
 Instructed NPO
 For Gastroduodenoscopy at 10:00am

23 June 2010
 With IVF of D5MB 1L x 60cc/hr and Kabiven x 40cc/hr on the left hand
 Responsive
 Not in distress
 V/S taken and recorded
 Assessed back pain due to Levoscoliosis – Pain Scale: 8/10
 Performed back massage
 Assisted on administering hot sitz bath
 ANATOMY AND PHYSIOLOGY
Part IV – A

A gene is the basic physical and functional unit of heredity. Genes, which are made up of DNA, act as
instructions to make molecules called proteins. In humans, genes vary in size from a few hundred
DNA bases to more than 2 million bases. The Human Genome Project has estimated that humans
have between 20,000 and 25,000 genes.

Every person has two copies of each gene, one inherited from each parent. Most genes are
the same in all people, but a small number of genes (less than 1 percent of the total) are slightly
different between people. Alleles are forms of the same gene with small differences in their
sequence of DNA bases. These small differences contribute to each person’s unique physical
features.

DNA, or deoxyribonucleic acid, is the hereditary material in humans and almost all other
organisms. Nearly every cell in a person’s body has the same DNA. Most DNA is located in the cell
nucleus (where it is called nuclear DNA), but a small amount of DNA can also be found in the
mitochondria (where it is called mitochondrial DNA or mtDNA).

The information in DNA is stored as a code made up of four chemical bases: adenine (A),
guanine (G), cytosine (C), and thymine (T). Human DNA consists of about 3 billion bases, and more
than 99 percent of those bases are the same in all people. The order, or sequence, of these bases
determines the information available for building and maintaining an organism, similar to the way
in which letters of the alphabet appear in a certain order to form words and sentences.

An important property of DNA is that it can replicate, or make copies of itself. Each strand of
DNA in the double helix can serve as a pattern for duplicating the sequence of bases. This is critical
when cells divide because each new cell needs to have an exact copy of the DNA present in the old
cell. DNA is a double helix formed by base pairs attached to a sugar-phosphate backbone.

The STK11 gene provides instructions for making an enzyme called serine/threonine kinase
11. This enzyme is a tumor suppressor, which means that it helps keep cells from growing and
dividing too fast or in an uncontrolled way. In addition to its role in regulating cell division, this
enzyme helps certain types of cells correctly orient themselves within tissues (polarization) and
assists in determining the amount of energy a cell uses. This kinase also promotes a type of
programmed cell death known as apoptosis. Through a combination of these mechanisms,
serine/threonine kinase 11 aids in the prevention of tumors, especially in the gastrointestinal tract,
pancreas, cervix, ovaries, and breasts. Serine/threonine kinase 11 function is also required for
normal development before birth.

Research has shown that the loss of this enzyme's function allows cells to divide too often.
The loss of the enzyme's tumor suppressor function likely underlies the increased risk of
gastrointestinal tumors, breast cancer, and other forms of cancer.

The STK11 gene is located on the short (p) arm of chromosome 19 at position 13.3. More
precisely, the STK11 gene is located from base pair 1,205,797 to base pair 1,228,433 on
chromosome 19.
GASTROINTESTINAL SYSTEM
The gastrointestinal tract (GIT) consists of a hollow muscular tube starting from the oral cavity,
where food enters the mouth, continuing through the pharynx, esophagus, stomach and intestines
to the rectum and anus, where food is expelled. There are various accessory organs that assist the
tract by secreting enzymes to help break down food into its component nutrients. Thus the salivary
glands, liver, pancreas and gall bladder have important functions in the digestive system. Food is
propelled along the length of the GIT by peristaltic movements of the muscular walls.

 The primary purpose of the gastrointestinal tract is to break food down into nutrients,
which can be absorbed into the body to provide energy. First food must be ingested into the mouth
to be mechanically processed and moistened. Secondly, digestion occurs mainly in the stomach and
small intestine where proteins, fats and carbohydrates are chemically broken down into their basic
building blocks. Smaller molecules are then absorbed across the epithelium of the small intestine
and subsequently enter the circulation. The large intestine plays a key role in reabsorbing excess
water. Finally, undigested material and secreted waste products are excreted from the body via
defecation (passing of faeces).

Oral Cavity
The oral cavity or mouth is responsible for the intake of food. Mastication refers to the mechanical
breakdown of food by chewing and chopping actions of the teeth. The tongue, a strong muscular
organ, manipulates the food bolus to come in contact with the teeth

Insalivation refers to the mixing of the oral cavity contents with salivary gland secretions.
The mucin (a glycoprotein) in saliva acts as a lubricant. The oral cavity also plays a limited role in
the digestion of carbohydrates. The enzyme serum amylase, a component of saliva, starts the
process of digestion of complex carbohydrates. The final function of the oral cavity is absorption of
small molecules such as glucose and water, across the mucosa. From the mouth, food passes
through the pharynx and esophagus via the action of swallowing.

Salivary Glands
Three pairs of salivary glands communicate with the oral cavity. Each is a complex gland with
numerous acini lined by secretory epithelium. Salivation occurs in response to the taste, smell or
even appearance of food.

Esophagus
The esophagus is a muscular tube of approximately 25cm in length and 2cm in diameter. It extends
from the pharynx to the stomach after passing through an opening in the diaphragm. The esophagus
functions primarily as a transport medium between compartments.

Stomach
The stomach is a J shaped expanded bag, located just left of the midline between the esophagus and
small intestine. It is divided into four main regions and has two borders called the greater and
lesser curvatures. Gastric contents are expelled into the proximal duodenum via the pyloric
sphincter. The inner surface of the stomach is contracted into numerous longitudinal folds called
rugae. These allow the stomach to stretch and expand when food enters. The stomach can hold up
to 1.5 litres of material. The functions of the stomach include:
1. The short-term storage of ingested food.
2. Mechanical breakdown of food by churning and mixing motions.
 3. Chemical digestion of proteins by acids and enzymes.
4. Stomach acid kills bugs and germs.
5. Some absorption of substances such as alcohol.

Most of these functions are achieved by the secretion of stomach juices by gastric glands in
the body and fundus. Some cells are responsible for secreting acid and others secrete enzymes to
break down proteins.

Small Intestine
The small intestine is composed of the duodenum, jejunum, and ileum. It averages approximately
6m in length, extending from the pyloric sphincter of the stomach to the ileo-caecal valve separating
the ileum from the caecum. The small intestine is compressed into numerous folds and occupies a
large proportion of the abdominal cavity.

The duodenum serves a mixing function as it combines digestive secretions from the
pancreas and liver with the contents expelled from the stomach. It is in the jejunum where the
majority of digestion and absorption occurs. The final portion, the ileum, is the longest segment and
empties into the caecum at the ileocaecal junction.

The small intestine performs the majority of digestion and absorption of nutrients. Partly
digested food from the stomach is further broken down by enzymes from the pancreas and bile salts
from the liver and gallbladder. These secretions enter the duodenum at the Ampulla of Vater. After
further digestion, food constituents such as proteins, fats, and carbohydrates are broken down to
small building blocks and absorbed into the body's blood stream.

The lining of the small intestine is made up of numerous permanent folds called plicae
circulares. Each plica has numerous villi (folds of mucosa) and each villus is covered by epithelium
with projecting microvilli (brush border). This increases the surface area for absorption by a factor
of several hundred.

Large Intestine
The large intestine is horse-shoe shaped and extends around the small intestine like a frame. It
consists of the appendix, caecum, ascending, transverse, descending and sigmoid colon, and the
rectum. It has a length of approximately 1.5m and a width of 7.5cm. The cecum is the expanded
pouch that receives material from the ileum and starts to compress food products into fecal
material. Food then travels along the colon. The wall of the colon is made up of several pouches
(haustra) that are held under tension by three thick bands of muscle (taenia coli). The rectum is the
final 15cm of the large intestine. It expands to hold fecal matter before it passes through the
anorectal canal to the anus. Thick bands of muscle, known as sphincters, control the passage of
feces. The mucosal surface is flat with several deep intestinal glands. Numerous goblet cells line the
glands that secrete mucous to lubricate fecal matter as it solidifies. The functions of the large
intestine can be summarized as:
1. The accumulation of unabsorbed material to form feces.
2. Some digestion by bacteria. The bacteria are responsible for the formation of intestinal gas.
3. Reabsorption of water, salts, sugar and vitamins.
Liver
The liver is a large, reddish-brown organ situated in the right upper quadrant of the abdomen. It
acts as a mechanical filter by filtering blood that travels from the intestinal system. It detoxifies
several metabolites including the breakdown of bilirubin and estrogen. In addition, the liver has
synthetic functions, producing albumin and blood clotting factors. However, its main roles in
digestion are in the production of bile and metabolism of nutrients. All nutrients absorbed by the
intestines pass through the liver and are processed before traveling to the rest of the body. The bile
produced by cells of the liver, enters the intestines at the duodenum. Here, bile salts break down
lipids into smaller particles so there is a greater surface area for digestive enzymes to act.

Gall Bladder
The gallbladder is a hollow, pear shaped organ that sits in a depression on the posterior surface of
the liver's right lobe. It consists of a fundus, body and neck. It empties via the cystic duct into the
biliary duct system. The main functions of the gall bladder are storage and concentration of bile.
Bile is a thick fluid that contains enzymes to help dissolve fat in the intestines. Bile is produced by
the liver but stored in the gallbladder until it is needed.

Pancreas
Finally, the pancreas is a lobular, pinkish-grey organ that lies behind the stomach. Its head
communicates with the duodenum and its tail extends to the spleen. The organ is approximately
15cm in length with a long, slender body connecting the head and tail segments. The pancreas has
both exocrine and endocrine functions. Endocrine refers to production of hormones which occurs in
the Islets of Langerhans. The Islets produce insulin, glucagon and other substances and these are
the areas damaged in diabetes mellitus. The exocrine (secretrory) portion makes up 80-85% of the
pancreas and is the area relevant to the gastrointestinal tract.

It is made up of numerous acini (small glands) that secrete contents into ducts which
eventually lead to the duodenum. The pancreas secretes fluid rich in carbohydrates and inactive
enzymes. Secretion is triggered by the hormones released by the duodenum in the presence of food.
Pancreatic enzymes include carbohydrases, lipases, nucleases and proteolytic enzymes that can
break down different components of food. These are secreted in an inactive form to prevent
digestion of the pancreas itself. The enzymes become active once they reach the duodenum.
 LABORATORY EXAMINATIONS
Part V

Laboratory Examinations

Date/
Normal Value Client Result Reason for Test Nursing Intervention
Laboratory Exam
21 June 2010
Clinical Chemistry
Sodium 136-145 mmol/L 133 mmol/L Blood sodium test is done to For acute severe hyponatremia
determine the amount of sodium associated with neurologic
present in the blood serum. The symptoms should be treated
test is mostly performed to check urgently with hypertonic saline at
for excess of sodium in the blood a correction rate that does not
called hypernatremia or deficiency exceed 1-2 mmol per L per hour,
of it which is known as and normo/hypernatremia should
hyponatremia. be avoided in the first 48 hours
For chronic hyponatremia, rapid
correction should be avoided
because it can lead to central
pontine myelinolysis.
In most cases of chronic
asymptomatic hyponatremia,
removing the underlying cause of
the hyponatremia is adequate
treatment. Otherwise, fluid
restriction (less than 1 to 1.5 L per
day) is the mainstay of treatment.
 Date/
Normal Value Client Result Reason for Test
Laboratory Exam
21 June 2010
Hematology
Hemoglobin 11.6 – 15.5 g/dL 7.3 g/dL Used to monitor previous medical
conditions and to check a person’s
health. This test measures the
amount of hemoglobin, a protein
found in red blood cells, which is a
good indication of the blood’s
ability to deliver oxygen to tissues
and organs and to transport the
waste product carbon dioxide to
the lungs, where it is exhaled.
Hematocrit 36 – 47 % 24.3 % Used to screen for anemia, a
deficiency of red blood cells, or to
monitor the treatment of an
existing anemic condition. Also
used in deciding whether or not to
give a blood transfusion.
RBC 4.2 – 5.4 mil/mm3 3.63 mil/mm3 May be used to indicate if there is
lack of oxygen in the blood or a
bone marrow problem; and if the
patient is suffering from anemia.
Differential Count
Nursing Intervention
>Low hemoglobin and hematocrit
places the patient on blood
transfusion.
>Advise patient that a healthy diet
containing vegetables and foods
high in iron can be beneficial.
>Low hemoglobin and hematocrit
places the patient on blood
transfusion.
>Advise patient to rest between
activities and avoid or stop
activities that make him short of
breath or make his heart beat
faster. Plan ahead and save energy
for the most important activities.
>Instruct client to eat a diet with
adequate protein and vitamins.
>Teach patient to drink plenty of
non-caffeinated and non-alcoholic
fluids.
Lymphocyte 19 – 48 % 10 % Lymphopenia >Treat underlying condition
>Withdraw or reduce dose of
causative drugs >Bone marrow
stimulating agents

Platelet 150,000 – 400,000 / 480,000 / mm3 Thrombocytosis >Treatment of underlying cause

mm3 >Aspirin
>Compression stockings
>Vitamin E - possibly used if
condition is caused by vitamin E
deficiency
MCV 82 – 98 fl 67 fl Microcytic anemia >Treatment of microcytic anaemia
is dependent upon the underlying
cause for the iron deficiency.
Treatments include:
 Investigation and treatment of
underlying conditions such as
coeliac disease, menorrhagia,
bowel cancer
 Dietary review and management
- to ensure adequate iron intake
in the diet
 Iron supplementation - used
when the patient is stable and
the underlying cause has been
identified, to restore
haemoglobin levels and
replenish iron stores
 Blood transfusion - for
management of severe anemia
where there is evidence of end
organ compromise such as
 cardiac ischaemia or cardiac
failure
 Green leafy vegetable
MCH 28-33 pg 20 pg Hypochromic anemia Treatments for Hypochromic
anemia (X-linked sideroblastic
anemia) include:
 Copper - possibly used for
related copper deficiency
 Vitamin B6 - possibly used for
related vitamin B6 deficiency
 Identify and treat underlying
condition. Iron supplementation,
vitamin C supplements are also
usually advised in order to
increase iron absorption
 Vitamin B6 to prevent iron
overload in mild cases. Severe
cases may require blood
transfusion and desferrioxamine
treatment to remove excess iron
MCHC 32 – 38 % 30 % Hypochromic anemia Treatments for Hypochromic
anemia (X-linked sideroblastic
anemia) include:
 Copper - possibly used for
related copper deficiency
 Vitamin B6 - possibly used
for related vitamin B6
deficiency
 Identify and treat
underlying condition. Iron
supplementation, vitamin C
supplements are also
usually advised in order to
increase iron absorption
 Vitamin B6 to prevent iron
overload in mild cases.
Severe cases may require
blood transfusion and
desferrioxamine treatment
to remove excess iron
Diagnostic Procedures

Date/ Diagnostic Nursing Intervention: Preparation for

Client Result Reason for Test
Procedure Pre- and Post-Procedure
Date of request: Diagnosis: To know the state and  Limit the intake of liquids for 24 to
25 August 2004  Hamartomatous Polyps condition of the patient’s 72 hours before the examination
Date of release: (Peutz-Jehger's Ployp) internal organs  Adequate colon cleansing provides
28 July 2007  Ileum and cecum with focal
optimal visualization and
adenomatous change.
Surgical Pathology  Hamartromatous Polyp, decreases the time needed for the
Consultation Report Infarcted Cecum. procedure
 Fibrosis with granulation  Secure informed consent
tissue, serosa, ileum, cecum  NPO post midnight before the test
Clinical diagnosis:  and ascending colon.  Post procedure: Observe for signs
Intussusception  Acute and chronic and symptoms of bowel
periappendicitis.
perforation
Specimen:  Fibrosis and granulation
Ileum to cecum, tissue, serosa, Ileum and
ascending colon, polyp  colon line of resection
 Reactive Hyperplasia,
Pericolic lymph nodes,
reactive
 hyperplasia

Gross/ Microscopic Description:

Specimen ileum, cecum, ascending
colon. The ileum measures 67cm.
long and 7cm. in diameter. There is
minimum mesenteric fat attached.
The external surface is gray brown
and smooth. These are three
polyphoid structures noted
adjacent to each other. The first
polyp measures 0.9x0.6x0.5 cm. It
has a light brown granular surface.
It is located 3.5 cm. from the
proximal line of resection and 2cm.
from the biggest polyp. The latter
measures 5x2.2x2.5 cm. Its surface
is light brown and nodular. This
polyp is seen 2cm. from the
proximal margin of resection. The
third polyp measures 2x1x0.8 cm.
and seen from 2.5 cm. from biggest
polyp. The rest of the ileal mucosa
is unremarkable. The attached
appendix measures 6cm. long and
0.7 in diameter. The external
surface is gray tan with prominent
blood vessels. The lumen measures
0.2 cm. The attached large intestine
measures 10 cm. long and 5 cm. in
diameter. There are two
pedunculated polyps seen at the
cecum. The pedunculated smaller
polyp has a gray brown surface and
measures 1.5x1.2x0.7 cm. The stalk
measures 3x3x2 cm. seen 1 cm.
from the line of resection. The
bigger polyp is seen 8 cm. from
distal line of resection.

 proximal line of resection,

s2b1
 distal line of resection
(colon), s2b1
 first polyp, ileum, s1b1
 Second polyp, ileum, s3b3
 Third polyp/ileum, s1b1
 random sections,
duodenum fibrinous
exudate, s3b1
  mesenteric node, s1b1
 appendix, s2b1
 polyp at cecum, s1b1
 bigger polyp at cecum, s8b3
 pericolic lymph nodes, s6b1
 submitted in a separate
container is a specimen
labeled as "polyp"

It is pedunculated and measures

2.1x1.5x0.8 cm which is supported
by stalk measures 0.4x0.7x0.7 cm.

Microscopic:
Sections from the polyps at ileum
and cecum shows prominent
glands lined by columnar
epithelium, some exhibiting
nuclear pseudostratifications and
mild nuclear atypia. Smooth muscle
bundles of variablethicness extends
from muscularis mucosal into the
polyp. One polyp at the cecum
shows areas of hemorrhage.
Serosal fibrosis and granulation
tissue compound of proliferating
blood vessels and inflammatory
cells are noted along the entire
segment of the intestine and colon.
The appendix shows mixed
inflammatory cells at the
perippendical area. All lymph node
(6) isolated from the pericolic fat
show reactive hyperplasia.
 Date/ Diagnostic Nursing Intervention: Preparation for
Client Result Reason for Test
Procedure Pre- and Post-Procedure
24 August 2004  There is evidence of minimal To have a more definitive view  NPO for six to eight hours.
ascites particularly in the of the abdominal organs of the  Check for allergy if an intravenous or
Whole Abdominal – pelvic patient oral contrast agent is used.
CT Scan  There is telescoping of the  Schedule barium studies after CT
distal small bowel, mesentery scan.
and vessels into large  Provide privacy.
Note: intestines up to the level of  Ensure safety.
16-slice spiral plain the transverse colon
and IV (w/o oral) indicative of intussusception.
contrast CT scan of the There is a nodular soft tissue
whole abdomen were density in the region of the
done splenic flexure.
 The liver is not enlarged
without discrete mass nor
dilate intrahepatic ducts.
Small focus of decreased
density is seen in the medial
left lobe which may relate to
focal fatty infiltration.
 The gallbladder, spleen and
adrenal glands are
unremarkable.
 The pancreas shows no focal
enlargement nor density
change. The pancreatic duct is
not dilated.
 Both kidneys are functioning
without hydronephrosis nor
opaque lithiasis.
 No evidence for enlarged
lymph nodes.
 The urinary bladder is intact.
The size of the uterus is
appropriate for age.
  There is a focus of sclerosis in
the sacral bone and ileum on
the right side. There is mild
deviation of the thoraco-
lumbar spine to the right. No
bone destruction seen.

Date/ Diagnostic Nursing Intervention: Preparation for

Client Result Reason for Test
Procedure Pre- and Post-Procedure
21 June 2010  Non-obstructive bowel gas To have a more definitive view  NPO for six to eight hours.
pattern. of the abdominal organs of the  Check for allergy if an intravenous or
Abdomen – Flat and  Minimal fecal material is patient oral contrast agent is used.
upright noted in the rect0-sigmoid  Schedule barium studies after CT
colon. scan.
 Flank stripes and psoas  Provide privacy.
Clinical history: shadows are intact.  Ensure safety.
Intussusception  No evidence of soft tissue
densities/calcifications.
Technique:  There is a small rounded
AP supine and upright opacity in the right hip joint
views area.
 There is levosclerosis in the
lumbar spine.
 Impression:
 Non-obstructive Bowel Gas
Pattern.
 Minimal Fecal Stasis
 Levosclerosis
 Bony Island, Right,
Unchanged
 DRUG STUDY
Part VI

Appropriate
Drug
Dosage for Why Client is
Dosage Classification & Nursing Intervention
24hours & Nursing Implications Receiving the
Route Mechanism of Action related to Effects of Drug
Drug Drug?
Frequency
Computation
metoclopramide 10 or 15 mg - Dopamine Antagonist - Advise patient to take - Assess patient for nausea, - Prevention of
(Reglan) four times daily without food; or take ½ vomiting, abdominal nausea and
1 tab - Blocks dopamine hour before meals. distention and bowel vomiting.
PO receptors in - Metoclopromide sounds before and after
TID chemoreceptor trigger should not be given in administration.
zone of the CNS. combination with any - Warn patient to about the
Stimulates motility of other medications, such drowsiness the drug can
the upper GI tract and as phenothiazines, that cause and told to avoid
accelerates gastric would lead to performing any hazardous
emptying. exacerbation of tasks.
extrapyramidal
symptoms.
Cinchocaine OD or one - Anti-inflammatory - Additional specific - Watch out for skin - For relief of anal
hydrochloride suppository therapy is required in atrophy (long-term itch
(Ultraproct) every other day - Fluocortolone exerts fungal infections. therapy), rarely allergic
an anti-inflammatory, - Inadvertent contact of skin reactions.
1 supp antiallergenic and the preparation with the
OD antipruritic effect. eyes should be avoided.
Capillary dilatation, Careful handwashing
intercellular edema after use is
and tissue infiltration recommended.
regress; capillary
 proliferation is
suppressed.
tramadol PO: 50-100mg - Miscellaneous - Taking with food or a - Inform patient that - For relief of
(Dolcet) q4-6h; not to Analgesic snack may help to tramadol may cause moderate to
100 mg exceed decrease nausea and vomiting. Offer severe pain
PO 400mg/day - Binds to mu-opioid gastrointestinal upset. cola or dry crackers to help
1 tab q8° receptors. Inhibits - Assess blood pressure relieve nausea.
reuptake of serotonin and respiratory rate - If dizziness, blurred
and norepinephrine in before and periodically vision, or drowsiness
the CNS. during administration. occur, nurse should be sure
- Assess bowel function to assist the patient with
routinely. ambulation.
- Educate patient about - Patient should be
injury prevention, such encouraged to report any
as the need to move and heart palpitations,
change positions slowly seizures, tremors, difficulty
and to avoid any tasks breathing, chest pain, and
that require mental muscle weakness.
clarity and alertness.
omeprazole PO: 60- - Proton-Pump - Administer 30-60 - Assess patient routinely - Commonly given
(Prilosec) 360mg/day Inhibitor minutes before meals. for epigastric or abdominal to prevent stress
40 mg TID - Administer antacids pain and frank or occult ulcers, which may
PO - Binds to an enzyme with, if needed. blood in the stool, emesis, be associated with
1 tab OD on gastric parietal cells - Nurse should double- or gastric aspirate. coma or other
in the presence of check the names and - Watch out for headache, severely
acidic gastric pH, dosages to ensure that diarrhea, constipation, compromising
preventing the final they are not confused abdominal pain, nausea, conditions.
transport of hydrogen with similarly named vomiting, flatulence.
ions into the gastric drugs.
lumen.
 THEORETICAL FRAMEWORK
Part VII – A

Virginia Henderson’s 14 Fundamental Needs

In 1966, Virginia Henderson’s definition of the unique function of nursing was a major
stepping stone in the emergence of nursing as a discipline separate from medicine. Like Nightingale,
Henderson described the patient as both healthy and ill individuals, acknowledged that nurses
interact with clients even when recovery may not be visible, and mentioned the teaching and
advocacy roles of the nurse.

Henderson (1966) conceptualized the nurse’s role as assisting sick or healthy individuals to
gain independence in meeting 14 fundamental needs:
1. Breathing normally
2. Eating and drinking adequately
3. Eliminating body waste
4. Moving and maintaining a desirable position
5. Sleeping and resting
6. Selecting suitable clothes
7. Maintaining body temperature within normal range by adjusting clothing and
modifying the Environment
8. Keeping the body clean and well groomed to protect the integument
9. Avoiding the dangers in the environment and avoiding injuring agents
10. Communicating with others in expressing emotions, needs, fears and opinions
11. Worshipping according to one’s faith
12. Working in such a way that one feels a sense of accomplishment
13. Playing or participating in various forms and creation
14. Learning, discovering, or satisfying the curiosity that leads to normal developmental
and health, and using available health facilities.

Virginia Henderson categorized nursing activities into 14 components, based on human

needs. She described the nurse's role as substitutive (doing for the person), supplementary (helping
the person), complementary (working with the person), with the goal of helping the person become
as independent as possible. In doing the nursing care for G.R., we based the intervention and
planning of care with Virginia Henderson’s nursing theory. We believe that nursing is merely
temporarily assisting an individual who lacks the necessary strength, will and knowledge to satisfy
1 or more of 14 basic needs. As a nurse we should assist and support the patient in life activities and
the attainment of independence.
 VII. NURSING CARE PLAN
Part VII – B

Nursing Nursing Expected

Assessment Planning Rationale
Diagnosis Intervention Outcome
Subjective: Bleeding Short term goal: Independent: Short term goal:
“Kulay itim yung related to polyps After 8 hours of Assessed vital signs, noting To keep an eye on the Goal met because the
dumi ko kagabi.” in the small nursing low blood pressure/severe improvement of the patient patient was able to
as answered by intestines interventions, the hypotension, rapid have stable vital signs
the patient when patient will be able to heartbeat and thread and no have signs of
asked what was have stable vital signs peripheral pulses bleeding such as
the color of her and will not have hematochezia and
stool. signs of bleeding Noted the physical signs To monitor dehydration and melena.
such as hematochezia (e.g. capillary refill, poor the amount of blood flow to
and melena. skin turgor) tissue

Objective: Weighed daily and Weight helps to assess fluid

- Pale skin and maintain accurate input balance Long term goal:
conjunctiva Long term goal: and output Goal partially met
- Cold to touch After 1 week of because the patient did
skin nursing Noted change in usual To monitor poor cerebral not show any signs of
- Weight: 40.5 kg interventions, the mentation/behavior/ perfusion and/or electrolyte bleeding. We were not
patient will be able to functional abilities (e.g. imbalance able to weigh the
slowly gain weight confusion, falling, loss of patient because we
and will no longer ability to carry out usual only handled her for 2
experience bleeding. activities, lethargy, days.
dizziness)

Auscultated bowel sounds. To evaluate degree of deficit

Noted characteristics of
stool (color, consistency,
frequency, etc.) that may
indicate bleeding(e.g.
melena)

Noted when the patient To evaluate degree of deficit

vomits blood
(hematemesis)

Dependent:
Administered IV fluids, as To maintain hydration
indicated.
 Nursing Nursing Expected
Assessment Planning Rationale
Diagnosis Interventions Outcome
Subjective: Imbalanced Short term goal: Independent: Short term goal:
“Kumakain naman nutrition less After 15 minutes of Assessed weight, age, body To provide comparative Goal met because the
siya madalas pero than body nursing build, strength, baseline patient and significant
sobrang kaunti requirements interventions, the activity/rest level and so others verbalized
lang kasi wala patient will be able forth. understanding of
siyang gana.” As to verbalize causative factors and
verbalized by the understanding of necessary
patient’s mother causative factors Identified client’s risk for To assess causative factors intervention regarding
and necessary malnutrition (e.g. intestinal the patient’s nutrition.
interventions surgery, prior nutrition
regarding her deficiencies).
Objective: nutrition.
- BMI = 14.8
(The patient is Ascertained understanding To determine what
40.5 kg; Healthy of individual nutrition information to provide Long term goal:
weight range to Long term goal: needs. client or significant others Goal not met. We were
her age and height After 1 week of not able to weigh the
is 54.13kg) nursing patient because we
- Pale conjunctiva interventions, the only handled her for 2
patient will be able Discussed eating habits, To appeal to clients days.
to demonstrate including food preferences, likes/desires
progressive weight intolerances/aversions.
gain toward goal
and display
normalization Assessed drug interactions, To assess factors that may
within her Body disease effects. affect appetite, food intake
Mass Index. or absorption

Noted total daily intake. To reveal changes that

Maintained diary of calorie should be made in client’s
intake, patterns and times dietary intake
of eating.

Auscultated bowel sounds. To evaluate degree of

Noted characteristics of deficit
stool (color, consistency,
frequency, etc.)

Promoted pleasant, To stimulate appetite

relaxing environment,
including socialization
when possible.

Weighed weekly or as To promote wellness

needed and document
results.

Emphasized importance of To enhance intake

well-balanced, nutritious
intake.

Dependent:
Consulted To implement
dietitian/nutritional team interdisciplinary team
as indicated. management

Collaborative:
Provided diet To establish a nutritional
modifications as indicated. plan that meets individual
needs
Encouraged client to To stimulate appetite
choose foods that are
appealing.
 Nursing Nursing Expected
Assessment Planning Rationale
Diagnosis Interventions Outcome
Subjective: Acute pain Short term goal: Independent: Short term goal:
“Maraming beses related to After 20 minutes of Performed a To assess Goal met because the
na sumasakit yung Levoscoliosis nursing comprehensive assessment etiology/precipitating patient verbalized
likod buong likod. intervention, the of pain to include location, contributing factors relief and pain scale
Dahil na rin po patient will be able characteristic, decreased from 8/10
ito ng pagiging to report pain is onset/duration, frequency, to 2/10.
kuba ko.” As relieved/ quality, severity and
verbalized by the Controlled and aggravating factors.
patient. pain scale will
decrease from 8
out of 10. Determined possible To assess
pathophysiologic/ etiology/precipitating
Objective: psychologic causes of pain contributing factors
- Pain Scale: 8/10 (e.g. fractures) Long term goal:
- Facial grimace Long term goal: Goal met because the
After 1 hour of patient verbalized
nursing Assessed client’s To assess ways to relief pain
intervention, the perceptions, along with etiology/precipitating when it occurs and
patient will be able behavioral and physiologic contributing factors demonstrated
to verbalize responses. relaxation skills.
methods to provide
relief and
demonstrate use of
relaxation skills Noted client’s attitude To rule out worsening of
and diversional toward pain and use of underlying
activities as specific medications, condition/development of
indicated for including any history of complications
individual. substance abuse.
Performed pain To evaluate client’s
assessment each time pain response to pain
occurs. Usually altered in acute
pain as timely intervention
is more likely to be
successful in alleviating
pain

Noted and investigate To assist client to explore

changes from previous methods for alleviation or
reports. control pain

Worked with client to To assist client to explore

prevent pain.. methods for alleviation or
control pain

Provided quiet, calm Provide non-

environment and comfort pharmacologic pain
measures. management

Suggested parent be To comfort child

present during procedures.

Collaborative:
Administered analgesics if To provide pharmacologic
indicated. pain management
 Nursing Nursing Expected
Assessment Planning Rationale
Diagnosis Interventions Outcome
Subjective: Anxiety Short term goal: Independent: Short term goal:
“Wala akong related to threat After 4 hours of Reviewed To assess level of anxiety Goal met because the
makausap dito at to or change in nursing familial/physiologic patient appeared
ayaw ko na sana health status intervention, the factors, current prescribed relaxed and verbalized
tumagal dito sa patient will be able medications and recent “Buti na lang andito
ospital.” As to appear relaxed drug history. kayo, kahit papaano
verbalized by the and report anxiety may nakakausap ako.”
patient. is reduced to a
“Ganyan lang manageable level. Identified client’s To assess level of anxiety
talaga siya. Laging perception of the threat
tahimik. Buti nga represented by the
at nandiyan kayo situation. Long term goal:
para may kausap Long term goal: Goal met because the
siya.” As After 3 days of patient identified
verbalized by the nursing Monitored physical To assess level of anxiety healthy ways to deal
patient’s mother. intervention, the responses for example, with and express
patient will be able palpitations/rapid pulse, anxiety.
to identify healthy repetitive movements,
ways to deal with pacing.
Objective: and express
- Poor eye contact anxiety.
- Restlessness Observed behavior To be aware of own
indicative of level of feelings of anxiety or
anxiety. uneasiness, which can be
clue to the client’s level of
anxiety
Established a therapeutic
relationship, conveying
empathy and
unconditional positive
regard.
Be available to client for
listening and talking.
Encouraged client to
acknowledge and express
feelings.
Acknowledged
anxiety/fear. Do not deny
and reassure client that
everything will be alright.
Provided comfort
measures (calm/quiet
environment, soft music,
and back rub).
Encouraged the client to
develop an
exercise/activity program.
To assist client to identify
feelings and begin to deal
with problems
To assist client to identify
feelings and begin to deal
with problems
To assist client to identify
feelings and begin to deal
with problems
To assist client to identify
feelings and begin to deal
with problems
May be helpful in reducing
level of anxiety by relieving
tension
 Nursing Nursing Expected
Assessment Planning Rationale
Diagnosis Interventions Outcome
Objective: Risk for activity Short term goal: Independent: Short term goal:
- Generalized intolerance After 2 hours of Identified factors that To assess factors affecting Goal met because the
weakness related to nursing could block/affect desired current situation patient verbalized
insufficient intervention, the level of activity. understanding of
nutrients in the patient will be able potential loss of ability
body to verbalize in relation to existing
understanding of condition.
potential loss of Noted presence of medical To track changes
ability in relation to diagnosis and/or regimen
existing condition. that has potential for
interfering with client’s
ability to perform a
desired activity level.
Long term goal: Long term goal:
After 2 days of Goal met because the
nursing Determined baseline To promote wellness patient participated in
intervention, the activity level and physical conditioning program
patient will be able condition. to enhance activities of
to participate in daily living.
conditioning
program to Discussed relationship of To promote wellness
enhance ability to illness/debilitating
perform and condition to inability to
identify alternative perform desired activities.
ways to maintain
desired activity
level. Provided information To promote wellness
regarding potential
interferences to activity.
Assisted client/SO with To sustain activity level
planning of changes that
may become necessary.

Referred to appropriate To enhance likelihood of

sources and/or success
equipment as needed.
 DISCHARGE PLANNING
Part VIII

Medications
 Instructed the patient to continue her medications as prescribed by the doctor.
 Emphasized the importance of compliance and strict adherence to dosage and the time
of intake of the medicines to attain the desired therapeutic effects.
 Stressed to the patient not to alter doses of medications and emphasize the importance
of continuous medication.

Environment
 Stressed to the relatives of the patient to have a clean, quiet, well ventilated room,
conducive to rest and relaxation and to reduce environmental stress that would
aggravate complications
 Instructed the relatives to promote a conducive environment to rest.

Treatment
 Encouraged patient to comply to the prescribe medications.
 Encouraged to have sitz baths twice a day.

Health Teachings
 Explained the importance of continuous medication, taking regular sitz bath and
following the prescribed diet in promoting wellness.
 Explained the importance of follow up care in updating the status of her health
condition.

Out-patient Follow-up
 Counseled the patient to have a follow-up care on the date the doctor instructed her to
return.

Diet
 Emphasized to the patient on no dark colored food in the diet
 Emphasized to the patient to avoid foods that can trigger more bleeding such as acid-
producing foods like caffeinated and decaffeinated beverages such as coffee, tea and
colas.

Spiritual
 Enlightened the patient to keep her positive outlook in life and encouraged the relatives
to support the patient and let her express whatever concerns and grief about the
diagnosis.
 IMPLICATIONS OF THE CASE
Part IX

Nursing Research
The case can be used as a baseline data for further research of the current management
of patients with Peutz Jegher’s Syndrome. There might be some information in this study that
can be of good use for future research. It is important to do research every now and then to gain
new information, better interventions and techniques to provide to the patients. Aside from
being beneficial as a simple academic informative material, this study might serve as a guide for
orienting people about the substance of the disease, Peutz Jegher’s Syndrome, and how this
disease affects many people. Therefore through this study, the researchers should have
introduced the symptoms (for early detection), treatment (for information), and management.

Nursing Education
This study can be a useful learning guide in nursing education to be used by future
students as a reference for future studies regarding Peutz Jegher’s Syndrome. Through this case,
the students will be able to assess the patient with any signs of gastrointestinal disease and be
able to provide appropriate care. Furthermore, the students will be able to do their nursing
intervention and at the same time have an idea of the rationale behind its actions. In this way,
they are acquiring a broader knowledge about the disease that they can use to further develop
their skills as nurses. They can examine it and if verified that valid, it may open a new door in
the practice of getting quality care. This study might also inspire other individuals to come up
with their own research about this disease.

Nursing Practice
This case study can be used as a tool in nursing practice because it provides nursing
interventions for patients with Peutz Jegher’s Syndrome. This study can give a good introduction
to the disease so that an established nursing action can be quickly utilized. And through
discovering and rediscovering, and trial after trial of innovative interventions and facilitation of
this condition, a more advanced nursing management may be developed. Through this study,
important information regarding this illness has been gathered which will be helpful on the
researchers to have a depth understanding on the said disease.